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pmc-6366086-1
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A 77-year-old man developed bilateral peripheral edema in August 2017. Persistent proteinuria and nephrotic syndrome were observed, and he was admitted to our hospital in September 2017. Past medical history included hypertension, cerebral hemorrhage, and rectal cancer. The rectal cancer was detected via a colonoscopy examination in July 2015, and a high anterior resection surgery with lymphadenectomy was performed in October 2015, with pathological diagnosis of rectal cancer, pT3N2bM0, pStage IIIC. Based on the sequelae of cerebral hemorrhage and performance status, no adjuvant chemotherapy was administered, and no recurrence was detected in follow-up. The patient’s family history was unremarkable. On admission, the blood pressure was 109/69 mmHg, the pulse was regular at 109 beats/min, and the body temperature was 36.8 °C. Physical examination revealed no abnormalities except for pitting edema of the limbs. Mild bilateral pleural effusion was confirmed by chest radiography. The size and the blood flow signal of both kidneys were normal in renal echography. Laboratory test results were as follows: total protein, 5.3 g/dL; albumin, 1.3 g/dL; serum creatinine, 1.07 mg/dL; total cholesterol, 293 mg/dL; glycosylated hemoglobin, 6.2%; white blood cell (WBC) count, 5000 cells/μL; hemoglobin, 12.3 g/dL; and platelet count, 23.7 × 104/μL. The patient was positive for hepatitis C virus (HCV) antibody, but HCV RNA level was low. Tests for hepatitis B surface antigen, hepatitis B surface antibody, and human immunodeficiency virus antibody were negative. Urinalysis results were as follows: urinary protein excretion of 10.1 g/day, sediment containing 1–4 red blood cells, 1–4 WBCs per high-power field, 1–4 granular casts per whole field, and oval fat bodies. Further serological study results were as follows: IgG, 891 mg/dL; IgA, 176 mg/dL; IgM, 90 mg/dL; C3, 149 mg/dL; and C4, 41 mg/dL. Anti-nuclear antibody was present at 40-fold in a homogenous and speckled pattern, but the specific antibody was absent.
A renal biopsy was performed the day after admission. Light microscopy (LM) showed that 9 of 42 glomeruli were globally sclerotic. Although the glomerular base membranes were thickened, spike formation was not observed (Fig. ). Mild mesangial proliferation was observed in a few glomeruli. There was no endocapillary proliferation or crescent formation. Tubulointerstitial or vascular damage was absent. Immunofluorescence showed diffuse granular staining for IgG (2+), and C3 (2+) along the glomerular capillary walls (GCWs). Staining for IgA, IgM, and C1q was negative. Regarding the subclasses of IgG, strong staining was observed for IgG1, IgG2, and IgG4 and weak staining for IgG3 (Fig. ). Electron microscopy showed subepithelial electron-dense deposits (Fig. ). Consequently, a diagnosis of MN of Ehrenreich-Churg stage 1 was made. Additionally, we performed immunostaining for PLA2R and THSD7A of the glomeruli and rectal cancer tissues with metastatic lymph nodes to discriminate between idiopathic and secondary MN (Fig. ). In the glomeruli, granular staining for THSD7A along the GCW was observed with negative staining for PLA2R. In the rectal cancer tissue, THSD7A staining was positive, showing a mainly luminal pattern, which was reported as the staining pattern of rectal cancer []. THSD7A staining of metastatic lymph nodes revealed deposition in the secondary lymph follicles and in the sinus. Based on the above findings, a diagnosis of THSD7A-associated MN was made, which was associated with rectal cancer. We suspected the recurrence of rectal cancer; however, there was no tumor recurrence on chest and abdominal CT examination and colonoscopy. No lymph node enlargement was observed.
Treatment of tumors cures tumor-associated MN [], but the patient did not want chemotherapy, and therefore he was kept on observation with supportive therapy. Consequently, nephrotic syndrome still persists, but obvious recurrence and metastasis of the primary tumor have not been observed.
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pmc-6366365-1
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A 25-year-old female with a history of sickle cell disease (on prophylactic penicillin VK) and venous thromboembolic disease (on oral anticoagulation with apixaban) presented to the emergency department with one week of right-sided neck pain and subjective fevers, and a one day history of trismus. Physical examination revealed warmth, swelling and tenderness to the right lateral neck near the angle of the mandible, with associated mild trismus (). Additional history revealed the presence of bilateral, subclavian, implanted venous access ports. We ordered a computed tomography (CT) of the neck with intravenous (IV) contrast ().
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pmc-6366366-1
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A 34-year-old woman with no significant past medical history presented to our ED with acute onset of suprapubic pain two hours prior to arrival. Pain was sharp, constant and non-radiating with associated nausea and vomiting. She denied any fever, vaginal bleeding, vaginal discharge, dark or bloody stools, flank pain, dyspnea, or syncope. The patient reported no history of pelvic inflammatory disease (PID) or ectopic pregnancy. She stated that she had a copper IUD placed approximately three years prior. Her initial vital signs included a blood pressure of 140/81 millimeters of mercury, pulse of 96 beats per minute, respiratory rate of 20 breaths per minute, and temperature of 98.1° Fahrenheit. Physical examination was remarkable for moderate to severe lower abdominal tenderness to palpation with associated rebound and guarding.
Although a urine pregnancy test was ordered shortly after the patient arrived, while walking to the restroom, the patient sustained an episode of lightheadedness and near-syncope. Immediately following this episode, point-of-care transabdominal pelvic sonography was performed to further evaluate the etiology for the patient’s presentation.
A focused assessment with sonography in trauma (FAST) protocol revealed free fluid in Morison’s pouch and the splenorenal space, as well as in the pelvis. Transabdominal pelvic sonography also showed evidence of an IUD within the uterus without evidence of an intrauterine pregnancy. Extensive pelvic hematoma was noted surrounding the uterus (, ). Transabdominal ultrasound examination of the adnexa showed a thick-walled circular structure in the left adnexa () demonstrating marked hypervascularity (“ring of fire” sign) (,) as well as fetal cardiac activity consistent with a live ectopic pregnancy. Given these findings, emergent gynecology consultation was obtained. Initial laboratory studies showed mild anemia and leukocytosis (hemoglobin 10.9 grams per deciliter, white blood cell count 12.4 × 109 per liter). Serum beta-human chorionic gonadotropin was 24,976 milli-international units per milliliter. The patient was taken emergently to the operating room where a ruptured left tubal ectopic pregnancy with one liter hemoperitoneum was noted, and salpingectomy was performed. The patient remained hemodynamically stable, and was subsequently discharged in good condition.
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pmc-6366367-1
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A 69-year-old woman presented to the ED with painless vision loss in her left eye. She stated that over the course of minutes, she completely lost vision in that eye, with onset approximately six hours prior to evaluation. Initially, there was concern for posterior circulation arterial stroke, as the patient had elected to stop taking her antihypertensive medications one year prior. A computed tomography (CT) of the head showed no evidence of intracranial hemorrhage, and a magnetic resonance imaging (MRI) evaluation of the brain showed no evidence of acute stroke. The patient’s fundoscopic exam was limited by constricted pupils, and she was transferred to a tertiary care academic medical center for ophthalmologic evaluation.
On arrival to the tertiary care center, the ED team performed a POCUS, linear probe, 12 MegaHertz (MHz) of the patient’s eye to evaluate for retinal detachment. No evidence of retinal detachment, vitreous detachment, or massive vitreous hemorrhage was found. However, the study demonstrated a widened and irregular optic nerve sheath, which measured over the normal limit of five millimeters (mm). Additionally, an area of hyperechoic signal was noted in the distal aspect of the optic nerve, raising concern for embolic event (). Additional radiologist review of the patient’s MRI showed no evidence of embolism in that area. Ophthalmology was consulted and performed a dilated fundoscopic exam, with direct visualization of a pale, occlusive object within the central retinal artery.
The patient was admitted to the neurology service for monitoring of permissive hypertension initially, and then resumption of an antihypertensive medication regimen. The timing of symptom onset was a contraindication for thrombolytic treatment. Rapid stroke-risk stratification demonstrated no echocardiographic evidence of cardiac source of her embolus, no right-to-left cardiac shunt, and no significant carotid stenosis on CT angiography. She was re-initiated on her outpatient antihypertensive medications, and on two-month follow-up she had no significant return of vision in her left eye and had no evidence of further embolic events.
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pmc-6366368-1
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A 50-year-old woman with widely metastatic melanoma presented to the emergency department with dyspnea. She was found to be tachypneic, hypoxic, tachycardic, and hypotensive. A non-rebreather oxygen mask was placed and her oxygen saturation improved mildly. We obtained a semi-erect chest radiograph (CXR) followed by chest computed tomography angiography (CTA) (–), due to concerns for a pulmonary embolism. The CXR revealed a depressed left hemidiaphragm and a left pleural effusion. The CTA revealed a massive left pleural effusion causing left lung atelectasis, rightward mediastinal shift, and depression of the left hemidiaphragm.
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pmc-6366369-1
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A 21-year-old incarcerated male was brought by ambulance to the ED for evaluation of bilateral shoulder pain. Prior to arrival the patient had suffered a witnessed, generalized tonic-clonic seizure at the penitentiary. He was stabilized by on-site medical personnel at the institution. During examination in the ED, the patient was lucid and oriented despite transitioning out from a postictal state. Upon questioning, he reported shoulder pain that he described as similar bilaterally – moderate to severe in intensity, sharp in nature with generalized radiation to adjacent joint structures and apprehensive to movement secondary to discomfort. He denied numbness, tingling and presence of stingers of the affected upper extremities. On physical exam, the patient was in mild distress, both shoulders resting in slight abduction and external rotation.
Both humeral heads were palpated along the anterior aspect of each glenohumeral joint with global, painful restriction of range of motion bilaterally without any evidence of peripheral motor, sensory or vascular deficit.
Plain radiographs confirmed bilateral subcoracoid dislocations, with the humeral heads lying anteriorly, medially and inferiorly in respect to the glenoid fossae (). We performed prompt reduction using modified Davos technique without anesthesia or analgesia, followed by sling immobilization and subsequent rehabilitation ().
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pmc-6366370-1
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A 30-year-old Asian male presented with a history of generalized tonic-clonic seizures an hour before presenting to emergency department. He had a similar episode three years prior for which he had not sought any medical evaluation. He was conscious and oriented on presentation, and physical examination was unremarkable. Non-contrast computed tomography (CT) of the head revealed multiple cystic lesions on both cerebral hemispheres in different stages (–).
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pmc-6366371-1
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An 81-year-old male was referred by his general practitioner with a troponin-T of 153 nanograms per liter (ng/L) (reference range <5 ng/L) and chest pain ongoing for 13 hours on arrival. Initial electrocardiogram showed 7-millimeter anterior ST elevation in leads V2–5. The case was discussed with cardiology at the nearest tertiary care center and plans were arranged for the patient’s transfer for percutaneous coronary intervention. Thrombolysis was withheld due to a known abdominal aortic aneurysm and a suspicious renal mass under investigation. While awaiting transfer, the patient suffered a ventricular tachycardia arrest, and cardiopulmonary resuscitation (CPR) was commenced. Point-of-care echocardiogram was performed, showing a hypokinetic myocardium. After four rounds of CPR, thrombolysis was given as a last resort. Repeat point-of-care echocardiography demonstrated irrecoverable injury; therefore, CPR was discontinued ().
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pmc-6366372-1
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A 78-year-old Hispanic male, a resident of Mexico, presented to the emergency department (ED) of a level I county trauma center with a complaint of jaw pain for the prior three days. On review of systems, the patient also complained of abdominal bloating. His medical history was only significant for hypertension, although he did not take any medications. The patient did not report having allergies, and he denied the use of tobacco, alcohol, or drugs. His vital signs were as follows: temperature 36.9° Celsius, blood pressure 165/109 millimeters of mercury, pulse 88 beats per minute, and respiratory rate 18 breaths per minute.
The patient appeared to have difficulty opening his mouth and exhibited dysphonia as a result. He had no reproducible pain on exam, but was uncomfortable when we attempted to open his mouth by force. While the oral exam was limited secondary to poor mouth opening, no caries or abscesses were appreciated. No lymph nodes were palpable and the remainder of the ear, nose, and throat exam was unremarkable. The patient’s abdomen was rigid and mildly distended, but non-tender. On examination, the medial aspect of the right forearm revealed a healing laceration, approximately 5 × 2 centimeters. When questioned about the wound, the patient stated he had received it at work two weeks prior when he fell off a tractor and into muddy water. He stated he had been seen by a doctor in Mexico for the wound and was given a topical medication, which he had been applying. When asked about immunization status, the patient denied receiving tetanus prophylaxis for the wound and stated that as far as he could remember he had never received any childhood or adult vaccinations.
The patient’s blood tests and computed tomography of the head and neck were within normal limits. Based on exam and history, the likely diagnosis of tetanus was made. His wound was debrided. Based on CDC guidelines, we administered tetanus immunoglobulin, tetanus diphtheria and pertussis vaccine, and intravenous metronidazole. The patient was admitted to the medical intensive care unit (MICU) for further treatment.
In the MICU, the patient was treated symptomatically with two milligrams of lorazepam as needed for muscle spasms. On hospital day four, he had an apneic event and subsequent cardiac arrest. He was intubated with return of spontaneous circulation. He was unable to be weaned from the ventilator due to continued trismus. On hospital day 11, the patient had a tracheostomy and percutaneous gastrostomy tube placed, given concern for prolonged course on the ventilator. He had continued improvement in mental status along with fewer episodes of tetany. On hospital day 16, the tracheostomy became dislodged without respiratory compromise, and so was discontinued. On hospital day 18, the patient was transferred to the general medical ward. He was started on a clear liquid diet and was able to advance to full liquids during his stay. He was discharged to home on hospital day 22 with the formal diagnosis of tetanus.
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pmc-6366373-1
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A 21-year-old woman presented to the ED with complaint of chest pain and shortness of breath. Prior to arrival to the ED she had an episode of near syncope. Her previous medical history included development of diffuse erythematous rash following a course of minocycline prescribed for acne three months prior to ED presentation. The minocycline was discontinued, and she was treated with 30 milligrams (mg) daily oral prednisone with improvement of the rash. Initial vitals included blood pressure of 81/68 millimeters of mercury (mmHg), heart rate of 121 beats per minute (bpm), and respiratory rate of 18 breaths per minute. She was afebrile (36.7ºC oral temperature) and had pulse oximetry (SpO2) of 100% on room air. Physical exam was within normal limits. Electrocardiogram (ECG) showed right bundle branch block and normal ST-T segments, but no previous ECG was available.
While in the ED the patient had an episode of syncope during peripheral venous catheter placement, and intravenous (IV) fluids were administered due to concern of vasovagal event. She was also administered 5 mg IV dexamethasone due to possibility of adrenal suppression from steroid use. Her systolic pressure improved. However, the patient complained of worsening chest pain and then became unresponsive with pulseless electrical activity arrest (PEA). Cardiopulmonary resuscitation (CPR) and Advanced Cardiac Life Support were initiated. She received two doses of 1 mg IV epinephrine with return of spontaneous circulation (ROSC) in normal sinus rhythm of 70 bpm and blood pressure of 72/48 mmHg. Due to persistent hypotension, norepinephrine infusion was administered with improvement of blood pressure to 88/56 mmHg. She was intubated for airway protection.
Due to concern for massive pulmonary embolus, computed tomography chest angiography was performed but was unremarkable. Point-of-care echocardiogram demonstrated no right heart strain and grossly reduced heart function. Telemetry demonstrated QRS widening and increasing bradycardia to 41 bpm. The patient then developed a second PEA arrest with ROSC after CPR and one dose of 1 mg IV epinephrine. She remained hypotensive with blood pressure of 60/40 mmHg despite norepinephrine infusion. Initial troponin-T measured was 8.56 nanograms/milliliter (ng/mL) (reference range 0.0–0.02 ng/mL) and complete blood count with differential showed leukocytosis of 17.5 × 103 cells/mL (reference range 4.0–10.0 × 103 cells/mL) and eosinophilia of 1.6 × 103 cells/mL r (reference range 0.0–0.4 × 103 cells/mL). The cardiology service was consulted and a formal echocardiogram demonstrated a severely reduced ejection fraction of 15% (normal range 55–70%).
The dermatology service was also consulted at bedside in the ED; concern for ANEM in setting of DRESS given the rapidity of onset of her cardiac dysfunction was discussed. Also, the differential diagnoses included coronary vasculitis, viral myocarditis, infiltrative cardiomyopathy, and sepsis. (She was administered 4.5 g piperacillin/tazobactam and 20 milligrams/kilogram (mg/kg) vancomycin, and 500 mg azithromycin IV.)
While in the ED the patient developed further hypotension with blood pressure of 61/40 mmHg despite multiple vasopressors (IV vasopressin and norepinephrine infusions) and had severe acidemia and hypoxemia despite high ventilator support. Ventilator settings were tidal volume of 360mL, positive end-expiratory pressure of 20 centimeters of water (cmH2O), and fraction of inspired oxygen of 100%, and respiratory rate of 28 breaths per minute. Arterial blood gas (ABG) revealed respiratory acidosis with pH of 7.06, carbon dioxide partial pressure (pCO2) of 78 mmHg (reference range 33–43 mmHg), low arterial oxygen (PaO2) of 61 mmHg (reference range 80–100 mmHg), and normal bicarbonate. Basal metabolic panel was within normal limits. Due to continued decline, she received cannulation for veno-arterial extracorporeal membrane oxygenation (VA-ECMO) within the ED. ECMO cannulation occurred within three hours of initial cardiac arrest. Once placed on ECMO she had significant improvement in her acidosis and hypoxemia on repeat ABG (pH 7.39, pCO2 41 mmHg, PaO2 135 mmHg) and was rapidly weaned from her vasopressors.
The patient was admitted to the cardiac intensive care unit (ICU) for further management. Due to concern of DRESS-induced myocarditis, dexamethasone with a dose of 1 mg/kg/day was administered intravenously. On hospital day (HD) eight, cardiac biopsy demonstrated diffuse active myocarditis with coagulative myocyte necrosis and mixed infiltrate including eosinophils consistent with DRESS myocarditis. She was decannulated from ECMO on HD 16. She was eventually discharged after 20 days in the ICU and 62 total days in the hospital with a life vest and continued cardiac follow-up. An echocardiogram performed three months later demonstrated improved ejection fraction of 34%.
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pmc-6366374-1
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A 48-year-old woman with history of congenital hydrocephalus and bilateral VP shunt placement presented to the emergency department (ED) with three weeks of progressively worsening, right-sided abdominal distension and pain. The pain was dull, constant, non-radiating, and unrelated to meals. She reported passing flatus and denied fevers, chills, nausea, vomiting, headache, visual changes, changes in urination, constipation, melena, or bright red blood in her stools. Her past surgical history was notable for placement of a right VP shunt terminating in her right lower abdomen (last revised 10 years prior) and a left VP shunt terminating in her left lower abdomen (last revised two years prior). She denied a history of other abdominal surgeries.
On examination, the patient appeared comfortable, was afebrile, and had a heart rate of 84 beats per minute, respiratory rate of 16 breaths per minute, blood pressure of 150/80 mmHg, and oxygen saturation of 99% on room air. Her abdominal exam was notable for morbid obesity and distension of the right upper and lower abdomen, which was dull to percussion. Her abdomen was minimally tender in the right upper and lower quadrants, but not rigid or tense. There was no erythema or other skin changes overlying her VP shunt reservoirs. Fundoscopic and neurologic exams, including gait, were unremarkable. A complete blood count, comprehensive metabolic panel, lipase, and urinalysis were normal. POCUS revealed a large fluid collection with septations in the soft tissue of the right abdominal wall as seen in and . Neurosurgery was consulted and requested a CT of the abdomen and pelvis with intravenous contrast, which confirmed a diagnosis of an abdominal pseudocyst as seen in . Given that the patient had no infectious symptoms or signs of VP shunt malfunction, she was scheduled for an urgent, outpatient revision of her right VP shunt.
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pmc-6366375-1
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A 38-year-old female presented to the emergency department (ED) with rash, dyspnea, odynophagia, and nasal congestion for the prior two weeks. During that time, she sought medical care twice. The first physician to evaluate the patient started her on antibiotics for a presumed upper respiratory infection (URI). Her symptoms did not improve after completing a 10-day course of amoxicillin; then a second medical provider prescribed her ciprofloxacin. She was on her eighth day of ciprofloxacin (i.e., total 18th day of treatment) when she presented to our ED with rash and dyspnea. She decided to come to the ED because her cough had worsened and become productive of sputum. She also complained of one month of fevers, chills, night sweats, and malaise. She denied any complaints of headaches, chest pain, palpitations, abdominal pain, genitourinary or neurologic symptoms.
Her past medical history was significant for adult-onset asthma and allergic rhinitis. Medications included fluticasone, ipratropium, and her recent courses of amoxicillin and ciprofloxacin. She had no known medication allergies but reported gastrointestinal intolerance to fish oil. Her family history was significant for a sister with multiple sclerosis. She was an Iranian immigrant who had moved to Baltimore six months prior to presenting in our ED. She was married with no children and denied ever using tobacco, alcohol or illicit drugs.
On physical exam, she was alert but appeared uncomfortable as she hobbled into triage that night. She was afebrile (36.7° Celsius) and mildly tachycardic (heart rate of 110 beats per minute). Her blood pressure was 102/68 millimeters of mercury, she was mildly tachypneic with a respiratory rate of 20 breaths per minute, and her oxygen saturation was 97% while breathing room air. She was well developed and well nourished, with an estimated body mass index of 22. Her head was normocephalic and atraumatic. Her oropharynx was clear; her neck was supple and no lymphadenopathy was detected. On auscultation, she was tachycardic with a normal S1 and S2, without any murmurs, gallops or rubs. She was mildly tachypneic without any accessory muscle use, retractions, or increased work of breathing. She was able to speak in full sentences without difficulty. The patient’s lungs were clear to auscultation bilaterally without wheezes, rhonchi, or rales. Her abdomen was soft and non-tender, and no lower extremity edema was present. She was alert, oriented and appropriately interactive.
On closer examination of the patient’s skin, her rash appeared to have three different morphologies. The first was located on her forehead and consisted of sub-centimeter papulovesicular eruptions with petechiae that were pruritic but not tender (). The second rash consisted of scattered hemorrhagic vesicles with purpuric macules and was located on her distal upper and lower extremities (). The third rash was an erythematous and indurated plaque at the base of the left foot, which was tender and made it painful for her to walk.
Initial laboratory results are shown in –. The patient’s electrocardiogram showed sinus tachycardia with normal intervals and without ST-segment or T-wave abnormalities. Bilateral multilobar infiltrates were revealed on chest radiograph (). A computed tomography (CT) of her chest confirmed the presence of bilateral multilobar infiltrates and a CT of her sinuses showed mucosal thickening throughout. An echocardiogram revealed a normal ejection fraction and no valvular pathology. No vegetations were seen. The patient was admitted to the hospital for further evaluation. A test then was performed, which confirmed the diagnosis.
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pmc-6366376-1
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A 23-year-old male presented to the emergency department (ED) with pain in his lower back radiating down his left leg. He had awoken from sleep five days prior with lower back pain radiating into both legs. He went to a hospital in another city where he was prescribed morphine, cyclobenzaprine, and naproxen. His pain was initially controlled enough that he could return to work while taking naproxen. The pain continued to progressively worsen and had begun to cause difficulty walking. He went to a family physician who suspected sciatic nerve pain and sent him to the ED for imaging of his spine.
On presentation to the ED, his pain was 7 to 8.5 out of 10. There was no history of trauma, and he had no bowel incontinence, urinary retention, or saddle anesthesia. He had no recent fevers, chills, or weight loss. His appetite was decreased due to his pain, and he had not had a bowel movement in three days. He had no significant past medical history and usually took no medications. He had consumed seven to nine alcoholic drinks and used cocaine the night before the pain began.
On examination, his temperature was 37.3°C, his heart rate was 96 beats per minute, his respiratory rate was 16 breaths per minute, his blood pressure was 124/60 millimeters of mercury, and his oxygen saturation was 100%. His abdomen was soft and non-tender. Testicular and rectal exams were normal. Palpation of the left sacroiliac joint revealed exquisite tenderness, identifying the more precise location of the lower back pain. Neurologic exam revealed normal tone, strength, and coordination in all extremities. Radiographs of the pelvis and sacroiliac joints were normal.
On laboratory workup, white cell count was 6.89×109/liter (L) (normal range, 4.5–11), serum hemoglobin was 100 grams (g)/L (normal range, 140–180), platelet count was 143×109/L (normal range, 150–350), and C-reactive protein (CRP) was 227 milligrams/L (normal range, <8). Peripheral blood smear showed increased polychromasia, some poikilocytosis with occasional teardrop cells, mature neutrophils, roughly 20% circulating blasts, and rare giant platelets, all consistent with acute leukemia. In the ED he was administered two milligrams (mg) of hydromorphone and 600 mg of ibuprofen orally. He was later admitted to the hematology service. He was diagnosed with precursor B-cell ALL and initiated on the Dana-Farber chemotherapy protocol. Two years post-diagnosis, his leukemia was in remission and he had recently completed his final cycle of chemotherapy.
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pmc-6366377-1
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A 74-year-old male with a history of metastatic prostate cancer presented to the emergency department with hypotension and shortness of breath. We assessed volume status using point-of-care ultrasound (POCUS) with a phased array probe in the subxiphoid orientation. This revealed a large inferior vena cava (IVC) thrombus extending from above the IVC bifurcation into the right atrium (, ). The patient was started on intravenous heparin and fluids. Computed tomography (CT) pulmonary angiogram revealed an occlusive pulmonary embolism (PE) in the right lower lobe.
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pmc-6366378-1
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An 18-year-old woman was brought to the ED by ambulance. Paramedics reported that the patient was on the phone with her mother and said she wanted to be dead. Her mother lives in another country and called emergency services. The patient was tearful and “very stressed” on arrival. Vital signs, a routine urine toxicology screen, and pregnancy test were unremarkable. She reported suicidal thoughts for about a week attributed to poor grades in college, family conflict, and financial obligations. She had missed several appointments with her therapist and prescriber and had recently run out of sertraline (Zoloft). She declined to provide her mother’s phone number.
The patient described a history of abuse at a young age. She had one prior psychiatric hospitalization after walking into traffic in a suicide attempt at age 15. Other episodes of self-harm started at age 10 and were non-suicidal in nature. Her biological father had minimal contact with the patient. Her grandmother had been diagnosed with schizophrenia. The patient denied access to firearms.
Concerned about multiple suicide safety risk factors, the emergency psychiatrist began a structured, single-session psychotherapy. The psychiatrist and patient wrote a timeline of events preceding the presentation (). In so doing, she provided more details of her history. Ten months prior, she had to leave her apartment due to conflicts with roommates. Beginning college, she worried about tuition and found two jobs. Despite several attempts to re-schedule her therapy appointments around her work schedule, the therapist’s office did not return her calls. The patient also revealed that a supportive stepfather lived nearby. The morning of her ED visit, she received another reminder about her tuition bill. She was talking with a roommate about this bill; however, she felt her roommate did not fully appreciate her challenges, and she then called her mother.
The psychiatrist and patient agreed all this would be stressful for anyone. Her affect evolved from tearful to more composed, and she identified some immediate goals: find a new therapist; talk with her school about a tuition grant; identify a tutor; and spend more time doing things she enjoys (). She agreed to let the resident call her mother who could help complete these tasks. The patient’s mother corroborated the patient’s history. In fact, the mother had already spoken with the school about help with tuition and had begun searching for new outpatient providers.
To complete discharge planning, a nurse made an appointment for the patient with a new outpatient provider. The patient completed a written safety plan and was offered a follow-up call. The family was apprised of local crisis resources. Within an hour, the evaluating psychiatrist felt that this patient’s acute risk was significantly mitigated through safety planning, mobilization of social supports, connection to treatment, and acute de-escalation to justify discharge. After six months, she had persistent resolution of suicidal thoughts without recurrent self-harm or inpatient hospitalization.
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pmc-6366379-1
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A 17-year-old female with history significant for twice-weekly migraines presented to the ED with 12 hours of a persistent right-sided occipital migraine described as constant, sharp, and 10/10 in severity. The patient had associated nausea, with no other neurologic symptoms, and no recent fever or head trauma. The patient took acetaminophen and sumatriptan at home, which helped for two hours, before subsequent recurrence. On exam, the patient had mild right occipital tenderness to palpation, with no midline spinal tenderness and no neurologic deficits. The patient received metoclopramide 10 mg in triage and one liter of normal saline. Approximately 40 minutes after initial treatment, the patient noted her pain had improved from 10/10 to 8/10 severity. The patient then received one milliliter (mL) injection of 1% lidocaine 1cm to the right GON. Approximately 60 minutes after medications had been given, and ten minutes after occipital nerve block, the patient noted her pain improved to 2/10. During follow-up phone interview at seven days, the patient noted her symptoms completely resolved one hour after discharge, and that over last seven days she had not had any further migraines.
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pmc-6366379-2
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A 48-year-old female presented to the ED with three days of persistent bilateral occipital pain that was constant, sharp in quality, and was 8/10 in severity. The patient had past medical history only significant for hyperlipidemia and migraines. The patient usually suffered one to two migraines per month. In addition to her headache, the patient also endorsed nausea and three episodes of emesis. She took sumatriptan, acetaminophen, and ibuprofen in the 48 hours prior to arrival with minimal relief. The patient denied any other symptoms. On exam, the patient was noted to have mild bilateral occipital tenderness to palpation and no neurological deficits or midline tenderness. In ED triage, the patient received metoclopramide 10 mg and ketoralac 15 mg intravenously. Approximately 60 minutes after the patient received these medications she was re-assessed and found to have persistent head pain rated at a 7/10 in severity. Bilateral GON blocks were administered with a total of one mL of 1% lidocaine to each site. At 15 minutes and 1.5 hours post-procedure, the patient reported pain improvement to 3/10. During follow-up phone interview at nine days post-emergency department visit, patient noted her pain had resolved over the course of 24 hours, with no recurrence of a migraine.
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pmc-6366379-3
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A 37-year-old male presented to the ED with past medical history significant for anxiety and once monthly migraines. The patient described the pain as originating from the back of his head and radiating forward. The pain was located only to the right side, was constant and sharp in nature, and rated at a 10/10 in severity. The patient had the pain for 12 hours. The patient noted that the pain was typical for his migraine; however, his typical home abortive medication, ibuprofen, did not work for him on this occasion. The patient also tried one hydrocodone/acetaminophen 5/325 three hours prior to arrival (which he had obtained during previous emergency department visits for the same head pain) but without improvement. The patient denied any recent head trauma, fevers, or neurological deficits. On exam, the patient had no midline spinal tenderness, no motor/sensory deficits, or cranial nerve abnormalities. The patient was noted to have right occipital tenderness to palpation. The patient was given metoclopramide 10 mg, one liter of normal saline, and diphenhydramine 25 mg by the ED triage physician. Approximately 45 minutes after the medications were given, the patient was re-assessed and stated his pain had improved from a 10/10 to an 8/10. The patient then received one mL of 1% lidocaine to the right GON. Approximately 60 minutes after the patient received the initial medications, and three minutes after the patient received the occipital nerve block, the patient reported the pain had improved to 2/10. Follow-up phone call interview conducted at day eight revealed that the patient’s migraine never recurred. The patient noted that his symptoms had completely resolved following the injection and that if he had a migraine again, he would preferentially seek out an occipital nerve block.
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pmc-6366380-1
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A 52-year-old female with hypertension and no other past history of cardiopulmonary disease presented to the emergency department (ED) in acute respiratory distress. Two days prior to arrival, the patient underwent a total laparoscopic hysterectomy, which was complicated by a prolonged surgical course of six hours and an estimated blood loss of 1,500 milliliters. The patient suffered from symptomatic post-operative anemia and was given two units of packed red blood cells on post-operative day one. The patient experienced mild shortness of breath shortly after the transfusion but was cleared by pulmonology for discharge after maintaining normal vital signs during a trial of ambulation and lacking the appropriate clinical evidence to support a diagnosis of TRALI. The patient endorsed progressively worsening dyspnea at home, which prompted her to return to the ED less than 24 hours after being discharged.
Initial vital signs were notable for tachypnea to 30 breaths per minute, an oxygen saturation of 77% on room air, tachycardia to 107 beats per minute, blood pressure of 177/94 millimeters of mercury, and an oral temperature of 101.8° Fahrenheit. The patient was in moderate respiratory distress with suprasternal retractions, accessory muscle use, diffuse rales, anxiety, diaphoresis, and speaking in short phrases. Her electrocardiogram showed sinus tachycardia without evidence of acute ischemia or infarction.
Chest radiography () and a computed tomography (CT)-pulmonary angiography () revealed bilateral pulmonary edema, which was not present on prior imaging. No pulmonary embolism was seen. The patient remained hypoxic to 90% oxygen saturation despite receiving eight liters of oxygen by non-rebreather mask. The patient was given 40 milligrams (mg) intravenous (IV) furosemide, 0.4 mg sublingual nitroglycerin, and one gram IV acetaminophen. Her respiratory status remained unchanged with these interventions, so noninvasive ventilation was initiated with continuous positive airway pressure at five centimeters water.
The patient was admitted to the medical intensive care unit given her need for noninvasive ventilatory support. She underwent diuresis and weaning of her respiratory support. On hospital day three, the patient was discharged following complete resolution of her respiratory symptoms.
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pmc-6366381-1
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A 34-year-old female with a history of methamphetamine-associated cardiomyopathy presented to the emergency department (ED) with generalized weakness, altered mental status, and chest pain. She reported a recent placement of an automatic implantable cardioverter-defibrillator at an outside hospital three months prior to current presentation and had a documented ejection fraction of 15%. Upon arrival to the ED, she was hypotensive with a systolic blood pressure ranging in the 40s to 70s millimeters of mercury and was hypothermic at 33.6 degrees Celsius. She appeared cachectic and had a 3/6 systolic ejection murmur at the left upper sternal border. We performed a point-of-care ultrasound (POCUS) to assess the patient’s cardiac function and found a large left ventricular (LV) thrombus measuring 5.8 × 2.8 centimeters (). Further views of the thrombus seen in the reveal a large hyperechoic density in the left ventricle. The patient was admitted to the intensive care unit for vasopressor support and thrombolytic therapy.
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pmc-6366382-1
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A 95-year-old female with a history of dementia and atrial fibrillation (not on anticoagulation) presented to the ED by ambulance from her skilled nursing facility due to hypoxia. The patient had been requiring 2–4 liters of oxygen via nasal cannula at her nursing facility; however, in the ED the patient’s oxygen saturation was 80% on a non-rebreather face mask. Physical exam was notable for tachycardia, tachypnea, use of accessory muscles for respiration, and somnolence. The patient did not have clinical signs of DVT such as unilateral leg swelling or calf tenderness. Of note, the patient had an allergy to iodinated contrast. POCUS was performed and showed evidence of a large mobile thrombus in the right ventricle on apical four-chamber view (). Further evidence of associated right heart strain was seen in the corresponding parasternal short-axis view. There was no evidence of a pericardial effusion. These ultrasound findings in combination with the patient’s clinical presentation were diagnostic of acute PE with right heart strain., The patient’s family arrived in the ED, and her code status was established as “do not resuscitate” with comfort measures only. Further imaging, fibrinolysis and thrombectomy were not attempted in accordance with the patient’s wishes.
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pmc-6366383-1
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A 38-year-old white male who is an avid skier presented with left calf pain described as sharp on the medial aspect that started after he fell while skiing about two weeks prior. During the fall, his left ankle was forced into extreme dorsiflexion. He had immediate onset of severe pain and had to stop skiing that day. He noticed swelling in the medial calf along with bruising of the area over the next few days. After about a week, he was able to ski again but had pain aggravated by active plantar flexion.
On physical examination of his left leg, his knee and ankle both appeared normal and had normal strength and range of motion with no tenderness. Sensation and pulses were normal. There was tenderness over the medial head of his gastrocnemius with a palpable, firm, four-centimeter ovoid mass. Pain was elicited during resisted plantar flexion. His right leg was completely normal.
Radiographs of the lower leg obtained in the ED were normal. A POCUS was then performed in the ED with specific focus over the mass in the medial head of the gastrocnemius, revealing a swollen, heterogeneous, disorganized mass in the medial head of the gastrocnemius, as demonstrated in –. Comparison views to the unaffected extremity, as seen in , further clarified the findings. We made a diagnosis of muscle tear in the medial head of the gastrocnemius.
The patient was instructed on exercises for strengthening and range of motion of the calf and ankle and specifically eccentric exercises that he should complete after an initial rest period of two weeks. On follow-up two months after the initial visit, his symptoms had nearly completely resolved. He reported mountain biking several times per week with only minimal pain after a long ride. The mass in the calf resolved, and he relayed no concerns about his leg for the upcoming ski season.
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pmc-6366384-1
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A 32-year-old female gravida 3 para 2 presented to the emergency department (ED) with two weeks of hyperpigmented macular and blistering rash involving bilateral upper and lower extremities () and trunk (). The patient was approximately 16 weeks pregnant at time of presentation. The rash was significantly pruritic. She denied constitutional symptoms or mucous membrane involvement. The patient was seen by obstetrics/gynecology consult who deemed her rash consistent with pemphigoid gestationis (PG). She was started on high-dose steroid therapy with improvement in the rash. ELISA (an enzyme-linked immunosorbent assay that measures autoantibody reactions to the bullous pemphigoid antigen [BP180] with 96% sensitivity and specificity for PG) showed our patient’s values elevated at 30.59 units (reference range less than 9.0 units). By the 35th week of pregnancy, her rash had resolved while on a stringent steroid regimen. She developed gestational diabetes that is being managed by her prenatal care provider.
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pmc-6366385-1
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A 55-year-old female presented to the emergency department with an enlarging forehead mass after a fall with head injury two weeks prior. She reported focal, tender swelling to her right forehead and headache. Physical examination revealed a two-centimeter, soft, pulsatile mass to her right frontotemporal region (). Point-of-care ultrasound (POCUS) with color Doppler revealed a dilated vascular structure with pulsatile, bidirectional flow – the “yin-yang” sign (). The diagnosis of traumatic superficial temporal artery (STA) pseudoaneurysm was confirmed by computed tomography (CT) angiography with three-dimensional reconstruction (). The patient’s pseudoaneurysm was surgically ligated and she recovered uneventfully.
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pmc-6366386-1
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A 39-year-old gravida one para zero woman at an estimated 15 weeks four days gestation presented to the emergency department (ED) with 10 days of progressive, severe abdominal pain. She also reported moderate vaginal bleeding for the prior several months along with intermittent nausea and vomiting. Six weeks prior to presentation, she was seen in clinic where her obstetrician performed a point-of-care ultrasound (POCUS). It was documented that the patient had an intrauterine pregnancy with an estimated gestational age of nine weeks and zero days.
In the ED she was tachycardic to 131 beats per minute and normotensive at 116/84 millimeters of mercury. Her exam was significant for tenderness to palpation of her entire abdomen with rebound and guarding present. Her labs were significant for a moderate anemia with a hemoglobin of 9.2 grams per deciliter along with a leukocytosis of 13,200 white blood count per millimeter cubed, and mild elevations of her alanine aminotransferase and aspartate aminotransferase at 76 units per liter (u/L) and 53 u/L, respectively. Limited POCUS identified a fetus measuring 16 weeks one day by biparietal diameter with a heart rate of 163 bpm. Oligohydramnios was noted. Obstetrics was consulted at that time. Shortly afterwards, the patient was taken for a formal ultrasound. This showed free fluid and clotted blood throughout her abdomen and was initially concerning for uterine rupture ().
The patient was taken to the operating room for an exploratory laparotomy and was found to have a ruptured left tubal ectopic pregnancy. She underwent a left-sided salpingo-oophorectomy and required four units of packed red blood cells. She did well post-operatively and was discharged home on post-operative day two.
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pmc-6366387-1
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An 11-year-old female was brought to the emergency department with left hip and knee pain as well as limping for three weeks. There was no fever or recent trauma. Physical examination revealed restricted range of movement due to pain on hip flexion, internal and external rotation. A point-of-care ultrasound (POCUS) performed by an emergency physician () raised the suspicion for her diagnosis when compared with right side (), which prompted expedited immobilization and pain control. POCUS was performed using a linear, high-frequency probe (14–5 MegaHertz) aligned parallel to the femoral neck. Subsequently, her pelvic radiograph () confirmed the diagnosis.
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pmc-6366388-1
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A 30-year-old man presented to the emergency department (ED) complaining of right-hand pain after punching a wall in anger approximately one hour prior to arrival. On examination, there was obvious deformity of the dorsal aspect of the hand with a palpable bony step-off extending across the distal aspect of the wrist. Neurovascular examination of the hand and digits was normal. We obtained standard posterior-anterior () and lateral () radiographs of the wrist.
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pmc-6366389-1
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A 48-year-old male with past medical history of hypertension, diabetes, and schizophrenia was brought to the ED for acute altered mental status and combative behavior at home. Family reported a history of hallucinations and a recent medication change to haloperidol (). In the ED the patient presented lethargic with a Glasgow Coma Scale (GCS) of 4, foaming from the oropharynx, and rigid. Vitals included an intravesical temperature of 109.6°F, blood pressure of 143/129 millimeters mercury (mmHg), pulse of 133 beats per minute (bpm), respiratory rate of 12 breaths per minute and irregular, and 100% saturation on high oxygen flow via nasal cannula. The patient’s total creatine kinase was 28.482 units per liter (U/L) and troponin of 0.75 nanogram per milliliter (ng/mL) with ECG revealing lateral depressions.
The patient was intubated for airway protection and immediately cooled with evaporative cooling measures. Additionally, the patient’s rhabdomyolysis was managed with vigorous hydration. The cardiology team determined the patient was not stable enough for urgent cardiac catheterization, and heparin drip was started. Given the patient’s hyperthermia and muscle rigidity, NMS was suspected and an intravenous one milligram per kilogram (mg/kg) bolus dantrolene was administered in the ED. He was admitted to the intensive care unit (ICU) with a diagnosis of NMS, rhabdomyolysis, respiratory failure, and non-ST-elevation myocardial infarction. The ICU treatment team began bromocriptine at a dose of 2.5mg per nasal gastric tube every six hours per neurology recommendations and cooling through Arctic Sun 5000 Temperature Management System™.
He also experienced multi-organ insult including hepatic shock and acute renal failure. Furthermore, he continued to experience labile temperatures with episodic fevers (). However, blood and urine cultures and cerebrospinal fluid (CSF) analysis were unremarkable. Additionally, he had acute loss of consciousness with wavering mentation, likely secondary to toxic metabolic encephalopathy, with GCS ranging from 4–11. After several failed multiple attempts for extubation, the patient subsequently required tracheostomy. He was discharged to an extended care facility for tracheostomy care after four weeks of inpatient management.
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pmc-6366389-2
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A 21-year-old male with past medical history of autism and psychiatric disorder on risperidone was brought to the ED by his family with concern of altered mental status. The family reported that the patient had been somnolent, nonverbal, febrile, and had developed an unsteady gait. The patient was presenting one-week post treatment with a depot dose of risperidone (). In the ED, the patient was nonverbal with a GCS of 11, rigid, somnolent but able to follow basic commands, and displayed masked facies. Vitals included a temporal temperature of 99.3°F, blood pressure of 146/97 mmHg, pulse of 125 bpm, respiratory rate of 20 breaths per minute and oxygen saturation of 98% at ambient air. Laboratory examination was significant for creatine kinase 1092 U/L. Urine drug screen, comprehensive metabolic panel, hematology, and CSF analysis were unremarkable. The patient’s ECG revealed sinus tachycardia with a heart rate of 102 bpm but was otherwise normal.
The patient was started on an intravenous 1mg/kg bolus of dantrolene, followed by 1mg/kg intravenously every six hours in the ED. He was admitted to the ICU for close monitoring. On hospital day two, he began to show improvement in alertness and cognition but remained mostly somnolent with no improvement in muscle rigidity, and he spiked a fever of greater than 102.3°F (). After four days, the intensive care team and neurology adjusted the dantrolene regimen to 40mg intravenously every six hours. Dantrolene was discontinued after 15 days and he was started on a 2.5mg dose of bromocriptine twice per day, which was subsequently adjusted to 5mg every eight hours.
By hospital day six, he was following basic commands and showing progressive symptomatic improvement. The patient continued to improve and by hospital day 10 demonstrated significant improvement in both gross and fine motor skills. He was subsequently discharged on hospital day 18 with continued bromocriptine treatment and close follow-up.
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pmc-6366390-1
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A 33-year-old obese male with a history of well-controlled type II diabetes and hyperlipidemia presented to the emergency department with a one-day history of recurrent non-bloody diarrhea and abdominal pain in the morning progressing to significant nausea, increased non-radiating abdominal pain, and multiple episodes of non-bilious, non-bloody emesis in the evening. The patient reported 8/10 non-radiating, sharp, epigastric abdominal pain upon arrival. Physical examination findings revealed tenderness and rigidity in the right lower quadrant. The patient had an initial white blood cell count of 22.9 cells/millimeter3 (mm3) anion gap of 16 milliequivalents per liter (L), glucose level of 203 millimoles per liter (mmol/L), and a lactate of 3.01mmol/L. A computed tomography (CT) of abdomen and pelvis with intravenous contrast showed a mild wall thickening of the terminal ileum with multiple reactive mesenteric lymph nodes in the right lower quadrant indicative of inflammation, and a small volume of hepatic gas in the left hepatic lobe (). Point-of-care ultrasound of the abdomen confirmed the presence of hepatic gas in the left hepatic lobe (). The patient received two L of normal saline and was reevaluated showing significant pain relief. Ciprofloxacin, metronidazole, and vancomycin were given to treat an infectious etiology causing terminal ileum inflammation and diarrhea. The patient was admitted with gastroenteritis and subsequently discharged.
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pmc-6366391-1
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A 38-year-old female with a history significant for depression and polysubstance abuse presented to the emergency department (ED) for altered mental status and decreased respiratory rate. She was placed in a resuscitation bay, where she was noted to be obtunded with minimal responsiveness to painful stimuli. She was also experiencing respiratory depression with bradypnea. Given clinical presentation and concern for opioid toxidrome with respiratory depression, the patient received two doses of 0.4 mg of naloxone.
Following administration, the patient’s depressed mental status resolved and respiratory rate increased. She subsequently became acutely agitated, requiring haloperidol for sedation. She was monitored in the ED, receiving supportive care and intravenous fluids. After she received haloperidol, she experienced altered mental status, which persisted for the next 12 hours. She did not have any respiratory depression during the monitoring after haloperidol administration, likely suggesting the altered mental status was related to the administration of the haloperidol.
Chart review revealed prior hospitalization for altered mental status, likely polysubstance overdose, with gas chromatography / mass spectrometry (GC/MS) during that admission positive for bupropion, venlafaxine, and kratom. During previous hospitalization, she did confirm that she had consumed kratom and otherwise denied current drug use. She was admitted to the hospital for continued altered mental status, which improved with supportive care over the next 24 hours. GC/MS analysis of her urine during this visit was positive only for the presence of kratom and did not show other opioids. The patient admitted to using kratom upon discharge, though she denied intentional overdose.
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pmc-6366392-1
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A 77-year-old male with a history of ventricular bigeminy presented to the emergency department complaining of arm tingling, pain, and poikilothermia. This occurred immediately after the patient reached to use the television remote control device. His right forearm and hand were dusky, cold, pulseless, and had delayed capillary refill compared to the left arm. Strength was intact but light touch sensation was decreased. The emergency physician (EP) performed a point-of-care ultrasound, which showed an occlusive distal brachial, proximal ulnar, and proximal radial artery thrombus (, ). Vascular surgery was consulted and within two hours of arrival the patient was in the operating room without any additional vessel imaging. An embolectomy was performed using a Fogarty catheter. Arterial flow was restored, the hand was revascularized, and the patient’s symptoms resolved. A transesophageal echocardiogram was performed postoperatively, which showed a left atrial appendage thrombus. He was started on lifelong anticoagulation and discharged from the hospital.
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pmc-6366946-1
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A 51-year old male patient with a chronic history of asthma managed with β2 adrenergic receptor agonists (salbutamol) was admitted to the asthma clinic at the University hospital of Verona (Italy) with wheezing, coughing, whistling sounds during breathing and worsening dyspnea, lasting for approximately 24 hours. The physical examination revealed wheezing during normal breathing, prolonged phases of forced exhalation and use of accessory muscles for breathing. As commonplace in these cases, the physician immediately prescribed a blood gas analysis for investigating the effectiveness of gas exchange and state of voluntary respiratory control. Venous rather than arterial blood was hence initially drawn by a nurse into a 3.0 mL heparinized syringe (Smiths Medical, Minneapolis, MI, United States), and delivered by hand to the central laboratory, for analysis of blood gases.
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pmc-6367106-1
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A 41-year-old male with a past medical history of intravenous drug abuse (IVDA) presented to our emergency department as a transfer following two days of medical management of rhabdomyolysis at an outside community hospital. He initially presented with weakness and numbness of the lower extremities. He had been using intravenous heroin and was lying supine on a floor for approximately 24 hours.
Upon presentation to our emergency department, the patient had pain in his bilateral lower extremities and no appreciable motor function below the level of the knee. He was in mild distress with a temperature of 37°C, pulse of 104 beats per minute, blood pressure of 146/56 mmHg, respiratory rate of 18 and oxygen saturation of 97% on room air. Examination of the lower extremities revealed erythema and tension in the bilateral posterior thigh compartments. In contrast, the medial thigh compartments bilaterally along with all four leg compartments were soft. Gluteal and buttock compartments were soft and compressible bilaterally. Motor strength testing revealed 1/5 strength bilaterally with manual testing of ankle dorsiflexion and plantarflexion, and knee flexion and extension. The patient was found to have bilateral sciatic nerve palsies, with no sensation bilaterally in the tibial nerve, deep and superficial peroneal nerve distributions. Both legs were warm and pink with brisk capillary refill along with 2+ dorsalis pedis pulses bilaterally. Provocative testing demonstrated pain with passive range of motion of the knees bilaterally and no pain with passive ankle dorsiflexion and plantarflexion. Lab work was significant for a creatinine phosphokinase (CPK) of 231,360 U/L.
Given the high suspicion for compartment syndrome, the patient underwent compartment pressure testing via Stryker pressure monitor system. Diastolic blood pressure recording measured 75 mmHg. The right posterior thigh compartment measured 75 mmHg and left posterior thigh compartment measured 30 mmHg. Based on the history and physical examination findings, the patient was diagnosed with bilateral thigh compartment syndrome likely secondary to his recent IVDA and subsequent immobilization and was taken to the operating room for urgent bilateral posterior thigh fasciotomies.
In the operating room, the right posterior compartment was approached first. The intermuscular septum was released with the fascia along the posterior compartment for full release of both the anterior and posterior compartments of the right thigh (Figure ).
Following fasciotomy, cautery was used to confirm the viability of the exposed muscles in the anterior and posterior compartments. The skin surrounding the incision for the compartment release was too tense for safe closure, and so it was decided to place a wound vac. A 22 x 8-cm negative pressure vacuum-assisted closure (VAC) dressing was then placed over the wound (Figure ).
Attention was then paid to the left thigh. Compartment pressures were rechecked. The left posterior thigh compartment measured 30 mmHg. Given these findings, the decision was made to forego the compartment release due to the morbidity associated with fasciotomies.
Serial wound vac changes were then carried out on postoperative day (POD) two and four. On POD six, we removed the wound vac and placed a DermaClose device for assistance with skin closure. On POD 10, his skin edges were closable and so he underwent complex wound closure approximately 20 cm in length.
Throughout operative treatment and recovery, the patient was given antibiotics and developed no further complications. His CPK levels returned to normal. He was discharged on antibiotics and was given counseling on the potential adverse effects of IV-drug abuse. He had a favorable recovery at two-week follow-up.
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pmc-6367108-1
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A 64-year-old female with past medical history of diabetes mellitus presented to the emergency department in November 2017 for left eye pain of two days duration followed by left-sided neck rash. She complained of intermittent, shooting, burning pain along the distribution of this rash. She was examined and found to have nonconfluent grouped vesicles on the posterior neck and diagnosed clinically with herpes zoster reactivation (shingles). Initial electrocardiography (EKG) was unremarkable (no conduction blocks or evidence of pericarditis). She was treated with valacyclovir and oral steroids and advised to seek urgent ophthalmological evaluation at a specialized center. She was readmitted in March of 2018 after presenting with altered mental status. EKG performed at that time showed a new first degree atrioventricular (AV) block as shown in Figure below.
Admission vitals were blood pressure of 96/58, heart rate of 65 beats per minute. Laboratory studies done on admission were unremarkable except for hypoglycemia at 32. Later on that day, she developed bradycardia at 49 beats per minute with blood pressure 117/63 mm Hg. Repeat EKG was performed, this time showing Second Degree AV block (Mobitz I) as seen in Figure .
She denied any fever, chills, arthralgias, myalgias, vomiting or diarrhea. She also denied any chest pain, palpitations, or shortness of breath. Electrolyte abnormalities were corrected and she was continuously monitored on telemetry and followed with serial EKGs (Figures -).
She had a positive chronotropic response with exertion and awakening. Echocardiography done during admission showed preserved left ventricular function (60%) with no wall motion abnormalities and mild grade 1 diastolic dysfunction. Electrophysiology evaluation was obtained, who advised outpatient monitoring within two weeks to evaluate for progression. The patient was not felt to be at high risk of progression due to preservation of the chronotropic response as well as absence of high-grade block.
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pmc-6367113-1
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A 72-year-old man with past medical history significant for a stroke and hypertension was undergoing routine evaluation for his muscular dystrophy. Transthoracic echocardiogram (TTE) revealed a right atrial mass measuring 1.5 cm x 2 cm (Figure ).
Transesophageal echocardiogram (TEE) results were consistent with this finding (Figures -).
He was started on anticoagulation and transferred to our facility for PMT using AngioVac (Figure ).
For this procedure, the right internal jugular vein and bilateral common femoral veins were accessed. Intracardiac echo was used. The intracardiac echo showed an approximately 1.5 cm x 2 cm mass in the right atrium. This was ultimately extracted with 0% leftover tissue burden. Pump time was approximately two minutes. 22 French AngioVac cannula had been fashioned with a heat gun. This was then introduced from the right internal jugular vein. Blood was delivered via the left common femoral vein. Intracardiac echo was performed from the right common iliac vein. Tissue was sent to pathology for further review which confirmed the diagnosis of PFE. The patient was then discharged home without the need for long-term anticoagulation.
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pmc-6367117-1
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A 22-year-old man presented with a complaint of left-sided non-radiating headache throughout the day for two months, with the pain worsening at night and associated with non-projectile vomiting. The patient managed to achieve some relief with oral analgesia. There were no symptoms of raised intracranial pressure. His symptoms worsened a week before the presentation of gradual painless reduction of his left visual field, particularly on the superior and inferior aspects. He also noticed binocular double vision with significant deviation of his eye. There was no history of recent head trauma, upper respiratory tract infection or sinusitis. He also denied any medical or surgical procedures, allergies and recreational or prescription drug use. He was a smoker of five pack years.
On examination, his vital signs were stable with no documented fever, and he appeared well, alert and conscious. On further examination of the visual system, his visual acuity (VA) of the right eye was 6/6, while the left eye VA was 6/7.5. There was partial ptosis of the left eye covering the visual axis with gross axial proptosis and mild exotropia of the left eye. There were, however, no signs of dilated conjunctiva vessels, cork-screw vessels or conjunctiva chemosis. Measurement with a Hertel exophthalmometer for the right and left eyes showed 15 mm and 22 mm, respectively, with a discrepancy of 7 mm between both eyes. Examination of the optic nerve function revealed no relative afferent pupillary defect (RAPD) and no defect in the Ishihara pseudoisochromatic plates for both eyes, but a relative 30% reduction in red saturation and light brightness of the left eye compared to the right. Extraocular movement of the left eye was limited for superior, temporal, inferior and nasal gaze. The patient also complained of binocular diplopia in all gazes. Examination of the anterior and posterior segments of both eyes appeared normal. Examinations of all cranial nerves were normal. Other related examinations revealed the absence of sinusitis, dental caries or odontogenic cause, otitis, facial furuncles or any breaks of skin around the face. There were no scalp abscess or any enlarged cervical lymph nodes.
Computed tomography (CT) venogram was done instead of the magnetic resonance imaging (MRI) because of resource constraints, and CT venogram was the best imaging modality that could be offered to the patient at the time. CT venogram (Figure ) was done, which revealed normal dural venous sinuses with vascular enhancing lesions on the right cavernous sinus with a filling defect, especially at the posterior part. Poor delineation of the cavernous segment of the right internal carotid artery was also observed with the left cavernous sinus concavity preserved. There was no dilatation or tortuosity of the bilateral superior ophthalmic vein with no filling defects. Other findings include mucosal thickening of the sphenoid, ethmoidal and bilateral maxillary sinuses, but the other sinuses and mastoid air cells were well aerated. There were no other significant brain findings. In view of strong clinical signs and radiological features that were strongly suggestive of CST, the patient was admitted urgently and started on intravenous ceftriaxone, intravenous cloxacillin and intravenous metronidazole with the diagnosis of the left cavernous sinus thrombosis in mind. The patient completed the intravenous antibiotics in a week and was discharged well with good vision in both eyes, reducing proptosis, resolved exotropia and binocular diplopia. Review in a week’s time showed marked clinical improvement with the patient achieving total clinical resolution in a month’s time.
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pmc-6367118-1
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A 54-year-old woman with a 20-year history of myelofibrosis (MF) being treated with ruxolitinib and complicated by pancytopenia and massive splenomegaly initially presented to an outside hospital with progressive dyspnea and generalized weakness. Laboratory evaluation at that hospital revealed worsened anemia compared to the patient’s baseline. Chest imaging demonstrated a large left pleural effusion for which she underwent thoracentesis. She was eventually transferred to our institution for further management.
On presentation to our institution, the patient’s blood pressure was 131/85 mmHg, pulse 90 beats/min, and respiratory rate 18 breaths/min. She had a temperature of 98.1F and an oxygen saturation of 98% while breathing supplemental oxygen via a nasal cannula at 2 L/min. Mild respiratory distress was apparent. On cardiac examination, she had a grade 2/6 systolic ejection murmur. Lung examination was notable for decreased breath sounds at the left base with dullness to percussion. Other pertinent findings were pallor, palpable splenomegaly, and lower extremity edema.
Abnormal laboratory values included a leukocytosis of 18.4 k/mm3 (normal range 4.8-10.8 k/mm3) with an increase in myeloid precursors, hemoglobin of 5.6 g/dl (normal range 12-16 g/dL), thrombocytopenia of 77 k/mm3 (normal range 160-410 k/mm3), and lactate dehydrogenase (LDH) of 882 U/L (normal range 125-220 U/L). Her coagulation parameters were unremarkable. Portable chest X-ray showed complete opacification of the left hemithorax with a rightward displacement of the trachea (Figure ). Urgent placement of a left chest tube yielded grossly bloody pleural fluid consisting of a red blood cell (RBC) count of 1,415,000/mm3 and an LDH of 1555 U/L. The approximate pleural fluid hematocrit of 14% was >50% of serum hematocrit consistent with hemothorax. She received three units of packed RBCs over the ensuing two days but surgical intervention was not pursued. There was no evidence of malignancy by cytology or flow cytometry. The chest tube was removed on hospital Day 9, as there was no active drainage with an interval decrease of pleural effusion and stable serum hemoglobin. Over the subsequent two days, however, her serum hemoglobin again dropped from 8.0g/dL to 6.5g/dL. On hospital Day 11, computed tomography (CT) angiogram of the chest, abdomen, and pelvis was performed to localize a source of bleeding. The CT angiogram showed a large, complex fluid collection in the left pleural space measuring 11.6 x 9.6 cm in cross-section with a hyperdense focus compatible with active extravasation (Figure ). The patient was taken to the operating room for urgent exploration. Approximately 2.5 liters of blood and blood clots were surgically evacuated. There was no evidence of a discrete bleeding source, but there was diffuse pleural oozing. Decortication of a thin fibrous peel was performed. She was extubated 24 hours postoperatively and transferred to the general medical floor after discontinuation of all chest tubes. A hematoxylin & eosin stained section of the patient’s pleura biopsied intraoperatively revealed maturing erythroid and myeloid cells with small clusters of erythroid and myeloid precursors (Figure ). Also present were atypical and dysplastic megakaryocytes that were highlighted by CD61 immunostaining (Figure ). The finding of trilineage hematopoiesis in a pleural sample is diagnostic of EMH at that location. Nuclear imaging with Technetium-99m sulfur (Tc-99m sulfur) colloid demonstrated diffusely increased uptake in the chest bilaterally, right greater than left, consistent with intrathoracic EMH (Figure ). As expected, there was decreased activity within the bone marrow compatible with the patient's history of myelofibrosis.
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pmc-6367119-1
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Here, we present the case of a 62-year-old Caucasian man who was treated for non-ST-elevation myocardial infarction, heart failure with reduced ejection fraction, community-acquired pneumonia, and, later, Staphylococcus hominis mitral valve endocarditis. He was scheduled to receive six weeks of intravenous vancomycin, however, on Day 32 of treatment, he developed whole body pruritus. Over the next day, an erythematous macular rash appeared on the torso and spread centrifugally to the extremities, including the palms and soles. By Day 33, the patient began to have desquamation, intense pain to the skin, and intermittent fevers. The desquamating rash evolved to include 80% of the total body surface area (BSA) without bullae. Although there was no obvious mucosal involvement, the patient consistently reported ageusia. Abnormal laboratory findings included: white blood cell count (WBC) 23.6 x 103/UL, peripheral blood eosinophilia 12%, erythrocyte sedimentation rate (ESR) 27 mm/hr, C-reactive protein 6.385 mg/L, and serum creatinine 2.3 mg/dL. His baseline laboratory values included WBC 10.5 x 103/UL (normal, 4.5 – 11.0 x 103/UL), eosinophils 2.9% (0.0 - 6.9%), ESR 11 mm/hr (0 - 22 mm/hr), and serum creatinine 1.3 mg/dL (0.6 - 1.3 mg/dL). There was no baseline C-reactive protein recorded (normal, <3.0 mg/L). Blood cultures were consistently negative. Serum alanine transaminase (ALT) and aspartate transaminase (AST) concentrations were within the normal range. The patient did not complain of any pulmonary symptoms, including cough, chest pain, or shortness of breath. The measurement of hemoglobin oxygen saturation by pulse oximetry remained above 95% throughout his hospital course.
Differential diagnosis discussion
We originally believed the diagnosis to be vancomycin-induced red man syndrome due to the presentation of an erythrodermic rash during concurrent vancomycin treatment. However, the vancomycin hypersensitivity reaction typically presents on the upper body, as opposed to the rash appearing over the 80% total BSA as seen in our patient. In addition, red man syndrome is a rate-related reaction that normally appears within minutes or hours of vancomycin infusion. Our patient had been receiving vancomycin for 32 days before manifestations of any hypersensitivity reaction and his skin continued to desquamate for two days after discontinuing vancomycin. Therefore, we did not believe the diagnosis was red man syndrome.
Due to the intermittent fevers and exfoliative rash following four weeks of drug administration, Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TENS) was considered as well. The skin lesions in SJS/TENS typically start on the face and thorax, with sparing of the palms, soles, and scalp. Mucosal involvement in the oral, ocular, or urogenital regions presents as painful crusts, and erosions occur in 90% of SJS/TENS cases. In contrast to the epidermal manifestation of SJS/TENS, our patient’s skin lesions were the most erythrodermic on the palms and soles. In addition, our patient had no visible oral, ocular, or urogenital mucosal tissue sloughing, erythroderma, or pruritus. Anemia and lymphopenia are common in SJS/TENS, however, our patient had neither hematologic abnormalities and, rather, had leukocytosis. Due to the lack of classic dermal, mucosal, or hematologic findings in SJS/TENS, this differential diagnosis was also excluded.
The RegiSCAR Diagnosis Score (Table ) was applied to predict the likelihood of DRESS syndrome as the leading diagnosis []. The patient received two points for peripheral eosinophilia of 22%. The patient’s skin rash contributed two points to the RegiSCAR score due to 80% of the total BSA rash extension and features of purpura and scaling. The lack of a skin biopsy contributed -1 point to the total RegiSCAR score. The serum creatinine levels, increasing from 1.3 mg/dL at baseline to 2.3 mg/dL, indicated renal involvement, which also contributed one point. The patient displayed many characteristic features of DRESS syndrome, leading to a RegiSCAR score of four, which correlates with a probable case of DRESS syndrome (Table ).
Treatment
Upon suspicion for drug-induced rash with eosinophilia and systemic symptoms, all antibiotics were stopped. However, for the next two days, the patient’s skin continued to desquamate, leukocytosis remained, and peripheral eosinophilia worsened, reaching a white blood cell count (WBC) of 31.5 x 103/UL with 14% eosinophils. On Day 3 following antibiotic discontinuation, the creatinine had decreased to 1.6 mg/dL, but peripheral eosinophilia continued to increase to WBC 28 x 103/UL with 22% eosinophils. Although the patient only completed five weeks of antibiotics, the decision was made to not complete the six-week antibiotic course. The patient was then given 80 mg of intramuscular triamcinolone (Kenalog) and topical 0.1% triamcinolone to be applied to the affected areas twice per day.
Outcome and follow-up
Two weeks after initiating steroids, there was a marked improvement in the erythema on his trunk and WBCs were down to 12.4 x 103/UL with resolved eosinophilia. At this point, the patient was discharged from the hospital without antibiotics. He was instructed to continue using the topical triamcinolone and Aquaphor ointment for symptomatic treatment at home. When seen at follow-up three months later, the rash had completely resolved except mild erythema to his hands and feet. His WBC count was normal with 2.9% eosinophils (normal 0.0-6.0%). At the four-month follow-up, the patient continued to note improvement in ageusia. Given the ongoing improvement in ageusia and lack of DRESS-like symptoms, no further workup was undertaken. He was not re-challenged with vancomycin. A lymphocyte transformation test, patch test, autoantibody profiling, and cytomegalovirus (CMV) serology were not acquired due to symptom resolution in the patient. At the five-month follow-up, the patient resumed his home blood pressure medications prior to his illness, with no sequelae.
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pmc-6367120-1
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A 47-year-old Caucasian male with a past medical history of uncontrolled diabetes mellitus, hypertension, gastroesophageal reflux disease, and anxiety presented to the emergency department with a chief complaint of right foot swelling. The patient reported that the swelling started two weeks prior to the presentation but denied any associated pain. The patient denied injury or trauma to the foot. An X-ray of the right foot revealed old fracture deformities but no evidence of an acute osseous lesion. A lower extremity Doppler ultrasound was performed and ruled out the presence of deep vein thrombosis (DVT). The patient also endorsed dysuria, dribbling, and urinary retention over the previous day but adamantly refused placement of a catheter. When the patient was told that he would be discharged from the emergency department, he began complaining of chest tightness, shortness of breath, and nausea, all of which he denied on a review of symptoms during the initial evaluation. He stated that the chest discomfort had been present all day, was non-radiating, and rated at a 4/10 in severity. Electrocardiography (EKG) and cardiac enzymes were within normal limits. The patient was admitted for further evaluation.
During the hospitalization, the patient developed multiple additional complaints that after appropriate workup, ultimately did not lead to a specific diagnosis. The patient worked with physical therapy and occupational therapy, which established that the patient was difficult to assess, as his functional mobility issues were inconsistent. He was noted to have deficits in balance, endurance, and safety awareness, which affected the patient’s ability to perform the activities of daily living. Of note, one of the physical therapists documented that the patient was witnessed alone in his room, walking without difficulty while texting on his phone.
On hospital day four, the patient was displeased when told that he was being discharged, as he desired further workup for his complaints. While preparing to leave, he had an unwitnessed fall in his room. The patient stated that he hit his head and was in pain but was unable to localize the pain. There was no evidence of acute trauma on physical exam. Regardless, the fall prompted a computed tomography (CT) scan of the brain without contrast to rule out any acute intracranial trauma. Incidentally, the CT scan revealed bilateral symmetric calcifications of the basal ganglia, putamen, caudate, thalami, dentate nuclei of the cerebellum, and cerebral white matter, consistent with Fahr's disease (Figure ). Laboratory values, such as parathyroid hormone (PTH), thyroid stimulating hormone (TSH), T3, and T4, were all within the normal reference limits. Serum calcium was mildly decreased, ranging from 8.1 - 8.4 mg/dL during the admission. After being informed of the CT scan findings, the patient revealed that his deceased mother suffered from a rare diagnosis. Further investigation, with the patient's permission, revealed a prior CT scan with similar calcifications in the basal ganglia and cerebellum, consistent with Fahr's disease.
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pmc-6367595-1
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PT, a 47-year-old male shop-worker, presented in October 2012 at age 44 with recurrent amnesia on awakening, characterised by disorientation and repetitive questioning which would last for minutes, in conjunction with 30 second ‘trance-like’ episodes during the day. As reported by his partner, these daytime episodes were sometimes accompanied by repetitive swallowing movements, followed by disorientation and repetitive questioning, and occurred in clusters of up to seven per day. PT himself was aware of some but not all of these episodes, with partial recollection of being unable to remember. He did report other memory changes, however, including an unexpectedly rapid loss of newly acquired memories over days, and an unusual difficulty in recalling both autobiographical events from his adulthood and familiar routes. Concurrently, PT developed olfactory hallucinations; uncharacteristic headaches with migrainous features; postural arm tremor, tingling legs, malaise and low mood. The olfactory hallucinations, which reminded him of candy floss, occurred independently of the amnestic episodes and could last for up to a day. He also reported some subjective blunting of his sense of smell. His medical history was unremarkable, aside from symptoms of depression during the previous year and a remote history of heavy alcohol use.
A timeline of PT's symptoms and investigations is provided in . Initial brain MRI showed subtly increased T2 signal in the right hippocampus, in the absence of any recent episodes. EEG was normal. Clinical examination revealed no focal neurological signs. Following an initial clinical diagnosis of TEA, PT was treated with lamotrigine (commencing at 25 mg and increasing incrementally up to 100 mg twice a day), resolving his trance-like and amnestic attacks, but not his other symptoms (namely headaches, tingling sensation in the limbs, and olfactory hallucinations).
Although PT satisfied criteria for a diagnosis of TEA, in view of his relatively young age for a diagnosis of TEA, varied neurological symptoms and subtle MRI changes, the possibility of an autoimmune epilepsy was raised. Screening for associated antibodies revealed high levels of serum NMDAR antibodies (strong binding persisted at a dilution of 1:500, live cell based assay and rodent brain immunohistochemistry). LGI1, CASPR2, GABA(B)R- and AMPAR-antibodies were negative. CT scan of thorax, abdomen and pelvis and testicular ultrasound showed no evidence of malignancy. CSF NMDAR-antibodies (now recommended to support this diagnosis) were not measured, but the combination of amnesia, mood disturbance, seizures, a movement disorder, imaging findings and high serum NMDAR antibodies made a diagnosis of NMDARE likely.
Initial treatment with intravenous methylprednisolone followed by oral maintenance therapy for 6 months led to partial resolution of symptoms, with a lower NMDAR-antibody level (1:100). Further treatment with plasma exchange followed by maintenance therapy with MMF and oral steroids substantially improved his symptoms and eliminated serum NMDA-receptor antibodies. Seven months later, relapsing symptoms were associated with the reappearance of the NMDAR-antibodies and a further course of intravenous methylprednisolone and plasma exchange was given. Repeat MRI now appeared normal (see a). PT is currently well on maintenance MMF, lamotrigine and a reducing dose of prednisolone.
Neuropsychological assessment was conducted in 2013 and 2015 using standard measures of: general cognitive ability (Wechsler Abbreviated Scale of Intelligence and Addenbrooke's Cognitive Examination-Revised – ACE-R), anterograde memory (Anna Thompson story from Wechsler Memory Scale 3rd edition, Rey Complex Figure Test), and executive function (verbal fluency tasks). Additional memory testing in 2015 included research measures of ALF (based on a modified administration of the Rey Auditory Verbal Learning test) and a modified Autobiographical Memory Interview (). Results for both 2013 and 2015 assessments indicated preserved general ability (estimated IQ = 109), with stable and preserved visuospatial skills, verbal and non-verbal reasoning, verbal generativity and retention of verbal material (stories) at 30-minute delay. Recall of visual information, however, was poor at both assessments. While initial encoding of information appeared to have worsened in 2015, PT now showed benefit from repeated exposure of material (e.g. in learning a name and address on the ACE-R). Further memory testing completed at the second assessment to evaluate accelerated long-term forgetting and autobiographical amnesia, indicated mild decrements in memory at standard delays, with evidence suggestive of accelerated forgetting after a 1-week delay, and pronounced autobiographical amnesia when comparing PT with 10 healthy older but IQ-matched controls (mean age = 59, IQ = 115) (b).
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pmc-6367745-1
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An 11-year-old boy was referred to our clinic for further diagnostic due to the persistence of the lower deciduous molars. The family history was without pathological findings. The clinical extra-oral examination revealed a kyphoscoliosis accompanied by a pectus excavatum with an age-appropriate habitus. Additionally, a myopia with moderate hypertelorism was obvious.
The intra-oral examination showed a mixed dentition at the beginning of the second dentition phase. The following deciduous teeth were found to be in situ: 53, 55, 63–65, 73–75, 84 and 85. Furthermore, the permanent teeth 14, 16, 12–22, 26, 36, 32–43 and 46 were erupted. In the vestibular region of teeth 43 and 84 an indolent, solid, smooth-margined swelling with a diameter of 20 mm was obvious.
The orthopantomogram (OPT) showed a retention of the teeth 33 and 45 at the base of the mandible. In the peri-coronal area of both teeth enlarged radiolucencies with a clearly visible margin in region 33 and 45 were obvious. Additionally, in the area between teeth 46 and 47, a diffuse osteolysis confluent with a peri-coronar osteolysis around the retained 47 was visible. Furthermore, the teeth 35, 34 and 44 were angulated disto-mesially and root resorptions at the teeth 73 and 85 were observed. (Fig. ).
In order to clarify the clinical and radiographic findings an incisional biopsy was taken from region 33 and 45 in local anesthesia. The histological examination proved the a keratocystic odontogenic tumor in both regions. Due to the diagnosis of bilateral KCOT the clinical suspicion of a NBCCS (Gorlin-Goltz syndrome) arose. Thus, multi-disciplinary consultations including radiology of the head-neck region, a dermatological screening, ophthalmological and orthopedic examinations were initiated. Furthermore, a genetic evaluation of the family including the entire generation of grandparents based on a sequencing of all PTCH1 exons and their intron-exon boundaries was performed. The genetic screening revealed a spontaneous mutation of the PTCH1 gene in the mother. Thus, the initial suspicion of a Gorlin-Goltz syndrome could be confirmed. However, the mother’s clinical findings were without pathology so that she can be considered as a carrier without any clinical gene expression.
Due to the genetic aberration revealed by the blood test a genetic counseling was recommended to the family. Considering the pathological findings, an interdisciplinary treatment plan for the adolescent patient was created. In order to find a satisfying treatment for the patient the options of marsupialization and enucleation of the cystic lesions were discussed with the patient and his family. Due to the long treatment period associated with the marsupialization enucleation of the lesions with simultaneous augmentation of the resulting defects was preferred.Follow-up of the physical screenings focusing on dermal and ophthalmic anomalies on a regular three-month interval. Radiological follow-up semi-annually focusing on osteolytic lesions in the jaw. Enucleation of the KCOTs with simultaneous bone grafting. Orthodontic treatment to level the dental arches and to maintain space for implant supported oral rehabilitation in region of 33 and the right mandible. Insertion of dental implants and prosthetic restoration after the cessation of the physical growth.
In order to estimate the extent of the bony defects remaining from the KCOTs, a cone beam computed tomography (CBCT, 3D-Exam, KaVo Dental GmbH, Biberach, Germany) was performed preoperatively. The bony defect in region 33 measured about 32 × 28 mm and the defect in region 45 was approximately 27 × 24 mm. The lesion in the right mandible showed a length of approximately 60 mm and a height and width of approximately 20 mm. Based on the data obtained from the CBCT, a model was created. Subsequently, a CAD/CAM milled, allogenic bone block was produced for the defect resulting from the enucleation in the right mandible (Tutoplast® Spongiosa, Tutodent, Neunkirchen a. B., Germany). The customized bone block is shown in Fig. . The cystectomy including the removal of the teeth 33, 45, 46 and 47 was performed in general anesthesia at the age of 12 years. It was possible to achieve an enucleation in toto that histologically confirmed a KCOT by instantaneous section diagnosis. In order to preserve the inferior alveolar nerve ostectomy or curettage were not carried out. Simultaneously, the reconstruction of the defects in region 33 and 45 were performed using preformed allogenic spongiosa blocks (Tutoplast® Spongiosa, Tutodent, Neunkirchen a. B., Germany). In the right mandible, the customized spongiosa block was inserted (Fig. ). The wound closure was achieved using a porcine pericardium membrane (Botiss Jason® membrane, Straumann GmbH, Freiburg, Germany) and absorbable sutures (Vicryl 3–0, Ethicon Deutschland, Johnson & Johnson Medical GmbH, Norderstedt, Germany). The extraction of the deciduous teeth 63 and 65 and a peri-coronal cystectomy at tooth 23 were carried out in the same session. Again, instantaneous section diagnosis revealed a KCOT around tooth 23. As the KCOT was only located peri-coronally and tooth 23 was retained in a vertical position closed to eruption the preservation of 23 was intended. Thus, a bracket for forced orthodontic movement was attached to the tooth.
In order to prevent mesial movement of the teeth in the left mandible, the orthodontic treatment was initiated directly postoperatively. This involved the fine adjustment of the dental arches with orthodontic movement of tooth 23 into the intended position. Furthermore, the angulated teeth 32, 34 and 44 were erected. Thus, the cuspid space in region 33 was kept open and the remaining front teeth could be stabilized in order to allow insertion of dental implants in the right posterior mandible.
In order to estimate the skeletal growth reserves a hand radiograph was performed at the age of 15 years. The method according to Björk is routinely used by pediatricians to determine the individual growth development and degree of maturation [, ]. This yielded a Ru-stage which implied the end of the physical growth according to ossification of the radius []. At this age, the patient’s height was already about 10 cm more than his parents’. The consultation of a pediatrician assumed the end of the growth period. Thus, it was decided to start the preparation of the implant placement.
For the detailed analysis of the bone volume, a CBCT was performed. The findings revealed an insufficient crestal amount of bone in region of tooth 33. Furthermore, a vertical deficit of about 8 mm in the right posterior mandible in regio 46 and 47 was obvious while the bone width was 12.9 mm. Only 9 mm of bone cranial of the mandibular canal could be measured providing an inappropriate crown to root relation. In general anesthesia, a lateral bone augmentation using xenogenic bone substitute (Cerabone®, Botiss GmbH, Zossen, Germany) was performed in region of tooth 33. Additionally, in order to overcome the vertical gap in the right mandible, the allogenic bone graft in region 45–48 was augmented by a discontinuous distraction osteogenesis (Vertical Alveolar Distraction, Type Cologne, Gebrüder Martin & Co KG, Tuttlingen, Germany). The healing was uneventful without any dehiscence or signs of inflammation. Subsequently, the distraction phase was set up for 18 days followed by a consolidation phase of 6 months (Fig. ). Thus, a bone height of 15 mm cranial of the mandibular canal was achieved while the bone width decreased slightly to 11.5 mm which, however, could considered sufficient for the insertion of dental implants (Fig. ).
The planning of the implant position was performed using the three-dimensional guided protocol of the NobelGuide® (NobelBiocare Deutschland GmbH, Cologne, Germany). In order to obtain samples for the histological examination of the augmented bone block a cylindrical trephine core of 3.5 mm diameter was harvested from region 45, 46 and 47. The samples were embedded in paraffin and stained using hematoxylin eosin. The histological examination revealed vital spongy bone trabeculae along avital bone tissue with evidence of increased remodeling. The implant insertion in areas 33, 45, 46 and 47 was performed according to the manufacturer’s protocol. Subsequently, four implants (SIC invent Deutschland GmbH, Göttingen, Germany) were placed in the regions of 33 and 45 (4.0 × 11.5 mm) and in regions of 46 and 47 (5.0 × 11.5 mm). The implants were allowed to heal for 6 months. During the healing period of the implants, the orthodontic fine adjustment was carried out. After 6 months, the implants were uncovered and provided with provisional crowns. An adaptation phase of another 6 months was allowed before incorporation of the final prosthetic restorations (Fig. ). The porcelain fused to base metal crowns were fixed on custom made abutments. At this stage of the treatment, the patient was 18 years old (Table ).
Regarding the histologically proven KCOTs a three-months radiological control interval was set up during the treatment period until the eruption of the last permanent tooth which was tooth 23 in the presented case. A sharply defined peri-coronal radiolucency around tooth 23 were found at the second control examination. Subsequently, the interval of radiographic examination has been extended to 6 months.
At the 6-months follow-up after insertion of the prosthetic restorations, OPT, lateral cephalogram and CBCT showed well osseointegrated implants without any sign of bone loss. No recurrence of the surgically removed KCOTs was observed. However, a new cystic lesion in the posterior part of the right sinus maxillaris was found. Tooth 17 was removed due to the close contact to the cystic lesion without complications. Histologic examination proofed another KCOT. At the 12-months follow-up, a lesion in the basal part of the left sinus maxillaris was observed. As the radiographic control showed a rapid progression of the lesion in the left sinus it was surgically removed under general anesthesia. Due to the strong adherence of the cyst to the bone it had to be resected. The resulting oroantral fistula was closed using a titanium supported membrane (Cytoplast® d-PTFE-Membrane, RIEMSER Dental, Greifswald, Germany). The histologic analysis again revealed a KCOT. Up to the patient’s age of 20 years, the follow-up in semi-annular intervals were without any pathologic finding (Fig. ).
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pmc-6367762-1
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A 7-year-old male French bulldog was referred to the Veterinary Teaching Hospital at CEU Cardenal Herrera University for a corneal mass on the right eye that had been enlarging over a 2-month period. The dog had a complete excision of the third eyelid gland 5 years before and developed an iatrogenic keratoconjunctivitis sicca that was being controlled with topical compounded 1% cyclosporine eye drops every 24 h and artificial tears every 8 h.
Initial ophthalmic examination revealed an exophytic pink to white gelatinous mass occupying approximately 70% of the central cornea on the right eye (Fig. a). Dazzle reflex was inconstant whereas menace response was negative. Fluorescein staining was negative. Slit lamp biomicroscopy examination (Kowa® SL-14, Kowa Company, Tokyo, Japan) showed severe increase of the corneal thickness, pigment deposit and superficial vascularization; the mass was infiltrating the corneal stroma. The rest of the ocular examination including Schirmer tear test-1 (19 mm/min, Sno-Strips®. Chauvin Pharmaceuticals Ltd. Essex, Great Britain) and intraocular pressure obtained by applanation tonometry (13 mmHg, Tonopen XL®, Mentor, Norwell, MA, USA) was within the normal limits. Indirect ophthalmoscopy could not be performed. The ophthalmic exam of the left eye was unremarkable, and no abnormalities were observed on physical examination.
At that time, the temptative diagnosis for the right eye was a corneal neoplasia; other differential diagnoses were granulation tissue and chronic inflammatory process. An excisional superficial keratectomy was performed under general anesthesia as previously described []. Histological findings were consistent with corneal carcinoma and transconjunctival enucleation was finally performed. The globe, conjunctiva and nictitant membrane were fixed intact in 10% neutral buffered formalin and submitted for histopathologic examination.
Locally expansive and infiltrative masses were observed arising from the corneal epithelium and extended into the deeper layers of the corneal stroma (Fig. b-d). Neoplastic cells were polyhedral with abundant eosinophilic cytoplasm and evident intercellular bridges and arranged in nest, cords or pseudoacini. These cells had large euchromatic nuclei with single prominent nucleoli. Abundant dyskeratotic foci (squamous differentiation) were observed in the neoplasia, but no keratin pearls were present. Anisokaryosis and anisocytosis were moderate, and the mitotic index was high (3–5 mitosis by high power field, Fig. e). An immunohistochemical panel was performed in order to better characterize the neoplasia: monoclonal mouse anti-human antibodies against pan-cytokeratin (clones AE1/AE3, “ready-to-use”), cytokeratin 5/6 (clone D5/16 B4; dilution: 1/50), cytokeratin 7 (clone OV-TL 12/30; dilution: 1/50) and muscle actin (clone HHF35, “ready-to-use”) from DAKO, Glostrup, Denmark. All neoplastic cells were strongly positive to AE1/AE3 pan-cytokeratin antibody (Fig. c), but only cells within the neoplastic corneal epithelium reacted with the CK5/6 (marker used for stratified epithelium, Fig. c). However, those cells located in the deeper layers of the corneal stroma and arranged as pseudoacini were both negative for CK5/6 and CK7 (specific marker for epithelial cell from glandular origin) and surrounded by elongated cells positive to muscle actin antibody (myofibroblasts cells, Fig. d). Neoplastic cells were near Descemet’s membrane but it was not invaded. Occasional blood vessels and fibroplasia were observed near the neoplastic foci. The corneal epithelium adjacent to the neoplasia was thickened and dysplastic. A mild inflammatory cell infiltrate, composed of a mixed population of lymphocytes and neutrophils, was also present at the tumor site. The stroma adjacent to the neoplastic tissue showed numerous melanin-laden macrophages. Based on the histological findings, a diagnosis of a poorly differentiated squamous cell carcinoma with deep stromal invasion was made.
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pmc-6367818-1
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A 26-years-old male patient presented with history of occasional mild central chest discomfort and mild dyspnea on exertion of 8-months duration. During last 4 months, the dyspnea (NYHA III) was progressing rapidly. He was receiving medications for congestive heart failure.
There was a continuous murmur over the precordium. His blood pressure in the right-arm was 135/54 mmHg. There was no systemic or pulmonary edema. Electrocardiogram was consistent with sinus rhythm, mild ‘ST-elevation’ in the ‘V1-V3’ leads and left ventricular enlargement (Fig. ). However, there was no evidence of myocardial ischemia.
The 2-D echocardiogram including color ‘Doppler’ revealed a dilated left ventricle and left atrium with turbulence in the left atrial cavity. A large cystic mass adjacent to the aortic root with continous flow was seen. The ejection fraction was 50–55%. A coronary angiography done through the right radial approach demonstrated normal left coronary artery with right dominance. The right coronary artery (RCA) was huge in size measuring 17–19 mm in diameter. For a better delineation of the course of the involved coronary artery and relationship of surrounding structures, we performed a computerized tomographic angiography (CTA) of the heart. It revealed normal origin of the coronary arteries. Left coronary artery measured 4.5 mm with normal branching pattern. There was a giant patent RCA with a diameter of 20 mm and a tortuous course. The dilated RCA traversed through the right atrioventricular groove posteriorly and eventually ended into a dilated sac on the posterior wall of the left atrium (Figs. and ). The dilated sac measured 30 mm in widest dimension and finally entered the floor of the left atrial cavity just above the mitral valve. There was no thrombus or stenosis of the coronary arteries. In addition, there was no evidence of a patent ductus arteriosus.
In view of recurrent chest discomfort, progressive dyspnea, cardiac enlargement, size of the involved coronary artery and location of the fistulous opening, the patient was considered for a surgical closure under standard cardiopulmonary bypass. The surgery was performed via median sternotomy, bi-caval cannulation, ascending aortic cannulation and aortic cross clamping. Alternating retrograde and antegrade crystalloid cardioplegia technique was used for maximum myocardial protection. The right coronary artery was hugely dilated in its entire course which was long, tortuous with friable surrounding tissue (Fig. ). Then right atrium was opened which showed no abnormality. Subsequently, the interatrial septum was opened, and the fistula was identified. It was located just above the mitral valve in the floor of the left atrium and the opening (1 cm) was guarded by a membranous windsock shape tissue (Fig. ). A complete resection of the redundant tissue was done and then the fistulous opening was closed using glutaraldehyde–treated autologous pericardial patch. The atrial septum and the right atrium were closed with running sutures. The weaning from cardiopulmonary bypass was uneventful. There were no ischemic changes and the patient remained in sinus rhythm after the procedure.
There was a good immediate and late postoperative recovery. Currently, (almost 6-months from surgery) the patient is receiving prophylactic aspirin and warfarin to prevent thrombosis or ischemic events. He will receive antibiotic prophylaxis and a careful 6-monthly follow up.
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pmc-6367833-1
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A 55-year-old postmenopausal woman presented in our outpatient department because of increasingly serious vaginal bleeding in the previous 2 years. She had experienced a caesarean delivery at 28 years old and underwent natural menopause at 53 years old. The bleeding, which followed a severe cough, was less than normal menses at first but later was heavy and left her clothes soaking wet. The patient reported that the bleeding was fresh blood, which could be stanched by compression in a sitting position. At the time of admission, a vaginal speculum examination showed that the vaginal wall had many varicosities and some bleeding spots. To exclude malignant diseases, the patient underwent colposcopy, and the varicosities and bleeding spots were further confirmed in the vaginal mucosa (Fig. a). However, iodine staining showed no lesions on the mucosae (Fig. b). In addition, the endometrial thickness was less than 4 mm according to transvaginal ultrasonography.
Simultaneously, we noticed that the patient had obvious venous varicosities in her left leg and that the diameter of the left leg was significantly larger than that of the right leg (Fig. a). She stated that the venous varicosities were found when she was 10 years old but not treated because of poor medical conditions. To exclude vascular malformation, we advised the patient to visit the vascular surgery department. On contrast-enhanced computed tomography (CT) scanning of the legs, several varicosities and hyperplastic soft tissue and muscle were found in the left leg but not in the right leg (Fig. b). To determine whether multiple arteriovenous malformations were present, the patient underwent a CT scan of the pelvis, which showed some venous varicosities in the left internal pudendal vein but not in the right vein (Fig. a). However, pelvic arteriovenous shunts and pelvic congestion syndrome were not found on the 3-D reconstructed image (Fig. b).
Then, deep arteriovenous shunts in the left leg were found by arterial angiography. The arteriovenous shunts were partly closed by an interventional arteriovenous fistula occlusion operation at Zhongshan Hospital, Fudan University, Shanghai, China. Aspirin and Plavix were given after surgery as anticoagulants for 1 month and were gradually stopped over the next 2 months. The vaginal bleeding was gradually alleviated and disappeared 5 months later. For long-term management, close observation and regular follow-up examinations were recommended.
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pmc-6368159-1
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A 42-years-old alcoholic man was evaluated for a 30-day history of abdominal pain and postprandial vomiting. He referred two previous hospitalizations for acute pancreatitis. Physical examination was unremarkable. Laboratory studies revealed a slight increase of pancreatic enzymes. Magnetic resonance imaging showed a 13 cm collection with thick content in the pancreatic neck. The upper endoscopy found an important bulging in the posterior gastric wall and the endoscopic ultrasound revealed a 15.7x7.2 cm pancreatic cyst, predominantly anechoic, with some mobile heterogeneous material, consistent with walled-off necrosis ().
Due to the diagnosis of symptomatic walled-off necrosis, we opted for an endoscopic drainage with a 10x10 mm HOT-AXIOSTM stent (). The patient was under general anesthesia. After puncture, there was an immediate output of a brownish secretion with necrotic solid components (). The total duration of the procedure was 20 min and the time required for drainage was 4 min. We did not use fluoroscopy and there were no immediate complications.
The patient was discharged three days later, asymptomatic. On the 7th post-drainage day, he underwent a new upper endoscopy and a pediatric endoscope was passed (5.4 mm in diameter) through the stent into the cyst cavity. Around 80% of the walled-off necrosis was collapsed and there was a small amount of fibrin with no clots or necrotic tissue. We washed it with 300 ml of 0.9% saline.
On the 14th post-drainage day, we performed another upper endoscopy and an ERCP for assessment of the main pancreatic duct. Firstly, we tried to introduce a typical gastroscope through the stent but it was not possible due to the complete collapse of the prosthesis. Then, we instilled contrast through the stent and we notice complete reflux back to the gastric chamber, confirming the complete collapse of the collection. Pancreatography revealed a stricture in the transition of the head and neck but no fistula. We performed a pancreatic sphincterotomy and placed a 7Fx10 cm straight plastic stent through the stricture. After deployment of the pancreatic stent, we removed the Hot-AXIOS using a foreign body forceps (). The patient was asymptomatic and was discharged 24 h after the ERCP procedure.
On the 30th post-drainage day, a new abdominal CT showed complete resolution of the pancreatic collection (). The patient is currently on two months follow-up and remains asymptomatic. An ERCP is planned on the 3rd month evaluation for removal of the pancreatic stent.
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pmc-6368363-1
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A 27-year-old male patient reported to the department of oral and maxillofacial surgery at Thai Moogambigai Dental College and Hospital in Chennai, Tamil Nadu, India. The patient’s chief concern was a pain in his left upper back tooth region lasting a week. The pain was dull, aching, continuous, and nonradiating, with no aggravating or relieving factors associated with the left upper back tooth region. The patient is healthy without systemic diseases or deleterious habits. On an intra-oral examination, we noted that all the third molars were clinically unerupted. On careful inspection, we noted his oral cavity appeared to be normal without any buccolingual swelling or mucosal changes. His mouth opening was normal. A dental panoramic radiograph revealed an accidental finding of cystic lesions associated with the lower third molars bilaterally. A review of his orthopantomogram revealed the upper third molar were impacted with a sinus approximation and a well-defined, unicystic radiolucency around the impacted lower third molars bilaterally. The lesions had bilaterally enclosed the crown of the horizontally impacted lower third molars from the cementoenamel junction, and they extended inferiorly to the apex of the distal root of the respective second molars (Figure ). Surgical excision of the lesion along with the impacted molars was planned under local anesthesia. A modified Ward’s incision was placed, and the mucoperiosteal flap was reflected. Bone guttering was done under copious saline irrigation, and the tooth was sectioned and removed. The soft cystic tissue was exposed, and we performed a complete enucleation of the cysts (Figures -). Carnoy’s solution was applied to cauterize the remnant cystic lining on the cavity wall junctions, and hemostasis was achieved. The obtained specimens were biopsied. Povidone-iodine was flushed into the sockets generously. Wound closure was done with a 3-0 silk suture. The patient was evaluated again three days later and had no paraesthesia. After one week, the sutures were removed and the wound healed satisfactorily. The histopathological report revealed a dentigerous cyst bilaterally. It showed a thin fibrous cystic wall lined by two-to-three cell layers of thick, nonkeratinized, stratified squamous epithelium. The connective tissue showed an inflamed infiltrate. Follow-up examinations at one, three, and six months yielded no degenerative changes around the third molar region, and no pockets formed in the distal aspect to the lower second molars (Figure ).
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pmc-6368426-1
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A 20-year-old male with a past medical history of seizures since the age of 14 and treated with divalproex sodium and topiramate presented to the emergency department with right lower quadrant abdominal pain. On presentation, all vital signs appeared normal. A complete metabolic panel and blood count were within normal limits (creatinine of 0.89 mg/dL and blood urea nitrogen of 14 mg/dL). An abdominal ultrasound was performed that demonstrated a nonspecific structure in the right lower abdomen with a vascular fatty central parenchyma, as well as an avascular hypoechoic peripheral parenchyma (Figures , ).
There were no acute findings on ultrasound. A computed tomography (CT) scan demonstrated no acute abnormality, but a supernumerary kidney fused to the lower pole of the native right kidney was visualized (Figures , ).
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pmc-6368428-1
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A 67-year-old Caucasian male, who been diagnosed for many years with irritable bowel syndrome, presented to our emergency room with a five-day history of abdominal pain, nausea, vomiting, and obstipation. The patient's past medical history also included hypertension and an appendectomy. His examination revealed a soft, distended abdomen. A computed tomography (CT) scan of the abdomen and pelvis was consistent with small bowel obstruction secondary to ileocecal intussusception (Figure ).
He was admitted to the hospital, and a laparoscopic right hemicolectomy with resection of the terminal ileum and an extracorporeal side-to-side stapled ileocolic anastomosis was performed (Figure ). The patient did well and was discharged from the hospital.
Gross pathology revealed two submucosal lipomas with an ischemic terminal ileum loop (Figure ). Histopathological examination of the leading point indicated mature adipose tissue consistent with a lipoma (Figure ).
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pmc-6368430-1
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This case involves a 60-year-old male with a history significant for benign prostatic hyperplasia, hypertension, and insulin-dependent diabetes who presented in July 2017 with diabetic ketoacidosis and Fournier’s gangrene. The patient’s treatment course for Fournier’s gangrene subsequently consisted of multiple operative debridements, an intensive care unit (ICU) admission, and ultimately a penectomy in July 2017 (Figure ). He continued his post-discharge follow-up at an outside hospital for recuperative care as he became homeless during this time. He received wound care with home healthcare and continued to undergo a follow-up in the outpatient clinic at the urology and plastic and reconstructive surgery departments, where he discussed his desire to undergo penile reconstruction. After discussing all treatment options as well as the risks and benefits of the surgery, the patient agreed to move forward with surgical reconstruction. The patient obtained consent for a free flap procedure, and plans were discussed to proceed with a radial forearm reconstruction of his penis.
This procedure involved phallus and neourethra construction utilizing a single radial forearm free flap. First, a 12-cm circumferential circle was marked and dissected out at the planned penis insertion site. Next, two branches of the dorsal penile nerve were carefully isolated and dissected out along with the left deep inferior epigastric and vena comitans that provided approximately 8 cm of pedicle length. An 8-cm segment of the great saphenous vein was also dissected out and transposed to the inferior epigastric vessels to assist with the flap anastomosis. The recipient vessels and nerves were now defined and attention was turned to harvesting the radial forearm free flap. The radial forearm free flap was lifted in the subfascial plane, while special attention was given to preserving the cephalic vein, the basilic vein, and the radial sensory nerve. The medial and lateral antebrachial cutaneous nerves were then isolated, and the flap was fashioned into a neophallus and urethra prior to vessel and nerve ligation. Once completed, the nerves and vessels were ligated, the flap was transferred to the groin for microsurgery, and the radial forearm donor site was covered with Integra (Integra LifeSciences, Plainsboro, NJ). The radial artery was anastomosed with the left deep inferior epigastric artery, two vena comitans were coupled to the greater saphenous vein, and two nerves were coapted to the dorsal penile nerve stump. The procedure was concluded and the patient was transferred to the ICU in stable condition. Postoperatively, the patient developed a 1 x 1.5-cm2 area of distal flap tip necrosis that resolved following conservative treatment with daily topical Silvadene. The patients remained in the ICU postoperatively for close flap monitoring and nutritional optimization, and he was discharged 14 days following the procedure. The postoperative course was complicated by a flap and donor-site infection requiring debridement of both sites with subsequent resolution of the infection. The patient is pending anastomosis of his neourethra to his native urethral stump by the urology department. The patient currently has a catheter in place for a urinary function that will be removed only after the neourethral anastomosis is complete. Should the patient desire the ability to maintain an erection for sexual activity, he would require an additional surgery for penile prosthesis placement. The patient has reported preserved sensation in the phallus and expressed satisfaction with his result.
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pmc-6368431-1
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A 67-year-old male with no significant past medical history presented to the emergency department with tremors and lethargy for one day. He stated that he started a five-day course of kidney cleansing regimen and had followed the instructions to the letter. For five days, his oral intake was limited to drinking over a gallon (3.7 liters) of fluid daily along with herbal tea. On the fifth day, he started to experience vague weakness, fatigue, nausea, tremor and restlessness. On general examination, he looked anxious and restless. Vitals were blood pressure of 158/84 mmHg, respiratory rate of 28 cycles per minute, pulse of 88 beats per minute, temperature of 98 F, and oxygen saturation of 98% on room air. His cardiovascular and respiratory exams were unremarkable. Initial laboratory evaluation revealed a serum sodium of 111 mmol/L. Based on history and laboratory finding, a diagnosis of acute severe hyponatremia was made and the patient was admitted to the ICU. Treatment was started with intravenous (IV) fluid. Isotonic saline 0.9% was started initially at a rate of 125 cc/hour, after two hours serum sodium increased from 111 mmol/L to 112 mmol/L. So the decision was made to infuse hypertonic saline 3% at a rate of 45 cc/hour. That leads to forced diuresis and rapid rise of serum Na from 112 mmol/L to 120 mmol/L on two hours serum Na follow-up. In order to prevent this fast correction of serum Na dextrose 5% was started at a rate of 75 cc/hour and serum Na was monitored every four hours in intensive care setting. After 48 hours since the time of admission, the patient clinically improved, serum sodium increased to 129 mmol/L and intravenous fluid administration was discontinued. He was counselled to discontinue the detoxification regimen and follow-up with his primary care physician after discharge.
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pmc-6368694-1
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The patient was a 28-year-old man who presented with a 2-week history of subtle personality changes, expressive aphasia, and severe headaches with nausea and vomiting. Imaging revealed a large mass in the left frontal lobe with significant surrounding vasogenic edema (Fig. a). He underwent gross total resection and pathology was consistent with a high grade neuroepithelial tumor with brisk mitotic activity and necrosis (Fig. , Additional file : Figures S1 and S2). The tumor was found to be IDH1 and TP53 mutant by amplicon-based next generation sequencing (NGS) targeting the hotspot regions of 27 cancer associated genes (not including ATRX), and had monosomy 1 and 19q loss by single nucleotide polymorphism (SNP) array (Figs. and ). A diagnosis of anaplastic oligodendroglioma was favored based on histologic appearance of the majority of the tissue with features suggestive of an oligodendroglioma in addition to preserved ATRX immunoreactivity, and atypical alterations in chromosomes 1 and 19. Treatment options were discussed including participation in the CODEL trial (NCT00887146), but ultimately started standard of care treatment with radiation therapy with plan for adjuvant procarbazine, lomustine, and vincristine (PCV) chemotherapy following radiation. Unfortunately, after resection and prior to starting radiation he developed somnolence, an intense headache, and diplopia. MRI of the brain revealed evidence of tumor regrowth in the original location and signs of impending herniation. He was started on dexamethasone and underwent emergent surgical decompression with tumor debulking. Pathology was again consistent with a high-grade IDH1-mutant glioma. His symptoms improved and he received intensity-modulated radiation therapy (IMRT) with a cumulative dose of 5940 cGy over 33 fractions. MGMT promoter methylation was tested upon recurrence and was found to be present.
Approximately two weeks after completing IMRT he experienced two focal seizures with secondary generalization resulting in right hemiparesis. Repeat MRI of the brain showed evidence of disease progression with interval development of a new enhancing lesion outside the radiation field involving the paramedian left parietal-occipital lobe extending to the superior surface of the left cerebellar tentorium (Fig. b). Due to newly reported low back pain, he underwent MRI of the spine which demonstrated extensive bony metastases. A whole-body 18Fluorodeoxyglucose (FDG) PET-CT scan was obtained and revealed innumerable hypermetabolic lesions throughout the patient’s body including lytic lesions virtually involving his entire skeleton and innumerable lesions in the liver, right lung, and left kidney. His clinical condition rapidly deteriorated and developed a clinical picture consistent with tumor lysis syndrome. Lactate dehydrogenase was elevated on admission at 5206 U/L (normal range 118–273 U/L) and increased to a peak level of 15,080 U/L. He became hyperuricemic (peak of 12.7 mg/dL; normal range 3.5–7.2 mg/dL) and hypophosphatemic (1.1 mg/dL; normal range 2.7–4.5 mg/dL) with evidence of acute kidney injury (creatinine from 0.6 mg/dL on admission to a peak of 1.9 mg/dL). Rasburicase was administered to treat his hyperuricemia. A biopsy of one of the liver masses in the right hepatic lobe (3.6 cm) was performed to rule out the presence of an independent neoplastic process given the very unusual aggressiveness of the cancer and widespread metastatic disease. The tissue sample showed prominent cellular necrosis with rare clusters of neoplastic cells, IDH1-mutant protein positive, consistent with metastasis of the patient’s known brain primary.
In addition to tumor lysis, the patient rapidly became thrombocytopenic (179 K/cu mm on admission to a nadir of 35 K/cu mm by day seven) and anemic (14.5 g/dL to a nadir of 5.8 g/dL), likely due to bone marrow infiltration of his cancer. Different chemotherapy regimens were considered and the choice was for temozolomide, as it is typically effective in diffuse gliomas and is able to cross the blood-brain barrier with even higher concentrations systemically. Five doses were planned (150 mg/m2) but the patient was only able to receive two administrations.
Unfortunately, the patient’s condition continued to deteriorate. He developed worsening renal failure, hypotension, and acute respiratory failure. He was intubated and mechanically ventilated, requiring vasopressor support. The patient developed cardiac arrest, was resuscitated and eventually transitioned to comfort measures. He was terminally extubated and passed away. An autopsy was declined.
Further analysis of the patient’s tumor tissue using a targeted, capture-based next generation sequencing panel covering the full coding regions of 644 cancer associated genes showed the presence of a number of additional genetic alterations (Table ), including an ATRX missense mutation, and amplification of MYCN, MET, and CDK4. There were no mutations in CIC or FUBP1. Other chromosomal variations, including gain of 7p and loss of 2q, 10p, 11p, 21q, and whole chromosomes 12 and 14 were also noted (Additional file : Table and Figure S3).
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pmc-6368759-1
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A 65-year old male patient was referred to our hospital with several space-occupying lesions in inferior lobe of right lung and enlarged lymph nodes in mediastinum and bilateral hilum detected occasionally by chest computed tomography (CT) scanning during the annual health examination in April 2015 (Fig. a). Sequential brain magnetic resonance imaging (MRI) showed a space-occupying lesion in left parietal lobe (Fig. a). One of the lesions in right lung obtained by percutaneous lung biopsy (PNLB) was proved to be adenocarcinoma (Fig. a). Subsequent drive gene analysis with the method of amplification refractory mutation system (ARMS) using a collected tissue sample suggested a deletion of EGFR exon 19 without T790 M mutation. Hence, a clinical diagnosis was made as adenocarcinoma in inferior lobe of right lung, in association with multiple lesions among bilateral lungs, enlargement of lymph nodes in mediastinum and bilateral hilum, and solitary lesion in left parietal lobe of brain suggesting metastasis (Fig. a). The patient was treated with two cycles of chemotherapy (pemetrexed plus cisplatin) as first-line therapy until June 2015, due to the delayed report of drive gene analysis. After the finish of chemotherapy, repeating chest CT scan revealed an increased lump in inferior lobe of right lung, as well as lymph nodes in mediastinum and bilateral hilum (Fig. b). Brain MRI in the same week showed a stable node (Fig. b). We therefore, evaluated the efficacy of the first-line chemotherapy as progressive disease (PD) according to response evaluation criteria in solid tumors (RECIST version 1.1). Icotinib was then administrated as the second-line treatment in June 2015. Subsequent CT scans, as expected, revealed a partial response (PR) for lesions in lung and a complete response (CR) in brain in the following 8months (Fig. c-e). In February 2016, regular chest CT scanning showed a secondary enlargement of neoplasm in primary location of right lung (Fig. f). Next-generation sequencing (NGS) technique using the patient’s plasma sample suggested an acquired T790 M mutation by frequency of 4% accompanied with the deletion of EGFR exon 19 by 7% (p.745–750 del. c.2235_2249 del GGAATTAAGAGAAGC. Figure a-b). The third-generation TKI of AZD9291, therefore, was prescribed as the third-line therapy in March 2016. After a ten-months duration of response (Fig. a-c), significant PD of lump in inferior lobe of right lung with atelectasis was detected again by chest CT scanning in January 2017 (Fig. d), while without any lesions in brain (Fig. d). Hence, two cycles of cytotoxic drug with docetaxel were administrated as the fourth-line management then. However, the re-enlargement of primary neoplasm in lung and multiply emerging lesions in brain signified a PD again in March 2017 (Fig. e), with symptoms of cough and hemoptysis aggravated seriously. Repeating NGS with plasma was developed on March 10th 2017. It was detected that the deletion of EGFR exon 19 (p745–750 del) with frequency by 78.3% and T790 M mutation by 0.2%, with concurring Leu792H mutation by 0.2%, rather than C797S mutation (Fig. c-d). In addition to those, other drive genes in the NGS panel including anaplastic lymphoma kinase (ALK), ROS proto-oncogene 1 (ROS1), V-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog (KRAS), neuroblastoma RAS viral oncogene homolog (NRAS), RET proto-oncogene (RET), V-raf murine sarcoma viral oncogene homologB1 (BRAF), receptor tyrosine-protein kinase erbB-2 (ERBB2), RAC-alpha serine/threonine-protein kinase (AKT1), discoidin domain receptor tyrosine kinase 2 (DDR2), fibroblast growth factor receptor 1 (FGFR1), MNNG HOS transforming gene (MET), phosphatase and tensin homolog (PTEN), phosphatidylinosito-4,5-bisphosphate 3-kinase (PIK3CA), and mitogen-activated protein kinase 1 (MAP2K1) were detected as wild type. In view of the primary resistance to chemotherapy, lack of potentially effective selection, and high frequency of EGFR exon 19 mutation, repeating icotinib was attempted as salvage treatment. After 2 months treatment, multiply lesions in brain were dramatically disappeared according to brain MRI (Fig. a), as well as symptoms of cough and hemoptysis relieved apparently. The repeating icotinib was administrated till November 30th 2017 (Fig. b-c), on which a sudden syncope happened. An emergency brain MRI suggested recurrence of tumors, with an enlarged lesion in left parietal lobe surrounded by encephaledema severely(Fig. d). Coinstantaneous chest CT scanning suggested a PD of the target lesion in right lung (Fig. d). After symptomatic treatment of dehydration with mannitol, whole brain radiotherapy (WBRT) was developed as palliative management. PNLB was operated again on December 18th 2017, result of which reconfirm adenocarcinoma in lung (Fig. b), accompanied with the deletion of EGFR exon 19 (p.745–750 del) with frequency by 87.5% and T790 M mutation by 9.4%, without Leu792H mutation any more obtained from plasma or tissue (Fig. e-f). Sixth-line treatment of repeating AZD9291 was restarted on January 6th 2018 due to the increased frequency of T790 M mutation. One month later on February 5th 2018, repeating pictures of chest CT showed a response of lump in right lung, with partial remission of lesions in brain as MRI presented (Fig. e). The variation of tumor markers including carcino-embryonic antigen (CEA, normal range, 0–0.5 ng/mL) and carbohydrate antigen 72–4 (CA72–4, normal range, 0–6.9 U/mL) are showed in Fig. for each visit from the initial treatment to the present. In addition, variations of tumor size during the whole treatment according to RECIST version 1.1 were listed in Table . The patient feels good without any symptoms and still receives AZD9291 treatment now. The overall survival has been 36 months and still in the extension.
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pmc-6368763-1
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A 30-year-old man was diagnosed as Cholangiocarcinoma with mediastinal lymph node metastasis stage IV. In July 2013, he received treatment at a local hospital for Crohn’s disease. In Nov. 2014, he received a liver transplantation; a huge tumor at hepatic portal was intraoperatively resected. The postoperative pathology report revealed a liver and hepatic portal poorly-differentiated adenocarcinoma with unresectable Cholangiocarcinoma metastasized to lymph nodes.
The MRI scan performed on Feb. 24th, 2015 showed a lesion in patient’s liver, therefore, he received lymph node resection on Apr. 13th, 2015. From Jun. 13th, 2015 to Aug. 14th, 2015, the patient received radiotherapy for hepatic portal and the area adjacent to inferior vena cava, with a total dosage of 45Gy. Afterward, the patient did not receive any further anti-cancer treatments, except follow-up visits. The PET/CT collected on Apr. 15th, 2016, showed lesions in mediastinum and liver. On Jun. 29th, 2016, the patient came to the Fuda Cancer Hospital. Firstly, aspiration biopsy was conducted and 10 I125 was seeded into the mediastinal tumor. On June 2017, when the patient came back the Fuda Cancer Hospital for follow-up check-up, biopsy result showed recurrent abdominal lymph node metastasis by experts’ consultation, therefore starting from June 2017, the patient only received γδ T cell immunotherapy to control his lesions, and the first γδ T cell infusion was scheduled on August 2017.
100 ml of blood was donated by a donor who had passed a health examination that included a check for infectious diseases. Following this procedure, a cell culture formula, which we developed (patent pending) that included zoledronic acid and a variety of interleukin was applied specifically to expand Vγ9Vδ2 T cells in vitro (culture media components and mechanism will be discussed in detail in our preparing article). With this formula, we can generally obtain 300–400 million of Vδ2 T cells at ~ 12 days. Figure shows a brief illustration on cell expansion and cell quality control as well as cell reinfusion, and Fig. indicates schedules of γδ T cell treatments and immunophenotypes monitoring (Additional file : Figure S1 and S2).
5 mL of peripheral blood was extracted from the patient each time, 1–3 days before receiving Vδ2 T cell treatment. Peripheral blood monocyte cells (PBMC) were isolated using the Ficoll recipe. Then immunofluorescence labeled cells were analyzed using flow cytometry (FACSanto™ II; BD Biosciences, San Jose, CA, USA). The analyzed immune cells mainly included T lymphocytes, NK cells, and γδ T cells.
During Vδ2 T cell treatment, tumor was routinely evaluated by using MRI imaging to monitor tumor size/area changes by the largest transverse diameter, particularly before and after treatment. The patient received plain and enhanced MRI 2 weeks before treatment, and then scanned periodically at the 3rd and 6th months after treatment.
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pmc-6368789-1
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A 56-year-old man was admitted to the hospital for the management of recurrent massive hemoptysis. The patient had a history of recurrent pneumonia, including an episode complicated by empyema requiring a left lower lobe thoracotomy, and recurrent sinus infections requiring surgery. In addition, he had a long-standing history of pneumatoceles, cystic bronchiectasis (Fig. ), multiple Aspergillomas on CT (Fig. ), and allergic bronchopulmonary aspergillosis (ABPA) with sputum cultures positive for Aspergillus. Aspergillomas had been treated in the past with intravenous (IV) amphotericin B and oral itraconazole, right upper lobe lung wedge resection, IV capsofungin therapy, and most recently, participation in the National Institutes of Health anti-microbial treatment protocol for HIES with trimethoprim sulfamethoxazole, posaconazole, and prednisone [] for the past 9 years. Other medical history included a diverticular abscess, a Mallory-Weiss tear, osteopenia, scoliosis, and gastroesophageal reflux disease. His family history was unremarkable. Prior investigations revealed eosinophilia, elevated serum immunoglobulin (Ig) E levels of 31,850 kU/l (normal range 0 to 115 kU/l), and normal IgA, IgG and IgM levels. The diagnosis of HIES was confirmed by the detection of a sporadic STAT3 mutation.
The patient first presented with persistent blood-tinged sputum and hematemesis 8 months prior to admission. At that time, esophagogastroduodenoscopy was done to rule out upper gastrointestinal bleeding. A chest CT with contrast for bronchial circulation showed a RLL basilar infiltrate consistent with blood, but no extravasation of contrast into the lung parenchyma. Arteriography of the right bronchial artery showed mildly abnormal vascularity without frank arterial extravasation or secondary angiographic signs of hemoptysis. The right bronchial and right 8th posterior intercostal arteries were embolized using 500–700 μm polyvinyl alcohol particles. The patient’s hemoptysis ceased and he was discharged home after 24 h.
The patient had no further hemoptysis for approximately 7 months and then developed sub-acute hemoptysis of approximately 10-20 cc per day for 2 weeks. After treatment with a course of oral antibiotics, he was admitted for evaluation. Chest CT without contrast showed no new abnormalities. Bronchoscopy did not demonstrate any active bleeding or an endobronchial source of bleeding. The hemoptysis improved and the patient was discharged home, but was re-admitted 23 days later after several episodes of hemoptysis > 200 cc. Laryngocospy was done to rule out sino-pharyngeal sources of bleeding. Percutaneous instillation of Amphotericin B paste into a cavitary mycetoma was considered [, ], but it was not clear which cavity was bleeding. Bronchial arteriography was repeated. Mild abnormal vascularity in the right lung was supplied by a slightly enlarged, tortuous right bronchial artery, but no active hemorrhage was observed. Notably, more intense bronchial vascularity is typically seen in the setting of Aspergillomas [, ]. Repeat embolization of the right bronchial artery and the distal right 6th intercostal artery was achieved using 250–355 μm polyvinyl alcohol particles. The hemoptysis stabilized and the patient was discharged after 4 days.
The patient experienced another episode of massive hemoptysis the following week and underwent interrogation of the pulmonary arteries via angiography. This revealed a small outpouching from a subsegmental pulmonary artery branch in the lateral segment of the right lower lobe (Fig. a). Contrast injection within the subsegmental branch confirmed the presence of the small pseudoaneurysm, with contrast actively extravasating into an adjacent cavity in the lung (Fig. b). The patient developed gross hemoptysis at this time, and was suctioned for 250 cc of blood. Embolization was achieved using seven 0.035 in. gauge Nester coils (Cook, Bloomington, IN) distal, across, and proximal to the orifice of the pseudoaneurysm, along with one 6 mm Amplatzer Vascular Plug II (St. Jude Medical, St. Paul, MN) in between the coils. After this branch was occluded, no further filling of the pseudoaneurysm or extravasation was seen (Fig. c), and the patient’s hemoptysis resolved. The patient had no further hemoptysis and was discharged home, with no further hemoptysis as of this writing.
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pmc-6368813-1
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A 16-year-old female patient was first administrated to our hospital because her right leg was 18 cm shorter than contralateral side (Fig. ). When she was 4-year-old, she contracted right tibia pyogenic osteomyelitis, leading to a 6-cm tibial absorption involving the distal epiphysis. The patient had no other past medical history. When she was 5, the tibia defect was reconstructed by her ipsilateral vascularized fibular graft. However, progressive leg length discrepancy developed with limited range of joint motion (ROM) (Table ). When she was 16, lengthening of FVFG with a unilateral external fixator was performed to correct the LLD (Fig. ). We executed an open osteotomy at the level of the middle metaphysis of the matured fibular graft. The patient received preventive intravenous antibiotic (Cefuroxime) for 72 h. The latency period was 7 days after the operation and distraction was performed at a rate of 1.0 mm per 36 h in four increments of 0.25 mm, and when the length of bone regeneration had reached approximately 6.0 cm, the distraction rate was reduced to 1.0 mm every 48 h []. Clinical and radiological examination was carried out every 15 days to assess new bone formation and the pin sites []. The rate was adjusted according to the discomfort and swelling of the limb and the quality of the regenerate bone like our previous study []. Partial weight-bearing was allowed as soon as union of the vascularized fibula graft on either junction was observed on radiographs. We achieved equalization with a unilateral external fixator in 26.5 months. The mean external fixation index was 44.2 day/cm. She had a pin-track infection and local inflammation, which were managed with pin care and oral antibiotics. The unilateral external fixator was removed when at least three of the four cortices were observed to be united on anteroposterior and lateral radiographs. She was able to walk without walking aids or braces, and to perform almost all activities of daily living with no difficulty based on the recommended criteria []. The results were divide into bone results and functional results. Based on the criteria recommended by Paley et al. [, ], bone result were excellent, and function result were good (Fig. , Table ).
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pmc-6368813-2
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A 17-year-old female patient was administrated to our hospital because her left leg was 9 cm shorter than contralateral side (Fig. ). When she was 6 years old, she was diagnosed as left tibia pyogenic osteomyelitis. She had no other past medical history. Her upper and almost the middle left tibia were absorbed. She received ipsilateral vascularized fibular graft to reconstruct bone defect at 8-year-old. The distal tibia was fused with fibula. Subsequently, progressive LLD developed. To correct the discrepancy, gradual length correction with a unilateral external fixator was started after being administrated to our hospital (Fig. ). The lengthening procedure was started at 9 years after fibular graft surgery. A lateral incision was used in the fibula for osteotomy. In order to sustain the anatomic axis, each set of pins was positioned in the same plane and perpendicular to the long axis of the proper lower limb alignment. The patient received preventive intravenous antibiotic (Cefuroxime) for 72 h. The latency period was 7 days after the operation and the rate of distraction was like case 1 according to our previous study []. Time to bone union was expressed in every 15 days and it was determined using the follow-up x-rays. Physiotherapy, daily nursing care, and regular follow-up were performed together with distraction. We achieved symmetry with a unilateral external fixator in 13.5 months. The mean external fixation index was 45.0 day/cm. The patient had a relapse clubfoot deformity gait when symmetry was achieved (Table ). Achilles tendon lengthening surgery was performed to correct the relapse clubfoot deformity (Fig. ). Partial weight-bearing was allowed as soon as union of the vascularized fibula graft on either junction was observed on radiographs. She can’t full weight-bearing until complete union of the vascularized fibula and massive bone allograft to host bone was evident. The results were divide into bone results and functional results. Based on the criteria recommended by Paley et al. [, ], bone result and function results were good. (Fig. , Table ).
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pmc-6368912-1
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An 89-year-old woman with no previous history of cardiac disease presented with chest pain of 3 days’ duration. The patient’s hemodynamics were stable, with a blood pressure of 150/90 mmHg and a heart rate of 78 bpm. The initial electrocardiogram showed ST-segment elevation in the inferior, posterior, and lateral leads ( and ). Transthoracic echocardiography showed normal-sized left and right ventricles, severe systolic dysfunction, moderate tricuspid regurgitation, and moderate mitral regurgitation with a left ventricular ejection fraction of 15%. She underwent coronary angiography, which showed anomaly in the shape of an SCA from the right aortic sinus of Valsalva and significant lesions at the common ostium and the proximal part of the right and left systems (). Coronary artery bypass graft surgery was recommended to the patient, but she refused. Accordingly, decision was made to perform percutaneous coronary intervention (PCI) with a 7-F right Judkins guiding catheter via the right femoral artery. Both right and left systems were wired with the Balance Middle Weight guide wires, and the lesions were dilated with the simultaneous inflation of two 2.5×15 MINI-TREK dilation balloons (Abbott Vascular, CA, USA). Thereafter, the lesions were Y-stented with two 3.5×115 XIENCE Xpedition stents (Abbott Vascular, CA, USA) and inflated up to 14 mmHg before the procedure was terminated (). The procedure was successful, and there were no complications ( and ). The patient was discharged 2 days later with no adverse complications.
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pmc-6368915-1
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We present the case of a 61-year-old man who was admitted to our center with sudden-onset chest pain. The patient was diagnosed with non–ST-elevation myocardial infarction. His electrocardiogram (ECG) showed normal sinus rhythm with inferolateral ST-segment depression (). The patient’s past medical history included controlled hypertension, non–insulin-dependent diabetes mellitus, peripheral vascular disease, hypercholesterolemia, iron deficiency anemia, hepatitis B, and being an ex-smoker.
The patient’s immediate angiogram revealed a right-dominant heart with severe distal left main stem disease and an 80% occlusion. The left anterior descending artery (LAD) was diffusely irregular with severe mid-vessel disease. It was noted that he had a high diagonal artery, in which the proximal portion was severely diseased. The left circumflex artery was both severely and diffusely diseased and very tortious. The right coronary artery was diffusely diseased. Due to the presence of diffuse multi-vessel involvement, the patient was deemed suitable for inpatient coronary artery bypass grafting surgery (CABG). He was medically stabilized and optimized for surgery.
Seven days following his initial presentation, the patient underwent CABG. On the induction of anesthesia and during the insertion of the central venous line into the right internal jugular vein, the patient developed an acute episode of sinus tachycardia followed by sinus bradycardia of 40 beats per minute. A bolus dose of adrenaline (5 μg IV) was given to maintain his heart rate. The preliminary impression was that the patient had sustained an infarct affecting the atrioventricular conduction system. Cardiopulmonary bypass (CPB) was instituted immediately, and the patient’s status was stabilized.
CABG was performed on the patient utilizing the left internal mammary artery to the LAD and the saphenous venous graft to the obtuse marginal and the posterior descending artery. The CPB time was 77 minutes, and the aortic cross-clamp time was 35 minutes. He was weaned off CPB support in a state of sinus bradycardia. Two epicardial pacing wires were placed, and the patient was externally paced.
Three days following the CABG surgery, the patient became hemodynamically unstable with alternate episodes of tachy-brady arrhythmia concomitant with atrial fibrillation. For rate control, he was loaded with intravenous amiodarone. His status necessitated inotropic support with noradrenaline to maintain an adequate blood pressure and urine output. Bedside transesophageal echocardiography ruled out cardiac tamponade and demonstrated no evidence of a left atrial thrombus.
Subsequently, an urgent consultation with our local electrophysiology team was sought. It was noted that the tachy-brady arrhythmia included multiple electrical pauses () alternating with episodes of atrial fibrillation. A decision was made to insert a dual-chamber permanent pacemaker (PPM). Following the insertion of the PPM, the patient experienced self-limiting episodes of atrial fibrillation associated with a fast-ventricular response rate; he was commenced on beta-blockers. Once the heart rate was controlled, he was discharged from the intensive care unit to a normal ward and made a complete recovery.
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pmc-6368917-1
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A 66-year-old man was hospitalized because of persistent fever and chills of 1 month’s duration. He had a history of cholecystectomy, common bile duct anastomosis to the small bowel 5 years previously, and pyonephrosis secondary to transurethral lithotripsy 1 month before the recent admission. He had no history of alcohol consumption and intravenous or illicit drug use.
Positive physical examination findings on the admission day included fever and mild right upper quadrant abdominal tenderness. Initial laboratory test results showed leukocytosis and elevated acute-phase reactants including the erythrocyte sedimentation rate and C reactive protein, while liver enzymes, creatinine, and electrolyte panel were within the normal limits. Three sets of aerobic blood culture, drawn on admission, were negative.
On day 1, computed tomography (CT) scan of the chest and abdomen revealed a filling defect in the IVC, protruding into the right atrium. Fluid accumulation in the retrohepatic and right perinephric space was found. In addition, a hypodense opacity medial to the caudate lobe of the liver, in favor of a hepatic abscess, was noted but not confirmed by the second report ().
Transesophageal echocardiography (TEE) revealed interatrial septal aneurysm, small-sized patent foramen ovale with a negligible flow, and no evidence of infective endocarditis. A large mass was detected at the origin of the IVC entering the right atrium, suggestive of a clot ().
Magnetic resonance imaging of the heart, abdomen, and pelvis was performed, and it confirmed the previous findings. It also revealed multiple hyper-intensity signals in the right perinephric space with a restricted pattern and ring enhancement, suggestive of abscess formation ().
In light of the imaging and clinical findings, a diagnosis of suppurative thrombosis of the IVC was made and intravenous antibiotic therapy with meropenem (1000 mg every 8 h) in combination with vancomycin (1000 mg every 12 h) was started. This combination was started on suspicion of nosocomial infection bearing in mind that the patient had pyonephrosis secondary to transurethral lithotripsy 1 month before the recent admission. Although experts recommend anticoagulation and endovascular surgery, since our patient’s symptoms had significantly improved after 1 week of antibiotic therapy, we decided to continue the intravenous antibiotics alone as the treatment strategy. Subsequent TEE showed a significant size reduction in the IVC mass. Moreover, a repeat CT scan of the abdomen and pelvis in week 10 showed ample resolution of the perinephric abscesses. Follow-up TEE in week 16 illustrated no residual thrombus in the IVC.
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pmc-6368980-1
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A 47-year-old woman was brought to our department with laryngeal burns sustained during a house fire. She wished to confirm the changes in the larynx. She was also counseled to relieve post-traumatic stress. During the first visit, the patient presented with a severe breathy voice, and was noted to have a cough. Although laryngeal edema was present, the airway was secure. Laryngeal movement was restricted during phonation. Laryngoscopic examinations showed movements of the vocal folds during respiration, but could not evaluate the vibratory patterns of the vocal folds during phonation because of the non-vibrating portion ().
Also, non-periodic vocal fold vibrations were invisible on laryngeal examination. HSV was used to observe the micro-vibrations in the non-vibrating portion. This means that the mucosa of the entire vocal folds was changed to a condition of increased stiffness. The absence of mucosal waves was also confirmed by digital kymography (DKG) and two-dimensional kymography (2D DKG) (-) ().
Acoustic analysis was performed to quantify the voice quality. Cepstral analysis of the voice signal was performed by analysis of dysphonia in speech and voice (ADSV; model 5109 v.3.4.2; KayPENTAX, Montvale, NJ). The results of cepstral peak prominence (CPP), low-to-high frequency spectral ratio (L/H spectral ratio), and mean CPP F0 are presented in . The CPP is a measure of the degree of harmony within voice signals. The L/H spectral ratio represents the mean ratio of the signal energy below 4,000 Hz to the energy above 4,000 Hz for the selected voiced data frames (). Additionally, an auditory perceptual assessment using the GRBAS scale was also performed. The GRBAS scale, which assesses the overall degree of dysphonia, as well as rough, breathy, asthenic, and strained parameters, is widely used in voice research and clinical practice (,). Initially, medication and voice therapy were planned as the patient complained of throat pain and dysphonia. An expectorant, bronchodilator, and antibiotic were prescribed for foreign body sensation and pain in the neck. Subsequently, intensive voice therapy was commenced for voice production, with effective glottal closure. Voice therapy techniques, such as vocal function exercises, accent method, and inhalation phonation were used
Additionally, an intracordal injection was considered as a second intervention for glottal contact. It was expected that the quality of the vocal fold mucosa and voice would improve based on the treatment plan.
Fortunately, there was no need to perform the injection because the voice quality was noted to have improved after 6 months of follow-up, and it was decided to administer medications alone and observe the patient without any additional treatment ().
An anterior web was noted to form during the recovery process, but this did not affect the patient’s breathing.
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pmc-6368982-1
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A 31-year-old woman gave birth to a full-term baby girl weighing 3.6 kg via spontaneous vaginal delivery at 40 weeks of gestation. The Apgar score was 9 after 1 minute; however, the child was noted to have a loud biphasic stridor with subcostal recessions, which did not improve with positioning. The respiratory rate was 60 breaths per minute and the pulse oximeter oxygen saturation was 100% under a head box with oxygen (5L/min). Liquor was clear. A physical examination of the cardiorespiratory system revealed normal heart sounds with transmitted sounds heard on auscultation of the lung fields. The case was referred to the otorhinolaryngology team on day 1 of life after being transferred from a district hospital. A bedside flexible nasolaryngoscopy examination revealed normal glottic and supraglottic structures.
A direct laryngoscopy with telebronchoscopy carried out on day 2 of life revealed a pulsatile narrowing of the tracheal lumen at the level of the thoracic trachea, suggestive of an external compression. An uncuffed endotracheal tube with an internal diameter of 3.0 mm was used to stent the narrowed segment to maintain airway patency. A contrast-enhanced computed tomography (CECT) of the thorax with a three-dimensional (3D) reconstruction confirmed a diagnosis of DAA, which was tightly encasing the trachea and esophagus (,).
Echocardiography did not show any associated intracardiac anomaly. Feeding was maintained via a nasogastric tube from day 1 of life. Although corrective surgery was scheduled at a cardiac center in Kuala Lumpur, the infant developed acute massive hematemesis on day 37 of life and succumbed to it prior to the surgery.
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pmc-6368983-1
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An 8-year-old-girl presented with principal complaints of pain in the left ear, bleeding from the mouth, difficulty in swallowing, change in the quality of voice and difficulty in breathing since one month. There was no preceding history of trauma to the neck or associated history of nasal obstruction or epistaxis. Personal and family history was not relevant. On clinical examination, there was a large submucosal bulge present over the left tonsillar fossa which crossed the midline, pushing the uvula to the opposite side and obscuring the oropharyngeal airway. The endolarynx could not be examined. On nasal endoscopy, bilateral choanae were blocked by a pinkish friable mass obliterating the whole of the nasopharynx. There were no palpable neck nodes. Hematological, liver and renal function tests were within normal limits. Contrast enhanced computed tomography (CECT) was suggestive of a large heterogeneous enhancing soft tissue density mass in the left parapharyngeal space with extension to the oropharynx, laryngopharynx, nasopharynx, prevertebral space, paravertebral region and reaching superiorly up to the base of skull (). The mass was causing displacement of the left carotid artery, complete compression of the internal jugular vein and significant narrowing of the airway.
Biopsy of the mass showed sheets and nests of round-to-oval tumor cells with hyperchromatic nuclei and scant cytoplasm ().
Brisk mitosis and large areas of tumor necrosis were seen. Immunohistochemically, the tumor cells were positive for CD99 and negative for smooth muscle actin (SMA), desmin and chromogranin immunostains. Weak periodic acid-Schiff (PAS) positivity was also noted ().
A CECT of the chest and abdomen revealed multiple lung nodules with lytic lesions in the dorsal vertebra suggestive of metastasis. Hence, a final diagnosis of EES of the parapharyngeal space with lung and skeletal metastasis was made.
In view of the disseminated disease, chemotherapy was started with vincristine, doxorubicin and cyclophosphamide at standard doses. The patient received six cycles of chemotherapy, and showed improvement in symptoms. Repeat CECT showed persistence of the parapharyngeal lesion with skeletal metastasis, although resolution of the lung nodules was noted. The patient was then given palliative local radiotherapy of 36 Gy in 20 fractions over 4 weeks. However, 1-month post radiotherapy, she presented with complaints of headache, vomiting and medial deviation of both eyes. Subsequent brain CECT revealed an intensely enhancing mass in the left frontoparietal region with mediastinal shift and uncal herniation. Following the CECT brain findings, palliative whole-brain radiotherapy of 25.2 Gy in 14 fractions was given at a rate of 1.8 Gy per fraction. The patient deteriorated further with rapid progression of local and systemic disease. Despite the best medical efforts, the patient succumbed to her disease 1 year after diagnosis.
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pmc-6368986-1
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A 51-year-old gentleman with a known history of hypertension presented with right-sided neck swelling, which had been progressively increasing in size for the past 2 years, along with dysphagia and odynophagia. The swelling was on the right side of the neck and was firm, non-mobile, and slightly tender to touch. It measured around 5 × 4 cm and was not adherent to the skin. Examination of the ear, nose, throat, and oral cavity was unremarkable, and flexible laryngoscopy revealed right vocal cord paresis. The patient had no complaint of voice change.A computed tomography (CT) scan of the head and neck with contrast was advised, and revealed a large heterogeneously enhancing solid lesion posterior to the right sternocleidomastoid muscle displacing the carotid space and medially extending inferiorly to the supraclavicular fossa. The mass measured 7.5×6.2 cm in size along its greatest dimensions ().
The patient underwent open biopsy with panendoscopy. The histopathology was inconclusive for malignancy; however, culture revealed S. aureus and Pseudomonas, with a raised CRP of 16.1 mg/dL. The patient was treated on the basis of an infectious etiology. The patient re-presented to us with complaints of rapidly increasing right-sided neck swelling for the past 3 months. A biopsy of an enlarged lymph node revealed papillary thyroid carcinoma.
The patient was electively taken for surgery and underwent total thyroidectomy with right-sided neck dissection. Intra-operatively, the right-sided lesion was extending from just above the clavicle to the base of the skull. It was adherent to the internal carotid artery and was involving the vagus and the accessory nerve, both of which had to be scarified during the excision of the lesion. The mass was also seen to be infiltrating the anterior-lying sternocleidomastoid and adherent to the internal jugular vein. In all, a radical neck dissection was carried out on the right side.
Histological examination of the thyroid gland showed multifocal papillary thyroid carcinoma involving both lobes with extrathyroidal spread. The tumor was composed of papillary structures with fibrovascular cores. The lining cells exhibited enlarged nuclei with focal grooves, pseudo-inclusions and overlapping (). The tumor involved 8/13 neck lymph nodes (), and was staged as pT3, N1. Histological examination of the separately sent 6×5×3.5 cm nodule showed a circumscribed cellular spindle cell neoplasm arranged in fascicles.
The tumor cells had hyperchromatic nuclei and eosinophilic characteristics (). Up to 11 mitoses/10 high-power fields (HPFs) were seen. The tumor cells were positive for Cytokeratin (CK) AE1/AE3,epithelial membrane antigen (EMA), Bcl2, CD99 and transducer-like enhancer of split 1(TLE1) ().
The tumor cells were negative for desmin, anti-smooth muscle actin (ASMA), and S100. The morphological and immunohistochemical features were consistent with synovial sarcoma. Translocation SS18 was detected by fluorescence in situ hybridization () Final diagnosis was conventional papillary thyroid carcinoma and monophasic synovial sarcoma of the neck.The patient was discharged in the subsequent days after neck drain removal and once stable. On follow-up fiberoptic laryngoscopy, right-sided vocal cord paralysis was seen, which was expected. Radiation and chemotherapy followed by radio-iodine ablation was advised after the case was discussed in the multidisciplinary tumor board meeting. The patient was disease free 6 months’ post-surgery and had started undergoing chemotherapy from another institute.
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pmc-6368990-1
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A 35-year-old male carpenter presented to our emergency department with a history of accidental penetrating injury on his neck caused by a piece of metal breaking from a saw when he was using and hit him on his neck. The patient had severe pain and bleeding from the wound entry site, which was aggravated on swallowing, speaking and on moving the neck. There was no history of difficulty in breathing, change in voice or cough.On clinical examination, the patient was conscious, well oriented and hemodynamically stable without any neurological deficit. On neck examination, there was an open wound, about 0.5 × 0.5 cm in size, in the lower third anterior aspect of the neck with surrounding neck swelling and tenderness (). Subcutaneous emphysema was present, extending from the inferior border of the mandible to the clavicle. Videolaryngoscopic examination showed normal vocal cord mobility.
X-rays of the cervical region and chest were taken immediately in the anteroposterior and lateral view. A soft tissue neck (STN) lateral view revealed a radiopaque foreign body shadow at the level of the C6–C7 vertebral body level (). This was further confirmed with contrast enhanced computed tomography (CECT) of the neck with fine 1-mm cuts, which also showed that the radio-dense foreign body lodged in the tracheoesophageal party wall at the level of second and third tracheal rings, corresponding to the C7 vertebral body ().
The patient was taken for immediate surgical exploration. A low tracheostomy was performed under local anesthesia at the level of the fourth tracheal ring, which was below the level of the penetrating wound. A Portex cuffed tracheostomy tube (size 7.5) was inserted, and general anesthesia (GA) was administered through the tracheostomy tube. The vertical incision for tracheostomy was extended superiorly up to the cricoid cartilage. The strap muscles were retracted, and the thyroid isthmus was retracted above. The small entry point was found on the anterior wall of the second tracheal ring (). We opened the second tracheal ring at the site of entry in a vertical fashion. The second tracheal ring was opened in an open-book fashion, and the foreign body was visualized embedded in the party wall between the esophagus and the trachea. The foreign body was gently removed, avoiding accidental slippage into the distal airway ().
Hemostasis was achieved and the opened second tracheal ring was approximated atraumatically using Prolene 3-0. The wound was closed in two layers. A nasogastric tube was inserted and removed on postoperative Day 2 (POD). On POD5, the patient was decannulated successfully and discharged from hospital without any complications.
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pmc-6369133-1
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A 20 yr old male patient was referred from a peripheral hospital to our institution intensive care unit having been operated on for perforated duodenal ulcer. He had presented with a long- standing history of peptic ulcer disease and had developed sudden onset of severe abdominal pain a day prior to admission. A plain radiograph done revealed free air under the diaphragm. Surgical intervention of a modified Grahams patch for a perforated duodenal ulcer was done. Post operatively, he was referred to due acute kidney injury(AKI).
At the ICU, his vital signs were an elevated heart rate of 140bpm, increased respiratory rate of 36 breaths per minute, low blood pressure of 80/50 mmHg and his temperature was 36.5°c. On examination, he had purulent discharge from his abdominal drain. His serum creatinine was elevated at 254 mmol/L (65–130) with an increased Blood Urea Nitrogen of 18.8 mmol/L (1.7–8.3). The arterial blood gas analysis (BGA): PH-7.354, PCO2 -3.57 kPa, PO2 – 8.26 kPa, HCO3 – 14.6 mmol/L, Base Excess- -9.6 mmol/l and SPO2 -91.3%. He was admitted with a diagnosis of hypovolaemic shock with intra-abdominal sepsis, compensated metabolic acidosis, and AKI. His initial management was intravenous fluids, empirical antibiotics: ceftriaxone, metronidazole and analgesics.
The patient was taken to theatre and intra operatively, 2 litres of purulent bilous material was found in the peritoneum and the previous repair had given way and there was now a large duodenal perforation with friable edges measuring approximately 1 cm in size at D1. A pyloric exclusion was done with a Roux-and-Y gastrojejunostomy and peritoneal lavage was done. Abdominal drains were left in-situ and he was taken back to the ICU.
The patient remained stable post operatively but was noted to have pus oozing from the incision site on post-op day three and on day four upon release of sutures he had a burst abdomen. He was also noted to be febrile with temperatures of 39 °C. An emergency laparotomy revealed a duodenal stump blow-out. Peritoneal lavage and repair of the duodenal stump was done and a “Bogota bag” as a method of temporary abdominal wall closure was chosen. He went to theatre twice weekly for peritoneal lavage and change of Bogota bag for an initial period of three weeks due to pus exudate from the abdomen and abdominal drains but as the effluent reduced, he went on demand basis. His clinical outlook was generally improving however the intra-abdominal infection persisted. The challenges we faced with the patient’s deranged physiology while in ICU are tabled as shown on .
shows the microbial profile that was grown and the antibiotic sensitivity patterns. These are most likely hospital acquired infections. His urinary cultures only once grew candida and all his tracheal aspirates grew nothing on culture. His antibiotic treatment changed from the empirical treatment with ceftriaxone and metronidazole and was tailored as per microscopy, culture and sensitivity results. Throughout his stay in the ICU he was put on H.pylori treatment, thromboprophylaxis, total parenteral nutrition and two hourly turning in bed to avoid bed sores.
After the fifteen laparotomies of which 12 were planned, the repair held and the intra-abdominal sepsis was controlled. The patient developed an entero-atmospheric low output fistula that was managed conservatively. He started passing stool ten weeks after his admission and he was allowed to feed orally as the parenteral nutrition was weaned off. Granulation tissue formed over his bowel and the low output fistula eventually closed. The patient was transferred from ICU to the ward having stayed there for ten weeks. The wound continued to be dressed in the ward for the next 2 months and healed with formation of a large ventral wall hernia. He was discharged home to be reviewed by the plastic surgery team at the surgical outpatient clinic to plan for reconstruction of the abdominal wall at a later time.
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pmc-6369201-1
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The patient was a 21-years-old Japanese male with no family history suggestive of immunodeficiency. He was born to non-consanguineous Japanese parents. He had experienced recurrent respiratory infections since childhood. At the age of 6 years, he was hospitalized with EBV-associated leiomyoma in his right bronchus, and complement deficiency (C2 and C9), low T-cell count, and reduced responses to phytohemagglutinin (PHA) and concanavalin A (ConA) were also found (). PID of unknown cause was suspected and Trimethoprim-Sulfamethoxazole (TMP-SMX) was started. He developed Yersinia enteritis at the age of 8 and pleurisy at the age of 9. After that, he did not experience severe infection for 10 years, even after discontinuing TMP-SMX at the age of 12. Chronic cough, purpura, edema, and pain of the lower limbs appeared at the age of 19. A skin biopsy was performed, which led to a diagnosis of leukocytic fragmentative vasculitis; however, immunosuppressive therapy was postponed due to his past medical history of immunodeficiency. At the age of 21, he was hospitalized with invasive Haemophilus influenzae infection, which had been stabilized following adequate antimicrobial therapy, and he also suffered from recurrent pneumonia caused by multiple pathogens. Extensive immunological evaluations showed dysgammaglobulinemia, with reduced IgG (608 mg/L) and IgG2 (109 mg/dL), elevated IgA (692 mg/dL), normal IgM (62 mg/dL), reduced IgE (<3 IU/mL), and reduced CH50 levels (16 U/mL) (), along with reduced lymphocyte proliferation (PHA 6,700 cpm and ConA 4,460 cpm). Lymphocyte subpopulation analysis showed reduced T cells, a paucity of B cells, and an increase of NK cells (). In CD3+ T cells, a markedly increased number of γδT cells was observed, and T cells were skewed to the memory phenotype, especially central memory T cells. The kappa-deleting recombination excision circles level was low but detectable, while the T-cell receptor excision circles level was undetectable. The patient exhibited normal production of specific antibodies against varicella zoster virus (VZV), mumps, rubella, and measles.
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pmc-6369256-1
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82-year-old Caucasian male, with history of prolapsed anal mass, was observed at the ER. Rectal examination revealed two large (2 cm and 1,8 cm), anterior, amelanocytic polyps, at 1 cm from the anal verge. The patient denied changes in bowel habits or other symptoms.
An elective trans-anal polypectomy was performed. Histopathology showed malignant melanoma: Mixed histology – Spindle and epithelioid cells; Thickness - 11 mm; 16 mitosis/mm2; positive margins. Immunohistochemistry showed positivity to PS100, CD117 and HMB45; and negativity to CAM 5.2, AE1/AE3, p63, CD34.
After a multidisciplinary team discussion, and due to the patient’s old age, it was decided to propose a second conservative approach: Wide local excision (WLE) (). This time a melanocytic nodule was observed at the same localization of the previous mass. Pathologic examination showed AMM with pure spindle cell histology; 3 mm thick; margins free from disease. A thoracic, abdominal and pelvic CT scan was performed: No signs of distant metastasis.
The patient was sent to the National Cancer Center for follow up.
A year later the patient presented with loco regional recurrence - Anal mass and palpable inguinal nodes, without any constitutional symptoms; Underwent surgery once again: WLE and bilateral inguinal and iliac lymphadenectomy. Pathology observed anal recurrence (free margins <1 mm) and lymph node metastasis (15 out of 35 lymph nodes).
Three months later, the follow-up PET-CT scan revealed a pulmonary metastasis at the right lower lobe and mediastinal lymph node involvement (). Due to disseminated disease and overall status, the patient did not undergo radio or chemotherapy.
Follow-up was performed every three months with clinical examination and imaging (PET-CT or CT scan). The patient died 32 months after the diagnosis, due to disease progression.
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pmc-6369259-1
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In this article, we present the case of a 54-year-old woman suffering from severe obesity with a body mass index (BMI) of 47.5 kg / m2 (weight: 104 kg, height 148 m). In anamnesis, chronic bronchopathy treated with corticosteroids and laparoscopic cholecystectomy, no family history of cancer.
She contacted our institute for bariatric surgery. The cross-disciplinary pre-operative assessment included clinical psychology interview, dietary evaluation, oesophagogastroduodenoscopy with biopsy, H. pylori identification and abdominal ultrasound. All the tests were negative for pathologies and, in agreement with the patient and the cross-disciplinary team, a surgical recommendation was given. A gastric bypass with fundectomy was performed laparoscopically.
At one and three postoperative months the patient showed a good weight loss: 95 kg at one month with 18.3% excess weight loss (%EWL) and 8.7% total body weight loss; 84 kg at 3 months with a 40.6% EWL and 19.2% total body weight loss.
At nine months she had an excellent weight loss: 72 kg with a 64.9%EWL and 30.8% total body weight loss, but she reported sporadic episodes of vomiting, asthenia and poor appetite.
The latter symptoms are common after bariatric procedures and are compatible with the normal outcome of the bypass procedure. For this reason, the patient was scheduled for the normal follow-up at one year.
One year after surgery, the patient contacted the department because of a worsening of her symptoms with vomiting and asthenia. Blood tests and X-ray with Gastrografin were carried out. Blood tests revealed slight anaemia (haemoglobin: 10.5 g/dl), and the X-ray with Gastrografin showed a regular progression of the contrast medium through the gastro-jejunal anastomosis and an absence of pathological findings ().
In the context of initial uncertainty, it was possible to subject the patient to a full gastroscopy, thanks to this technique which allows for an easy exploration of the bypassed stomach. It showed the presence of a 6 mm antral ulcer on which biopsies were performed. The results indicated that it was an intestinal type G3 gastric adenocarcinoma. After this diagnosis, preoperative staging was performed with abdominal and thoracic CT scan with contrast dye, which resulted in negative metastasis and the gastric lesion was not visible. Cancer markers tested were in the normal range (CEA, Ca19.9 and alphafetoprotein).
The patient underwent laparoscopic degastroresection with D2 lymphadenectomy. The operation lasted 230 min, with no intraoperative complications. The post-operative period was normal and the patient was discharged on the fifth day.
The definitive histological analysis documented the presence of a gastric adenocarcinoma of the antrum pT1bN0M0 with 22 negative lymph nodes removed. After an oncological evaluation, the patient underwent standard follow-up without chemotherapy or radiotherapy.
The follow-up at two years showed no recurrence of disease.
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pmc-6369457-1
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A 62-year-old woman presented with a one-month history of sudden painless visual loss in the right eye. On examination, best corrected visual acuity (BCVA) was 20/20 in both eyes. Intraocular pressure was 21 mmHg in both eyes. Dilated funduscopic examination in the right eye revealed retinal emboli inferior to the optic disc obstructing a small arteriole associated with retinal ischemia (Figures and ). The left eye was unremarkable. There was no prior past ocular history. The patient had hypertension and hypercholesterolemia and had an extensive smoking history.
Fluorescein angiography revealed delayed retinal perfusion along the inferior arcade in the right eye (Figures and ). Optical coherence tomography showed normal foveal contour with inner retinal ischemia and thickening, consistent with an acute inferotemporal branch retinal artery occlusion (). The patient was started on Latanoprost at nighttime in the right eye to lower the intraocular pressure in hopes to increase reperfusion of the retina. An extensive cardiovascular workup was done, and significant carotid artery stenosis of less than 70% was found. She was started on plavix and aspirin by her cardiologist. The patient continued with BCVA 20/20 OU vision and persistent highly refractile peripapillary emboli; retinal ischemia resolved. Her OCT showed inner retinal atrophy (Figures and ).
Unexpectedly, the patient presented with a three-day history of sudden painless visual loss OD a year and a half after her initial presentation. BCVA was counting fingers (CF) in the right eye. Fundus exam revealed new superotemporal retinal ischemia associated with two new emboli. OCT demonstrated thickening and hyperreflectivity of the inner retinal layers consistent with an acute BRAO OD (). The patient refused fluorescein angiography at that time. Urgent workup revealed worsening stenosis to 80% in her right common carotid artery. The vascular surgery referral prompted a right carotid endarterectomy with a carotid stent placement approximately two months following her presentation with acute vision loss.
After endarterectomy, vision in the right eye improved from counting fingers to 20/200 and 20/250 at 2 months and 6 months, respectively. Postoperatively, retinal whitening resolved and reduced intraretinal edema was noticed (). One year after endarterectomy, visual acuity was 20/30. The retinal swelling had resolved ().
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pmc-6369461-1
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A 13-year-old female patient was postoperatively admitted to the intensive care unit (ICU), following a spondylodesis procedure due to severe spinal malformation (). The girl's medical history was remarkable for a genetically confirmed diagnosis of SMA type 1 (Werdnig–Hoffmann disease) within her first six months of age (homozygous deletion of the survival motor neuron 1 (SMN1) on exon 7, 5q chromosome, with two copies of the SMN2 gene).
On the third ICU day, the patient developed metabolic acidosis. Arterial blood gases revealed pH 7.17 (reference 7.35–7.45), partial pressure of oxygen (PaO2) 12.40 kPa (reference 11–13 kPa), partial pressure of carbon dioxide (PaCO2) 4 kPa (reference 4.7–6.0 kPa), bicarbonate (HCO3−) 10.7 mmol/L (reference 22–26 mmol/L), and base deficit −13 mmol/L. Lactate was normal with a value of 0.8 mmol/L (reference 0.56–2.0 mmol/L). The anion gap was 14 mmol/L, and the corrected value for the albumin anion gap was 26 mmol/L (reference 3–11 mmol/L).
Before admission, the patient's respiratory function was impaired. She had significant respiratory muscle weakness and poor cough ability, and she required at home the use of noninvasive ventilation (NIV) and mechanically assisted coughing (MAC). She was intubated prior to surgery and extubated after a difficult and prolonged weaning following admission to the ICU. At the time of examination, she was breathing spontaneously and was supported intermittently with NIV. Despite her having had a gastrostomy tube at an earlier stage of life, during hospitalization, she was fasted for three consecutive days, the day of the surgery and the next two postoperative days, in light of the presumed risk of pulmonary aspiration. Furthermore, she was apyrexial, intravenously hydrated with 0.9% normal saline, and not on any inotropic or vasopressor support since her vital signs implied hemodynamic stability (systolic blood pressure > 110 mmHg and heart rate < 90 bpm). Common causes of metabolic acidosis with a high anion gap, namely, uremia, diabetic or alcoholic ketoacidosis, and lactic acidosis, were excluded. Renal and liver functions were normal, and no sign of infection was evident since inflammatory markers were negative and white blood cell account was normal. Total parenteral nutrition, and other agents, such as valproic acid and salicylates, which can potentially induce acidosis, had not been administered.
The pediatric patient had no history of DM, and she presented normal glycated hemoglobin (4.4%, reference 4.0–5.7%) and plasma glucose levels. However, her reduced body mass index-for-age (14.8 kg/m2) and, accordingly, the extremely low levels of serum urea (1.78 mmol/L, reference 2.5–8.0 mmol/L) and serum creatinine (8.8 mmol/L, reference 52–96.8 mmol/L) prompted the ICU consultation team to investigate the likelihood of ketosis and ketoacidosis. Plasma β-hydroxybutyrate assay was unavailable on the premises; nevertheless, urine analysis revealed a severe degree of ketonuria (acetoacetate >7.84 mmol/L; urine value 4+).
Upon establishment of the diagnosis of euglycemic ketoacidosis, our patient was started on a fat-free enteral nutrition enriched with carbohydrates and proteins via her gastrostomy feeding tube. The acidosis resolved completely within 48 hours; urine ketones were negative at the time. No bicarbonate or insulin infusion was administered. The patient was discharged to the ward four days later.
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pmc-6369462-1
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A 70-year-old woman with a history of diabetes mellitus type 2, hypertension, gastroesophageal reflux disease, and hypothyroidism presented with urinary incontinence. Evaluation showed a tender left vulvar lesion, which she stated had worsened over the prior nine months. The clinical impression of the vulvar lesion was lichen sclerosus et atrophicus. The patient was using an over-the-counter topical treatment (zinc oxide cream), with no amelioration of symptoms. Later, triamcinolone acetonide was tried, without improvement.
Over a period of months, the lesion slightly decreased in size but irritation and tenderness increased. Physical examination before the biopsy showed a one-centimeter ulcerated lesion with lichenoid change, involving introitus and clitoral hood. A biopsy was performed and histopathologic examination showed an overall well circumscribed lesion () with a subepidermal, horizontally oriented, plate-like proliferation of pale appearing squamous epithelial cells, with numerous points of connection with the overlying epidermis and peripheral palisading (). Interconnecting bands of epithelial cells give the lesion a fenestrated or reticulated pattern appearance. Numerous keratocysts are noted at the base, while, notably, no visible granular layer is present (). An eosinophilic basement membrane is notable around tumor islands. Elastic fibers appear to be condensed below the lesion and a patchy and mild lymphocytic infiltrate is also present around and within the tumor.
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pmc-6369479-1
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We report the case of a 69-year-old woman without significant history, who suffered from chromium-cobalt intoxication following total hip arthroplasty (THA). This patient underwent primary arthroplasty to the left hip in 2013 in another orthopedic department, with ceramic bearing. Postoperative course was uncomplicated until 2016 when she experienced three dislocations. Unipolar revision surgery for instability was performed, with per-operative ceramic acetabular liner fracturing in order to remove it. Dual-mobility cup with metal-on-polyethylene components was then implanted. Less than a year after revision surgery, the patient presented to our hospital with fever, asthenia, tachycardia, weight loss, and left groin pain. First clinical and biological evaluation suggested prosthetic joint infection. Plain radiographs and CT scan showed massive collection around the left prosthesis (Figures and ). A few days later, the patient reported rapid cognitive decline with behavioral disorders, lack of memory, and brutal hearing and visual loss with worsening of asthenia and weight loss (15 kg in 6 weeks due to decreased oral intake, attributed to dysgeusia with metallic taste). Puncture of the periprosthetic collection was performed, revealing metallosis. Neurological exam with MRI showed heavy metal accumulation in the basal ganglia and caudate nucleus (). Ophthalmologic and otologic examinations confirmed metal damages to the eyes with bilateral papilledema and to the ears with sensorineural hearing loss limited to 55 dB.
Heavy metal blood levels revealed huge concentrations of cobalt 24808 nmol/L (normal: 0.3-9) (i.e., 1461 μg/L) and chromium 1268 nmol/L (normal: 1-5) (i.e., 65.9 μg/L). Urinary dosage also revealed explosive levels of cobalt 8234 μg/g of creatinine (normal < 2) and chromium 151 μg/L (normal < 1). Regarding these systemic symptoms of metal toxicity, our patient received immediate calcicodisodic EDTA (ethylenediaminetetraacetic acid) and DMSA (dimercaptosuccinic acid) chelation.
One-stage bipolar revision surgery was also performed shortly after, in our institution. Considerable black-liquid collection (around 500 mL, cobalt dosage in liquid > 1 g/L, culture negative for infection) was found around the prosthesis with black staining in periprosthetic tissues and gluteal muscles necrosis. Every component was removed and the cobalt-chromium femoral head was severely worn, with a hole in it (). Laboratory exam of the head revealed an 18 g of metal loss, explaining heavy metal intoxication. Ceramic particles around polyethylene and chromium-cobalt head were also found, contributing to metal debris formation. We performed a large and exhaustive synovectomy and debridement of the muscles necrosis and were obliged to implant a dual mobility cup in order to maintain stability of this multioperated hip.
All symptoms along with hip pain rapidly improved after treatment. Cognitive functions were progressively restored. Patient regained audition and vision at functional level. Asthenia, behavioral disorders, and memory loss also disappeared. New MRI examination at 3 months showed reduced T1 hypersignal of the basal ganglia and caudate nucleus. These improvements accompanied a decrease in cobalt and chromium levels. Serum cobalt level went down to 34 and 17 μg/L at, respectively, 1 and 6 months follow-up. Chromium serum levels were at 50 and 40 μg/L at 1 and 6 months. Urinary cobalt level was at 483 μg/g of creatinine and urinary chromium level at 82 μg/L at 6 months. Standard X-rays at the six-month follow-up showed no radiological complications of the left hip revision (). Patient could walk without limping or crutches and the Harris Hip Score was 75/100.
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pmc-6369487-1
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A 59-year-old man presented in December 2017 an indurated lesion of the anal margin causing burning sensation, measuring 1 cm ().
The biopsy revealed moderately differentiated squamous cell carcinoma. Using immunohistochemistry, irregular/heterogenous positivity for p16 protein was observed ().
The detection of High Risk-HPV DNA (HPV 16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 59, 66, and 68) from the paraffin-embedded sample using the BD onclarity HPV assay (BD diagnostics, Sparks, USA) was negative [].
The tumor was classified cT1 and treated by radiotherapy until February 2018. In May 2018, after a period of complete response, the patient noted the reappearance of an indurated and painful area near the anal margin. The patient underwent excision in June 2018.
Macroscopically, an irregular and ulcerated lesion occupying the near totality of a mucous ellipse measuring 26x15 mm was observed. This lesion was covered by a white coat.
Microscopically, the tumor consisted of nests of invasive squamous cell carcinoma, moderately differentiated. Lateral margins were positive. Using immunohistochemistry, tumor was negative for p16 (clone ink4a E6H4, ready to use, Roche). Immunoreactivity of p53 (clone DO-7, 1:200, Dako Agilent) appeared continuous and limited to the periphery of invasive nests, with strong intensity (). The tumor was classified rpT2Nx.
Gene mutation testing was performed by NGS, as we have previously described [, ], with a panel of 50 genes described in . One mutation was found: G279fs∗4 (c.833.834insGAGTCGAAACTCCACGCACAAACACGGACAGGAC) frameshift mutation of the TP53 gene.
In addition, the detection of High Risk-HPV DNA was negative [].
Due to the positive margins and the classification, PET-CT was realized and was negative. Complementary resection was performed in July 2018.
Macroscopically, 2nd resection showed an ulcerated mucous ellipse.Microscopically, borders of the ulceration revealed a thickened epithelium with parakeratosis, elongated rete ridges, disorderly basal cell layer, prominent intercellular bridges, and mitosis. Using immunohistochemistry, just like in 1st resection, lesion was negative for p16. Immunoreactivity of p53 appeared limited to the basal and suprabasal layers of the epithelium, with weak to moderate intensity ().
According to all these pathological data, the diagnosis of differentiated-type intraepithelial neoplasia (DIN) was suggested.
Gene mutation testing was performed in this DIN-like lesion, but no mutation was found.
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pmc-6369490-1
|
A 21-year-old man with a past medical history of asthma presented with acute worsening shortness of breath overnight with no identifiable trigger. The patient had experienced this symptom for three weeks although less severe initially. He also stated that the symptom was accompanied by cough, chest tightness, and pain across the anterior chest but could not clearly describe the characteristics of the pain. The difficult breathing was worsened with lying flat. He denied any history of smoking. On initial presentation, his vital signs included a temperature of 36.7°C, a blood pressure of 119/83 mmHg, a heart rate of 105 beats/min, respiratory rate of 18 breaths/min, and an oxygen saturation of 97% on room air. The patient was 170.2 cm tall and weighed 57.2 kg and BMI of 19.79 kg/m2. Physical examination revealed a distressed and ill-appearing male. Cardiopulmonary examination was notable for tachycardia, tachypnea, and decreased breath sounds in both upper lung fields. Laboratory results showed mild leukocytosis with a white blood cell count of 12.9 × 109 cells/L, 72% neutrophils, and 16% lymphocytes. His hemoglobin level was 16.2 g/dl with a hematocrit of 48.6% and platelet count of 243 × 109 cells/L. The blood biochemical profiles were unremarkable. Chest X-ray (CXR) () showed bilateral large pneumothorax (>2cm) with minimal bilateral pleural effusions and significantly compressed mediastinum.
A diagnosis of SBPSP was made. Chest tubes were placed bilaterally with immediate improvement in breathing and tachycardia. The right- and left-sided chest tubes drained serosanguinous fluids, 5 ml and 10 ml, respectively. Patient's clinical condition continued to improve and a follow-up CXR immediately following the procedures () showed a decrease of pneumothorax in both sides. However, during the hospital course, the chest tubes continued to have an air leak and the follow-up CXR continued to demonstrate residual pneumothorax without full lungs expansion. Computed tomography (CT) scan without contrast of the chest revealed subpleural blebs in both of the upper lobes (). Due to continuous air leak without full lung expansion with conservative management, the patient was referred to thoracic surgery evaluation. One week later, our patient underwent bilateral VATS with apical bleb resection, bilateral pleurectomy, and bilateral doxycycline pleurodesis. Biopsy of the apical blebs and parietal pleura of both lungs showed fibrosis and granulation tissue, negative for any atypical cells suspicious for malignancy or Langerhans cell histiocytosis.
On the subsequent outpatient follow-up visit, the patient had been doing well six months following surgery with no recurrence of pneumothorax ().
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pmc-6369501-1
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A 83-year-old woman presented with a visual acuity of light perception in the left eye for four years. The patient underwent detailed ophthalmological examinations including slit-lamp biomicroscopy, intraocular pressure measurement (applanation tonometry), dilated fundus examination (+90 D), and B-scan ocular ultrasonography. On initial examination, visual acuity was 0.2 (Snellen equivalent) in the right eye and light perception was in the left eye. Slit-lamp biomicroscopy revealed left hard brown cataract and right pseudophakia. Specular microscopy was within acceptable limits (cell density >2000 cells/mm2, hexagonality >50%) in the left eye. Intraocular pressure measurements were normal bilaterally. Right fundus examination showed macular atrophic changes. However, dense cataract did not allow a detailed fundoscopic evaluation in the left eye. B-scan ocular ultrasonography revealed no retinal detachment or intraocular mass.
Phaco surgery was performed under retrobulbar anesthesia due to poor patient compliance. A 2.75 mm superior clear corneal incision was created, and anterior lens capsule was stained with trypan blue dye. Central part of the anterior lens capsule was punctured with a cystotome following OVD injection into the anterior chamber (AC). A continuous curvilinear capsulorhexis was created. Two corneal side port incisions were made using a 20-gauge blade two clock hours away from the superior corneal incision. After hydrodissection, central vertical groove was formed, and nucleus was cracked into two halves (Figures and ). Using the phaco needle, core nucleus (which is the rock-hard part of the lens) of the two halves was hollowed sideward in the capsular bag (using low vacuum (linear increment with an upper limit of 80 mmHg) and power (linear increment with an upper limit of 80% torsional amplitude) settings) (Figures and ). A dispersive OVD was injected into the AC to protect corneal endothelium. The nuclear halves were removed from the capsular bag using the phaco tip, and each half was pushed to one side on the iris plane with assistance of OVD (Figures and ). Capsular bag was inflated with OVD, and a foldable IOL was implanted (Figures and ). Thus, posterior capsule moved backwards and got fully secured. Then, nuclear halves were chopped into small pieces and removed as far as possible from the corneal endothelium, which is also protected by dispersive viscoelastic (Figures , , , and ). After nucleus removal, the remaining cortical material and OVD were aspirated with bimanual irrigation/aspiration (I/A). Corneal incisions were hydrated, and intraocular antibiotic injection was performed.
demonstrates the steps of the phaco surgery.
At first day postoperatively, biomicroscopic examination revealed normal findings except grade 2 corneal edema in the left eye. At 1-week control visit, corrected visual acuity was 0.7 (Snellen equivalent), cornea was clear, and minimal macular atrophic changes were present in her left eye. Postoperative control specular microscopy was planned at 1-month visit, whereas the patient was out of follow-up.
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pmc-6370013-1
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A 35-year-old woman who presented with a 5-day history of chest tightness and right leg pain was admitted to our emergency department on October 22, 2017. The patient had delivered (first pregnancy) 48 days prior and had an unremarkable medical history. Upon physical examination, dullness to percussion was noted over her right lower lung. Swelling, tenderness, and ecchymosis were present in the right medial thigh. The circumference of the right thigh was 53.5 cm, while that of the left thigh was 49 cm. Computed tomography angiography of the aorta showed a large amount of pleural effusion in the right thoracic cavity and partial right pulmonary collapse (see Fig. A). Under B-mode ultrasound guidance, thoracentesis was performed, and bloody pleural effusions were drained. Her white blood cell count was 17.9 (109/L), with 75.8% neutrophils; hemoglobin was 70 (g/L), and platelets were 238 (109/L). Prothrombin time (PT) was 15.20 s, and activated partial thromboplastin time (APTT) was 68.40 s. An APTT mixing study showed that her APTTs were 70.12 s, 30.45 s, and 60.40 s at 0, 1, and 2 h, respectively. Factor IX activity was 107.8 (%), factor XI activity was 66.9%, and factor VIII activity was 12.6%. The Bethesda assay showed a FVIII antibody titer of 7.4 Bethesda units (BUs). The diagnosis of AHA was confirmed.
The regimen for this patient included aPCC (10 U/kg intravenously 3 times daily), hFVIII (20 IU/kg intravenously twice daily), prednisone (1 mg/kg orally once daily), and plasma (400 mL intravenously once daily). Two weeks later, the ecchymosis in her medial thigh improved, and PT and APTT were 17.70 s and 20.30 s, respectively. FVIII activity was 127.30%, and the FVIII antibody titer was 0 BU. After prednisone was tapered to 10 mg orally once daily, the patient was discharged. At the 2-month follow-up visit, her pulmonary computed tomography revealed that the pleural hemorrhage had subsided (see Fig. B). Prednisone was withdrawn at a rate of 20% every 2 weeks. The patient is now undergoing 6-month follow-up and has shown no recurrence.
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pmc-6370060-1
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An 80-year-old man was diagnosed with squamous cell carcinoma of the left cheek area (Fig. ). We planned a wide excision with local flap coverage under local anesthesia. He underwent wide excision with a 4-mm safety margin, and the final defect size was 2.5 × 3 cm2 on the lateral subunit of the cheek unit in view of the facial aesthetic unit concept. We covered the defect with a 3 × 5.5 cm2-sized Ω-variant Type IIA KDPIF from the anterior side of the defect, in the light of the facial RSTL and the aesthetic unit concept. Both the insetting of the flap and the primary closure of the donor site were achieved without tension or drain placement. The flap survived completely without postoperative complications. The final appearance was evaluated after a 6-month follow-up period, and the patient was satisfied with the final outcome, providing a subjective satisfaction score of 8. The postoperative cosmetic outcome evaluated by 3 independent plastic surgeons was rated as good (2 raters) and excellent (1 rater).
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pmc-6370060-2
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An 82-year-old woman was diagnosed with a basal cell carcinoma in the left nasolabial fold area after a punch biopsy (Fig. ). We planned a wide excision with local flap coverage under local anesthesia. She underwent wide excision with a 4-mm safety margin and the final defect size was 2 × 3 cm2 on the medial subunit of the cheek unit, in view of the facial aesthetic unit concept. We covered the defect with a 2 × 5.5 cm2-sized Type IIA KDPIF from the upper-lateral side of the defect, in the light of the facial RSTL and the aesthetic unit concept. Both the insetting of the flap and the primary closure of the donor site were achieved without tension or drain placement. The flap survived completely without postoperative complications. No tumor recurrence was observed during the 6-month follow-up period, and the patient was satisfied with the final outcome, providing a subjective satisfaction score of 9. The postoperative cosmetic outcome evaluated by 3 independent plastic surgeons was rated as excellent (2 raters) and good (1 rater).
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pmc-6370060-3
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An 81-year-old woman was diagnosed with squamous cell carcinoma in the temporal region after a punch biopsy (Fig. ). We planned a wide excision with local flap coverage under local anesthesia. She underwent wide excision with a 5-mm safety margin and the final defect size was 3 × 3.5 cm2 on the lateral subunit of the forehead unit in view of the facial aesthetic unit concept. We covered the defect with an Ω-variant Type III KDPIF, wherein the sizes of the medial and lateral flaps were 1.5 × 5 cm2 and 2 × 6 cm2, respectively, in light of the facial RSTL and the aesthetic subunit concept. Both the insetting of the flap and the primary closure of the donor site were achieved without tension or drain placement. The flap survived completely without postoperative complications. No tumor recurrence was observed during the 10-month follow-up period, and the patient was satisfied with the final outcome, providing a subjective satisfaction score of 9. The postoperative cosmetic outcome evaluated by 3 independent plastic surgeons was rated as excellent (2 raters) and good (1 rater).
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pmc-6370060-4
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A 62-year-old man was diagnosed with basal cell carcinoma of the nose after a punch biopsy (Fig. ). We planned a wide excision with local flap coverage under general anesthesia. He underwent wide excision with a 4-mm safety margin and the final defect size was 3 × 2.5 cm2 on the dorsal subunit of the nose, in view of the facial aesthetic unit concept. We covered the defect with an Ω-variant Type III KDPIF, with a flap size of 1.5 × 3.5 cm2 from each dorsal side wall subunit, in light of the facial RSTL and the aesthetic unit concept. Both the insetting of the flap and the primary closure of the donor site were achieved without tension or drain placement. The flap survived completely without postoperative complications. No tumor recurrence was observed during the 12-month follow-up period, and the patient was satisfied with the final outcome, providing a subjective satisfaction score of 10. The postoperative cosmetic outcome evaluated by 3 independent plastic surgeons was rated as excellent by all the raters.
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pmc-6370112-1
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A 39-year-old woman was scheduled to undergo split thickness skin grafts as part of a reconstructive program following extensive burns. She was burned in a house fire 2 years previously, when she suffered 93% total body surface area burns, with a burn index of 85. Her history was significant for having undergone 39 reconstructive operations over 2 years. Her neck was reconstructed using a graft from the latissimus dorsi, and both femoral veins were occluded due to multiple accesses and indwelling catheters. A subclavian venous catheter had been inserted once before under ultrasound guidance. Physical exam showed her weight was 49 kg, and her height was 155 cm. Most of her body was covered by hard contracted skin. There were no peripheral veins evident on inspection.
To obtain venous access for this operation, we searched for veins using a linear probe ultrasound device (6–15 MHz, SonoSite Edge, FUJIFILM SonoSite, Inc., Washington, USA) on the chest and arm. This revealed accessible veins, including the subclavian and axillary veins on the anterior chest, and the brachial vein of the right arm. An indwelling catheter in the right brachial vein was considered as the first choice to avoid possible complications of using the subclavian or axillary veins such as pneumothorax or hemothorax. The right brachial vein measured 3 mm by ultrasound imaging.
The right arm had restricted motion range due to extensive contractures. She was positioned in the right semilateral position to gain easy access to the medial side of the arm by supination and abduction. A pillow was placed behind her back to maintain this position. The skin of the arm was prepped with 1% chlorhexidine alcohol solution and covered with a sterile drape. The ultrasound probe was covered with a sterile plastic probe cover. The indwelling venous catheter was placed using sterile barrier precautions. Local anesthetic (5 mL of 1% lidocaine) was injected, and a 20G catheter-over-the needle (48 mm) was inserted. Ultrasound-guided venous catheterization was performed. To access the brachial vein, we used the short-axis out-of-plane approach to avoid mechanical complications of unanticipated artery or peripheral nerve injuries, and then used the long-axis in-plane approach to penetrate the anterior vein wall and cannulate the vein. After inserting the cannula, a guide wire was placed through the cannula using the modified Seldinger technique. We placed a single lumen polyurethane catheter (3 Fr., SMAC Plus, Nippon Covidien, Japan), a commercially available central venous catheter (there is no commercially available midline catheter in Japan). Successful insertion of the catheter was confirmed with ultrasound imaging (Fig. ). The catheter entered the vein 6 cm from the skin entry site (Fig. ). After placing the catheter, we checked venous flow using color Doppler imaging (Fig. ).
Surgery was performed the following day with no adverse events. A split-thickness skin graft was harvested from the head, and grafted to the neck. The skin graft became infected several days later and the midline catheter was used for administration of antibiotics. A second skin graft was performed again postadmission day 16 and the catheter removed on postadmission day 24. She was discharged without complications.
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pmc-6370124-1
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A 72-year-old woman was referred to our emergency department from a dialysis clinic because of general discomfort and decreased blood pressure within 30 min after hemodialysis was initiated. Her status on arrival was as follows: blood pressure, 85/54 mmHg; heart rate, 122 bpm; and body temperature, 35.8°C. Her consciousness was clear. She complained of general discomfort, but no chest or abdominal pain was observed. Physical examination revealed a pale conjunctiva and soft abdomen. No bloody/tarry stool passage was observed. She had diabetes mellitus and uremia and underwent regular hemodialysis. She had undergone surgery for a left forearm arteriovenous pseudoaneurysm 15 days before, and a temporary hemodialysis catheter was inserted via the left femoral vein on the day after surgery. She had undergone hemodialysis via a left femoral catheter several times without any problems until this time. Blood examination revealed the following values: white blood cells, 27,580/μL; hemoglobin, 7.2 g/dL; platelets, 269,000/μL; glucose, 347 mg/dl; glutamic oxaloacetic transaminase/glutamic pyruvic transaminase, 29/10 IU/L; C-reactive protein, 13.36 mg/L; sodium, 136 mmol/L; potassium, 5.9 mmol/L; creatine phosphokinase, 78 IU/L; creatine kinase-muscle/brain, 5.3 ng/mL; troponin I, 0.039 ng/mL; pH, 7.114; and HCO3−, 13.8 mmol/L. Her blood pressure recovered after normal saline hydration and packed red blood cell transfusion. Owing to the incomplete regular hemodialysis and hyperkalemia, she was referred to the hemodialysis department to undergo hemodialysis again. Reduced blood pressure and change in consciousness developed soon after hemodialysis was initiated. She was intubated and referred back to the emergency department. Subsequently, central venous catheter insertion, fluid resuscitation, and vasopressor administration were performed. Blood examinations were repeated. Computed tomography was performed to exclude vascular emergency and revealed a double-lumen catheter tip protruding from the left iliac vein and hematoma in the left retroperitoneal space and pelvic cavity (Fig. ). Blood examination results revealed the following values: white blood cells, 26,840/μL; hemoglobin, 3.9 g/dL; platelets, 182,000/μL; pH, 7.018; HCO3−, 10.2 mmol/L; lipase 64 U/L; creatine phosphokinase, 152 IU/L; creatine kinase-muscle/brain, 9.6 ng/mL; troponin I, 0.106 ng/mL; and lactate, 11.5 mmol/L. She was then admitted to the intensive care unit for further care. The left femoral hemodialysis catheter was removed, and follow-up computed tomography revealed resolution of the retroperitoneal space hematoma. She was transferred to the ordinary ward 18 days later with stable hemodynamic status. Unfortunately, the patient developed hospital-acquired pneumonia and suspected arteriovenous shunt infection, and died from respiratory failure and sepsis on the 34th day after being transferred to our hospital.
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pmc-6370162-1
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A 21-year-old Chinese male patient has a history of epilepsy for 10 years. He presented with complex partial seizure (CPS) and secondary tonic-clonic seizure (sGTCS) with a frequency of 3–4 times a week. CPS was described as transient consciousness-lost companied with oropharynx automatism. He insisted on antiepileptic therapy of Oxcarbazepine (1200 mg/day) without improvement. In the past, he has given up the therapy of topiramate and valproic acid due to unsatisfactory effect. After this admission, his mother planned to make a schedule for epilepsy surgery. However, within 2 weeks recently, the patient developed another new seizure type of unilateral or bilateral-shoulder jerks frequently. In admission, physical examination was negative, then scalp EEG was performed, but with normal result. No synchronized, Jerk-locked spike or spike-slow wave has been found (Fig. ). Magnetic resonance imaging (MRI) showed focal schizencephaly in right temporal-parietal lobe (Fig. ). Mini-Mental State Examination (MMSE) score was 30/30. According to the patient's clinical seizure, we made a suspected diagnosis of myoclonic seizure but lacking objective evidence, and his MRI changes, previous seizure types should be considered carefully. In this situation, we need to explore another electrophysiological technique to help begin diagnosis. And we chose the Jerk-locked back-averaging technique, which employed EEG electrodes and EMG electrodes for the upper limbs (according to the 10/20 EEG system) and correlated averaged EEG responses with EMG bursts used as a trigger. In other words, we named the bilateral-shoulder jerks as EMG trigger point, made 15 times averaged superposition of Jerk-locked EEG before the trigger point. In order to ensure the data reliable, we applied 64-channel Event-related evoked potential machine produced by Neuroscan Compumedics Limited with a sampling rate of 1000 Hz. The filter setting was from 0.3 Hz to 70 Hz. The patient was tested in an electrically shielded room. During the data-processing, baseline correction, artifact rejection, filter settings, segmentation, and simple averaging were performed, respectively. After the examination, a time-locked, evoked negative cortical averaging potential that precedes the EMG activity has been found (Fig. ), and was shown as symmetrical bilaterally in both hemisphere (Fig. ), which was different from scalp-EEG (Fig. ). The evoked EEG-potential began at 50 ms prior to the movement onset with amplitude of 6.5 and 7.5 μV. Such results may indicate: (1) bilateral-shoulder jerks were cortical myoclonus and may be myoclonic seizure probably; (2) such myoclonic seizure may be generalized. In order to verify such assumption, we named the unilateral-shoulder jerks as EMG trigger point, made the same analysis again, obtained the same result fortunately. Combining the suspected seizure with electrophysiological results described above, we made a clinical diagnosis of myoclonic epilepsy (generalized seizure). After the additional therapy of Levetiracetam (1.0 g/day) for 4 days, such bilateral-shoulder jerks have disappeared and sGTCS seizures have also decreased gradually. After the communication with the patient's family, they decided to give up the epilepsy surgery and insisted on antiepileptic drug. In the following visit, the patient had a better prognosis with low seizure frequency.
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pmc-6370563-1
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Case 1: A 20-year-old man with NF1 visited our clinic hoping to reduce the enlargement of his left lower limb. Examination confirmed a soft and drooping tumour encircling the thigh (A). DPN was diagnosed, and we planned a volume reduction surgery. To control haemorrhage, preoperative TAE to the superior gluteal artery and the deep femoral artery was performed with Serescue™ (Astellas Pharma Inc., Tokyo, Japan) by interventional radiologists.
Under general anaesthesia, debulking surgery was performed. The skin of the lateral thigh turned purple, probably because of ischaemia following TAE. Enclosing the discolouration area, a 3-kg tumour was resected above the fascia level. The tumour’s pedunculation and flexibility enabled us to temporarily ligate at the tumour’s base (B). The total amount of bleeding was 500 mL; transfusion was not needed. Postoperative course was satisfactory, and the patient was discharged 17 days after surgery. One year after the surgery, he attends the hospital on foot (C).
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pmc-6370563-2
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Case 2: A 40-year-old man, diagnosed with NF1 in his childhood, was referred to our department with complaints of walking difficulty. His right lower limb had begun to enlarge since his 30 s. Eventually, he had become unable to walk by himself because of his huge and heavy leg (A). We established that his symptom was caused by DPN in his leg, characterised by an elephant-like pigmented cutaneous tumour.
We planned serial excision with the resection area being from the buttock to the posterior thigh. Before the procedure, interventional radiologists performed TAE with Serescue™ to the feeding vessels originated from the superior and inferior gluteal arteries and the deep femoral artery of the affected side. An occlusion balloon was placed within the right internal iliac artery (IIA) to prevent uncontrollable critical haemorrhage.
Under general anaesthesia, we resected the tumour in the posterior thigh towards the head (A). In the buttock area, torrential bleeding from the wound occurred. The occlusion balloon in the IIA was inflated, and the bleeding was reduced temporarily. The total intraoperative blood loss was 4970 mL. We transfused 1960 mL of concentrated red cells and 1680 mL of fresh frozen plasma. The resected tumour weighed 5 kg with confirmed porous particles occluded in the arteries (C).
To prevent further bleeding from the wound edge, additional TAE was performed to the superior and inferior gluteal arteries immediately after surgery. The wound edge became gradually necrotic and dehiscent (B). Finally, we performed debridement and wound closure operation 4 weeks after the first surgery.
The second debulking surgery in the lower leg was conducted 4 months after the first debulking surgery. At this time, we could apply a tourniquet to the thigh area. The total blood loss during the second surgery was 420 mL, and TAE was not conducted. Wound healing was not delayed. The patient left the hospital walking by himself (C).
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pmc-6370567-1
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A 64-year-old Saudi female of the Eastern Province was referred to our institute with a several-month history of unilateral progressive nasal obstruction and recurrent episodes of epistaxis. There was no history of anosmia, nasal discharge, nasal congestion, sneezing, facial pain, or headache. Her past medical history included a known case of hypertension and diabetes. Her family history was otherwise unremarkable.
A rigid endoscopic examination of the nose showed a large, left-sided nasal polypoidal mass with smooth overlying mucosa that occluded the whole left nasal cavity (). The remaining ear, nose, throat, head, and neck examinations were normal, and there was no palpable lymphadenopathy.
A CT scan of the paranasal sinuses with contrast showed a left anterior lobulated nasal mass of undetermined origin at the level of the cartilaginous part of the nasal septum with mild heterogeneous post IV contrast enhancement and bone remodeling. There was no extension to the paranasal sinuses, and no obvious bone invasion was observed ().
Endonasal endoscopic excision of the left nasal cavity mass was performed under general anesthesia. Intraoperative findings demonstrated a semifirm vascular mass attached to the anterior face of the middle turbinate and septum at the same level. The mass was excised completely from its attachment, and the pedicle was cauterized. Histological examination of the mass revealed three fragments of soft to firm tan-gray tissue, the largest measuring 1.8 × 1.6 × 0.6 cm, and the smallest measuring 0.8 × 0.6 × 0.5 cm (). The lesion was positive for vimentin and S100 and negative for desmin, NSE, and SMA. The final histopathology confirmed the diagnosis of schwannoma of the nasal septum.
The patient has received regular follow-up care for three years with no recurrence.
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pmc-6370578-1
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Patient 1: A 25-year-old male underwent total gastrectomy including a D3 lymph node dissection for advanced gastric carcinoma, preoperatively staged as T3 (SS), N0, M0, cStage II. Peritoneal lavage showed poorly differentiated adenocarcinoma (Class V). Pathological diagnosis showed the depth of invasion was T2 (invading the muscularis propria). He received adjuvant chemotherapy, biweekly paclitaxel for one year and oral administration of S-1 (TS-1; tegafur, gimeracil, oteracil potassium). At 13 years after resection, there is no evidence of recurrence.
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pmc-6370578-2
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Patient 2: A 63-year-old male underwent distal gastrectomy with D2 lymph node dissection for a type 0-IIc + IIa tumor, preoperatively staged as T1b(SM), N0, M0, cStage IA. Pathological diagnosis showed the depth of invasion was T2 (invading the muscularis propria). Although he began receiving adjuvant chemotherapy, a recurrence was seen on computed tomography scan in the mediastinal and para-aortic areas four months postoperatively. He died 21 months later.
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pmc-6370578-3
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Patient 3: A 69-year-old male underwent total gastrectomy with D2 lymph node dissection for two separate primary gastric carcinomas. Both tumors invaded the muscularis propria (T2) and had lymphatic invasion. He has received adjuvant chemotherapy with docetaxel/S-1 and is free of recurrence at 14 months postoperatively.
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pmc-6370578-4
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Patient 4: An 83-year-old male underwent distal gastrectomy with D2 lymph node dissection for a type III tumor, preoperatively staged as T3(SS), N1, M0, cStage IIb. Pathology examination showed the depth of tumor invasion as T1b(SM2) with significant lymphatic and vascular invasions. There were tumor cells in the lymphatics of the gallbladder, which was incidentally resected for cholelithiasis. He is receiving S-1 monotherapy.
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pmc-6370588-1
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A 68-year-old male presented at an external hospital with painless obstructive jaundice and a loss of weight of 5 kg over the last two months. Except for a Billroth II gastric resection in 2011 for ulcer disease, no previous abdominal surgery was noted. Secondary diagnoses included a smoking history of 10 pack years, arterial hypertension, and a mild type 2 diabetes treated with metformin. The diabetes lasted for several years and was not new onset. Using contrast-enhanced multi-phase computed tomography (CT), a tumor in the head of the pancreas associated with a distal biliary obstruction was diagnosed suspicious for pancreatic cancer (). The Ca 19-9 serum level was extensively elevated with 2100 U/ml. Since there were no imaging signs of distant metastasis nor signs of local irresectability, the patient underwent surgical exploration on September 18, 2017 at the external hospital. Due to questionable infiltration of the caval vein and suspicious lymphadenopathy, the exploration was broken off. Histology of a sample biopsy of the hepatoduodenal ligament revealed fragments of a moderately differentiated adenocarcinoma. Postoperatively, due to persistent jaundice, the patient received percutaneous transhepatic bile duct drainage (PTCD) for combined external/internal drainage which was changed on October 6, 2017 to a self-expanding metal stent (SEMS) for permanent recanalization of the bile duct (). With regard to the suspected locally advanced ductal adenocarcinoma of the pancreas, a palliative systemic chemotherapy was anticipated.
Subsequently, the patient presented at the Katharinenhospital Stuttgart as a referral center for hepatobiliary and pancreatic surgery seeking a second medical opinion. The patient again underwent staging, which revealed no signs of liver or lung metastasis (). Upon an interdisciplinary tumor board decision, the patient was surgically re-explored on October 24, 2017. Intraoperatively, no contraindications against pancreatic head resection, and particularly no infiltration of the caval or portal vein were found. Thus, the patient underwent pylorus-preserving duodenopancreatectomy. The early post-operative course was uneventful. However, on post-operative day 10, the patient had to be reexplored due to an ischemic perforation of the transverse colon. A discontinuity resection of the transverse colon was performed with preparation of a colostomy and a long Hartmann stump. The further clinical course was uncomplicated and the patient was discharged on postoperative day 30 in good health condition.
Final histopathology of the surgical specimen surprisingly revealed a pT3, pN0 (0/12), R0, G2 ductal adenocarcinoma arising in the background of an ITPN (). The tumor had a maximum diameter of 5.9 cm and showed wide infiltration of the duodenum. All resection margins were clear and lymph nodes were free of metastasis. To rule out a neuroendocrine tumor, immunohistochemistry was performed using Cytoceratin-7, Chromogranin, Synaptophysin, and Trypsin (). Since the tumor did not show expression of these markers, a neuroendocrine tumor and an acinus cell differentiation were excluded. According to the postoperative tumor board decision, adjuvant systemic chemotherapy with Gemcitabine and Xeloda was administered. The therapy was well tolerated by the patient and no severe complications were observed.
A follow-up re-staging CT in March 2018 revealed no signs of recurrent disease or distant metastasis. The patient therefore underwent restoration of intestinal continuity by an ascendodescendostomy. The postoperative course was uneventful and the patient was discharged on postoperative day six. To date the patient is in a good clinical condition without signs of recurrent disease and with a non-insulin dependent diabetes mellitus treated orally with metformin as preexisting prior to surgery.
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pmc-6370615-1
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The patient was a 12-year-old male who had his first seizure attack at the age of 10, and had four types of epileptic seizures. The first type of seizure was tonic axial seizures characterized by flexion of the neck and body and the extension of four extremities for several seconds. The tonic seizures could last several seconds. This type of seizure occurred predominantly at night and the frequency was about 4–5 times per week. The second type was atypical absence seizures, which manifested as a sudden loss of consciousness and the resuming of normal activity right after the seizure. This type of seizure could last about 15 s and the frequency was 4–5 times per week. The third type was the myoclonic seizure, presented as prominent myoclonic jerks of bilateral upper limbs. The frequency of this type of seizures was 2–3 times per day. The last type of seizure was the generalized tonic-clonic seizure, which could last about 5 min. The mean frequency of this type of seizure was <1 time per week.
The patient was a full-term infant with no history of perinatal asphyxia, head injury, encephalitis, and febrile convulsions. His family history was unremarkable. He had a mild degree of intellectual impairment and learning disability after the onset of the disease. The physical examinations were normal. Auxiliary examinations, including blood routine examination, serum biochemical examination, thyroid function, autoimmunity antibody, and blood ammonia, were normal. The electrocardiogram was normal and the QT interval (QTc) was 372 ms. There was no lesion on the brain MRI. The Wechsler Intelligence Scale showed a borderline cognition impairment. The electroencephalogram (EEG) before treatment showed that there was a large number of 3 to 5 Hz slow waves with middle and high amplitude in the anterior region in the background. Fast rhythms bursts of 16 to 20 Hz and multiple-spike-and-slow-waves of 0.5 to 1 Hz were observed during the sleep period. Slow spike-and-slow-waves of 1.5 to 2.5 Hz were observed during awake time ().
The patient was diagnosed with Lennox-Gastaut syndrome after considering his multiple types of epileptic seizures, mental retardation, and typical electroencephalographic features. He was refractory to a multiple anti-epileptic drugs treatment, including sodium valproate (8 mg/kg/day), levetiracetam (50 mg/kg/day), clonazepam (0.0375 mg/kg/day), topiramate (3.75 mg/kg/day), and lamotrigine (2.5 mg/kg/day). Whole exome sequencing (WES) identified a novel heterozygous KCNT1 mutation (chr9:138649026; c.625C>T; p.Arg209Cys) inherited from his father. This missense mutation was highly likely to cause the dysfunction of the KCNT1 channel and led to a gain-of-function phenotype. This alteration had not been previously reported and was not found in the ExAC database (), and was predicted to be likely pathogenic.
This study was approved by the human research ethic committees of Xuanwu hospital capital medical university. Written informed consent was obtained from all participants and guardians of minors for the quinidine therapy and the publication of this study. Additive quinidine therapy to our patient was initiated at 12 years of age. The doses of the above anticonvulsants remained unchanged. In the month before quinidine therapy, the patient had 16 tonic seizures, 12 atypical absence seizures, 10 myoclonic seizures, and 1 generalized tonic-clonic seizures.
After admission, the quinidine therapy was initiated with 5 mg/kg/day in 3 divided doses under electrocardiographic (ECG) monitoring. The QTc ranged from 361 to 415 ms, with an average of 378 ms (the normal limit of QTc is within 450 ms). After 1 month of treatment, the dose of quinidine was titrated to 10 mg/kg/day and he had 13 tonic seizures during this month. The dose was maintained during the following 2 months. QTc was in normal range (391–436 ms). There was also no other adverse effect of quinidine. At the fourth month, the dose of quinidine was increased to 13.75 mg/kg/day in 3 divided doses. The frequency of tonic seizures ranged between 4 and 6 times per month. The mean QTc interval was 383 ms. As no adverse effects were experienced, the dose of quinidine was maintained to 13.75 mg/kg/day during the following 4 months. The patient had 4 tonic seizures per month. The frequency of tonic seizures subsided by 75% (), whereas the frequency of the other types of seizures was not reduced significantly.
Video EEG (VEEG) was performed regularly during treatment and the number of epileptic discharges was counted by four expert technicians who had not seen the patient's clinical information. One slow spike-and-wave complex or one episode of paroxysmal fast rhythms in EEGs were counted as one epileptiform discharge. 1323 epileptiform discharges were recorded during a 24-h video EEG before the treatment of quinidine. We subsequently applied the 24-h VEEG every 3 months to evaluated the efficacy of the quinidine treatment. The total number of epileptic discharges was 512, 652, and 598 in the next 3, 6, and 9 months, respectively. The epileptiform discharges decreased by 54.80% ().
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pmc-6370624-1
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“Greg” is a male in his twenties and was a Sergeant (SGT) in the United States Army. He was an unrestrained passenger in a Humvee rollover in 2014. He suffered intracranial hemorrhages, presented unresponsive, and was intubated. Greg was medevacked, stabilized, and returned to the United States for ongoing treatment. He was referred to music therapy by a nurse case manager for cognitive deficits due to severe TBI. Greg received a music therapy assessment in January 2016 and received weekly individual and small group sessions for 15-months as part of outpatient treatment.
Upon initial music therapy assessment, it was determined that Greg was experiencing expressive speech issues in addition to memory and coordination deficits. The music therapist consulted with speech therapy to create integrative treatment approaches. Music therapy sessions were designed to assist his breathing, speech fluency, and rhythmic phrasing. A few months into music therapy, Greg and a fellow service member were working on a song that supported shared speech and cognition goals. The music therapist determined that it was appropriate to offer performance as an opportunity to: (1) encourage active participation in treatment, (2) enhance self-practice, and (3) engage with an audience while applying skills learned in music therapy. Greg and his peer were working on the song, “We Didn’t Start the Fire” by Billy Joel and opted to perform it as a duet. In his interview “Greg” stated, “I chose that song, because I knew for a fact that it would help with my speech...and it helped with my memory.”
Therapeutic singing, rhythmic cueing, and vocal prosody exercises were used to address hypernasality, slurred speech, fluidity, intonation, and articulation. Greg stated, “[Music therapy] helped my pronunciation of words, [performance] pushed me harder in music therapy, which in turn pushed my brain harder to focus on my words and my motor functions.” He furthermore acknowledged how performance had helped him address vocal and cognitive changes experienced since his accident: “My voice changed since I got hurt...I didn’t really want to talk to people... With music therapy, I learned not to be ashamed of my voice, and that it won’t get better if I don’t talk. It helped me with my memory because I had to remember lyrics, chord changes, and everything else...it made a great impact overall.” Through performance in music therapy, Greg gained insight into personal struggles and was motivated to continue engaging in independent practice and performance. He shared, “Watching expressions of people I don’t even know...seeing and feeling that someone you’ve never met before [is] showing happiness and joy from your performance…that is what is really empowering.”
Greg participated in monthly performances as a therapeutic tool to provide awareness of his successes in rehabilitation goals. Eventually, he was able to simultaneously sing and play, which is a high-level cognitive process and an ongoing goal. He recounts, “Between music therapy sessions, I was rehearsing. Every time I got stuck on a word, I went back to my room and [would] say it, and my speech got clearer. It made me feel amazing after [performing] because, honestly I noticed every time I did something, people would be amazed.” Performance supported Greg in changing from an internal focus to being able to communicate with others using music. This was observed by a shift in song selections from those that solely challenged his speaking/singing ability to songs that he connected with on a deeper level. Intentional song selection eventually led to Greg writing original compositions that reflected his belief systems and included messages to inspire himself and others. He spoke about the emotional benefits and comfort that he experienced from performance in music therapy, “With music, that’s your friend, your therapist, and everything beyond, and it doesn’t have anything bad to say about you ever.”
When Greg started music therapy, it was difficult and uncomfortable for him to speak on command or initiate conversations. Through the process of preparing for performances, which also included verbal song introductions, he continued to make clinical gains post-music therapy treatment, including speaking at his retirement ceremony and national public performances. Greg medically retired in May 2017 and is currently attending college with aspirations to become a motivational speaker. He reflected about his experiences performing in music therapy, “[Performance] worked my body harder. If I did not have music therapy, I would not be doing as well as I am today. I [still] incorporate what music expresses in my life.”
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pmc-6370624-2
|
“Chad” is a male in his thirties and was a Staff Sergeant (SSgt) in the United States Marine Corps. He served multiple combat deployments over his 13 years of service, during which he experienced blast exposure. Upon homecoming, Chad was diagnosed with mild TBI, chronic PTSD, and other combat-related psychological health concerns. He initially engaged in individual and group music therapy through the intensive outpatient program. Chad continued music therapy for 1 year post-IOP, in weekly individual sessions.
Chad’s music therapy goals addressed pervasive symptoms of chronic PTSD, as well as mild TBI issues of cognition, emotional regulation, and expression. Music therapy sessions incorporated various interactive experiences (e.g., active music making, lyric analysis, songwriting) to assist him in processing his military service and positively reframing self-perception and identity. Chad often selected songs by preferred artists to express things that were difficult to communicate prior to music therapy. He recounts, “Something about music made it easier to talk about issues that were bothering me. We spoke about issues that I hadn’t even discussed with my [psycho]therapist at that point.” After 8 months of individual sessions, Chad opted to perform a song at a Creative Arts Café that he had been preparing for his wedding. The integration of personally meaningful music into music therapy motivated his engagement in performance. Lyric analysis and songwriting facilitated emotional expression, as Chad modified songs to reflect his values. He shared, “I was able to add a spin on the song from my perspective, with help from the music therapist, which involved changing lyrics at the end, “Mercy,” look what’s become of us/One by one we turned it around/Maybe carry on just a little bit longer/God’s gonna give us what we need” (adapted from “Mercy” ).
A song of particular significance that Chad subsequently performed was an arrangement of “Walls” by Kings of Leon. He reflected on the song’s symbolism,” ‘When the walls come down,’ that’s how I felt music therapy was helping me...my walls coming down.” Chad’s musical engagement often inspired feelings of hope and reduction of personal guilt as reflected in his comment, “I feel like singing or even whistling means that there is some kind of happiness inside me, and it’s not all dark...Music therapy is like God answering my prayers. A light in the darkness, and a reassurance that while I have experienced horror, I am not the cause of it all.” He shared how performing helped him reduce isolation, “Performances can help to reconnect with people and even give others a chance to understand me more.” The reciprocal experience of self-understanding while feeling “heard” by the audience allowed for continuous progression in treatment.
As Chad began preparing for his transition from active duty, he transferred skills developed through performance to continue growing in his life and relationships. His evolving interpretations of songs he performed in music therapy reflected his progression in treatment. For example, when he first used “Mercy” in sessions, the meaning was focused on in-the-moment needs, specifically symptom management. It progressed to address broader areas, namely humanization and becoming a better partner. Ultimately, Chad sang and played bass and percussion in a multimedia project of “Mercy” that he presented this to his wife, family, and friends at his wedding. He speaks about how the spirituality of the song aligned with his transformation in recovery, “The first line in the song is, “Don’t give up”. Even though I felt like I had so many times, I didn’t give up, and to this day I am alive and not giving up. It was a sad song for me when the demons were in control, but God transformed the song into healing when it took on a new meaning for my wife and I, when we listened to it together. It transformed again as we grew as a couple, and we both went through really hard times along the path to recovery. [My wife] stayed with me through everything...there was no way I could let this song not be a part of our wedding day and music therapy was the reason it became a part of it.” Chad medically retired in July 2017.
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pmc-6370629-1
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Our present study included a Han Chinese family. The proband is a 30-year old man of non-consanguineous Chinese parents (). Proband (II-1) was clinically diagnosed with NSHL. Proband’s younger brother (II-2) also identified and diagnosed with NSHL. Proband’s father (I-1) and mother (I-2) are phenotypically normal. Clinical diagnosis has been done at Gansu Provincial Maternity and Child-care Hospital, LanZhou, China.
Clinical diagnosis of the proband and proband’s younger brother has been done on the basis of complete medical history, family history and detailed physical examination. Proband and his younger brother’s auditory test has been performed with otoscopic examination and pure tone audiometry (PTA) followed by the brainstem evoked response audiometry (BERA). In addition, hearing threshold of proband and his younger brother were evaluated or estimated by the air-conduction pure-tone average thresholds ranging from 250 to 8000 Hz. However, PTA, BERA and hearing threshold were also evaluated for proband’s father and mother and no abnormality was found.
In PTA test, hearing level was categorized into five groups; viz., normal (<20 dB), mild (20–40 dB), moderate (41–70 dB), severe (71–90 dB), and profound (>90dB). Here, hearing threshold was calculated based on the average of the right and left ears. In order to understand the structural abnormality of the temporal bone, computerized tomography (CT) scan was performed. Vestibular function analysis was done by caloric stimulation.
Proband and his family members denied to have any previous potential causes of acquired hearing loss. In addition, audiological history of this family has been obtained to understand the age of onset, gradual progression rate and presence of other related audiological symptoms. Four family members (proband, proband’s younger brother and their parents) underwent a clinical otorhinolaryngological examination. Moreover, tympanogram was performed and middle-ear associated causes of (HL) were excluded. In addition, other ophthalmological and pedo-neurological examinations was performed.
This study was approved by the ethics committee of the Gansu Provincial Maternity and Child-care Hospital, LanZhou, 730050, China, in accordance with the recommendations of the Declaration of Helsinki. Written informed consent has been obtained from all the participant of this study in accordance with the Declaration of Helsinki.
The proband (II-1, 30-year old) was presented with bilateral hearing loss and admitted to our hospital. Proband’s detailed and comprehensive medical report showed that there is no abnormality in vestibular organs. Caloric stimulation test also supported it. In contrast with other NSHL cases, here the proband’s speech perception ability was not impaired. PTA test result revealed that the hearing perception level were 60, 80, 90, 95, and 100 dB for the right ear and 70, 80, 85, 95, and 100 dB for left ear for stimulation at 0.125, 0.25, 0.5, 1, and 2 kHz frequencies, respectively, (). Computed tomography scan revealed no abnormality in medial and inner ear with normal temporal bone.
The proband’s younger brother (II-2, 25-year old) was presented with bilateral hearing loss and admitted to our hospital. He was not identified with any abnormality in vestibular organs. His speech perception ability was quite well. The PTA test for proband’s younger brother (II-2) revealed that the hearing threshold 55, 60, 85, 95, and 100 dB for the right ear and 60, 75, 85, 95, and 100 dB on the left ear at 0.25, 0.5, 1, 2, and 4 kHz frequency range, respectively, (). The CT scan of temporal bones was normal.
Both these patients are clinically diagnosed with (NSHL). The patient’s parents are phenotypically normal ().
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pmc-6370643-1
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Patient 1 was a 2-year-old girl with a history of perinatal hypoglycemia and a 1.5-year history of ES. No other types of seizure were noted. MRI revealed bilateral parietal–occipital encephalomalacia. Interictal scalp EEG revealed sharp waves mainly over the bilateral parietal–occipital regions . Video-EEG captured 40 episodes of ES, which presented as blinking accompanied by rapid jittering throughout the body, with ictal EEG showing generalized high-voltage slow waves followed by a diffuse electro-decrement with superimposed fast activity [i.e., typical ictal EEG pattern for ES ()].
Mean seizure frequency during the baseline period was 35.90 ± 23.95 times per day, in spite of taking clonazepam, sodium valproate, lamotrigine, and topiramate. She underwent one block of tDCS treatment at 1 mA. A current intensity of 1 mA was used due to her age, however all other tDCS parameters remained the same as mentioned above. During the treatment period and the first month of follow-up, mean seizure frequency was 44.23 ± 22.36 and 55.33 ± 35.56 times per day, respectively. She remained seizure free throughout the next 3 months of follow-up. Mean seizure frequency for Patient 1, who was classified as a positive responder, is shown in .
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pmc-6370643-2
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Patient 2 was a 3-year-old girl with a 6-month history of ES. MRI revealed no evidence of lesions. Scalp EEG revealed sharp and slow wave complexes mainly over the bilateral parietal–occipital–posterior temporal regions . Two types of seizure were captured by ictal video-EEG: (a) 77 episodes of ES, which presented as nodding accompanied by rapid lifting of the upper limbs, with a typical ictal EEG pattern for ES ; (b) one episode of ES followed by a tonic seizure, which presented as sudden nodding, lifting, and stiffening of the upper limbs for several seconds, with EEG showing a spike rhythm following a typical ES pattern .
Mean seizure frequency at baseline was 100.33 ± 18.44 times per day. She was taking sodium valproate, clonazepam, topiramate, lamotrigine, and levetiracetam. The patient underwent three blocks of tDCS treatment at 2 mA. Mean seizure frequency during the first, second, and third months of follow-up was 52.09 ± 24.73, 14.17 ± 8.77, and 26.12 ± 9.23 times per day, respectively . As Patient 2 experienced a more than 50% reduction in seizure frequency for 12 months, she was defined as a positive responder.
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pmc-6370643-3
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Patient 3 was a 7-year-old boy with a 3-year history of ES. MRI revealed no evidence of lesions. Scalp EEG revealed sharp waves mainly over the bilateral parietal–occipital–posterior temporal regions . Two types of seizure were captured by ictal video-EEG: (a) 14 episodes of ES, which presented as nodding, blinking, deviation of the right corner of the mouth, accompanied by rapid lifting of the upper limbs, with a typical ictal EEG pattern for ES ; (b) one episode of ES followed by a tonic seizure, which presented as sudden nodding, lifting, and stiffening of the upper limbs for several seconds, with EEG showing a spike rhythm following a typical ES pattern .
Mean seizure frequency at baseline was 9.41 ± 5.30 times per day. He was taking sodium valproate, clonazepam, and topiramate. The patient underwent six blocks of tDCS treatment at 2 mA. Seizure frequency at the first follow-up was 7.68 ± 5.38 times per day. During the first 19 days of the second follow-up, seizure frequency was 9.11 ± 5.49 times per day. Beginning the 20th day of the second follow-up, he remained seizure-free for 6 months. Thus, he was identified as a positive responder. Mean seizure frequency for Patient 3 is shown in .
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pmc-6370643-4
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Patient 4 was a 3-year-old girl with a 1-year history of ES. MRI revealed enlargement of the right temporal horn of the lateral ventricle. Scalp EEG revealed sharp and wave complexes mainly over the posterior temporal–parietal regions, with higher amplitude on the left side electrodes . One type of seizure was captured by ictal video-EEG: 146 episodes of ES, which presented as nodding and lifting of the bilateral upper limbs, with typical ictal EEG patterns for ES .
Mean seizure frequency at baseline was 4.28 ± 2.61 times per day. She was taking sodium valproate, nitrazepam, levetiracetam, and topiramate. The patient underwent one block of tDCS treatment at 2 mA. Her seizure frequency began to decrease during the second month of the follow-up period and reached a 76.64% reduction by the third month . Thus, she was identified as a positive responder.
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