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pmc-6256137-1
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A 17-year old girl was admitted to a local hospital for acute abdominal symptoms. She had a previous history of HSP with typical cutaneous and articular manifestations and good response to oral steroid therapy, at the age of 12. Ten months after the first HSP occurrence, she relapsed presenting the same clinical features. Renal involvement was always absent. At age 16 she developed erythema nodosum which was successfully treated with oral steroid therapy.
On admission to the local hospital she presented with fever, vomiting, diarrhea and epigastric pain. Symptoms had begun 5 days before and had gradually worsened. She was not under any medications and did not report any recent infection. Physical examination was unremarkable except for abdominal pain on deep palpation especially in the epigastric region, without hepatomegaly or splenomegaly; Blumberg and Murphy signs were negative. There was no skin rash at admission.
Laboratory analysis showed slightly increased C-reactive protein (CRP) (2.3 mg/dL, normal value < 0.29 mg/dl). During hospitalization, gastrointestinal symptoms worsened despite intravenous (IV) proton pump inhibitor and empiric antibiotic treatment. Three days after admission, a few petechiae appeared on arms and feet. The patient was thus transferred to our hospital.
On arrival she was febrile and presented continuous projectile vomiting. Vital parameters were normal except for mild tachycardia (140 bpm). Few petechial lesions were present on forearms, hands, and insteps. Lungs and heart examination was unremarkable. The abdomen was diffusely painful and tender, especially in upper quadrants, without rebound tenderness or organomegaly. Rectal examination did not show any bleeding, anal fissures, ulcers, abscesses, fistulae or scarring. Musculoskeletal examination was also normal.
Blood exams were unremarkable, except for mild leukocytosis (leucocytes 18,120/mm3) and elevated CRP (4.36 mg/dL). Erythrocyte sedimentation rate (ESR), procalcitonin (PCT), transaminases, total and direct bilirubin, amylase, lipase, coagulation profile were within normal range. Stool culture, testing for Rotavirus and Adenovirus, Clostridium difficilis toxin and antigen were negative. Hemoccult was positive on three stools specimens. Urinalysis showed no significant abnormalities. Anti-Saccharomyces cerevisiae antibodies (ASCA) and anti-neutrophil cytoplasmic antibodies (ANCA), were performed in the workout for inflammatory bowel diseases and resulted negative. On the other hand, fecal calprotectin dosage was elevated (>300 mg/Kg, normal value: <50 mg/Kg). Immunological laboratory tests (IgG, IgA, IgM levels, IgG subclasses, and lymphocyte subpopulations) were normal.
Abdomen ultrasound showed scarce peristalsis, slight ascites, hypervascularization and wall thickening of pylorus and second portion of duodenum. Plain abdomen X-ray and CT scan showed no significant abnormalities.
For the persistence of abdominal pain, an esophagogastroduodenoscopy was performed, which revealed pyloric edema, multiple hyperemic and hemorrhagic lesions with round shape in the duodenal bulb and descending duodenum, some of them were ulcerating (Figures , ). Histological examination showed active but non-specific inflammation with eosinophilic component and IgA deposition (Figure ).
Overall, clinical manifestations, the results of laboratory analysis and the macroscopic and histopathological aspects of pylorus and duodenum, prompted the diagnosis of HSP relapse with initial GI involvement.
On admission, the patient was started on total parenteral nutrition and IV antibiotic treatment with ceftazidime (150 mg/kg/day IV in three divided doses) and metronidazole (40 mg/kg/day IV in three divided doses).
After endoscopy, high-dose IV methylprednisolone (30 mg/Kg/day) for 3 days, followed by oral steroids (prednisone 1 mg/kg/day) was started with dramatic improvement of gastrointestinal and cutaneous symptoms, which disappeared within a week. Enteral nutrition was gradually started again and she was discharged on oral prednisone.
Four weeks later, abdomen ultrasound did not show any abnormalities. Periodic urinalysis and fecal occult blood tests were normal. Oral steroid therapy was gradually tapered in 6 weeks.
At 1 year follow up, the patient was asymptomatic and showed no recurrence of skin rash or gastrointestinal symptoms.
Written informed consent was obtained from the patient's parents for the publication of this case report. As the patient's age was 17 years, we also obtained her informed consent.
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pmc-6256173-1
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Patient 1. A 2-years-old female infant was referred to the First Affiliated Hospital, Jinan University, due to elevated serum total bile acids (TBA) discovered for 21 months. At the age 3 months, she was admitted to Women and Children's Hospital of Quanzhou because of jaundice for 2 months. Laboratory test revealed that the serum levels of TBA, direct bilirubin (DBIL), aspartate transaminase (AST) and alanine transaminase (ALT) were all elevated (Table ), and she was thus diagnosed to have cholestatic liver disease. After being treated with intravenous reduced glutathione and ademetionine-1,4-butanedisulfonate for 9 days, which were commonly used in cholestatic patients with elevated alanine transaminase levels, her jaundice was alleviated, but the liver function indices remained abnormal (Table ). Then oral ursodesoxycholic acid was given and she was discharged at the age 3.3 months. During the subsequent follow-up over 20 months, she showed normal anthropometric and neurobehavioral development without abnormal appearance, and her cholestasis resolved completely since the age 7 months. However, persistently raised TBA levels were observed on repeated biochemical analyses (Table ). So she was referred to our hospital for further investigation and management when aged 2 years.
The patient was the elder sister of two monochorionic diamniotic twins who was delivered by cesarean section at the gestation age of 37 weeks and 2 days with the birth weight 2.25 kg and body length 45.0 cm. Her father is a hepatitis B virus (HBV) carrier who was clinically healthy but with slightly elevated serum TBA level on biochemistry analysis, and her mother was physically and biochemically healthy (Table ). There was no family history of any genetic disease.
Physical examination revealed a body weight of 12.0 kg, height 83.5 cm and head circumference 46.0 cm. No jaundice was observed in the skin and sclera. No stridor, crackles or crepitus was heard in the two lungs, and the heart sound was normal without any murmurs. There was no abdominal distention, and the liver and spleen were non-palpable. Physiological reflexes were normal and no pathological reflexes could be found on nervous system examination. On biochemical analysis, the TBA level reached 173.8 μmol/L (0–10 μmol/L) as other indices were normal (Table ).
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pmc-6256734-1
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This was an 85-year-old woman, right-handed, insulin-dependent, hypertensive, obese with body mass index of 42, who had fell over her right upper limb ( ). She did not have any neurovascular changes after the fall.
Patient had a cemented RSA performed 6 years before the fracture because of massive rotator cuff injury. Before the fracture, she had an active arch elevation motion of 60°, abduction of 50°, lateral rotation of 40° and medial of 50°.
We did not observed loosening of RSA components, according to criteria defined by Sanchez-Sotelo et al.(
The non-surgical management of the injury was discussed with patient. However, because of her clinical conditions (obesity and diabetes) e better control of pain, we decided for the surgery.
In surgery, we used lateral approach due to possibility of proximal extension access, and visualization and protection of radial nerve all the way along its course. A 3.5mm locking plate (DePuy, Synthes®) associated with cerclage and osteosynthesis with loops were used ( ).
Patient used a simple sling for 2 weeks, and an immobilized elbow to 90° degree, whereas passive movements of the limb were done. In the third week, the patient begun active movements, and 3 months after surgery, she evolved with consolidation of the fracture and the limb regained the same function level it had before the surgery ( ).
In an one year follow-up after the procedure, we evaluated patient function based on Disabilities of the Arm, Shoulder and Hand (DASH) index that was translated into Brazilian Portuguese,( her score was 78, being 0 the best score and 100 is worse.
Patient’s active arch motion reduced for degree of elevation of 45°, abduction of 40°, lateral rotation of 40° and medial of 30°.
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pmc-6256830-1
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A 69-year-old male patient who was being followed without treatment for CLL in Rai stage 2 for 6 months presented with abdominal pain and jaundice. Laboratory tests were as follows: leukocytes: 55,140/µL, lymphocytes: 51,240/µL, hemoglobin: 5.3 g/dL, platelets: 46,000/µL, indirect bilirubin: 2.89 mg/dL, haptoglobin: 2 mg/dL, lactate dehydrogenase (LDH): 1585 U/L, and DAT positive for Immunoglobulin G (IgG) (no titer provided). Imaging studies showed compressing conglomerate lymph node masses in the abdomen. The patient was started on steroid and BR treatments. The hemoglobin value rose to normal levels and DAT became negative after 3 cycles of BR. The patient received 6 cycles of BR chemotherapy and steroids were interrupted at the 5th month of treatment. The patient has been followed in remission for 1 year.
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pmc-6256830-2
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A 75-year-old female patient who was being followed without treatment with the diagnosis of CLL in Rai 0 stage for 8 years was admitted due to weakness and fatigue. Laboratory tests were as follows: leukocytes: 78,840/µL, lymphocytes: 67,020/µL, hemoglobin: 6.3 g/dL, platelets: 255,000/µL, indirect bilirubin: 2.58 mg/dL, LDH: 504 U/L, haptoglobin: 1 mg/dL, corrected reticulocyte count: 5.2%, and DAT positive for IgG (4+). The patient was started on steroid treatment and subsequently BR therapy was added due to increased lymphocyte doubling time. After the first cycle, the DAT titer dropped to 3+. Hemoglobin value rose to normal levels and DAT became negative after 3 cycles of BR. Steroids were ceased at the 7th month of treatment; The patient completed 6 cycles of BR and has been followed in remission for 1 year.
While the standard approach in CLL patients with AIHA is steroids, systemic chemotherapy is recommended in refractory cases and in patients requiring treatment for CLL []. Although first-line therapy in CLL patients is the fludarabine-cyclophosphamide-rituximab regimen, the wide use of BR chemotherapy, especially in advanced-age patients, has brought up the application of this combination in patients with AIHA [,]. In a recent study including 26 CLL patients who had AIHA and received BR, the response rate was 81% for AIHA and 77% for CLL []. Similarly, our patients also responded well in terms of CLL and AIHA. The most striking point was that DAT became negative in a short period of time (after 3 cycles of BR).
In conclusion, in addition to being a plausible option in advanced-age CLL patients, BR seems to be an important treatment of choice in terms of eliminating the poor prognostic factor of DAT positivity and assuring safe cessation of steroid treatment due to rapid achievement of DAT negativity.
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pmc-6256967-1
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A 21-year-old woman without former medical history had a bicycle accident resulting in her right-hand traumatism. No fractures were detected on X-rays. Wrist sprain was suspected because of persistent swelling. A plaster cast was applied for 15 days. Physiotherapy, including manual drainage, was performed. Two months later, the hand was still inflated and painful. Algodystrophy was suspected following a bone scan. Venous Doppler ultrasound of the upper limb excluded the diagnosis of phlebitis. The hand was so swollen (a) and painful that the young woman stopped her studies. Massage, manual drainage, and intravenous biphosphonate treatment were delivered without improvement. The patient was suspected of self-injury with strangling threads at wrist level. MRI angiography ruled out arteriovenous malformation. Two series of intravenous immunoglobulin were delivered without improvement. The final proposed diagnosis was a psychiatric disease corresponding to Munchausen syndrome or Secretan's syndrome, a possible etiology of hand lymphedema. Personality tests and psychiatric evaluations were done but the results were negative. A total impairment of the hand complicated by recurrent local infection appeared. The rheumatologists organized a consultation with specialists in lymphology. The first specialist decided to continue the physiotherapy treatment. The patient asked for a second opinion from in our center. The noncontrast magnetic resonance lymphography (NCMRL) demonstrated local lymph flow (LF) blockage at the wrist. It was associated with lymphatic malformations of the entire forearm, justifying a surgical procedure. An extrafascial dermolipectomy was first performed (a). Local advanced flaps were performed to close the hand. This first step was essential to remove all fibrous and nonfunctional tissue obstructing lymphatic circulation. This allowed for the removal of the strangulation at the wrist. One month following dermolipectomy, the time necessary for the hand to heal later, a free flap containing some lymph nodes VLNFT was performed. The autologous donor flap harvested at groin level (b) was transferred to elbow level. It was supplied by superficial circumflex iliac artery and contained lymph nodes, lymphatic vessels, and fat. One arterial and one venous anastomosis were performed to connect flap vessels to perforate branches (artery and vein) around the elbow (c). Lymphatic vessels were kept intact, allowing the spontaneous anastomoses to appear, which are the signs of lymphatic growth. The postoperative course was uneventful with rapid functional recovery. At the 1-year follow-up, the hand was nearly normal (b) and there is no lower limb lymphedema ( and ). The patient returned to the university and could play the piano and the guitar again.
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pmc-6256967-2
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A 26-year-old woman without former medical history had had a right-hand lymphedema for 4 years, which evolved quickly within a few weeks. The origin was apparently a burn of the hand. Six months after analgesic treatment, the patient was referred to a lymphology center. She was told that it was a chronic disease without any possibility of recovery and could be treated by massages, lymphatic drainage, and bandages. Unable to work because of hand impairment, she lost her job and could not find a new job because of the monstrous aspect of her hand (). Her referring physician could not find any solution and the patient was referred to a pain treatment center. At that time, the hand weighed 6 kg. The lymphedema was painful and required daily morphine intake. Via social networks, the young woman found a “lymphedema group” that mentioned treatment by VLNFT, so she came to our center. An obstruction of the LF at elbow level was confirmed by NCMRL (a) compared to normal aspect (b). A two-stage surgical procedure was decided consisting of an extrafascial dermolipectomy of the hand, followed, 1 month later, by a VLNFT from the groin area to the elbow. The same surgical procedure as described in case 1 was performed. In the postoperative course, forearm compression by bandage was maintained. The hand quickly ameliorated (a) and was functional after 6 months, allowing the patient to work again. The postoperative NCMRL showed new LVs at elbow level (b) and normal LF (c).
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pmc-6256995-1
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AASM is a 60-year-old female born in Boa Viagem, CE, Brazil and living in Diadema, SP and is a retired farmer. At the first consultation in July 2015, the patient had a one-year history of progressive dysphagia and sore throat. She had a smoking history of 50 pack-years, but quit one month before the appointment. She used to consume alcohol but stopped 6 months prior, and she has used a dental prosthesis for 40 years. At the physical exam, there was a reddened lesion involving the soft palate that was more prominent on the uvula and both anterior and posterior right pillars. There were no palpable cervical masses. Upon endoscopy, there was no involvement of the larynx or nasopharynx. Biopsy of the lesion on 07.16.2015 showed in situ squamous cell carcinoma. The face, neck and chest CT scan on 08.12.15 showed a thickening area and asymmetrical enhancement of the right soft palate, extending to the left tonsillar fossa and uvula. There were no suspicious cervical lymph nodes. The diagnosis was OPSCC, clinically staged as cT1 N0 M0.
On 10.02.2015, the patient underwent TORS, with the successful excision of a left tonsillar fossa tumor, with free frozen section margins. Simultaneous SLNB was performed with no complications. The final pathology report showed a moderately differentiated squamous cell carcinoma located on the left tonsillar fossa, with 1.2 cm in its greatest diameter and 0.3 cm thickness with free margins. No angiolymphatic or perineural invasion were found. Lymph node metastasis was identified in one of two SLNs removed with the left level II dissection. The final pathological staging was pT1 pNs1 M0. On 11.26.2015, the patient underwent a left modified radical neck dissection and a right elective selective neck dissection of levels I, II and III, because the lesion extended beyond midline. The pathological report found no additional metastatic lymph nodes. The patient had a great outcome after both surgeries without any complication. The hospital stay was short. and the follow-up period was uneventful.
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pmc-6257468-1
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A 13-year-old healthy female student with no medical illness presented with sudden onset loss of vision in both eyes for three days. It was associated with pain on eye movements. She had her first dose of an HPV vaccination three weeks prior to the presentation and reported having flu-like symptoms after the vaccination. There was no history of limb numbness, weakness, or walking difficulty. The bowel opening and micturition were normal. On examination, visual acuity in the right eye was counting fingers and left eye was hand movement. Both pupils were sluggish and a relative afferent pupillary defect (RAPD) was not apparent. Both anterior segments were unremarkable. Funduscopy showed a diffuse hyperemic swollen disc with dilated vessels in both eyes. The left eye was found to be more profound than the right eye (Figure ). The macula was normal in both eyes. There was no sign of retinitis or choroiditis. She was admitted to the ward for further investigation. Urgent computed tomography (CT) of the brain, orbit, and paranasal area was performed. Both optic nerves had homogenous enlargement which was suggestive of optic neuritis. The left optic nerve was slightly prominent compared to the right side (Figures -). The brain and paranasal views were normal. An erythrocyte sedimentation rate (ESR) showed 6 mm/hour and the white cell count was within normal limit. Blood screening for infective and autoimmune processes was unremarkable. Mantoux test was negative. A diagnosis of bilateral optic neuritis post-HPV vaccination was made. She was treated with intravenous methylprednisolone, 125 mg four times a day (qid) for three days, followed by oral prednisolone 1 mg/kg/day for 11 days. Her visual acuity improved significantly to 6/6 bilaterally after one week of treatment. Fundus examination showed healthy optic discs. Optic nerve functions were normal. She has been under eye clinic follow-up for two years and does not show any sign of CNS demyelinating syndrome.
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pmc-6257471-1
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A 58-year-old female patient with a past medical history significant for a migraine headache was referred to the neurology clinic for complaints of paresthesias in the left foot for nine months. Paresthesias were described as tingling that was precipitated every time she tried to put her right foot on top of the left foot. She further described that she would feel ‘zapp’-like sensations over last two toes on the dorsum of the left foot. She denied associated weakness or similar complaints in the right foot. She also denied a prior history of diabetes mellitus, ankle trauma, sprains, arthroscopies or ankle surgeries.
Neurological examination was remarkable for precipitation of paresthesias on the dorsum of the left foot with percussion on the dorsum of the left ankle joint (positive tinel sign). Sensations were intact to gross touch and pinprick. There was no evidence of a foot drop, and the motor strength was 5/5 in all extremities. Deep tendon reflexes were intact.
Initial laboratory workup revealed white blood cell (WBC) count of 4.89 x 103/nl (normal: 3.5-10.5 x 103/nl), hemoglobin of 13.4 g/dL (normal: 12-15.5 g/dL) and mean corpuscular volume of 93.5 fL (normal: 81.6-98.3 fL). Thyroid stimulation hormone (TSH) level was 1.720 micIU/mL (normal: 0.270-4.200 micIU/mL). Blood urea nitrogen and serum creatinine levels were 17 mg/dL (normal: 6-20 mg/dL) and 0.79 mg/dL (normal: 0.5-1.2 mg/dL), respectively. Vitamin B12 level was 597 pg/mL (normal: 230-1245 pg/mL). Electrodiagnostic studies were done and reported to be normal with no evidence of neuropathy, radiculopathy or tarsal tunnel syndrome. Nerve conduction study (NCS) of the medial and lateral branches of the right and left superficial peroneal nerves (SPN) showed no significant side-to-side difference in antidromic sensory responses, as shown in Table . The patient then underwent a neuromuscular ultrasound evaluation of bilateral DCPNs at the level of the ankle. The study revealed a significantly enlarged left DCPN with a cross-sectional area of 27.88 mm2 at the level of the ankle, as compared to the cross-sectional area of the right DCPN, which was 10.8 mm2, as seen in Figure .
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pmc-6257490-1
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The first case was a 24-year-old woman with a 13-year history of T1D complicated by end-stage renal disease (ESRD) requiring hemodialysis. At the time of surgery, she weighed 61.9 kg and received a pancreas from a seven-year-old, 25 kg donor as part of her SKP. The patient had normal endogenous pancreatic function within 24 hours, independent of exogenous insulin with normal amylase and lipase function. At six months post-op, she had a normalized HgbA1c of 5.1% and C-peptide increased from <0.1 to 4.2 ng/mL.
A small peripancreatic fluid collection seen in the one and two-month follow-up scans resolved by three months; presumptive diagnosis was hematoma. Sonographic measurements of the transplanted pancreas were taken postoperatively, showing a size of 10.4 cm x 2.9 cm (CC x AP). Three-month follow-up average measurements showed minimal enlargement with measurements of 9.9 cm x 3.4 cm (CC x AP).
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pmc-6257490-2
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The second case was a 55-year-old woman with T1D complicated by gastroparesis and ESRD requiring peritoneal dialysis. At the time of surgery, she weighed 49 kg and received a pancreas from a seven-year-old, 35 kg donor as part of her SKP. This patient also had near immediate normal endocrine and exocrine pancreatic function. By six months after transplant, pre-transplant HgbA1c of 7.2% dropped to a post-transplant Hgb A1c of 5.4% and C-peptide increased from <0.1 to 1.3 ng/mL.
Immediate postoperative sonographic size of the transplant pancreas was 9.5 cm x 3.6 cm (CC x AP). Three-month follow-up showed a decrease in the size of the transplant to 6.1 cm x 1.8 cm (CC x AP). The sonographic appearance of the pancreatic allograft remained normal throughout all studies, with no complicating events.
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pmc-6257490-3
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A 47-year-old male with T1D complicated by ESRD and diabetic retinopathy received a SKP. At the time of surgery, he weighed 56 kg and the weight of the six-year-old pediatric pancreatic donor was 25 kg. His postoperative course was complicated by a small bowel obstruction requiring lysis of adhesions and an internal hernia requiring reduction, within one month after his initial transplant surgery. His endocrine and exocrine function were normal after surgery. At six-months follow-up, his preoperative HgbA1c of 8.4% had dropped to 4.6% and C-peptide increased from <0.1 to 2.5 ng/mL.
Immediate postoperative sonographic size of the transplant pancreas was 7.8 cm x 1.6 cm (CC x AP). Three months postoperatively, his transplanted pancreas was 8.0 cm x 1.9 cm (CC x AP).
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pmc-6257492-1
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A 24-year-old G2 P0 patient with history of chronic hypertension and chronic unspecified kidney disease diagnosed in infancy and followed by a nephrologist was admitted for one-day history of epigastric and right upper quadrant pain. The pain was sharp, intermittent with an intensity of 9/10. On admission day, the pain increased two hours after eating spicy tacos. She denies any exacerbating or alleviating factor. The patient also vomited six times after eating.
The patient also reported painful uterine contraction. On physical exam, the patient was hemodynamically stable. The vaginal exam revealed 3–4 cm dilated and effaced cervix.
On admission, laboratory results were consistent with microangiopathic hemolytic anemia (severe thrombocytopenia, anemia, worsening kidney function) (Table ).
The delivery team was consulted for dilation and evacuation for a possible pre-eclampsia/HELLP syndrome. Failure of thrombocytopenia to improve after dilation and evacuation led to consider TTP and aHUS. TTP is treated with plasma exchange, and aHUS is treated with anti-complement therapy. TTP was considered and treated presumptively given the severity of its complication: intracranial bleeding. Creatinine level was monitored to assess the response to treatment. A lack of improvement would change the presumptive diagnosis of TTP to aHUS. ADAMTS 13 level was sent prior to any transfusion. ADAMTS level was low at 44% favoring aHUS. The patient entered in remission when she was treated with Eculizumab.
Imaging and genetic tests
Chest X-ray showed basilar opacities, likely moderate pleural effusions (Figure ).
The abdominal sonogram demonstrated an atrophic right kidney with increased echogenicity, slightly prominent tortuous veins at the splenic hilum which may represent focal perisplenic varices.
Genetic testing
DNA sequencing and multiplex ligation-dependent probe amplification (MLPA) analysis did not identify any significant variant of the genes tested: CPH, CFI, MCP, THBD, VFB, C3, DGKE, ADAMTS 13, C4BPA, C4BPB, LMNA, CFTHR1, CFHR3. Genetic variants identify only 50–60% of aHUS cases.
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pmc-6257493-1
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We present the case of a 27-year-old African American male who was shot with a 9 mm handgun during an attempted home invasion and burglary. An initial axial non-contrast computed tomography (CT) of the chest and abdomen was done that showed a bullet found anterior to the abdominal midline. The bullet had ricocheted off of the T12 vertebral body and penetrated the inferior vena cava (IVC) (Figure ).
The patient was then taken to the operating room for emergency exploratory surgery. The IVC was clamped and the patient was intubated and placed on life support. A follow-up chest radiograph 48 hours later demonstrated a 9 mm bullet that was seen in a different location projecting over the right atrium of the heart. This differs from the initial CT scan (Figure ).
The two-dimensional anterior-posterior view of the chest radiograph conducted could not confirm the spatial orientation or anatomic location of the bullet. Therefore, a non-contrast sagittal CT confirmed that the bullet had migrated from the IVC into the right atrium of the heart (Figure ).
The concerns in the further management of the trauma and cardiothoracic surgery teams included the possibility that the bullet could cause myocardium perforation, pericardial injury, or further migration into the patient’s pulmonary artery. Interventional radiology was consulted for the possibility of performing an endovascular extraction of the bullet through the right internal jugular vein (IJV). The interventional radiology team agreed to proceed with the extraction, and the patient was brought to special procedures. The patient’s right neck was prepared and draped in a sterile fashion to initiate venous access into the IJV. Using ultrasound guidance, access was obtained into the right IJV using a micropuncture kit. Through the micropuncture sheath, a Benson wire was advanced into the right IJV, superior vena cava (SVC), and ultimately the IVC. A 10 cm by 11 cm bright tip sheath was then advanced over the Benson wire, into the right IJV. A 12 mm by 20 mm snare device was advanced over a 6 French sheath into the right atrium. Ultimately, the bullet was snared with tension placed on the ensnare device, which lassoed the bullet against the guiding sheath (Figure ).
The serrated edges of the bullet made the removal difficult once snared, and numerous attempts to reposition the bullet were made to allow for preservation of the surrounding structures. The bullet was then retrieved once it was certain to have a clear path for removal out of the right atrium, SVC, and right IJV (Figure ).
A venogram of the proximal-most aspect of the right IJV as shown in Figure was conducted once above the level of the clavicles to evaluate vascular injury, extravasation, or hemorrhage.
Surgical incision and cut down of the right IJV inferior to the percutaneous access site was conducted in order to provide a controlled exit site for the bullet. Vessel loops were placed around the right IJV superior and inferior to the incision. After the surgical incision, control of bleeding, and evacuation of blood, the bullet was identified (Figure ).
The snare device and vascular sheath were removed, and a multi-layer surgical closure of the right IJV was performed.
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pmc-6257518-1
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A 41-year-old Ukrainian male with a past medical history of acquired immunodeficiency syndrome (AIDS) and syphilis presented to the emergency department with a complaint of sudden-onset left buttock pain for one day. The pain was described as severe, progressive, continuous, radiating to the left foot, associated with numbness and burning sensation of the left foot and foot drop. The patient also reported a palpable mass in the left buttock. The patient was diagnosed with human immunodeficiency virus (HIV) seven years ago and his most recent cluster of differentiation (CD)4 count was 20; he was not receiving treatment for AIDS. He was also a diagnosed case of latent syphilis for 12 years. His past surgical and family history was non-contributory. He denied smoking as well as alcohol and illicit drug abuse. The patient was sexually active with one male partner. The patient denied any fever, back pain, urinary/bowel habit changes, swelling in any other part of the body, history of trauma, sexual dysfunction, and appetite/weight changes.
Pertinent findings during the physical examination of the lower limbs include diminished deep tendon reflexes (DTRs) of the left lower extremity, with a static response of plantar along with left foot drop. Sensation to touch was diminished on the left side in the L5, S2, and S3 dermatomal areas and was completely absent below the left ankle. The motor and sensory examination of the right lower extremity was completely normal. Local exam of the left buttock showed a firm, fluctuant, freely mobile mass measuring 4 x 4 cm with no overlying skin changes.
The baseline investigations were unremarkable except hemoglobin (Hb): 9 g/dl, blood urea nitrogen (BUN): 18 mg/dl, and serum creatinine (Cr): 1.25 mg/dl. A left hip X-ray revealed a soft tissue density. Computed tomography (CT) scan of the pelvis confirmed a solid mass. A biopsy of the left buttock mass was performed which showed high-grade BL, a type of NHL (Figure ). The patient was referred to the oncology department and was started on intensive high dose chemotherapy along with highly active antiretroviral therapy (HAART).
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pmc-6257526-1
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A 47-year-old female was presented to a private hospital with a history of recurrent
facial paralysis and left hemiparesis. The patient had no other pertinent past
medical history. After a marked right-sided visual field defect developed, various
investigations disclosed a tumor in the right parieto-occipital region. A cranial
mass was identified at cranial magnetic resonance (MR) imaging () and she underwent neurosurgery operation. It was
surgically resected and found to be a 3-cm spherical red mass with central necrosis,
sharply demarcated from the surrounding brain tissue by a thin condensation of
fibrous tissue. It was composed entirely of well-formed, blood-filled, anastomosing
sinusoidal-type spaces formed by branching trabeculae of loose fibrocellular stroma
covered by an endothelial cell lining, generally single-layered with occasional
doubling (). Mitoses were quite
uncommon. This lesion was reported as a hemangioma, although the atypical location
and histologic features were noted. These immunohistochemical examination was
determined as metastatic angiosarcoma. She also got adjuvant chemotherapy. The
regimen of the therapy administered by medical oncology department was doxorubicin
(75 mg/m2) and ifosfamide (7500 mg/m2) with a duration of 6
cycles of combination treatment.
About 6 weeks after neurosurgery, she reappeared with shortness of breath and
pericardial friction rub but no increased jugular venous pressure or dependent
edema. She was transferred to our institution and underwent transesophageal
echocardiography, which showed a homogeneous mass that involved the free wall of the
right atrium. The patient then underwent computed tomography (CT), MR imaging, and
angiography (). Findings from the mass
suggested a diagnosis of cardiac angiosarcoma as 3x3 cm. Electrocardiogram showed
normal sinus rhythm with nonspecific ST wave changes. Chest X-ray revealed
cardiomegaly and a pericardial effusion, confirmed by echocardiography. An abdominal
ultrasound showed only an enlarged liver. By evaluation of our heart team, we
decided to proceed with complete surgical resection of primary tumor.
Full-arterial monitoring was performed under general anesthesia. Standard aortic
arterial and bicaval venous cannulation were performed. Cardiopulmonary bypass (CPB)
was achieved. Cardiac arrest was achieved with blood cardioplegia. After
cross-clamping, surgical field was exposed with right atriotomy. The tumor, as well
as part of the right pericardium, were resected (). Because of tumor involvement of anterosuperior leaflet,
tricuspid valve was reconstructed with ring annuloplasty. A piece of bovine
pericardium was used to reconstruct the right atrial wall via continue suture
technique. And due to resection of the right coronary artery with the tumor, distal
anastomosis of coronary bypass surgery with saphenous vein was performed (). Proximal anastomosis was performed
with side clamped as well. She has weaned from the CPB smoothly. Heparin
neutralization with protamine was performed.
Aortic cross-clamp time was 36 minutes and total perfusion time was 65 minutes.
During the operation, mean arterial pressure was maintained at 70 mmHg. By the end
of CBP, the patient was transferred to intensive care unit (ICU) with a
hemodynamically stable condition. She was extubated on the postoperative
7th hour.
Total drainage was 500 cc. After one night staying in ICU, the patient was discharged
in good condition after the 7th day of the operation. Follow up
echocardiographic controls revealed clean cardiac cavities. The control routine
biochemistry was normal and 9th month PET scan was also clean.
In the present report, a patient with cardiac angiosarcoma and distant metastases was
treated with a combination of chemotherapy and surgical resections. The patient
responded well to neoadjuvant chemotherapy, and the tumor and metastases decreased
to an extent that made complete surgical resection feasible. Surgical resection was
successful in removing the mass and subsequent chemotherapy was used to destroy any
remaining tumor cells.
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pmc-6257529-1
|
A 57-year-old man with hypertension, chronic kidney disease, chronic pulmonary
obstructive disease, a 33 cm2/m body mass index (BMI), and a history of
ureterorenoscopy four years earlier was admitted to the emergency department with
flank pain. Renal colic due to urolithiasis was suspected. A computed tomography
(CT) scan without contrast revealed multiple renal cysts and two calculi larger than
1 cm on the right renal calix, an aortic aneurysm 57 mm in diameter, and
retroperitoneal hematoma ( and ). The patient's general condition
deteriorated, he became hypotensive and lost consciousness. The classical triad of a
ruptured AAA is easily identified with the aid of radiological imaging instead of
palpation of a pulsatile mass. The patient was immediately transferred to the
operating room and aortobiiliac graft replacement was performed with an 18x9 mm
bifurcated graft for a ruptured infrarenal AAA. Initially, manual compression at the
level of renal artery and vein was performed by the surgical team, and the admission
of bolus doses of noradrenalin and crystalloids by the anesthesia team provided
enough time to expose the neck of the aneurysm and to perform proximal clamping to
the infrarenal aorta. Distally, both iliac arteries were exposed and clamped. After
proximal and distal control, 5000 units of heparin were administered intravenously.
Longitudinal aortotomy was performed. The aneurysm sac was free of thrombus, and the
ostium of the inferior mesenteric artery was patent. A tear was identified on the
left side of the aorta (). Our initial
strategy to implant a tubular graft was changed based on the need to excise the
aortic bifurcation due to its friable tissue. The inferior mesenteric artery without
sufficient backflow was replanted ().
The patient was transferred to the intensive care unit. He was extubated after 48
hours. Continuous positive airway pressure with a mask was maintained for four days
postoperatively. In the postoperative course, the patient underwent several
hemodialysis sessions, was oxygen dependent, and experienced paralytic ileus.
Hemodialysis was needed daily after postoperative day 2, even though sufficient
urine output was present on postoperative day 5. On postoperative day 6, abdominal
distension was apparent despite nasogastric decompression. Oral administration of 50
ml of contrast in 500 ml of water was used 8 hours before radiography to evaluate
the prolonged postoperative ileus ().
As there was passage to the colon, we continued to follow up with dexpanthenol 500
mg twice/day, neostigmine 0.5 mg once/day, and an enema twice/day. Oral contrast was
administered again on postoperative day 9 to profit from its stimulant effect on
intestinal peristalsis[. The need
for hemodialysis and the postoperative ileus resolved 10 days after surgery, and the
patient was mobilized without oxygen support. During the postoperative course, the
patient was hypertensive, and tachycardia was present. He was on sinus rhythm and
oral metoprolol 100 mg twice/day was given to keep the rhythm at 100 beats/min. His
blood pressure was controlled with nitroglycerin and alpha (α) and calcium
(Ca) channel blockers. We investigated the potential causes of this condition.
Two-dimensional echocardiography was performed; the results showed normal ejection
fraction and mild tricuspid insufficiency. Re-evaluation of the initial preoperative
CT scan revealed a left suprarenal incidentaloma with 4 cm diameter (). The presence of a left suprarenal
adenoma on the CT scan with an unenhanced density greater than 10 Hounsfield units
resulted in the suspicion that a pheochromocytoma was the etiology of the patient's
hypertension[.
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pmc-6257534-1
|
Case 1: 65-year-old male, with a penetrating aortic ulcer (PAU) on the descending
thoracic aorta. On the CT angiography (), it seemed to start right after the origin of the left subclavian
artery, extending to the descending thoracic aorta, with a maximum diameter of
57 mm.
Patient was selected for endovascular treatment. The initial planning suggested
that the endoprosthesis could be anchored in Zone 3[ with the free flow zone over the left
subclavian artery.
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pmc-6257534-2
|
Case 2: 67-year-old male, with a descending aorta aneurysm and distal involvement
of the aortic arch with a maximum diameter of 10.0 x 9.2 cm ().
|
pmc-6257534-3
|
Case 3: 75-year-old male, being followed-up by the Cardiology team for severe
aortic valve stenosis with an ejection fraction of 30% in New York Heart
Association (NYHA) functional class III ().
|
pmc-6257534-4
|
Case 4: 68-year-old male, with asymptomatic infrarenal abdominal aortic aneurysm
extending to the iliac arteries with maximum diameter of 67 mm ().
|
pmc-6257534-5
|
Case 5: 64-year-old male, 3 years follow-up for Stanford type B chronic aortic
dissection, with acute abdominal pain, undergoing surgical treatment ().
|
pmc-6257534-6
|
Case 6: 67-year-old female, Stanford type A chronic dissection, with acute
dyspnea and chest pain undergoing ascending aorta replacement ().
|
pmc-6257588-1
|
A 70-year-old female patient was admitted to our cardiovascular department for chest distress for 11 hours. The patient presented with shortness of breath at rest and difficulty in laying in the recumbent position. She had been diagnosed with hypertension and diabetes mellitus several years prior and was on oral medication for their treatment. After clinical examination, she was diagnosed with coronary atherosclerotic heart disease and subsequently underwent coronary angiography and stent implantation (Fig. A and B), with heparin (total 5500 units, including arterial intrathecal injection of 2000 units and intravenous injection of 3500 units) administration during the operation. After coronary angiography, she was prescribed aspirin (100 mg per day), TiGraylo (90 mg once every 12 h), and enoxaparin sodium (0.4 mL once every 12 h subcutaneously) for 3 days, and her chest distress and shortness of breath had relieved. On the third day after the operation, she was diagnosed with hematochezia without abdominal pain.
Physical examination revealed mild abdominal tenderness, especially on the left lower quadrant, with signs indicative of peritoneal irritation. A neoplasm-like mucous clot connected to the bowel was found in the anus. Laboratory tests showed declined levels of hemoglobin (102 g/L), prolonged prothrombin time (13.4 s; normal range 9.8–12.3), and an international normalized ratio of 1.2 (normal range 0.9–1.1). Colonoscopy revealed a submucosal mass, covered with a partially ruptured mucous membrane and with some clots in the cavity, which caused obstruction in the sigmoid colon, hindering further examination (Fig. A and B). Abdominal CT showed submucosal hematoma in the sigmoid colon and the density of the mass did not significantly change on enhanced CT scan. The left flexure of the colon was thick, indicating that it could also be a hematoma (Fig. A and B). Clinical symptoms did not resolve with conservative treatment, which included anticoagulant cessation, total parenteral nutrition, and blood transfusions. Two days later, the patient complained of fever, body temperature of 38.8°C, with aggravated lower abdominal pain, and abdominal distention, and physical examination revealed peritoneal irritation. The patient underwent emergency exploratory surgery, which revealed a huge submucosal hematoma in the sigmoid colon and a necrosed bowel, 40 cm in length, with a clear boundary. Sigmoidectomy and end colostomy were performed. Pathological examination showed left-half colonic hemorrhage with necrosis (Figs. and A, B). Considering that the patient had a high risk for developing an embolism 24 hours after the surgical intervention, she was prescribed enoxaparin sodium (0.4 mL once daily, subcutaneous injection). Six and 9 days later, clopidogrel (75 mg per day) and aspirin (0.1 g per day) were added, respectively, and enoxaparin sodium administration was stopped. The patient was administered the same 2 antiplatelets until May 2018, after which she was prescribed oral clopidogrel bisulfate (50 mg per day) and aspirin (0.1 g per day). She did not have intestinal bleeding and occult blood test was negative on the latest follow-up.
Patient consent was obtained, and approval for the study was granted by the ethics committee of the Fourth Affiliated Hospital, Zhejiang University School of Medicine.
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pmc-6257600-1
|
A 31-year-old woman, of Indian origin and known to have TA, was booked for an antenatal care appointment at the 11th week of her third gestation in April 2017. Her earlier two pregnancies in 2008 and 2010 resulted in the normal vaginal delivery of a full term and healthy singleton each. However, her second pregnancy was complicated by pre-eclampsia. The patient was incidentally diagnosed through a routine chest X-ray, which revealed a widened superior mediastinum (Figure ) in June 2014.
Further physical examination revealed an absent left radial pulse. Subsequently, a computed tomography (CT) pulmonary angiogram confirmed the presence of a dissecting aortic aneurysm, which measured 7 cm in length and was inferior to the origin of the left subclavian artery (occluded) (Figure ). The CT scan also revealed an atrophic right kidney and a hypertrophied left one. Thus, the patient was placed on prednisone as well as methotrexate and booked for endovascular repair surgery. The woman later successfully underwent endovascular stent grafting of the thoracic aorta in April 2015. After developing pneumonia, the patient was switched from methotrexate to azathioprine (AZA) and maintained on amlodipine (5 mg), aspirin, and prednisone.
The lady's antenatal care was under the feto-maternal unit (FMU) at the Women’s Hospital. Simultaneously, the patient was regularly followed by her rheumatologist and vascular surgeon. The patient resumed her medication during pregnancy; and serial ultrasound scans revealed a normally developing fetus. The antenatal period was uneventful except for gestational diabetes mellitus. In November 2017, the patient presented at 38+ weeks gestation for an elective cesarean section and bilateral tubal ligation as advised by the multidisciplinary team. Thus, the patient delivered a healthy baby girl, weighing 2279 g, and her postpartum period was uneventful. The patient was counselled about breastfeeding while on AZA and told that the current evidence does not suggest any risk from AZA during pregnancy and while breastfeeding. One month following her delivery, the patient presented for a follow-up appointment with her vascular surgeon and was asymptomatic. Also, her blood pressure (BP) measurement and inflammatory markers were within the normal range.
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pmc-6257626-1
|
A 22-year-old male was referred to our outpatient hepatology clinic for an evaluation of elevated liver enzymes. The patient was first informed about the hepatic dysfunction one year ago at the time of enlistment in the army in the Trinidad. He had a history of heavy alcohol consumption, at least once every two weeks, for about five years. However, he stopped drinking one year ago. He also reported intermittent, crampy, waxing and waning abdominal pain. Review of the systems was negative for any abnormalities. He was a professional soccer player with excellent health. There was no prior history of drugs, herbal medications, vitamins, or supplements. His mother was diagnosed with breast cancer; however, family history was negative for genetic disorders. On admission, he was hemodynamically stable. Physical and neurological examinations were unremarkable.
Laboratory studies revealed a fasting blood sugar 96 mg/dL (normal, 70-100 mg/dL) and HBA1c 5% (normal, <6%). His complete blood count, hemoglobin, hematocrit, serum albumin, serum electrolytes, renal function tests, and coagulation profile (international normalized ratio, 2.2; prothrombin time, 12 s) were within the normal limits. Serum creatine phosphokinase, aldolase, lactate dehydrogenase, calcium, vitamin D, vitamin B12, and cortisol levels were also within normal ranges. Adrenal and thyroid functions were normal. The details of his liver function testing are provided (Table ).
Hepatitis serologies, human immunodeficiency virus, serum ferritin and total iron-binding capacity for hemochromatosis, autoimmune workup, ceruloplasmin for Wilson's disease, and alpha-1 antitrypsin deficiency testing were all negative. Right upper quadrant ultrasound was inconclusive for gross biliary or hepatic abnormalities. Subsequently, an uneventful percutaneous liver biopsy was performed. Pathological examination of the biopsy specimen showed polyglucosan inclusions in the hepatocytes that were resistant to diastase, consistent with LBs (Figure ).
Periportal hepatocytes had large ground-glass inclusions (Figure ).
The probable causes were ruled out based on the standard set of investigations. Although it was an atypical presentation, the patient was deemed to suffer from Lafora disease in light of the consistent histopathological features indicating the presence of LBs in the hepatocytes. He was educated about the disease and genetic testing was recommended. However, he had to travel back to the Trinidad and unfortunately, could not come for a follow-up visit.
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pmc-6257648-1
|
A 33-year-old Taiwanese man presented to the emergency department with bilateral limb weakness (more severe in the lower limbs than in the upper limbs). The patient's vital status on arrival was as follows: blood pressure, 160/86 mmHg; heartbeat, 96 bpm; and, body temperature, 36.3 °C, with clear consciousness. He denied experiencing recent trauma, back pain, chest pain, abdominal pain, headache or dizziness, or a fever episode. Physical examination showed no specific findings. Neurological examination showed weakness in the muscles of the bilateral upper limbs (muscle power score 4) and of the bilateral lower limbs (muscle power score 3). Muscle weakness was more severe in the proximal site than in the distal site. Blood examination included complete blood count, tests for renal and liver function, measurement of electrolyte levels (including potassium, sodium, and calcium levels), and tests for thyroid function. Blood examination showed normal complete blood count, normal renal and liver function, and normal potassium (3.5 mmol/L, normal range 3.5–5.1 mmol/L), sodium, and calcium levels; however, the examination showed impaired thyroid function (thyroid stimulating hormone: 0.04 uIU/mL, normal range 0.34–5.60 uIU/mL; free T4: 1.96 ng/dL, normal range 0.61–1.12 ng/dL). A 12-lead electrocardiogram showed normal sinus rhythm. Brain computed tomography without contrast showed no obvious intra-cranial lesion. Intravenous potassium infusion (20 mEq/L) with normal saline was prescribed for the patient. After the treatment, he felt a decrease in limb weakness. The clinical diagnosis favored TPP. This diagnosis was based on the clinical condition (bilateral muscle weakness that was more severe in the proximal site than in the distal site), age and race (33 years, Asian), family history (no history of periodic paralysis), blood examination results (hyperthyroid status but no obvious signs or symptoms of hyperthyroidism such as tremors, palpitation, or body weight loss), and imaging findings (brain computed tomography showed no obvious lesion); all these conditions matched the characteristics of TPP. The patient was discharged from our emergency department with a scheduled follow-up in the endocrine outpatient department (follow-up period of approximately 2 months). Methimazole (5 mg 3 times per day orally) was prescribed for controlling hyperthyroidism. He did not have any paralytic attack after discharge from our emergency department. Follow-up blood examination showed the following results: thyroglobulin antibody, <20 IU/mL and microsomal antibody, 36.9 IU/mL. Thyroid ultrasonography performed in the outpatient department showed no obvious mass or goiter.
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pmc-6257653-1
|
A 56-year-old man presented to us with chronic, progressive midline low back pain associated with right lower extremity radiculopathy. The patient described the pain as radiating and achy. On the physical exam, the patient had decreased sensation in the lateral aspect of the right lower extremity. Radiological findings demonstrated multilevel central stenosis and neuroforaminal narrowing with a degenerative scoliotic deformity of the lumbar spine (Figures , ). A conservative trial for up to a year with physical therapy, chiropractic and interventional pain modalities provided partial and short-lasting relief. As the pain continued to progress, the patient decided to proceed with surgery.
Various surgical options were considered. Eventually the patient underwent an ALIF at the L3-S1 levels using a retroperitoneal approach followed by a T10-pelvis posterior spinal fusion and a right L4-L5 laminotomy and decompression in two stages (Figure ). The post-operative course was complicated by abdominal distention that occurred on post-operative day eight. Computed tomography (CT) scan of the abdomen and pelvis demonstrated a large collection of retroperitoneal fluid measuring 11 x 9 x 22 cm in the left pelvis and left flank area with mass effect on the left kidney and ureter causing hydronephrosis (Figure ). After a consult with the general surgery team and the approach surgeon, the patient underwent ultrasound-guided drainage of fluid with temporary drain placement.
The fluid was serosangionous in nature with a high output of greater than 1500 cc/day. Fluid gram stain showed no organisms and cultures also revealed no growth. He was discharged home with the drain in place for a total of 22 days after which the drain was discontinued upon clinic follow-up. The lymphocele recurred four days after the drain was removed, and the patient presented back to the emergency room with recurrence of abdominal distension. A new drain was placed again in the fluid collection, and was finally removed for paucity of drainage three weeks later. The patient’s symptoms as well as the lymphocele resolved and did not recur.
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pmc-6257654-1
|
During routine dissection and removal of cerebral structures of a 56-year-old female at death cadaver, duplicated branches of the AComA were observed. After further inspection, both arteries were found to originate from the anterior medial aspect of AComA (Figure ). This was realized to be an extremely rare case of duplication of the MACC, which to our knowledge has never been reported before. The left MACC had a diameter of 0.60 mm and the right MACC had a diameter of 1.02 mm. The left A1 and A2 measured 1.96 mm and 1.74 mm, respectively, while the contralateral A1 was fenestrated measuring 1.06 mm and 1.07 mm joining into a common A2, which measured 2.04 mm. No hypoplasia was observed with the AComA although its course exhibited a variation in diameter of 1.01 mm (right side) and 1.13 mm (left side) (Figure ). The left recurrent artery of Heubner measured 0.76 mm in diameter while the right one measured 0.80 mm in diameter. No other cerebral vascular variants or pathologies was observed.
|
pmc-6257711-1
|
A 52-year-old male was brought to the trauma center after a high speed motor cycle collision. The patient was not wearing a helmet and had altered mental status on the scene requiring emergent intubation with a King laryngeal tube (Ambu Inc., MD, USA). On arrival at the trauma center his airway was secured with an endotracheal tube and a cervical collar was placed. He had bilateral breath sounds and his oxygen saturation was 100%. His distal pulses were 2+, blood pressure 98/60 mm Hg and pulse rate was 89 beats per minute (bpm). He had a negative focused abdominal examination for trauma (FAST) exam. His Glasgow Coma Score (GCS) was three with pupils two mm bilaterally and reactive. On log roll there was no rectal tone, but the patient had received succinylcholine 100 milligram (mg) for the endotracheal intubation. A foley catheter was placed and no hematuria noted, his pelvis was stable. The patient’s repeat blood pressure was 70/49 mmHg and pulse 85 bpm with a weak thready radial pulse and warm extremities. He was given two units of packed red cells and two units of fresh frozen plasma. His repeat blood pressure was 79/62 mm Hg as measured by a right femoral arterial line. A massive transfusion protocol was initiated with the patient receiving another five units of packed red cells, four units of fresh frozen plasma, one pack of platelets, and one gram of Tranexamic acid. Simultaneously the right femoral arterial line was exchanged for the 7 French introducer catheter and connected to the arterial line with good waveform. The REBOA catheter was placed at 42 centimeter (cm) into zone one. Inflation was done with 2 milliliters (ml), then 4 ml, then 6 ml of saline for a total of 6 ml of saline with improvement in the blood pressure to 112/62 mm Hg. The REBOA catheter was locked and sutured in place. His abdominal X-ray (Figure ) showed good placement and the catheter was placed to continuous invasive monitoring. His repeat FAST and chest X-ray were negative for intrathoracic effusion or intraabdominal free fluid. Norepinephrine was added for suspected spinal shock given his warm extremities with dilated peripheral veins. Prior to leaving the trauma resuscitation area his systole blood pressure was 125 mm Hg and went up to 195 mm Hg on arrival at the CT scan suite. The REBOA balloon was deflated slowly 30 minutes after inflation and prior to the completion of computed tomography (CT) scans. He was also weaned off the norepinephrine prior to the completion of CT scans, and 0.6 mg of atropine was given for bradycardia in the 40s. He was brought to the intensive care unit for further resuscitation. The patient sustained diffuse axonal injury and subarachnoid hemorrhage. The patient's post resuscitation GCS was 11T. The patient had neurogenic shock secondary to C5 level comminuted fractures involving lateral masses and lamina bilaterally, C6 level comminuted distracted fracture of the spinous process, C7 level fracture of the left lateral mass and left lamina and 5 mm anterolisthesis of C5 on C6 with cord contusion, facet joints causing a perched right C5 facet. The patient also had T1, T2, T3, T5, T7, T8, and T9 level vertebral body fractures and contusion. The patient also had bilateral mandible fracture, avulsion of the right earlobe, and right first and second ribs fracture.
The neurogenic shock resolved and the REBOA catheter was removed 78 minutes after initial placement. The patient did not require further transfusion of blood products or vasopressors. Lovenox for venous thromboembolism prophylaxis was started on hospital day 2. The patient underwent stabilization of his cervical spine and fixation of his mandibular fractures, and reattachment of the right earlobe. At the six-month follow-up the patient was independent of the ventilator, masticated with assistance, and was participating in spinal cord injury rehabilitation.
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pmc-6257712-1
|
A 67-year-old male with a past medical history of congestive heart failure, non-ischemic dilated cardiomyopathy, atrial fibrillation, and pacer-dependent heart block, presented to the University of Miami emergency department with refractory complex partial seizures. Due to the fact that the patient was pacemaker-dependent, he was unable to undergo MRI. Therefore, a contrasted CT scan of the brain was performed, which demonstrated a heterogeneously enhancing left frontal mass just anterior to the left motor cortex thought to be either a primary central nervous system tumor or a metastatic lesion (Figure ).
A full metastatic workup was conducted with no primary lesion detected. Given the location of the lesion, further imaging was thought to be necessary for two reasons: 1) to further elucidate the differential diagnosis of the lesion and 2) to help safely guide resection or biopsy of a lesion in close proximity to eloquent brain tissue. As a result, cardiothoracic surgery was consulted for the possible replacement of the patient’s current pacemaker with an MRI-compatible alternative. The patient subsequently underwent the removal of their pacemaker and replacement with a Surescan Medtronic DDD Pacemaker RVDR01 (Minneapolis, MN, US). Following this procedure, an MRI was performed and demonstrated a multi-lobulated, relatively uniformly enhancing left frontal lesion (Figure ). Due to the lack of a definitive diagnosis, the decision was made to perform an awake craniotomy with MRI-assisted stereotactic guidance and intraoperative electrocorticoraphy for a definitive diagnosis. The lesion was able to be resected with the initial pathology suggestive of Mycobacterium tuberculosae brain abscess. The patient was placed on anti-tuberculosis medication and intravenous (IV) antibiotics and discharged home. Approximately one year later, he returned to the hospital after suffering a partial seizure with subsequent right upper extremity weakness. He was found to have a recurrence of his left frontal lesion and was again taken to the operating room for surgical resection. Repeat imaging at one year did not demonstrate any further recurrence of his previously seen lesion.
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pmc-6257712-2
|
A 75-year-old male with a past medical history of sick sinus system status post the implantation of a pacemaker in 2004 presented to our service with complaints of chronic numbness in his lower extremities beginning approximately two years prior. He was initially diagnosed with peripheral neuropathy. His numbness was insidious in onset, and he initially presented on his bilateral feet. Over time, the patient’s symptoms progressed to the point where he would experience bilateral thigh numbness, which worsened with ambulating and, subsequently, lower abdominal numbness and urinary retention. His physical exam at that time demonstrated hyper-reflexia in his lower extremities bilaterally along with decreased hip flexor strength and bilateral Babinski signs. Due to concern for myelopathy and the inability to perform an MRI given the patient’s pacemaker, a CT myelogram was performed and demonstrated a likely intramedullary lesion at the T3 level (Figure ).
The quality of the CT was sub-optimal due to part of the dye being injected in a subdural location. A positron emission tomography (PET) computed tomography (CT) was subsequently performed, which seemed to confirm that the lesion was neoplastic and likely intramedullary in location. Given the advanced age of the patient and multiple comorbidities, further clarity on the nature of the lesion was needed in order to drive a treatment plan and, as a result, cardiothoracic surgery was consulted for the replacement of the pacemaker with an MRI-compatible alternative. The patient subsequently underwent the removal of their pacemaker and replacement with a DDD Medtronic Advisa SureScan pacemaker (Minneapolis, MN, US). Subsequent MRI demonstrated that the lesion was in fact not intramedullary, but presented as a dorsal, extramedullary dural-based lesion at T3 causing significant canal stenosis and cord compression, likely representing a meningioma (Figure ). Given the location and suspected pathology, it was felt to be surgical despite the patient’s advanced age and comorbidities. The patient subsequently underwent T2-T4 laminectomy with resection of meningioma. At three months follow-up, a repeat MR with gadolinium demonstrated complete resection of the tumor and the patient remained clinically improved two years post-resection with no evidence of recurrence.
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pmc-6257712-3
|
A 38-year-old female [] with a past medical history of Hodgkin lymphoma, treated with chemotherapy and radiation, along with radiation-induced heart conduction defect status post pacemaker placement, presented to a hospital in Mexico with a chief complaint of occipital headaches, dizziness, dysarthria, and imbalance. As a result, a CT scan was performed and demonstrated a midline superior cerebellar mass with surrounding edema, early hydrocephalus, and a small tentorial subdural hematoma (Figure ).
The patient was transferred to our service for further workup and treatment. At the time of her initial presentation, the lesion seen on the CT scan was favored to represent metastatic disease with lymphoma high on the differential diagnosis. Due to the presence of a non-MRI-compatible pacemaker, an MRI was unable to be performed at that time and a full metastatic workup was otherwise unrevealing. Ultimately, given the uncertainty regarding the pathology of the lesion and, as a result, the optimal treatment modality, the decision was made to replace the patient’s pacemaker with a DDD Medtronic Advisa SureScan MRI-compatible pacemaker. Postoperatively, an MRI of the brain was performed, which demonstrated an enhancing, likely extra-axial mass favored to represent a meningioma. As a result, the patient was taken to the operating room for a suboccipital craniotomy. A gross total resection was achieved and pathology returned as a high-grade peripheral nerve sheath tumor. Unfortunately, the patient developed a postoperative hematoma in her surgical cavity, which required emergent evacuation and the placement of an external ventricular drain and, ultimately, a ventriculoperitoneal shunt. The patient, however, made a full recovery and was discharged with plans for adjuvant chemotherapy and radiation.
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pmc-6257712-4
|
A 50-year-old male presented with a chief complaint of chronic back pain since 1997. He had a history of lumbar surgeries, including a right-sided L4-L5 and L5-S1 hemilaminectomy in 1999. The patient reported that after a car accident in the distant past, he “lost feeling” in his right leg and was told he had severe nerve pain and damage. His chief complaint during his visit to our office was a weakness in his left quadriceps along with back and leg pain and numbness. On physical exam, the patient had subtle weakness in the left hip flexor and left quadriceps, as well as the extensor hallucis longus and the gastrocnemius on the right. He had decreased pinprick in the L3 dermatome on the left but sensation otherwise appeared grossly intact. Deep tendon reflexes were 1+ to 2 throughout bilaterally. Nerve conduction studies revealed axonal loss and motor neuropathy of the peroneal nerve on the right along with chronic irritation of the right L5 nerve root but were otherwise unrevealing. Unfortunately, the patient had a non-MRI-compatible pacemaker, which prevented him from undergoing an MRI. A plain CT of the lumbar spine demonstrated multi-level spondylosis with severe loss of disk height at L3-L4, L4-L5, and L5-S1. In lieu of an MRI, a CT myelogram was performed (Figure ), which was inconclusive but suggested possible multi-level neural foraminal stenosis at L3-L4, L4-L5, and L5-S1. Notably, a majority of the contrast was located dorsally, which may have been related to operator technique.
The patient indicated that he would not be interested in undergoing a large fusion surgery, and so we discussed the possibility of performing selective nerve root blocks, and if these were effective, foraminotomies to offer some symptomatic relief for his leg pain. However, given progressively worsening leg pain and a lack of adequate imaging and unrevealing nerve conduction studies, we requested that the patient have his pacemaker changed with an MRI-compatible alternative to enable the performance of an MRI scan. At that time, the patient was already scheduled for a battery change and, therefore, elected to undergo full replacement of his pacemaker with an MRI-compatible alternative. Following the completion of this surgery, an MRI was performed, which while demonstrating multi-level spondylosis, did not show any significant central canal or neuroforaminal stenosis (Figure ). As a result, we advised the patient that while his axial back pain may be improved by a fusion surgery, foraminotomies were unlikely to result in any significant symptomatic relief.
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pmc-6257714-1
|
This is a case of a 53-year-old female patient who presented to our hospital for a cosmetic deformity. She suffered from a direct trauma to her lateral right thigh two years ago. She had no recall of a hematoma formation. She had no continuous pain but feels traction on hip abduction. Her main complaint is the difficulty to wear tight clothes.
A lower extremities MRI was performed showing a 5 x 7 x 2 cm cystic cavity that created a gliding plane between the subcutaneous tissue and the underlying muscular fascia (Figures , ). With the patient history in mind the diagnosis of pseudolipoma was postulated.
The pseudolipoma is marked preoperatively (Figure ) and a 5 cm incision on the caudal end of the mass is highlighted. We chose this incision in order to access this fibrotic adherence that caused the stair step deformation.
The operation was performed under general anesthesia. The surgical field included the whole leg. The incision was carried to the subcutaneous tissue and blunt dissection was performed to the underlying fascia. A finger dissection was performed between the muscular fascia and the posterior capsule of the pseudolipoma and a cautery dissection on the anterior surface of the mass. After extraction, the tumor consisted of a serous filled capsular formation (Figure ).
After removal and wound suture we can notice a depression in the area (Figure ).
An extensive rigotomy using a Coleman three cannula was performed to remove any remaining adherences.
A total of 20 cc of fat were extracted from the inner thigh using a tumescent solution with a Coleman three cannula and reinjected by a fanning technique to achieve regular contour. Aesthetic results three months following the operation were encouraging and showed a regular contour without any hump sign or skin depression (Figure ).
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pmc-6257738-1
|
A 73-year-old Caucasian male with a biopsy-proven diagnosis of stage IV poorly differentiated adenocarcinoma of the lung, epidermal growth factor receptor mutation negative, anaplastic lymphoma kinase translocation negative, and PD-L1 tumor proportion score of 20% was started on carboplatin, pemetrexed, and pembrolizumab. The patient was receiving the chemotherapy regimen every three weeks and prior to initiating cycle two, he developed generalized weakness. On presentation, he expressed subjective progressive weakness of the lower extremities (LEs) greater than the upper extremities (UEs). The physical exam was significant for 3/5 motor strength and absent deep tendon reflexes in the bilateral upper extremities (UEs) and LEs. Given the clinical presentation, an irAE secondary to an IO agent was suspected, with a differential diagnosis that included Guillain-Barré syndrome (GBS) versus myasthenia gravis-like syndrome. Lumbar puncture revealed albuminocytological dissociation in the cerebrospinal fluid (CSF) of 68 g/L, which further supported AIDP. The paraneoplastic panel was negative. Infectious workup, including CSF cultures, cytogenetic studies, polymerase chain reaction (PCR) for herpes simplex virus, cytomegalovirus, and serum venereal disease research laboratory (VDRL) was also negative. Methylprednisolone, along with intravenous immunoglobulin (IVIG), were initiated. Despite five infusions of IVIG, the patient’s strength diminished to 2/5 in the bilateral UEs and LEs. IVIG treatments were stopped and plasmapheresis was initiated. On Day 8 of hospitalization, the patient was transferred to the intensive care unit (ICU) for worsening respiratory status, as indicated by the measured lowest negative inspiratory force (NIF) of -20 cm H2O and forced vital capacity (FVC) of 1.1 L. Fortunately, the patient’s respiratory status improved with plasmapheresis and high-dose corticosteroid treatments and he never required mechanical ventilation. The patient received a total of eight sessions of plasmapheresis with a gradual recovery of his motor function. At Day 25 of hospitalization, the patient’s strength in his right UE was 5/5, left UE 3/5, and bilateral LEs 3/5. He was discharged to an acute rehabilitation facility on low-dose prednisone with instructions to follow up in the outpatient department. Unfortunately, the patient was re-admitted one month after discharge with another irAE and was subsequently placed in hospice care.
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pmc-6257738-2
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An 81-year-old Caucasian male with a diagnosis of melanoma on the right anterior chest wall and a wide local excision was found to have a recurrence in the right lung four months after initial diagnosis. Pembrolizumab was initiated, however, treatment was switched to dabrafenib and trametinib once molecular studies confirmed positivity for the BRAF V600R mutation. Nine months after diagnosis, magnetic resonance imaging (MRI) of the brain with and without contrast revealed multiple metastatic lesions in the brain (Figures -), prompting the administration of the second cycle of pembrolizumab in addition to dabrafenib and trametinib. One month following the second cycle of pembrolizumab, the patient was admitted to the hospital with progressive weakness described as originating in the bilateral LEs and then spreading to his bilateral UEs. His neurological exam revealed a strength of 2/5 in the bilateral UEs and 0/5 in the bilateral LEs with areflexia and no bulbar muscle weakness. A lumbar puncture showed an albuminocytologic dissociation with elevated CSF protein (56 g/L), which supported our diagnosis of AIDP. Electromyography was consistent with motor and sensory neuropathy. Infectious workup revealed negative CSF cultures and VDRL. Antibodies to ganglioside GM1 for GBS were negative. He was promptly started on methylprednisolone and IVIG for five days. On Day 1 of treatment, he developed acute hypoxic respiratory failure, requiring mechanical ventilation. After five days of treatment without any clinical improvement, plasmapheresis was initiated. On Day 14 of hospitalization, he developed a generalized tonic-clonic seizure. A computed tomography (CT) scan of the brain without contrast revealed a hemorrhage within one of his metastatic lesions and associated vasogenic edema (Figure ). On the same day, care was withdrawn and the patient expired shortly after.
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pmc-6257896-1
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A 35-year-old white male patient presented complaining of pain in the submandibular region and purulent secretion from a lingual caruncle with slightly increased volume in the region. Panoramic radiography showed a dense radiopaque mass in the region corresponding to the left submandibular gland (). However, plain radiographs are not appropriate for the diagnosis of calcified inflammatory diseases []. As a result of the magnification, geometric distortion, and superimposition of structures on the panoramic radiography, cone beam computed tomography (CBCT) was used to evaluate the lesion. The CBCT showed a hyperdense image of 24.35 mm × 9.35 mm ().
Ultrasound is usually used as the first exam to evaluate the salivary glands because it is noninvasive and with low cost [].
Ultrasonography showed an increased volume of submandibular gland with diffuse echogenicity changes associated with salivary duct dilatation, viewed as a partially circumscribed hyperechogenic image measuring 1.9 cm × 1.1 cm × 0.5 cm located in the adjacent sublingual region ().
Prior to the surgical procedure, the InVesalius software () was used to assess the ratio between sialolith's volume and submandibular gland involved for analysis of gland resection. After 3D reconstruction, it was possible to reduce bone transparency and observe the morphology of sialolith, which was isolated from other structures in order to allow the evaluation of its area, volume, and position. CBCT image shows the area corresponding to the sialolith, which was manually segmented (). The addition of volume (123 mm3) provided us a new perspective regarding not only the extension and size of the lesion (in mm3) but also the analysis of morphological aspects of the salivary calculus, thus confirming the need of surgical treatment.
After indication for surgery, the patient was placed on supine position with the left side of the neck exposed, showing the upper cervical skin 4 cm below the mandibular angle, where incision and opening of the flap were performed with an electrocautery device superficially placed on the platysma muscle. Next, the mandibular and cervical rami of the facial nerve were located, dissected, and protected. The posterior facial vein was clamped and sectioned, with the stump being isolated in order to facilitate the separation of the mandibular ramus cranially along with the upper skin flap. Next, the soft parts of the submandibular gland were dissected, exposing the anterior belly of the digastric muscle before sectioning of the small vessels to allow mobilisation of the gland and its complete resection. A fragment of soft tissue measuring 70 mm × 28 mm × 17 mm having irregular surface, elongated shape, blackened colour, and fibrous consistency was collected ().
Microscopic examination revealed fragments of salivary gland lobules showing excretory duct containing granular basophilic material and mineralised areas with a concentric deposition pattern compatible with a sialolith. In other areas, one can observe stroma composed of dense connective tissue exhibiting intense lymphoplasmacytic inflammatory infiltrate and congested blood vessels. From within, it was possible to identify areas showing salivary gland lobules, with the majority presenting acini replaced by the proliferation of duct-shaped structures exhibiting a double-layer of cells among the scant amount of connective tissue. In some areas, the proliferation of duct-shaped structures was found with intense lymphoplasmacytic inflammatory infiltrate. In the focal area, one could also observe the presence of exogenous material of yellowish colour and refractive aspect, exhibiting strange body reaction characterised by the presence of multinucleated giant cells ().
In the postoperative period, the patient reported paresthesia in the region of the lingual nerve. Therefore, a protocol with low laser therapy with an energy per point from 1.0 to 4.0 joules was applied. After this procedure, a considerable improvement in paresthesia was observed and the evolution of the cicatrization process was also accelerated allowing the patient to recover the regular movements of the neck, previously limited by edema. The residual scar was satisfactory and did not significantly compromise the aesthetics of the patient.
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pmc-6257899-1
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A 64-year-old female presented to the emergency department (ED) in February due to “wine-colored urine.” This was preceded by two weeks of viral upper respiratory illness (URI) symptoms, including fevers, chills, cough, headache, and malaise. Two days prior to presentation, she received azithromycin with no improvement in her symptoms. She had no past medical history and took no medications prior to this illness. Her family history was notable for a daughter with systemic lupus erythematosus.
Laboratory data included hemoglobin 11.7 (11.7–15.0 g/dL), total bilirubin 3.4 (0–1.2 mg/dL), and direct bilirubin 0.8 (<0.9 mg/dL). Urine was red and hazy with a moderate amount of blood. The sediment contained 4 + bacteria, 0–3 red blood cells (RBCs), and 11–25 white blood cells (WBCs) per high-power field (HPF). It was negative for nitrites, leukocyte esterase, urobilinogen, and bilirubin. Chest X-ray and CT abdomen/pelvis enhanced with oral and intravenous contrast did not reveal any acute pathology. She was started on nitrofurantoin for suspected urinary tract infection and discharged home. Urine cultures later showed no evidence of bacterial growth.
Four days later, she returned to the ED due to low-grade fevers, weakness, and persistence of urine discoloration. Physical exam was notable for scleral icterus and jaundice. Her hemoglobin decreased to 8.9 g/dL with total bilirubin 2.9 mg/dL, direct bilirubin 0.7 mg/dL, AST 84 (<36 U/L), ALT 42 (<46 U/L), alkaline phosphatase 75 (38–126 U/L), reticulocytes 4.4 (0.7–2.8%), lactate dehydrogenase (LDH) 2,397 (100–220 U/L), and undetectable haptoglobin. The peripheral smear showed polychromasia, occasional nucleated red blood cells but no schistocytes or red blood cell agglutination. Urine was amber-colored containing a large amount of blood, moderate amount of bilirubin and urobilinogen <2.0 mg/dL. Urine staining for hemosiderin was negative twice. Coagulation studies were normal. The direct antiglobulin test (DAT) was positive for C3d and negative for IgG. Testing for cryoglobulins and cold agglutinins was negative. Mycoplasma serology was positive for IgG and negative for IgM. Testing for HIV, hepatitis A, B, and C, Epstein–Barr virus, and antinuclear antibody was all negative. Glucose-6-phosphate dehydrogenase assay was adequate. RSV nucleic acid was detected via real-time polymerase chain reaction testing from a nasopharyngeal swab (Xpert® Xpress Flu/RSV). Testing for the Donath–Landsteiner (DL) antibody was positive, confirming the diagnosis of paroxysmal cold hemoglobinuria.
Management consisted of warming the patient and avoiding exposure to cold temperatures. She did not require any transfusions and by hospital day two, her hemoglobin stabilized and urine discoloration resolved over the ensuing two weeks. Two months after initial presentation, the repeat DL test was positive, along with DAT positive for C3d and negative for IgG. The follow-up hemolysis panel was notable only for mild elevation of LDH (245 U/L) with normal haptoglobin and reticulocyte count. Six months after initial presentation, the DL test was negative and hemoglobin was normal.
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pmc-6257903-1
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The patient was a 49-year-old woman with a body mass index (BMI) of 40.8 kg/m2 who underwent a laparoscopic sleeve gastric resection on May 2018 in our centre. She did not take drugs, and had no history of significant diseases. Six days after surgery, she presented with tachycardia, dyspnea and fever (38 °C). The abdomen was tender, without signs of peritonitis. Blood tests revealed an increased white blood cell count of 11.84 migl/mmc, a C-reactive protein level of 10.23 mg/dl and a procalcitonin level of 0.74 ng/ml. A TC scan of the abdomen was performed after oral administration of water-soluble contrast medium, and it was suggestive of a proximal staple-line leak with abdominal collection in left hypochondrium and left lumbar. A surgical laparoscopic management was decided: two abdominal drain tubes were placed, and a 8.5 Fr, 2-cm lenght double-pigtail stent was endoscopically delivered by our reference endoscopists through the fistula orifice into the collection (). A repeat scan after administration of oral water-soluble contrast performed 10 days after endoscopic procedure revealed the presence of pigtail drainage with an endo-luminal end and an extra-luminal end, with an associated blind-ending cavity of about 3 cm. About 20 days after pigtail placement, a control CT scan was performed, which showed the external tip of the tube in close proximity to the medial side of the spleen, with partial intra-splenic displacement, but without parenchymal or vascular damage. The presence of air in the subcapsular region confirmed intrasplenic displacement of the drain (). At that time, the patient was completely asymptomatic and underwent endoscopic pigtail removal, without any bleeding. The last CT scan confirmed no laceration of the splenic parenchyma and no vessel injury or extravasation of contrast medium. At a 3-month follow-up the patient presented no further complications.
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pmc-6258146-1
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A 72-year-old man presented with a history of multiple resections of CSCC located in his face, back and upper extremities. Almost 2 years subsequent to the latest excision, he first noted a palpable right axillary mass. A computed tomography (CT) of the chest revealed a 10 cm right axillary nodal conglomerate, additional supraclavicular lymphadenopathies and pulmonary nodules concerning for metastatic disease. These findings were subsequently confirmed by an FDG-PET/CT, and a right axillary mass biopsy was consistent with moderately-differentiated CSCC. First-line therapy with cisplatin 75 mg/m2 combined with 5-fluorouracil 1000 mg/m2 days 1–4 was recommended, and accompanied by significant gastrointestinal toxicity (diarrhea/mucositis). Re-staging scans revealed a reduction in size of the previously appreciable lesions. A salvage right axillary lymphadenectomy was attempted at an outside institution, revealing involvement by CSCC of 15 out of 16 resected lymph nodes. Early disease progression led to the indication of second-line therapy with carboplatin AUC 5 and paclitaxel 175 mg/m2, given every 21 days. Following cycle 6, repeat imaging showed disease progression in the lungs and multiple lymphadenopathies: right axillary, cervical, retropectoral and mediastinal. Of note, no signs of bone involvement were noted, despite degenerative findings and a previously described fracture attributed to osteoporosis.
At that point, the patient transitioned his care to our service. Following extensive debate at a multidisciplinary tumor board, decision was made to proceed with third-line nivolumab. After a thorough discussion and clarification regarding the off-label use of the anti-PD-1 agent, the patient received the first dose of nivolumab 3 mg/kg given intravenously every 14 days. Except for non-limiting fatigue, the patient had a remarkable tolerance to nivolumab, accompanied by early clinical response (reduction of right axillary/retropectoral mass). Re-staging PET-CT performed after 3 months of therapy revealed reduction in size and resolution of the metabolic activity of right axillary, cervical, retropectoral and mediastinal lymphadenopathies; pulmonary nodules were no longer appreciable. However, new areas of hypermetabolism were noted in C7, T1, T6 and L1 vertebral bodies, along with new lytic lesions, as well as increased metabolic activity in T3, T4 and T7 pedicles (Figs and ).
Due to the limited treatment options in this setting and remarkable response of visceral/nodal lesions, and based on a shared decision, continued treatment with nivolumab followed by short-interval repeat (6–8 weeks) scans was recommended. The patient decided to proceed with his treatment locally, at an outside facility, and returned for a reevaluation almost 5 months later, still receiving nivolumab and with no new adverse events. A new PET-CT showed metabolic response in the previously identified bone lesions, now described as sclerotic, and sustained control of pulmonary nodules and lymphadenopathies (Figs and ). Following more than 12 of nivolumab therapy, the patient continues to endure clinical benefit, with prolonged disease control and good tolerance.
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pmc-6258150-1
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The 32-year-old female attended the outpatient clinic of our hospital in June 2017. She complained of blurred vision for the past 2 years in her left eye. The patient had previously undergone the operation for cerebellar hemangioblastoma 3 years ago. Family history revealed that the patient’s mother had blindness in one eye and died of renal carcinoma. Upon initial examination, the patient’s BCVA was NLP OD and 0.04 OS. Her intraocular pressure was 39 mmHg OD and 17 mmHg OS. Fundus examinations showed several retinal hemangioblastomas in the temporal area of the left eye. Retinal traction detachment was noted due to the formation of proliferative membranes connected to the hemangioblastomas (Fig. ). Because of a complicating cataract, the fundus was not visible in the right eye, and B ultrasound scan showed proliferative vitreoretinopathy. She underwent PPV, hemangioblastoma resectomy and oil temponade in the left eye. BCVA improved to 0.2 OS postoperatively.
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pmc-6258161-1
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A 50-year-old woman native of Haiti presented to the Cayenne hospital in French Guiana with complaints of persistent fever, mild cough, asthenia and anorexia. She reported medical consultation three months before for febrile nasal obstruction and cough treated by amoxicillin-clavulanic acid without ameliorations. She lived in French Guiana for 20 years.
Diagnosis of HIV infection was further achieved, showing a high viral replication level (HIV viral load = 7,300,000 copies/mL) along with a deeply immunocompromised status (CD4 count = 22 cells/mm3). A full-body computer tomography scanner (CT scan) showed a complete opacification of right maxillary sinus filled with flocculent calcifications (Fig. ). This typical aspect along with an absence of osteolysis or osteocondensation was evocative of an Aspergillus spp. fungus ball. Additionally, CT scan showed bilateral inferior alveolar opacities (also visible during chest radiography), hepatosplenomegaly and disseminated lymph nodes up to 2 cms.
Diagnosis protocol included for microbiological examinations: sputum, bronchoalveolar lavage (BAL), maxillary sinus puncture, bone marrow, osteomedullar, node and hepatitis biopsies. Potassium hydroxide (KOH) direct examination and May-Grünwald Giemsa (MGG) stained smears were negative except for the sputum and the BAL. They showed an association of yeast and a gram-negative bacterium further identified in culture as Candida albicans, Klebsiella pneumonia and Pseudomonas fluorescens. Histopathology examination including Gomory Methenamine-Silver Grocott (GG) and Periodic Acid Schiff (PAS) staining have been practiced on the liver, the osteomedullar biopsy and the BAL fluid and were negative. Serologic analysis revealed no detection of Aspergillus spp. galactomannan antigen nor specific antibodies for Histoplasma or Leishmania.
Patient suddenly developed a respiratory distress syndrome along with a severe sepsis-like infection, after a prolonged well-supported fever. Antifungal therapy with Voriconazole was introduced due to presumption of an Aspergillus spp. maxillary infection that may be associated with pulmonary aspergillosis. Antimicrobial therapy with Piperacillin-tazobactam was introduced to cover a potential bacterial pulmonary infection (Klebsiella spp. and Pseudomonas spp.). Finally, the patient rapidly died of a respiratory distress syndrome, three days after the beginning of the pharmacological treatment and before the H. capsulatum infection was diagnosed.
Indeed, H. capsulatum was isolated from the sinusitis aspirate and from cervical adenopathy biopsy after 20 and 15 days of culture, respectively using Sabouraud/chloramphenicol/gentamicin agar media at 30 °C. The other fungal cultures (bone marrow, liver biopsy, blood, urine, BAL and expectorations) remained sterile after 2 months of incubation. Retrospectively, after a slide discoloration, a GG staining was performed on sinus aspirate and allowed to identify ovoid 2–4 μm yeasts evocative of Histoplasma capsulatum var. capsulatum, previously not visible during MGG staining (Fig. ). Additionally, a H. capsulatum real-time PCR analysis developed by the Academic Laboratory of Parasitology and Mycology, Cayenne Hospital, French Guiana [] was retrospectively performed on expectorations, BAL fluid and node biopsy and tested positive, confirming the diagnosis.
Several underlying pathologies have been revealed during post-mortem analysis. First, lymphoma diagnostic was evocated following direct examination of bone marrow, osteomedullar and node biopsies and was confirmed as a B lymphocyte-depleted Hodgkin’s disease by immunophenotyping (CD20+ CD3+ CD30+ ki67+). Then, disseminated Mycobacterium avium infection was diagnosed by PCR on positive culturing of BAL, bone marrow, liver biopsy and node biopsy.
A search for published cases of H. capsulatum sinusitis was conducted in PubMed database using the keywords “Histoplasma capsulatum”, “sinusitis”, “rhinosinusitis”, “histoplasmosis”. Only cases in which imaging confirmed sinusitis and H. capsulatum was documented by histopathologic direct examination or culture were included in the review. Studies in any language were considered and reviewed. Extracted data included age and gender, patient origin, underlying disease, clinical signs, symptoms duration, site of infection, direct examination and culture results, CT scan images of the sinus, treatment and outcome.
Eight articles reporting 8 cases of H. capsulatum sinusitis from 1993 to the present were included. These cases are summarized in Table [, –].
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pmc-6258364-1
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This is a case report of a 41 year old male patient who suffered a knife inflicted injury to the face. He was referred to our department 20 months after the incidence complaining of persistent discomfort with downward gaze of the left eye. He reported being agitated and feared a possible sight-threatening condition after the alleged assault. He was transferred to an emergency department where he was examined by an ophthalmologist who reassured him that his globe was intact and sutured the deep cut wounds related to his upper and lower eyelids. He was reassured that the facial edema would subside and the neurosensory deficit he was suffering from would eventually recover. Empirical antibiotics, Analgesia and tetanus prophylaxis were prescribed and the patient was discharged from the emergency department. He was unsatisfied with the resultant scar in his left eyelids and six months later was re-examined by a plastic surgeon that performed blepharoplasty. The persistent complaint of discomfort related to his left eye led him to seek medical advice again before being referred to our department.
Systematic thorough clinical and radiographic examination was performed. Upon inspection, it was noted that the left globe was displaced superiorly with increased scleral show (a). Bimanual palpation was done and a hard object could be felt behind the orbital rim of the left eye; although it was not visible externally. No evidence of diplopia was noted by ophthalmological examination. Plain radiography (Paranasal sinuses view (P.N.S) and lateral skull view) revealed a radiopaque foreign body (FB) lodged inside the orbital floor and directed downward & posteriorly (b and c). Computed tomography (CT) scan was ordered to precisely localize the foreign body and its relation to the surrounding structures. The foreign body was found penetrating the facial skeleton at the level of the orbital floor and transgressed the maxillary antrum and its tip reaching the pterygoid plates of maxilla (d–f).
Management of this type of injury has no formally documented guidelines in literature. The decision for surgical removal of this foreign body was made based on the patient's chief complain, general surgical principles and facial esthetics. An extraoral approach under general anesthesia was chosen and a preoperative informed consent was obtained. The orbital floor was exposed through a subciliary incision and the intraorbital portion of the foreign body was found firmly anchored to bone. A vertical osteotomy from the orbital rim to the anterior wall of the maxillary sinus was performed to avoid extraction of the object along its path of insertion. A wire-twister was used to firmly grasp the foreign body which was retrieved without any significant bleeding and was found to be a stainless steel knife blade (a). The orbital floor and rim were carefully evaluated and the need for internal orbital reconstruction was found unnecessary.
At that point it was noted that a portion of the foreign body, considered the blade tip, was not retrieved. A C-arm was used in an attempt to guide its localization and removal but provided insufficient information. The risks of proceeding with a blind surgical approach in the complex maxillofacial region outweighed the benefits of removing the retained foreign body and the decision to terminate the surgery was made. The possibility of returning at a later stage for a more extensive procedure after obtaining a new patient consent and the required radiographs was considered. The patient was discharged the same day of the operation and was regularly monitored in the first two weeks postoperatively (b–d).
At the first and third months of the follow up period, the patient has made rapid recovery with no signs or symptoms of infection. At 1 year of postoperative follow up, clinical examination showed normal symmetrical eye position and no ectropion (a) as well as improvement in the neurosensory deficit. At 2 years of postoperative follow up, radiographic examination showed bone healing of the previous defect at the orbital rim (b). It also demonstrated that the impacted portion of the foreign body has remained lodged in the exact same position (c). The option of leaving or retrieving the foreign body was presented again to the patient but he ensured us to be extremely satisfied with the outcome and refused to proceed with further surgical procedures.
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pmc-6258389-1
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A two-year-old girl was referred to our institute due to incidentally discovered hydronephrosis. She was investigated for abdominal pain, and abdominal ultrasound revealed SFU grade 4 right hydronephrosis without clear hydroureter (Fig. -a). Initially, vesicoureteric reflux was excluded based on a normal VCUG. A MAG3 renal scan revealed a hydronephrotic right kidney with reduced global cortical uptake, no response to Lasix, and split renal function of 32% on the right side. The patient was admitted electively, and right RGP showed right mid-ureteral stricture with a length of 1 cm (Fig. -b). Subsequently, the patient underwent laparoscopic excision of the stricture segment and ureteroureterostomy (Fig. -c, d). A pathological report indicated predominant sever chronic inflammation with foreign body giant cell infiltration of the ureteral wall with severely stenosed lumen. Right RGP was performed at the time of stent removal and showed smooth passage of contrast media up to the pelvicalyceal system (Fig. -e). An ultrasound examination performed 30 months postoperatively revealed SFU grade 1 hydronephrosis.
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pmc-6258392-1
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A female, 16-year-old, Standard Poodle was presented at a private veterinary clinic. The owner reported several changes in the mental status 1 year prior to clinical examination. These changes were recognized as confusion and reduced awareness, impaired recognition of human family members and loss of previously learned abilities. Two days prior to clinical examination, the animal presented with a generalized tonic-clonic seizure. Subsequently, the animal presented with a poor appetite and lethargy, with circling and ataxia.
The neurologic exam revealed acute right-sided abnormalities characterized by postural reaction deficit, circling, and head and neck turn in addition to left-sided head tilt, positional nystagmus, and ataxia. The owner declined to perform a magnetic resonance imaging exam.
The clinical signs evolved to lateral recumbency and the dog was unable to feed itself, characterizing a poor prognosis. Therefore, the dog was euthanized and submitted for necropsy at the Department of Pathology of the School of Veterinary Medicine and Animal Science (FMVZ), University of São Paulo (USP). The brain was entirely fixed in 10% buffered formalin and routinely processed for histology. Sections of the brain were stained with hematoxylin and eosin, and were also submitted for immunohistochemistry for detection of APP and glial fibrillary acidic protein (GFAP). Briefly, several sections of CNS were submitted to antigen retrieval with citrate buffer pH 6.0. Then, the slides were incubated with primary antibodies anti-APP (Millipore, Darmstadt, Germany) and anti-GFAP (Dako, Agilent, Santa Clara, California, USA) diluted at 1:200 and 1:14000, respectively. The antigen-antibody binding was visualized using EnVision FLEX System kit (Dako, Agilent, Santa Clara, California, USA), according to manufacturer’s instructions. As a positive control for APP staining, a brain of dog with traumatic brain injury with axonal degeneration was used. A brain of a young dog without significant histological lesions were used as negative control for APP labeling.
Grossly, the lateral ventricles were mildly distended, with flattened gyri and mild widening of sulci.
Histologically, intraparenchymal and leptomeningeal blood vessels from many areas of CNS, including the myelencephalon up to cerebral cortex were mildly to severely thickened by an acellular and hyaline material. On the right paramedian region, extending from the thalamus, at the level of diencephalon and mesencephalon junction to the oculomotor nucleus, at the level of rostral colliculus, there was a locally extensive, well demarcated, lacunar area with marked loss of neuroparenchyma tinctorial properties with coagulative necrosis surrounded by a rim of severe spongiosis (Fig. ). Within the area of necrosis, multifocally, neurons had a pyknotic nuclei and hypereosinophilic cytoplasm (neuronal necrosis). There was also necrosis and degeneration of glial and endothelial cells, characterized by pyknotic and karyorrhectic nuclei (Fig. ). The perivascular compartments of surrounding vessels were infiltrated by a low number of histiocytes (Fig. ). Within the area of spongiosis there was a large number of swollen axons forming axonal spheroids (axonal degeneration). GFAP immunolabeling showed an increase in the number, complexity, and thickness of astrocytic process (astrogliosis) surrounding the necrotic lesion (Fig. ). Within the necrotic area, the astrocytic processes were not evident. GFAP was mildly detected within degenerated and necrotic astrocytes inside the necrotic area. These findings are compatible with an acute area of ischemia/infarction with initial reactive gliosis with minimal inflammation.
Multifocally, lacunar areas of subacute infarction were visualized within the cerebellum, mainly involving the granular layer and adjacent white matter (Fig. ). The areas were characterized by a high number of granular cells with pyknotic and karyorrhectic nuclei, with liquefaction of neuropil, admixed with a moderate number of foam macrophages (Gitter cells) and axonal spheroids (Fig. ). These areas were surrounded by a moderate number of new-formed blood vessels with hypertrophied endothelial cells (neovascularization) with adjacent areas of hemorrhage and mild perivascular cuffing containing histiocytes. Prominent astrogliosis, demonstrated with GFAP immunolabeling, involving also the Bergman glia were visualized at the periphery of these necrotic areas (Fig. ).
Chronic lacunar areas of ischemia were also present within the telencephalon at the level of basal nuclei and hippocampus. These areas were pallor, well demarcated with neuron loss and with a marked proliferation of capillaries with hypertrophied endothelial cells. There was a mild to moderate spongiosis with an increase in the number of glial cells (gliosis), including a moderate number of cells with an abundant eosinophilic cytoplasm and an eccentric nuclei (gemistocytes). Rare perivascular cuffing of small number of histiocytes were visualized. Within these areas there was a marked increase in the number of astrocytes (astrocytosis) and astrogliosis as demonstrated by GFAP immunolabeling (Fig. ).
In additional to the areas of ischemia/infarcts, within the medulla at the level of obex, there were multifocal areas of hemorrhage within the reticular formation and several nuclei (Fig. ). The white matter of many areas, including telencephalon to the myelencephalon, were variably degenerated.
APP immunolabeling were visualized more frequently within the wall of intraparenchymal blood vessels (Fig. ), and occasionally affecting leptomeningeal vessels. Intensity of APP immunolabeling varied from mild to severe, according to Attems []. APP affected vessels from many areas of the CNS, but had a patchy distribution, characterized by APP-positive areas with adjacent APP-negative areas. Frontal lobes and temporal lobes were more affected when compared to occipital lobes. Other affected areas included the hippocampus, diencephalon, mesencephalon and myelencephalon. The cerebellum was less affected. Rarely, vessels with dyschoric change were observed, which consists in additional spreading of APP from vessels to adjacent neuropil (Fig. ). Importantly, a large number of hyalinized vessels weren’t immunolabeled for APP, especially those with a higher wall thickness. Within the periphery of the acute infarction, swollen and degenerating axons were immunolabeled for APP (Fig. ).
At the level of rostral commissure (basal nuclei) and thalamus there are locally extensive areas where a multifocal staining for APP was visualized within the neuroparenchyma. Within these areas, two types of APP staining were seen. Small, up to 25 μm in diameter, areas of staining containing a round, dense and darkly stained central region (Fig. ) were visualized in addition to other labeled areas, in which a central darkly stained core was not present (Fig. ) [].
The brain of a young dog used as negative control didn’t exhibit APP immunostaining within CNS vessels or within the neuroparenchyma (Fig. ).
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pmc-6258396-1
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A 60-year-old Asian (Chinese) woman complained of discovering a cervical pulsatile mass. She also complained of dysphagia and dyspnea symptoms. She has a history of Meniere’s disease.
When admitted in the vascular surgery ward, her vital signs were stable and she complained of dizziness. A solid pulsatile 4 × 4 × 5 cm mass could be palpitated at the right cervical and supraclavicular fossae. There was no tenderness of the mass. The pulse of her right carotid artery and right branchial artery were normal. Her body temperature was 36.7 °C, heart rate was 108/minute, respiration rate was 20/minute, and oxygen saturation was 98% at administration. Bilateral upper extremity blood pressure was equal at 132/70 mmHg. The laboratory findings were white blood cells (WBC) 10.01 × 109/L and neutrophils 6.92 × 109/L; a liver function test revealed: aspartate aminotransferase (AST) 10.8 μmol/L, albumin 39.7 g/L, and D-dimer 0.92 μg/mL. There was no special family history or other genetic information of our patient. She has no history of smoking tobacco and alcohol consumption. There was no special prior social, employment, and environmental history of this patient.
Computed tomography angiography (CTA) showed a pseudoaneurysm at the right cervical area (Fig. ). Contrast came from the initial location of right common carotid artery. The rupture was located on bifurcation of right common carotid artery and right subclavian artery. The diameter of the pseudoaneurysm was 4 cm. Her esophagus and trachea were slightly compressed by the pseudoaneurysm. Digital subtraction angiography (DSA) confirmed that the rupture area of the pseudoaneurysm came from innominate artery bifurcation. The artery tear lesion had a diameter of 0.5 cm (Fig. ).
Before the intervention, a monitor was used to make sure our patient’s vital signs were stable. Cardiac type B ultrasound evaluated that her cardiac function was normal. No special drug was given to her. After the preoperative preparation, the endovascular treatment was arranged in the catheter theater of our hospital. She was in a supine position. We successfully punctured her right femoral artery and right branchial artery. Then two through accesses were built using Amplatz guidewires: one was from right femoral artery to right carotid artery and the other was from right branchial artery to ascending aorta. In order to keep right carotid artery and right subclavian artery patent, two covered stent grafts from guidewire accesses were placed into innominate artery bifurcation. The two Fluency® Plus covered stent grafts (Angiomed GmbH &Co Karlsruhe, Germany) of 12 mm × 6 cm and 12 mm × 8 cm were pre-positioned in innominate artery partially, also in right carotid artery and right subclavian artery partially. A “roadmap” angiography made the delivery process precise. The two pre-positioned stent grafts were delivered and they were partially “kissing” in innominate artery. The other part of the two stent grafts was in distal site of right subclavian artery and right carotid artery separately. Balloon expansion was performed in both stent grafts. DSA after the stent grafts deployment showed the pseudoaneurysm was repaired, no contrast went into the pseudoaneurysm anymore.
Two weeks later, CTA showed that the two stent grafts were in satisfactory position, both the right carotid artery and the right subclavian artery were patent (Fig. ). No contrast went into the pseudoaneurysm. No pulse of the cervical mass could be palpitated after the procedure was finished. There was no need to resect the mass because the compressive symptoms had disappeared. No infection was noted at the lesion area when she came back 1 month later. Her symptoms of dysphagia and dyspnea were relieved 1–2 weeks later. She was alive and well at 1-year follow-up. She had no special complaints and she refused another CTA when we communicated with her by telephone.
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pmc-6258450-1
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A 49-year-old male presented to the urology clinic after incidental detection of a renal mass. He was asymptomatic, without any hematuria, flank pain, constitutional symptoms, or prior urological history. His past medical history was remarkable for morbid obesity, hypertension, atrial fibrillation, asthma, osteoarthritis, and gastro-esophageal reflux disease. His only prior surgery was a pannulectomy. He reported no relevant family history. Physical examination was unremarkable, except for an obese abdomen and a large ventral hernia. Patient weighed 400 lbs., having previously weighed 500 lbs. His bloodwork showed a hemoglobin of 131 g/L, creatinine of 96 umol/L, and eGFR of 80 ml/min/1.73m2.
A CT scan of the abdomen was done as part of a workup for abdominal pain. This revealed a large exophytic heterogeneous mass measuring 9.1 × 9.1 × 10.5 cm in the lower pole of the left kidney (Fig. ). There was no lymphadenopathy, regional invasion, or distant metastases seen. Bilaterally there were renal cysts without hydronephrosis or hydroureter. A pre-operative CT scan of the chest and bone scan were both negative for metastatic disease. A renogram showed that the large left renal mass was poorly functioning and that there was significant tubular dysfunction affecting both kidneys symmetrically. The function was estimated as 43% on the left and 57% on the right. Review of CT with urology and radiology was suggestive of T2A, N0, M0 renal cell carcinoma. Because of the high likelihood of RCC diagnosis, pre-operative biopsy was offered to the patient, but felt to be unnecessary.
Four months after presentation, he underwent an uncomplicated open left partial nephrectomy. He recovered expectantly post-operatively. The tumor was confined to the kidney with negative surgical margins; pathological stage was pT2a, Nx, Mx.
Since the patient’s surgery, he has been seen in follow up every 6 months with CT imaging. To date, he has remained without evidence of any local or distant tumour recurrence.
Gross examination revealed a firm-to-rubbery 10 cm tumor located in the lower pole of the left kidney. The tumor was tan brown with areas of hemorrhage and a pale yellow scarred area measuring 3.2 cm.
Microscopic examination showed a solid renal tumor. The cells were intermediate to large in size with partially vacuolated eosinophilic cytoplasms. The nuclei were round (non-resinoid) and without prominent nucleoli or apparent perinuclear halos. The tumor was classified as ISUP nucleolar grade 1 of 4. (Fig. ). There was no necrosis, sarcomatoid change or increased number of mitotic figures. The tumor cells stained positive for PAX8, AE1/AE3, CAM 5.2, p504S, and EMA. The tumor cells were negative for SDHB, CD117, CK7, CK20, CD10, vimentin, RCC, S100, HMB-45, Melan-A, myogenin, SMA, calretinin, inhibin, DOG1, E-cadherin, and CD56 (Fig. ).
The absence of SDHB staining by immunohistochemistry confirmed SDH -deficient RCC. Most individuals with SDH -deficient RCC have underlying germline mutations in one of the SDH genes. The patient subsequently underwent genetic counselling and germline mutation analysis of the SDH genes was carried out. This revealed a pathogenic mutation in the SDHB gene.
Since there is an increased risk of paragangliomas and pheochromocytomas in SDHB mutation carriers, surveillance for these neoplasms was carried out. A baseline CT scan of the neck/chest/abdomen/pelvis, utilized as the patient’s body habitus precluded MRI scanning, revealed no significant abnormalities aside from post-operative changes post partial nephrectomy. Similarly, baseline 24-h urinary collection for metanephrines and catecholamines was within normal limits. He will continue to undergo annual or biennial biochemical and radiographic surveillance for PC and PGL. Additionally, genetic testing has been offered to family members, who are at risk of inheriting the SDHB mutation.
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pmc-6258560-1
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Patient 1: A 53-year-old woman was diagnosed with stage IV lung adenocarcinoma (T3N2M1). CGP detected an EGFR exon 19 deletion and she was started on afatanib and had a partial response. After progression at 8 months, CGP was performed on a new biopsy and showed an EGFR exon 19 deletion and T790M mutation. She started osimertinib and had a partial response but had progression at 5 months. CGP on a new biopsy found an EGFR exon 19 deletion and C797S mutation, but the EGFR T790M mutation was not detected. She was started on gefitinib and osimertinib and tolerated it well. Follow up at 3 months showed a mixed response with 2 lesions progressing, 2 lesions stable and no new lesions. At 6 months, new metastases were observed. Further CGP testing on a pleural fluid sample at 6 months found the original EGFR exon 19 deletion with no EGFR T790M or C797S mutation, but instead a C797N mutation.
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pmc-6258560-2
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Patient 2: A 41-year-old man was diagnosed with stage IV lung adenocarcinoma with metastases to the brain. Genomic testing revealed an EGFR exon 19 deletion. The patient was treated with afatinib for 23 months until progression. Liquid biopsy testing found the EGFR exon 19 deletion in addition to an EGFR T790M mutation and EGFR amplification. The patient was started on osimertinib and responded well, but progressed after 8 months. The patient stopped osimertinib treatment and started on a clinical trial with a cMet inhibitor. At that time, CGP found the original EGFR exon 19 deletion, an EGFR C797S mutation not detected in previous tests, and no evidence of the prior EGFR T790M mutation.
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pmc-6258717-1
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A 71-year-old man with a history of chronic lower extremity deep venous thrombosis (DVT), pulmonary emboli, and pulmonary hypertension on home oxygen and Ventavis® (iloprost) presented for PTE. During the rewarming period after PTE, bright red blood was noted in the endotracheal tube. A bronchoscope was used to suction 750 ml of blood from the airway. After an unsuccessful attempt to place a left sided Arndt endobronchial blocker (Cook Medical, Bloomington, IN,USA) with a standard adult bronchoscope the decision was made to attempt to exchange the single lumen 8 mm endotracheal tube (ETT) to a 41F left sided double lumen tube (DLT). The initial DLT exchange over a tube exchanger was unsuccessful. The patient was extubated and a DLT 41F left sided tube was placed under direct visual laryngoscopy and position confirmed with a 5.2 mm distal tip diameter bronchoscope. Approximately 1L of blood was suctioned from the left endobronchial lumen. Single right lung ventilation was initiated and separation from cardiopulmonary bypass (CPB) was successful. Prior to transfer to the cardiac surgical intensive care unit (CSICU), the left lung bleeding slowed and two-lung ventilation was initiated. Respiratory failure in the post-operative period resulted in a need for veno-venous extracorporeal membrane oxygenation (VV ECMO). The bleeding stopped overnight and the DLT was exchanged for a standard ETT. The patient developed acute respiratory distress syndrome (ARDS) and multi-organ failure. Comfort measures only were initiated, and the patient expired on postoperative day (POD) 9.
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pmc-6258717-2
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A 71-year-old man with a history of CTEPH presented for PTE. During the rewarming process following PTE, bright red blood was noted in the airway. Bronchoscopy revealed bleeding from the right lung. Placement of a right sided Arndt endobronchial blocker (Cook medical, Bloomington, IN, USA) was attempted but was unsuccessful due to copious bleeding. The 8.0 mm single lumen ETT was exchanged for a size 9 mm single lumen ETT. Then a right sided 9Fr Arndt endobronchial blocker was placed to contain the bleeding with the aid of a 6.0 mm outer diameter therapeutic bronchoscope. The weaning from CPB continued successfully, and the patient was transferred to the ICU. The patient's postoperative course was complicated by renal failure, reintubation for hemoptysis, and poor neurologic recovery. Due to the multiple complications, comfort only measures were initiated and the patient expired on POD 20.
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pmc-6258717-3
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A 54-year-old woman with past medical history of COPD and CTEPH presented for PTE. During the CPB weaning process high airway pressures were noted along with bright red blood in the airway. Bronchoscopy noted significant blood coming from the right bronchus. Initial placement of an Arndt endobronchial blocker was attempted but unsuccessful. The 7.5 mm ETT was then exchanged for a size 8.5 mm ETT with the use of a Glidescope® video laryngoscopy (Verathon, Seattle, Washington, USA). After placement of the 8.5 mm ETT, a right sided Arndt endobronchial blocker was effectively positioned with a 6.0 mm outer diameter therapeutic bronchoscope. Significant pulmonary hypertension and hypoxemia remained and the decision was made to place her on venoarterial (VA) ECMO. The patient had a protracted complicated postoperative course. She was eventually weaned from ECMO but developed renal failure and ARDS. In conjunction with the patient's family wishes, comfort only measures were initiated and the patient expired on POD 12.
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pmc-6258717-4
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A 65-year-old woman with history of obesity, OSA, and CTEPH presented for PTE. During rewarming, bright red blood was noted in the ETT. Bronchoscopy demonstrated bleeding from the left lung. Initial placement of an Arndt endobronchial blocker and ETT exchange over a stylet were unsuccessful. The patient was extubated and direct laryngoscopy was utilized to place an 8.5 mm ETT. A 9Fr Arndt endobronchial blocker was then placed in the distal left main bronchus with the aid of a 6.0 mm outer diameter therapeutic bronchoscope. After removal of blood from the airway with bronchoscopy, two lung ventilation with low tidal volumes and PEEP was instituted. CPB could not be weaned due to poor oxygenation and hemodynamic instability so VA ECMO was instituted. Her postoperative course in the ICU was complicated by ongoing renal, hepatic, and right ventricular failure as well as ARDS. She expressed a desire for comfort measures only and expired on POD 25.
A synopsis of clinical factors is seen in Table and relevant clinical algorithm in Figure .
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pmc-6258873-1
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The present patient was a 45-year-old woman who exhibited no subjective symptoms. She visited the Takahashi Breast and Gastroenterology Clinic for breast cancer screening. A mass measuring 1.5 cm was palpated in the E region of the left breast. MMG showed a tumor shadow in the left S region. There was a high-density area inside the tumor, suggesting bone or cartilage formation, and a diagnosis of category 3 on MMG was made (a and b). US revealed a poorly demarcated tumor measuring 14.3 × 14.8 × 10.7 mm with relatively smooth margins and inhomogeneous high-echo content (a and b). Color Doppler US showed a hypovascular pattern (c). CNB was performed. The linear shadow passing from the right side transversely and slightly obliquely downwards, anterior to the tumor was the needle used in the biopsy (d). The needle passed through the solid tumor steadily and accurately (e).
The pathological findings of the CNB specimen indicated PA. The tumor showed proliferation of small cells forming glandular duct-like structures or anastomosed funicular structures, and the cells in the basilar portion were spindle-shaped and transitioned to the stroma. Cartilaginous metaplasia, calcification, and ossification were observed in some areas. Intraductal papilloma was also observed in some areas. The diagnosis of PA was made based on the histological picture of CNB (a, b, 3c).
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pmc-6259042-1
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A 63-year-old, G3Y3, postmenopausal, morbid obese woman presented to the outpatient clinics with the complain of vaginal bleeding. Physical examination was compatible with atrophy in vulva, vagina and cervix. Pap smear test was reported as negative. Transvaginal sonography revealed endometrial thickness of 12 mm. Following endometrial biopsy was reported as atypical, complex endometrial hyperplasia. Thus, laparoscopic hysterectomy and bilateral salpingo-oophorectomy and omental biopsy were performed. 10 mm telescope and advanced bipolar energy modalities were used during surgery. First, 10 mm trocar was directly inserted through subumbilical incision of 1 cm. laparoscope was inserted following insufflation of 3–4 l co2. Three 5-mm-trocars were inserted abdominally through 2 ipsilateral inguinal and single contralateral inguinal incisions. Rumi (R) II uterine manuplator was used for uterine manipulation. The frozen sections of hysterectomy specimen was compatible with a myometrial invasion more than 1/2. Therefore, bilateral pelvic lymphadenectomy was processed. We recognized that the right obturator nerve was transected accidentally by 5-mm Ligasure(R) during right obturator lymph node dissection (). It was seen that the nerve was transected in a full-thickness manner, besides thermal injury was occured at nerve ends. Following debridement (1 mm in size) of thermally injured areas,the nerve ends were reapproached (). An end-to-end reanastomosis without tension was performed by epineural sutures (4-0 polypropylene) via laparoscopy (, ). The duration of repair was 21 min, while overall operation session was 180 min. Volume of blood loss was 150 mL. No marked loss of adductor function was observed during early postoperative period. The patient was discharged on day 3, postoperatively. On month 2 of the operation, the patient reported numbness at medial aspect of thigh and minimal difficulty in climbing upstairs. Complete clinical recovery was detected on month 6. No neurological deficit was detected in the muscle group innervated by obturator nerve in her EMG at the end of the 6th month, postoperatively.
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pmc-6259044-1
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Herein we report the case of a Caucasian 50-year-old woman who had been operated in 2013 for a 18 mm breast invasive ductal cancer which was poorly differentiated.
The initial breast cancer was located in the external upper right quadrant; it was negative for hormone receptors, HER- 2/neu overexpressing, ki67 30%, and associated with lymphovascular invasion. First the patient underwent a right side quadrantectomy and lymphoadenectomy for a lymph node metastasis (3/10). Then she underwent a total body CT scan without contrast, because she was allergic to the contrast. Finally, a bone scintigraphy was also carried out. In the end, no body metastases were found. According to Saint Gallen criteria she was a pT1cN1aM0.
From a genetic test, the patient did not present a germ line mutation for BRCA 1/2.
After one month she started receiving adjuvant chemotherapy. The patient underwent a 4-cycle treatment with AC then taxolo treatment x 12 weeks and herceptin x 54 weeks. In addition to that she underwent local radiation therapy.
The follow up surveillance showed good general conditions until December 2017.
In January 2018 the patient reported about the appearance of local cutaneous nodes in the right breast, that were merely diagnosed as skin lesions. The increase of lesions despite the topical therapy made the patient decide to ask us for a second opinion. At the clinical assessment we assumed the presence of multiple cutaneous metastases in the breast.
The patient underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) for staging work-up. Owing to her allergy to the contrast, it was impossible to carry out a total body CT or breast MRI. The FDG PET/CT showed increased FDG uptake in the right breast, in the omolateral internal mammary chain, in the left axillary lymph nodes and left subclavicular lymph nodes.
Consequently, it was useful to perform a the FDG PET/CT and lymphoscintigraphy to detect unpredictable contralateral axillary lymph node metastases from a second primary breast cancer. Later in May, she underwent right rescue mastectomy and left mastectomy (the latter at her request and in order to discover an eventual CUP-syndrome) together with a left lymphadenectomy. But she refused recostruction with prosthesis.
The final pathological diagnosis revealed a right side poorly differentiated multiphocal breast invasive ductal cancer, the biggest lesion being 1 cm, with extensive dermal angiolymphatic diffusion. The tumor was ER/PR negative, Her-2 positive as before and ki67 was 10%.
It only revealed a cystic fibrosis in the left breast and no evidence of tumor.
As regards the left axilla the final pathological diagnosis showed a bigger and palpable lymph node and 2 additional subclavicular lymph nodes which were positive for metastases and 5 additional axillary lymph nodes which resulted negative. Prognostic factors in axillary lymph nodes were ER/PR negative, Her-2 positive and ki67 was 60%.
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pmc-6260197-1
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An 83-year-old male patient presented to the hematology-oncology clinic with the past medical history of small bowel obstruction status post resection, basal cell carcinoma of the skin, actinic keratosis and colon adenocarcinoma status post left hemicolectomy with negative postoperative surveillance for relapse by serial carcinoembryonic antigen levels and serial colonoscopies. He developed progressing pancytopenia. His laboratory workup revealed, hemoglobin 9.1 g/dl, platelet count 76,000/ul, white blood cell (WBC) count 2700/ul with 59% neutrophils, and absolute neutrophil count 1600/mm3. He had no reported past medical history of hematological disorders. No sign or symptom or any laboratory workup was indicative of systemic infection or inflammation. The patient’s home medications did not include any antimetabolite nor he was ever treated in the past with any antineoplastic agents or radiation therapy. The patient was a lifetime nonsmoker with occasional alcohol drinking. Vitamin B12 and folic acid levels were normal and HIV tests were negative, respectively. Bone marrow aspiration was performed which revealed myelodysplasia with ring sideroblasts (Figures -). Cytogenetic results delineated complex abnormal karyotype with monosomy of chromosomes 5, 7, 20, partial deletion of 5q, and abnormalities consistent with high-grade myelodysplasia. The patient did not give consent for the proposed treatment with low dose chemotherapy. Thus management was begun with weekly intravenous (IV) erythropoietin administration along with as needed blood transfusion.
Six months post-diagnosis, the patient was presented to the emergency department with the chief complaint of pain, swelling and redness of the left wrist-joint. He was afebrile and physical examination exhibited a swollen joint which elicited pain upon movement. X-ray of the wrist was evident for chondrocalcinosis (Figure ). Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were elevated. Lab workup revealed WBC count 1.8 x 10⁹/L with 8% bands. Taking into account his leukopenia and inflamed wrist joint, the patient was empirically started on antibiotics for the clinical suspicion of septic arthritis but a minimal improvement in the symptoms was noticed. Synovial fluid extracted from the radiocarpal joint was grossly turbid with WBC count 6897/mm3 and polymorphonuclear granulocyte 87%. Gram staining was unremarkable and fluid cultures were negative for microbial growth. Crystal analysis of synovial fluid demonstrated few positively birefringent rhomboid intracellular CPPD crystals. Treatment with IV steroid was begun which culminated in a dramatic improvement of symptoms. Antibiotics administration was sustained due to a possibility of coexisting septic arthritis in the clinical context of leukopenia. The patient was discharged home on a steroid taper and a short duration course of antibiotics. Post-discharge, outpatient workup remained negative for antinuclear antibody, rheumatoid factor and cyclic citrullinated peptide antibody.
A month later, the patient presented again with abrupt onset of right knee pain accompanied by joint swelling, tenderness and redness. X-ray indicated degenerative changes (chondrocalcinosis) of the knee joint most remarkable around the medial femorotibial compartment (Figure ). Levels for ESR and CRP were elevated. Intracellular CPPD crystals were identified on synovial fluid analysis. The patient was treated with steroids and antibiotics (due to persistent leukopenia). The patient improves clinically. Colchicine could not be started for prophylaxis of pseudogout due to persistent leukopenia.
A highly suspicious bout of pseudogout flared up again within a month with the symptoms of sharp lower back pain, localized to the lumbar area with redness surrounding overlying skin. The patient was afebrile. WBC count was 3500/ul. Degenerative changes were visible at L4-L5 on computed tomography (CT) spine with no contrast enhancing lesion identified on imaging (Figure ). Considering history of previous flares of pseudogout, the patient was started treatment empirically with steroid in addition to antibiotics. Upon improvement, he was discharged home. Considering the evidence-based benefits of azacitidine in abeyance of the autoimmune phenomenon in MDS, the patient was then eventually started on azacitidine by his hematologist. No further acute CPPD crystal-associated acute flares of arthritis have been noted till date since the initiation of the therapy with azacitidine.
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pmc-6260234-1
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This 39 year old woman presented in 2015 with abdominal pain and distention. By CT and biopsy, a diagnosis of signet ring adenocarcinoma of the appendix was made. She was taken for cytoreductive surgery plus HIPEC with mitomycin C on 10/23/2015. Following this she was treated with 12 cycles of FOLFOX chemotherapy. In the fall of 2017, she began noting abdominal tightness. In March 1, 2018, CT scans showed extensive ascites with tethering of the small bowel within the pelvis (). She experienced a 20 lbs weight loss. It was thought that her partial intestinal obstruction was due, at least in part, to the tense ascites. In March of 2018, she underwent multiple paracenteses followed by a single cycle of intraperitoneal chemotherapy with 5-fluorouracil. There was transient relief of symptoms but abdominal pain and vomiting recurred which led to an exploratory surgery on 03/14/2018. The abdomen was found to be frozen and gastrostomy placement was impossible because the stomach was fixed within the left upper quadrant as a result of her prior extensive cytoreductive surgery. Postoperatively, she required continuous nasogastric suctioning. On 03/26/2018, she underwent percutaneous placement of an esophagostomy tube by an experienced thoracic surgeon (PGK) []. A left-sided pneumothorax that occurred with the tube placement was treated by a chest tube. Throughout her hospitalization and on hospital discharge she was maintained on TPN. Intermittent pain was controlled with a Fentanyl transdermal patch (Janssen Pharmaceuticals, Inc., Titusville, NJ). This symptom management and nutritional support has continued for 4 months without incident while the patient searched for further treatment options. The patient died as a result of progressive disease on 05/02/2018.
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pmc-6260360-1
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A 60-year-old male patient was admitted in the Emergency department of our Hospital with symptoms of abdominal pain with 2 weeks of evolution and progressive worsening and acute low gastrointestinal bleeding. The patient denied nausea, vomiting, fever and weight loss. He didn’t have personal history of medical or surgical diseases. The patient also didn’t have any history of familiar malignant disease.
On initial clinical observation the patient was haemodynamically stable. The examination of the abdomen revealed a palpable painless mass in the periumbilical region, left hypochondrium and left flank. There was evidence of low gastrointestinal bleeding (hematochezia). The initial haemoglobin value at admission was 133 g/dl. An emergency non-total colonoscopy showed no gastrointestinal lesions that could cause the bleeding. An Angio-CT was requested and showed a large exophytic mass arising apparently from the 4th duodenal segment and first jejunal segment with approximately 20 × 14 x 13 cm, with vascularization provided from branches from the upper mesenteric artery, with vascular dilatations inside the tumor and apparent intratumoral bleeding, without intraperitoneal bleeding ().
The patient’s condition deteriorated, and he became haemodynamically instable, with new episode of low gastrointestinal bleeding (hematochezia). The haemoglobin value dropped to 8,6 g/dl, requiring fluid resuscitation and blood transfusion.
Due to haemodynamic instability the patient was submitted to emergency laparotomy, in witch a large tumor was found arising from the first jejunal segment (2 cm after duodenual-jejunal flexure) (e and ). A segmental enterectomy was performed, resecting the mass and adjacent jejunum and the 4th duodenal segment. A side-to-side manual anastomosis was performed between the 3rd duodenal segment and the jejunum ().
The post-operative period ran without complications. The patient started oral feeding at the 7th post-operative day, after performing a gastrointestinal oral contrast study that confirmed the integrity of the anastomosis. The patient was discharged at the 11th post-operative day.
The histopathologic exam of the surgical specimen showed a gastro-intestinal stromal tumor of epithelioid cell nature, with low mitotic count (<5 per 50HPF), significant mucosal ulceration, high vascularization and tumoral necrosis. The imunohistochemical analysis revealed positivity for CD117 (c-kit) and DOG-1. The tumor was categorized as T4N0 stage IIIA. A mutation on c-KIT was found in exon 9. According to size, location and mitotic count, the tumor was categorized as high-risk GIST.
The patient was evaluated by Medical Oncology and started Imatinib therapy (400 mg per day). The follow-up continues, and the patient is free of disease recurrence for 3 years.
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pmc-6260367-1
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A 28-year-old female, who was a childminder, injured her left knee during repeated deep flexion of the knee to comfort the children. She had a pain in the lateral side of the knee and standing-up was the most painful motion for more than 3 months. On physical examination, the knee was restricted in extension to 5° without instability, and the McMurray test [] was positive. X-ray showed lateral joint space widening compared to the right knee (a). Magnetic resonance imaging (MRI) showed a horizontal high signal cleavage throughout the body of the DLM (b, c).
After finding that there was no tear on the femoral side of the DLM and confirming its stability by a probe using two standard anterior portals (a), a minimal part of the central portion of the DLM was removed using an additional far-anteromedial portal (b, c). Then, the middle and posterior part of the inferior-leaf was removed with a meniscal punch, and the anterior part was removed with a shaver using an inferomeniscal portal (d, e) []. About half of the width of the remaining stable superior-leaf was preserved (f, Video). Finally, the knee was arthroscopically checked its smooth flexion and extension without clicking.
Postoperatively, the patient had free knee range of motion (ROM) and weight bearing. All activities were allowed at 1 month postoperatively.
Two years after the surgery, the patient had no pain and no restriction of ROM. X-ray showed slight narrowing of the lateral joint space, but no other degenerative changes (a). On MRI, the remaining superior-leaf maintained about half its width (14.0 mm) with no intrameniscal signal changes, and no progression of coronal/sagittal extrusion (b, c).
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pmc-6260367-2
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A 34-year-old female had felt pain in her right knee while running. She had a pain in the lateral side and running was the most painful motion for more than 3 months. On physical examination, knee extension was restricted to 10°. The McMurray test [] was positive without instability. X-ray showed no remarkable findings (a). MRI showed that the DLM had a horizontal high signal cleavage. Surgery and postoperative therapy were performed as in the first case (b–d).
Two years after the surgery, the patient had no symptoms while running, and had no restriction of ROM. X-ray showed slight narrowing of the lateral joint space (e), and MRI showed a thin lateral meniscus that had maintained its width (12.1 mm) with no progression of coronal/sagittal extrusion.
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pmc-6260368-1
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A 62-year-old man with NF1 was scheduled for elective surgical treatment of an asymptomatic, enlarged and diffusely thickened appendix that remained after curative antimicrobial treatment of acute appendicitis because he hoped for an antimicrobial treatment 2 months ago. Physical examination revealed multiple neurofibromas and café-au-lait spots on the skin. He had no other co-morbidities other than having NF1. Laboratory analysis revealed hemoglobin, 15.9 g/dL; white blood cell count, 5.33 × 103/μL; platelets, 17.9 × 104/μL; serum total protein, 7.9 g/dL; serum albumin, 4.8 g/dL; total bilirubin, 0.8 mg/dL; aspartate aminotransferase, 27 IU/L; alanine aminotransferase, 21 IU/L; and lactic acid dehydrogenase, 173 IU/L; C-reactive protein, 0.073 mg/dL. The serum levels of tumor markers were normal, including carcinoembryonic antigen, 3.2 ng/ml and carbohydrate antigen 19–9, 7.1 U/mL. Contrast-enhanced computed tomography (CT) demonstrated an enlarged and diffusely thickened appendix (a, b). Colonoscopy showed thickened appendiceal mucosa projecting to the cecum without evidence of abscess or jellylike liquid (). A sample of the appendiceal mucosa was obtained by colonoscopy and pathologically revealed to be benign. The patient was preoperatively diagnosed with treated acute appendicitis with chronic appendiceal inflammation versus appendiceal neoplasms. Laparoscopic cecectomy was performed. The resected specimen revealed a thick, enlarged and fibrotic appendiceal wall. There was no evidence of appendiceal rupture or serosal mucin extravasation (a). Histopathological examination showed a single layer of atypical mucinous epithelial cells lining the appendix (b). Multiple neurofibromas were observed in the muscle layer, submucosa and mucosa of the appendix (c). Micro plexiform neurofibromas were observed in the neuroplexus of the appendix. Immunohistochemical examination showed positive staining for S-100 (d). Pathologically, the patient was diagnosed with appendiceal neurofibroma of NF1 with LAMNs. His postoperative course was unremarkable. He was discharged on post-operative day 3 and remained in good heath 7 months after surgery.
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pmc-6260384-1
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A 64-year-old Caucasian man fell in a bathtub approximately 9 months prior to presentation. At the time of the injury, he suffered multiple left-sided nonunion rib fractures (4th to 9th) and was treated with a chest tube and analgesia. He had developed chronic pain from the injury to the point in which he was unable to sleep at night and heard a clicking sound every time he took a deep breath. On the CT scan of the chest, the rib fractures were displaced and on exam, the ribs were mobile with reproducible pain and clicks on palpation. The patient was taken to the operating room for an open reduction and internal fixation with MatrixRIB fixation plates (DePuySynthes, West Chester, PA, USA).
The procedure was performed by a board certified thoracic surgeon (MPK). On the day of the operation, the patient had a dynamic CT scan, with one scan taken with maximal inspiration and one scan taken with maximal expiration in the right lateral decubitus position with the left side up. The CT scan was imported into the electromagnetic navigation system software (Veran Medical Technologies Inc, St. Louis, MO). We first marked the rib fractures on the planning software as a target. The software demonstrated 2.2 cm rib fracture displacement between inspiration and expiration (A). Next, we identified the skin that was directly superficial to the site of the fractured ribs using the SPiN Perc™ protocol (B). Intraoperatively, a bronchoscopy was performed for electromagnetic calibration (A). This allows the accurate matching of the patient’s anatomy to the virtual anatomy seen on the CT scan. Then, cutaneous sites of all fractures were identified and marked with image guidance (B-C). These markings served as a guide for the incision’s location. After incision, the dissection was continued to the chest wall sparing the latissimus dorsi and serratus anterior. The rib fractures were readily identified with accurate correlation with the cutaneous sites (D). The fractures were freed from the bony callus and were fixed with MatrixRIB plates (DePuySynthes, West Chester, PA, USA) in a conventional fashion (E). A closed suction drain was left in the operative field. The patient recovered well postoperatively and reported immediate improvement in chest wall pain. He was discharged on postoperative day 2 with non-narcotic analgesics.
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pmc-6260389-1
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Patient one, a 3-month-old female patient, presented in March 2015. Her parents had noticed a rapidly growing maxillary swelling during the previous month. The patient’s medical history was insignificant. On examination, a firm swelling measuring 3 × 4 cm was detected on the anterior maxilla. The overlying mucosa was ulcerated in the middle, with a deciduous incisor exfoliating through the lesion ().
Multislice Computed Tomography (CT) revealed a well-defined osteolytic lesion encroaching on the right anterior maxillary wall. Incisional biopsy, performed by a team led by author FAM, confirmed a diagnosis of melanotic neuroectodermal tumour of infancy. Subsequently, a second surgery was performed in April 2015, with tumour excision via a transoral approach (, ).
Possibly due to the conservative nature of the surgical excision and/or tumour seeding, a recurrence of the lesion occurred four months later in August 2015. Via a Weber Ferguson approach, a right subtotal maxillectomy was performed to resect the recurrent tumour with a safety margin of 1 cm. Histopathology affirmed the diagnosis of MNTI. The patient’s subsequent recovery was uneventful; she has been followed up for over three years, with no incidence of recurrence clinically or radiographically (, ).
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pmc-6260389-2
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The second patient was a 4-month-old female infant, who presented to our unit in December 2015 after her parents noticed a progressively growing left maxillary mass of gradual onset.
On examination, a well-defined firm mass of the left maxilla was detected. The lesion was roughly 4 × 5 cm in size and smooth in texture, with an ulcer measuring 1 × 1 cm located at the lesion’s surface. Computed Tomography revealed an expansile lesion of the left maxilla with poorly-defined margins (, ).
An incisional biopsy revealed a diagnosis of Melanotic Neuroectodermal Tumour of Infancy. Histologically, the specimen showed groups of round cells with abundant cytoplasm and pale nuclei, surrounding nests of neuroblast-like cells possessing scant or fibrillar cytoplasm. Immunohistochemistry confirmed the specimen was positive for both HMB45 and Synaptophysin.
A thorough work-up was subsequently performed, including Computed Tomography of the chest, abdomen and pelvis to rule out any metastasis; this was negative for any tumor spread.
Via a Weber Ferguson approach, a surgical team headed by author ME performed a left subtotal maxillectomy and the tumour was excised with a safety margin of 1 cm. The surgical defect was closed primarily with the use of a buccal fat pad and no reconstructive procedure was taken (, ).
A follow-up CT was taken 18 months postoperatively, with no recurrence detected. Accordingly, a minor residual soft tissue defect in the left premaxilla was closed via a local flap in July 2017. The patient has been followed up for over two years following the MNTI excision, with no signs of recurrence clinically or radiographically ().
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pmc-6260394-1
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A 51-year-old man was admitted to our surgical unit with a diagnosis of chronic pancreatitis. He was suffering from episodes of continuous abdominal pain for the last one year. The pain was radiated from the right upper quadrant of the abdomen to the back and was associated with nausea, partially relieved by injectable analgesics and aggravated by food ingestion. This challenge led to the loss of appetite then to weight loss which also worsened by the presence of occasional episodes of malabsorption and the development of insulin dependent diabetes mellitus. There was no family history of the similar condition in parents, siblings or first-degree relatives.
Moreover, there was no history of abdominal trauma in the past. The patient had a history of admissions to different hospitals for the recurrent pain attacks. However, apart from this illness, he had never been to hospitals for any other medical or surgical condition. He was on analgesics, insulin therapy, and pancreatic enzyme supplementation and had never been allergic to the exposed medications.
The physical examination showed an emaciated man looking older than his chronological age, appeared pallor but no clinical evidence of jaundice. Abdominal examination was normal. Chest examination found no remarkable findings, and the rest of the physical examination was normal. The laboratory examination showed an increased level of blood sugar, normal levels of serum amylase, renal function tests and liver function test (LFT). No attempt was made to establish the insufficiency of the exocrine pancreatic function. Chest X-Ray and ECG were normal.
Plain radiology showed opacities in the middle and right upper quadrant abdomen (a). CT abdomen was performed to rule out other associated pathologies like pancreatic pseudocyst, pancreatopleural, pancreatogastric or pancreatocolonic fistulae as the surgical procedure would have been different in the presence of any of the complications. Contrast-enhanced abdominal CT scan revealed impacted stones at the ampulla within the distal bile duct and pancreatic duct. The remaining parts of the pancreas showed normal result with no associated features of chronic pancreatitis (b). Since the contrast-enhanced abdominal CT scan indicated impacted stones in the distal bile duct at the ampulla, MRCP was performed. Initial MRCP suggested stones within the main pancreatic duct (MPD) in the head of the pancreas and dilated common bile duct suggested stone in the distal part of it (c).
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pmc-6260394-2
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A female patient age 48-years-old was admitted with a diagnosis of obstructive jaundice with chronic pancreatitis. She was suffering recurrent abdominal pain in the back for the last eight months and recurrent jaundice in the last three months and diagnosed with hepatitis at a district hospital before referred to our hospital.
Physical examination found slight jaundice at the sclera and skin, with abdomen within normal limit. Chest examination found no remarkable findings. Laboratory results showed raised the level of blood sugar, but we found normal values of serum amylase, renal function tests and Liver Function Tests (LFT’s). Chest X-Ray and ECG were normal.
Plain radiogram (a) showed opacities and areas of dilatation in the region of the biliary tract and pancreatic duct. MRCP was performed, and the result suggested a radiolucent stone in the distal of the common bile duct and the duct of the pancreas (b).
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pmc-6260394-3
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Female, 60-year-old, was hospitalized with recurrent upper abdominal pain. The patient complained about right upper quadrant pain and recurrent yellowish of her eyes. One month previously, she underwent open cholecystectomy due to cholelithiasis. Physical examination revealed jaundice of the skin and sclerae and tenderness in her epigastric region. Laboratory tests showed total bilirubin 7.05 mg/dL, direct bilirubin 5.62 mg/dL, Gamma GT 475 U/L, and Alkaline Phosphatase 511 U/L. Other laboratory values were within normal limit.
On her plain abdominal radiogram, there were opacities in the abdomen region suspected as stone in common bile duct and pancreatic duct (a). MRCP revealed opacities suggested as stones in the pancreatic duct and distal of the common bile duct (b and c).
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pmc-6260398-1
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A 61-year-old female patient was referred to our department for laparoscopic excision of GIST in the stomach. Prior to our treatment she underwent an esophagogastroduodenoscopy due to epigastric pain and occasional vomiting. Endoscopy showed erosive gastritis, a hiatal hernia, and a submucosal tumour located along the lesser curvature on the posterior wall of the antrum (). The tumour had a central eroded depression but otherwise appeared to be covered with normal mucosa. Several biopsies of the tumour were taken using the bite-on-bite technique. Unfortunately, only scant samples of the submucosa were obtained, which precluded a histological diagnosis of the tumour.
Endoscopic ultrasonography confirmed a 22 × 13 mm submucosal well-defined solid tumour arising from the fourth, muscular, layer of the stomach (). The tumour appeared to be nodular, with slightly heterogeneous echogenicity. Endoscopic ultrasonography did not demonstrate a significant ulceration or ductal structures. In the diagnostic work-up of abdominal pain and vomiting the patient had undergone transabdominal ultrasonography, which showed no focal lesions in the liver. Before surgery, we performed another endoscopy where the lesion was tattooed proximally and distally.
Laparoscopy was performed, and on initial exploration of the abdominal cavity, we discovered a tattooed submucosal tumour on the greater curvature of the stomach. Laparoscopic excision was performed and the specimen was recovered in a retrieval bag. The abdominal cavity was irrigated with saline and the fluid aspirated. An abdominal drain was inserted beneath the stomach. The fascia and skin wounds were sutured. The postoperative course was uneventful and the patient was discharged on the fifth postoperative day.
Resection samples were sent for histopathological analysis. The histopathologic analysis report about 6.6 × 3.5 cm excision gastric wall. The mucosa and serosa have normal appearance, muscularis propria and submucosa include a gray yellowish node 15 mm in diameter. The tumour did not involve the resection margins. Histopathological analysis confirmed an ectopic pancreas, 15 mm in diameter ().
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pmc-6260410-1
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Our first patient is a 30-year-old female with a medical history of systemic lupus erythematosus (SLE) complicated by stage V lupus nephritis. She underwent deceased donor renal transplant in 2005 and has been on chronic immunosuppression with tacrolimus 2 mg twice daily and mycophenolate mofetil 500 mg twice daily for approximately 10 years prior to presentation. Ten years after transplant, the patient presented with a chief complaint of diarrhea consisting of 4-5 loose, watery bowel movements daily. Her symptoms were accompanied by mild nausea, anorexia, and abdominal cramping, as well as an unintentional 15-pound weight loss. On admission, she did not have leukocytosis (WBC 4.5 B/L), but she was anemic with a hemoglobin count of 6.1 g/dL and had an elevated creatinine level of 1.6 mg/dL from 1.1 mg/dL baseline. An extensive workup for diarrhea was performed which revealed a positive fecal lactoferrin, normal fecal fat content, and antitissue transglutaminase antibody, a negative Clostridium difficile stool toxin, and a positive stool infectious panel PCR for sapovirus. The patient was treated initially with intravenous fluids and antidiarrheal medications. Mycophenolate mofetil dose was decreased gradually and eventually discontinued. Azathioprine was started in place of mycophenolate mofetil, and the patient did have some improvement in the consistency of her stools but the overall large volume output persisted. Nitazoxanide 500 mg orally (PO) twice daily was initiated, and the patient's diarrhea improved within 3 days. She was treated for 7 days total with this medication, and a repeat stool infectious panel 1 month after therapy demonstrated a negative sapovirus PCR.
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pmc-6260410-2
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Our second patient is a 70-year-old female with end-stage renal disease (ESRD) secondary to after streptococcal glomerulonephritis who received a kidney transplant in 2011. The patient was maintained on tacrolimus 4 mg twice daily, prednisone 5 mg daily, and mycophenolate mofetil 500 mg daily. She presented four years after transplant with intermittent diarrhea for 4 months duration and significant weight loss. On presentation, her creatinine was 3.4 mg/dL from a baseline of 1.2 mg/dL on prior studies. The patient was found to have a positive stool infectious panel PCR for norovirus during an inpatient hospital evaluation. Clostridium difficile study was negative, and fecal fat content was within normal limits. Mycophenolate mofetil was discontinued due to concern that her symptoms may be a result of medication side effect. She was subsequently started on azathioprine 50 mg daily for immunosuppression. With no real improvement in symptoms, she was treated with a 3-day course of 500 mg PO nitazoxanide twice daily with modest improvement. Two months later, the patient's symptoms reoccurred, and she received a second, longer course of nitazoxanide for 3 weeks. Her symptoms resolved within a week of treatment but again relapsed over the subsequent 3 months. Stool biofire PCRs were persistently positive for norovirus when checked monthly over the following 5-month time period. Finally, she was started on a 3-week course of nitazoxanide high dose (500 mg PO every eight hours). Initially, this was difficult to tolerate due to gastrointestinal distress, and the frequency of administration was decreased to twice daily. The patient completed the three weeks of nitazoxanide with resolution of her gastrointestinal symptoms. She has been asymptomatic since the prolonged course, and repeat stool PCR for norovirus 10 days after completion of therapy is negative.
The last patient is a 78-year-old male who underwent renal transplant in 2014 due to ESRD as a result of severe diabetic nephropathy. The transplant was complicated by interstitial fibrosis and tubular atrophy, requiring high doses of immunosuppressant medications. These medications included mycophenolate mofetil 500 mg twice daily, tacrolimus 4 mg twice daily, and prednisone 10 mg daily. The patient presented to our hospital 3 years after renal transplant with an evidence of altered mental status, lethargy, and progressive weight loss. Per his family, he was having significant diarrhea associated with agitation and confusion. On admission, he had severe metabolic acidosis and an elevated creatinine level of 7.8 mg/dL from baseline of 2.7 mg/dL, which required urgent hemodialysis. His metabolic derangements improved, but he remained confused. The patient was having significant watery stool output. Infectious workup was negative for Clostridium difficile infection but confirmed norovirus on a stool infectious panel. Initially, the immunosuppressant medications, primarily mycophenolate mofetil, were held in hopes of improving his diarrhea. When symptoms did not dissipate, he was started on oral nitazoxanide 500 mg twice daily empirically. The patient's diarrhea did improve within 72 hours of nitazoxanide initiation, and he was treated with a full 14-day course. Simultaneously, the patient developed acute transplant rejection which was confirmed on renal ultrasound. This was thought to be due to decrease in immunosuppressant medications. Since that time, hemodialysis has been reinitiated. Unfortunately, a repeat stool infectious panel after therapy was not collected as the patient was lost to follow-up from both the infectious disease and transplant nephrology clinics.
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pmc-6260438-1
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A 14-year-old male had an extensive flame burn in the right side of neck, face, and scalp, anterior chest, and right upper limb at the age of 2 years.
No past medical or surgical history. No known drug history, no family history of any genetic disorder, patient and both parents are non-smokers.
He received multiple skin grafting procedures and an amputation of the right hand. The burn to his scalp was treated with a burr hole to the outer cortex to allow granulation tissue to cover the scalp, and then the wound was skin grafted.
The patient chronically continued to have unstable skin over the scalp and kept developing recurrent ulceration. On 29 March 2012, the patient was admitted throw the clinic due to his chronic condition of recurrent open wounds for many years.
A plan was set for the removal of the unstable ulcer and scarred skin of the scalp and coverage with an omental free flap and split thickness skin graft ().
Under general anesthesia with endotracheal intubation, the patient was in supine position, with draping of the head circumflex, face, abdomen, and left thigh down to the knee.
The General Surgery team was consulted for laparoscopically harvesting the omental flap and was performed by Dr I.Anwar based on the left gastroepiploic artery. The right superficial temporal artery was identified to be the recipient vessel ().
The ENT team did a right neck exploration to explore the neck vessels as a backup in case the right superficial temporal artery was not suitable.
The free omental flap based on the left gastroepiploic artery was anastomosed to the right superficial temporal artery. The micro procedure of omental free flap was performed by DR F.Hashem, Plastic surgeon.
The patient was given heparin 2000 U intraoperatively. The flap was viable with bleeding with a positive Doppler signal.
After establishing flap revascularization, all the excising unstable scarred and ulcerated skin covering the scalp was resected, and the underlying irregular skull was smoothed down using a flat burr. The omental flap then covered the scalp, and a split-thickness skin graft from the thigh was placed over the omental flap ().
The patient was monitored postoperatively in intensive care unit. Post-operative care for microsurgery and wound care for skin graft over omental flap.
The hospital course was uneventful. The patient was kept on IV anticoagulants (dextran and heparin) for 5 days.
The patient tolerated the procedure well. No wound complications were reported during his two years follow-up (). Re-Exploration nor revision of the surgery were needed
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pmc-6260439-1
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A 30-year-old male (170 cm/60 kg, BMI = 20.8) suffered the HBV-related cirrhosis complicated by portal hypertension. His model for end-stage liver disease (MELD) score was 19. His liver anatomy was modal with a standard liver volume (SLV) of 1220cm3 []. Transplantation was considered due to his deteriorated general and liver condition. His 34-year-old brother (170 cm/70 kg, BMI = 23.9) volunteered for living donation. He underwent a comprehensive living donor evaluation including MRCP and the CT image-based three-dimensional reconstruction (IQQA-liver, EDDA, US) for volumetric assessment and angiography. The volume of his right lobe (i.e. segments 5–8) without middle hepatic vein (MHV) was 672cm3, representing 54.3% of his entire liver. The graft to recipient body weight ratio (GRWR) was 1.32% and the liver remnant was 45.7% of the total liver volume, which fulfills the safety criteria for both recipient and donor. Donor’s hepatic vasculature anatomy was modal with the presence of portal vein bifurcation and a single right branch of a proper hepatic artery. MRCP showed normal biliary confluence with a single right hepatic duct. MHV tributaries in Segment 5, Segment 8 ventral and dorsal areas were ＞5 mm in diameter and need to be reconstructed for the recipient . Informed consent was obtained from both donor and recipient, which were characterized by a morbi-mortality of this complex procedure and the innovative nature of the pure laparoscopic technique. It’s decided that any incident occurring during the innovative procedure that might compromise the donor safety or graft integrity would prompt conversion to laparotomy. The procedure was approved by the ethics committee of our institute.
A surgical team (XM, HW, YX) with an experience of over 500 cases of laparoscopic major hepatectomy performed the donor procedure. The donor was placed in a supine position with right upper abdomen slightly elevated and right arm suspended. Four trocars were placed as shown in . A pneumoperitoneum was created and maintained at 13 mmHg. The cystic duct and artery were clipped and divided. The portal pedicle was opened with the right hepatic artery and right portal branch dissected free and taped, during which some small portal branches into the caudate lobe were suture-ligated and divided. After cutting the round and falciform ligaments, the right lobe was mobilized with sectioning of the short hepatic veins at anterior aspect of the inferior vena cava. The right hepatic vein (RHV) was then dissected and taped. 2 ml of ICG (0.5 mg/ml) was directly injected into the right portal branch through a scalp needle. The fluorescent territory of the right lobe was clearly visualized through the fusion image mounted on the laparoscopy system (Pinpoint, Stryker, US). The right hepatic artery and portal branch were briefly clamped to reveal the main portal fissure and to confirm the transection plane a–c. The intra-operative ultrasound (IOUS) was used to identify the course of MHV, which would be preserved for the left lobe. The liver capsule was opened using a harmonic scalpel. Parenchyma was divided tracing the fluorescence borderline of right lobe. A Cavitron Ultrasonic Surgical Aspirator (CUSA) was used for parenchyma dissection. Bipolar coagulation and clips were used for hemostasis. The Segment 5, 8 ventral and dorsal venous tributaries were double clipped and divided. When the right hepatic duct level was reached, 20 ml of ICG was bolus injected intravenously. The biliary confluence and right hepatic duct were then clearly visualized and sectioned. The liver section was then completed and the right hepatic vein was isolated. A 12-cm suprapubic incision without muscle sectioning was made allowing for the insertion of a retrieval bag. The right lobe was put into the bag before the right hepatic artery, portal branches and hepatic vein were a securely clipped and divided d–f. The graft was quickly retrieved to the back table and perfused with cold UW solution. Then the right hepatic ducal stump on the liver remnant was sealed by suture. The warm ischemia time was 6 min 20 s. The V5, V8v and V8d were recanalized on the back-table using cryopreserved iliac artery allografts . The recipient procedure and perioperative management followed our institutional protocol. The donor procedure took 420 min with a blood loss of 100 ml. The recipient procedure took 380 min with a blood loss of 450 ml. Neither required blood transfusion. The donor recovered uneventfully and was discharged from hospital on POD 7.
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pmc-6260440-1
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A 81 years old female presented to the Department of Surgery with a history of right breast erythema and edema associated to breast pain, with over two weeks duration and progressive worsening.
She linked the appearance of these signals with an episode of breast trauma and she denied the presence of previous breast nodules, nipple drainage, nipple retraction, fever and constitutional symptoms.
The patient was multiparous (three pregnancies and three births) and nursed all children.
She had multiple medical co-morbidities but there was no past history of breast pathology or family history of breast malignacy.
Physical examination demonstrated mammary asymmetry because right breast was bigger and tender (). The outer quadrants and the periareolar region had inflammatory signs with orange peel skin (). There was no palpable masses or nipple changes. The left breast was normal.
She had the right upper limb swollen and palpable axillar and supraclavicular lymph nodes ().
The principal suspicion was an inflammatory breast cancer.
The ultrasound of the right breast revealed skin thickening and tissue densification involving principally the external quadrants, and it was identified an irregular hypoechoic mass with 8 cm × 5 cm and multiple axillary and supraclavicular enlarged lymph nodes. The ultrasound of left breast and of left axilla was normal.
Core needle biopsy of an axillary lymph node and of the breast mass identified morphological and immunophenotypic features consistent with diagnosis of primary non-Hodgkin DLBCL.
The CT scan of the chest confirmed right breast alterations identified in ultrasound and ipsilateral axillary and supraclavicular lymph node enlargement. No other enlarged lymph nodes were observed. CT scans of the brain, abdomen and pelvis were normal. Bone marrow aspiration was negative. Serum lactate dehydrogenase level was normal.
After confirming diagnosis, the therapy plan included R-CHOP x8 (Rituximab (375 mg/m2, D1), cyclophosphamide (750 mg/m2, D1), doxorubicin (50 mg/m2, D1), vincristine (1.4 mg/m2, D1), prednisone (50 mg twice a day, D1–D5)). She also received radiotherapy (4500 cGY) to the breast and regional lymph nodes with 180 cGY daily fractions.
She is currently well at follow-up 24 months after presentation and without evidence of residual disease.
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pmc-6260447-1
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A 21-year-old female with no prior encounters presented to the emergency department with right iliac fossa pain and a history concerning for appendicitis. She reported a background of having been involved in a serious motor vehicle accident overseas 3 months ago which resulted in the death of her sister. She has also required an emergency laparotomy and bowel resection with prolonged ICU stay.
On examination, a midline laparotomy scar was evident and the patient was tender over the right iliac fossa with no features of peritonism. Her white cell count was equivocal and C-reactive protein unremarkable. A pelvic ultrasound scan was performed which was unsuccessful in identifying the appendix. On CT, the appendix was again not visualised, however there were no secondary signs of appendicitis.
The patient was admitted for observation and serial assessment, after 24 h of admission, it was noted that patient required significant amounts of opioids with no clinical evidence of appendicitis nor biochemical evidence to suggest inflammation. The acute pain service as well as the drug dependence teams were consulted. After extensive investigation into her background, it was discovered that she had 23 different aliases and countless hospital presentations with drug seeking behaviours. Her overseas injuries were falsified and her sister was also alive and well. Shortly after the discovery, the patient discharged against medical advice.
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pmc-6260530-1
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A 48-year-old woman presented with a rapid enlargement of a preexisting goiter without compressive symptoms. Her past medical history included type 2 diabetes mellitus, hypertension, goiter, and primary hypothyroidism for fifteen years. There was no past or present history of smoking and her family history was unremarkable.
She was complaining of productive cough for two weeks. However, she did not have any history of fever, night sweats, or anorexia.
Clinical examination showed a normal body temperature, a body mass index of 35.88 kg/m2, a blood pressure of 120/80 mmHg, a regular pulse of 89 beats/min, and a normal respiration rate of 20 breaths/min. The lung breath sounds were normal without any rales being heard. Cervical examination revealed a plunging multinodular goiter without any lymphadenopathy. Other systemic and regional examinations did not show any abnormalities.
The blood routine tests showed a fasting blood glucose of 7.19 mmol/l, a plasma creatinine level of 49 μmol/l, a C-reactive protein level of 5 mg/l (reference range < 5 mg/l), an erythrocyte sedimentation rate of 57 mm/first hour, a red blood cells count of 4.38 ∗ 106/mm3, a total hemoglobin concentration of 12.8 g/dl, a white blood cells count of 6800/mm3, a neutrophil count of 3640/mm3, and a lymphocyte count of 2220/mm3. Liver function tests were normal.
The thyroid function tests disclosed normal serum thyroid stimulating hormone (TSH) level at 0.5 μIU/ml (reference range: 0.35-4.94) and normal free thyroxin (FT4) level at 9.14 pmol/L (reference range: 8.5-25) on daily 100 μg of levothyroxine.
Thyroid ultrasound showed a heterogeneous multinodular goiter. Her chest X-ray showed a mediastinal enlargement and a suspicious lesion located at the upper lobe of the right lung. Cervical and chest computed tomography scan revealed an enlarged plunging multinodular thyroid gland (right lobe: 113 × 39 × 41 mm, left lobe: 90 × 53 × 44 mm) with an extension of the right lobe into Barety's space () and multiple bilateral lung nodules.
Sputum smear microscopy was negative.
Thyroid cancer was suspected. Fine needle aspiration (FNA) cytology was not available for technical reasons. Surgical treatment was indicated and the patient underwent a total thyroidectomy. Multiple lung biopsies were also performed using a left anterior minithoracotomy through the fifth intercostal space.
Histopathological examination showed a benign multinodular hyperplasia with epithelioid cell granulomas and giant cells (). These morphological signs were compatible with multiple tuberculous foci of the thyroid gland. The histopathological examination of the lung biopsies showed foci of granulomatous inflammation along with caseous necrosis ().
The diagnosis of tuberculosis involving both the lung and the thyroid gland was established and the patient was treated with antituberculosis drugs for 6 months.
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pmc-6260546-1
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A 34-year-old primipara with pycnodysostosis was scheduled for an elective caesarean delivery in week 37 + 4 of pregnancy. The patient was 140 cm in height and weighted 60kg. She had no known allergies apart from nonsteroidal anti-inflammatory drugs and was not taking any prescribed medication. She had a history of multiple fractures, including 8 vertebral compression fractures in the thoracic and lumbar spine, but was otherwise healthy. Prior general anesthetics involving intubations had been without complications.
Prior to the caesarean delivery the patient was evaluated in a preoperative assessment by an experienced anesthesiologist.
The airway was assessed using the Simplified Airway Risk Assessment (SARI), which consist of 7 parameters: mouth opening, thyromental distance, Mallampati score, movement of the neck, ability to protrude the jaw, body weight, and a history of previous difficult intubation. A summed SARI score >3 indicates possible difficult intubation []. The patient had a SARI score of 3, due to inability to protrude her jaw (1 point) and a thyromental distance less than 6 cm (2 points). She had a modified Mallampati score of II. The overall dental status was good and she had no prior dental work done.
Physical examination of the spine revealed a slightly accentuated lumbar lordosis, which reduced with flexion. No scoliosis was detected.
Given her medical history, current pregnancy, and physical examination the initial plan was spinal anesthesia. Patient was also consented for general anesthesia. On the day of surgery the anesthesiologist responsible for the operation chose neuraxial anesthesia with an epidural approach. The patient was monitored with 3-lead electrocardiography, pulse oximetry and noninvasive blood pressure. The vital signs were all within normal ranges. Intravenous access was secured with an 18-gauge (G) cannula. Cefuroxime 1.5g and 1000 mL of Ringers-Acetate were administered. The epidural catheter was placed preoperative by an experienced anesthesiologist. Under strict aseptic conditions the epidural catheter was inserted at the L2-L3 level with an 18G Tuohy needle with the patient in the sitting position. The catheter was inserted via midline approach using the loss of resistance to saline technique. Loss of resistance was obtained at 5.5 cm, and the catheter was placed 3.5 cm into the epidural space. The procedure was uncomplicated. During the procedure the patient experienced a short period of paraesthesia in the left leg, which quickly resolved. There was negative aspiration for blood and cerebrospinal fluid. The catheter was tested with 2 mL of 2% lidocaine without onset of spinal anesthesia.
Epidural anesthesia was preformed with incremental doses of a solution consisting of 2% lidocaine with epinephrine 5μg/mL, sodium bicarbonate 8.4mg/mL, and fentanyl 3.75μg/mL. The epidural was dosed in aliquots with intermittent aspiration; a total of 6 mL was administered. The patient achieved satisfying level of analgesia above the Th4 level and the caesarean delivery was performed. It took 4 minutes from the beginning of the procedure to the delivery of a healthy boy. 10 international units of syntocinon were administered. Perioperative bleeding was estimated at 300 mL. The entire procedure took 26 minutes. The postoperative period was without complications.
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pmc-6260547-1
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A 47-year-old female with a past medical history of hypertension presented to the emergency department at Lincoln Hospital, Bronx, New York, with altered mental status. As per the patient's daughter, she was found confused in the bathroom, with last well known time eight hours prior to her presentation. Upon examination in the emergency department, she appeared altered, nonverbal, but responsive to painful stimuli, involuntarily opening and closing her eyes, and able to protect her airway. The patient was afebrile, tachycardic to 130 with a blood pressure of 140/90. Neurological exam was remarkable for left gaze preference, spasticity of all extremities, bilateral lower extremity hyperreflexia, occasional myoclonus, and positive Babinski sign bilaterally. She was also noted to be moving all extremities purposelessly. All labs were normal including complete blood count, basic metabolic panel, creatinine kinase, and troponin. Serum levels of salicylate, acetaminophen, carbamazepine, lithium, valproic acid, and alcohol were negative, and urine toxicology screen was also negative for any illicit drugs. EKG showed normal sinus rhythm.
Initial brain CT was negative for hemorrhage or any infarcts []. A few hours later, the patient became more lethargic, unable to protect her airway, and, given the high risk for aspiration, she was intubated and admitted to the medical ICU. Lumbar puncture was done and CSF analysis was negative for bacterial meningitis, as well as CMV, HSV PCR, toxoplasma, west Nile virus, and oligoclonal bands. She had a repeat CT the next day that demonstrated large areas of low attenuation in both cerebral hemispheres consistent with acute ACA and MCA infarcts [Figures and ]. In view of the patient's presentation and absence of classical stroke risk factors, CT angiography and vasculitis work-up were sent including ANA, c-ANCA, p-ANCA, ESR, CRP, antiphospholipid antibodies, Factor V Leiden, prothrombin gene mutation, protein C, protein S, cryoglobulins, and complement C3 and C4 levels. Results of the vasculitis workup were negative, excluding vasculitis as a cause for her presentation.
Brain CT angiography was remarkable for acute bilateral ACA and large right MCA territory infarcts. The intracranial segments of the ICA were diffusely decreased in caliber; there was a complete occlusion of the right MCA from its origin and at least moderate stenosis of the M1 and M2 segments of the left MCA. ACAs were patent but diffusely decreased in caliber, and prominent lenticulostriate vessels were noted in the basal ganglia bilaterally [Figures and ]. Repeat brain CT the next day showed worsening edema with mass effect on the lateral ventricle and midline shift; thus neurosurgery was consulted and the patient had a left hemicraniectomy. However, mental status remained poor; the patient had a tracheostomy and percutaneous endoscopic gastrostomy and was transferred to a long-term acute care facility.
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pmc-6260550-1
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A 91-year-old female presented with a 4-day history of subcutaneous as well as intramuscular hemorrhages of the upper and lower extremities. In her past medical history, she had thoracic and lumber compression fractures. Laboratory investigations revealed a severe anemia (Hb 4.6 g/dL) with a prolonged APTT (93.3 sec; control 27.8 sec). Initially, she was taken care of with red blood cell (RBC; total 12 units) and fresh frozen plasma (FFP; total 8 units) transfusions. On day 5, interventional radiology was carried out to examine femoral vasculatures because of severe extravasation in the right thigh; however, no abnormalities were noted. On day 10 of admission, she was found to have an FVIII activity level <1.0% and an anti-FVIII inhibitor level of 110.6 BU/mL, in addition to positive lupus anticoagulant (LAC) (phospholipid neutralization method 14.5 sec; reference <7.9 sec). A diagnosis of AHA was therefore assigned with positive LAC. Following consultation with a hematologist, the patient received 36 doses (4.5 doses/day) of rFVIIa (NovoSeven®; 90 µg/kg/dose) and prednisolone (1.2 mg/kg/day and then tapered). On day 33, combined cyclophosphamide (CPA; 1.7 kg/kg/day) and prednisolone therapy was initiated, which was administered for 5 weeks. Within 7 weeks, a CR was obtained.
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pmc-6260550-2
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A 93-year-old male patient presented with hematuria, epistaxis, and a subcutaneous hemorrhage in the region of the right shoulder two weeks after admission for influenza B pneumonia. Laboratory investigations revealed anemia (Hb 7.5 g/dL), a prolonged APTT (73.1 sec; control 27.6 sec), and microscopic hematuria (>100/HPF). Five days later, the patient was diagnosed as having AHA when laboratory investigations revealed an FVIII activity level <1.0% and an anti-FVIII inhibitor level of 8.8 BU/mL. After consultation with a hematologist, the patient was administered six doses of rFVIIa (NovoSeven®; 90 µg/kg/dose) over 2 days in combination with prednisolone (0.6 mg/kg/day and then tapered). Within 5 days, the hematuria and subcutaneous hemorrhages improved. AHA was controlled within 2 weeks, and a CR was obtained.
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pmc-6260550-3
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A 92-year-old female presented with generalized subcutaneous hemorrhages for a few days. Three weeks before, she had undergone surgery for a left femoral trochanteric fracture. Because of anemia (Hb 7.3 g/dL) and a prolonged APTT (55.2 sec; control 29.0 sec), she was administered RBC (10 units) and FFP (2 units) transfusions, and oral prednisolone (0.5 mg/kg/day and then tapered) was started. On day 7 of admission, laboratory investigations revealed an FVIII activity level of 3.0% and an anti-FVIII inhibitor level of 10.0 BU/mL. A diagnosis of AHA was therefore assigned. The patient received two doses of rFVII (NovoSeven®; 90 µg/kg/dose), and prednisolone (0.5 mg/kg/day and then tapered) was continued as maintenance therapy. AHA was controlled within 7 weeks, and a CR was obtained. The patient remained stable for 2 years. However, she then developed further subcutaneous hemorrhages. Laboratory investigations revealed an FVIII activity level of 6.2% and an anti-FVIII inhibitor level of 2.1 BU/mL. With a diagnosis of AHA relapse, reintroduction of prednisone (0.6 mg/kg/day) was performed.
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pmc-6260550-4
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A 90-year-old male presented with a left gluteal intramuscular hemorrhage. The patient was in a state of chronic heart failure and was anemic (Hb 6.4 g/dL) with a prolonged APTT (69.2 sec; control 28.0 sec). Based on the information that he had previously been diagnosed as having AHA, a relapse of AHA was suspected, and the patient was immediately managed with RBC infusion (2 units), APCC (Feiba®; 100 U/kg/dose, three doses), and prednisolone (0.6 mg/kg/day and then tapered). Later, it was confirmed that the patient had an FVIII activity level <1.0% and an anti-FVIII inhibitor level of 240 BU/mL. With the above measures, the patient remained in a state of no bleeding but FVIII activity <50% over the subsequent several months. Thereafter, he developed repeat intermittent cutaneous hemorrhages, which required three further periods of hospitalization. During each admission, minimal doses of rFVIIa (NovoSeven®; 90 µg/kg/dose, two doses each) were administered to control the bleeding with a limited effect. Due to the persistence of high anti-FVIII inhibitor levels, the patient was transferred to a tertiary center for further management.
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pmc-6260550-5
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An 80-year-old male presented with soft tissue hemorrhages in the left forearm and right lower extremity for 3 weeks, which progressed compartment-like symptoms []. He was anemic (Hb 8.0 g/dL) with a prolonged APTT (78.4 sec; control 25.3 sec). In his medical history, the patient had been noted to have leukocytosis two years earlier and was diagnosed to have chronic neutrophilic leukemia (CNL) after admission. Laboratory investigations revealed an FVIII activity level <1.0% and an anti-FVIII inhibitor level of 190 BU/mL. A diagnosis of AHA was therefore assigned in association with CNL. The patient was administered 16 doses of APCC (Feiba®; 100 U/kg/dose) and four doses of rFVIIa (NovoSeven®; 90 µg/kg/dose), and hemostasis was achieved. Inhibitor eradication was achieved using prednisolone (0.6 mg/kg/day and then tapered) and two doses of rituximab (375 mg/m2/dose). During the treatment for AHA, reactivation was noted around the 7th week of treatment, but administration of two more doses of rituximab was successful in eradicating the inhibitor. Together with the treatment of AHA, the underlying CNL was also treated with hydroxycarbamide (Hydrea®; 500 mg/day) and controlled well. A CR of AHA was attained within 26 weeks.
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pmc-6260550-6
|
A 62-year-old female presented with hemorrhagic shock a few hours after a tooth extraction and was referred to the emergency department []. The patient had been treated for noncompensated liver cirrhosis during the past several years. Laboratory investigations revealed severe anemia (Hb 4.6 g/dL) with a prolonged APTT (>100 sec; control 28.1 sec) and low fibrinogen (58 mg/dL; reference 200–400). She was taken care of by RBC (4 units) and fresh frozen plasma (FFP; total 32 units) transfusions with hemodynamic and respiratory care until day 8 when laboratory investigations revealed an FVIII activity level <1.0% and an anti-FVIII inhibitor level of 11.9 BU/mL. A diagnosis of AHA was therefore assigned. However, due to the liver cirrhosis, hemostasis was attempted using plasma exchanges (two courses of 30 units each) rather than the administration of APCC (Feiba®) or rFVIIa (NovoSeven®). Inhibitor eradication was achieved using a combination of prednisolone (1.0 mg/kg/day and then tapered) and cyclosporine A (CSA; 3.0 mg/kg/day). AHA resolved within 10 weeks, and a CR was obtained. Unfortunately, the patient died of liver failure a year after the onset of AHA.
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pmc-6260552-1
|
A 53-year-old man was admitted for mobility of tooth. The dentist suggested the presence of a mass located at the tooth root by physical examination. Computed tomography revealed a well-demarcated radicular cyst of 4 cm in diameter at the tooth base. Carcinomatous infiltration of squamous cell carcinoma was observed in the excisional biopsy of the lesion. In microscopic evaluation, tumoral infiltration revealed features of moderately differentiated squamous cell carcinoma. Malignant cells infiltrated the underlying connective tissue stroma in solid groups and sheets. Numerous neutrophils were present within the cytoplasm of the malignant cells as well as in the surrounding stroma. The internalized neutrophils were intact (). Desmosomal connections were observed between the tumor cells in some areas. The tumor cells showed moderate cellular pleomorphism. The diffuse immunoreactivities of P63 and CK5/6 were determined in the malignant cells by immunohistochemical staining. Additionally, perineural invasion was found, whereas vascular invasion was not observed. Because surgical margin was positive for tumor cells, partial maxillectomy and bilateral neck dissection was performed. Bone infiltration was present. Furthermore, diffuse neutrophilic emperipolesis was observed in cancer cells by microscopic evaluation. Some of the neutrophils in the tumor cells revealed degenerative changes by high-power field (×1000) microscopic evaluation (Figures –), while some neutrophils included apoptotic bodies. Nearly one year later, local relapse developed and additional therapeutic manipulations including surgery, radiotherapy, and chemotherapy (cisplatin) were done. Tumor recurrence occurred in the periparotid and right neck lymph nodes after six months (). Chemotherapy (cisplatin) and radiotherapy were performed for the recurrence. Two more relapses developed in the right neck, left submandibular lymph nodes and in the superficial and deep soft tissues of the neck three months apart. Tumor showed continuity along the surgical margin in the excised biopsy sample and a pericapsular invasion at the submandibular lymph node. Chemotherapy was continued. The endmost tumor recurrence was in the palatine tonsil and posterior parotideal region. Following unresponsive chemotherapy, pembrolizumab treatment was started eight months prior to this study (). A complete response occurred following the sixth dose of pembrolizumab. Secondary adrenal insufficiency and pulmonary reactivation tuberculosis developed as the side effects of treatment. Tuberculosis was identified by PCR and compatible chest CT findings. Pembrolizumab was interrupted and antituberculous treatment was started. Pembrolizumab was commenced when the tumor progressed to a 15 cm mass (). Following the fourth dose of pembrolizumab, the tumor regressed to 4 cm () and the patient is currently alive for four years.
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pmc-6260651-1
|
A 65-year-old Asian woman was admitted with exertional dyspnea. She had a medical history of hypertension, hyperlipidemia, and coronary artery disease that had required transcatheter intervention. Her family history also included coronary artery disease. Her social history was unremarkable with respect to environmental and workplace exposures, and she did not smoke or consume alcohol. A physical examination upon admission revealed no signs of cardiac congestion or neurological deficits. Her vital signs were a systemic blood pressure of 143/86 mmHg, pulse rate of 89 beats per minute, and a body temperature of 35.9 °C. Chest x-rays at the time of admission showed cardiomegaly with protrusion of the right first aortic arch, and electrocardiography showed an incomplete right bundle branch block of normal sinus rhythm. Coronary angiography performed because of her history of coronary artery disease revealed no significant coronary arterial stenosis. However, a concurrent pressure study revealed oxygen step-up in gas sampling between the SVC and right atrium (RA) with a pulmonary blood flow/systemic blood flow ratio (Qp/Qs) of 2.07, even though pulmonary artery pressure was normal in the pressure study and the atrial septum was intact on echocardiography. Contrast-enhanced computed tomography (CT) revealed that the RUPV flowed into the SVC (Fig. ) without any other congenital heart conditions, including an ASD and a dilated RA and right ventricle (RV). Isolated PAPVR was diagnosed on the basis of CT imaging findings, and surgery was indicated on the basis of the patient’s symptoms and the hemodynamic pressure findings.
The surgical approach was via a median sternotomy. The SVC was cannulated near the confluence of the innominate vein, sufficiently above the RUPV. A cardiopulmonary bypass was established, and cardiac arrest was applied. The RA was incised longitudinally. The intact atrial septum was incised in the cranial direction from the superior aspect of the fossa ovalis, and this new ASD formed a hole with an approximate diameter of 15 mm after the right and left atrial endocardia were closed using continuous 5-0 polypropylene sutures (Fig. a). An intra-atrial baffle comprising an autologous pericardial patch was sutured from the inferior border of the new ASD to the cavoatrial junction to separate the cavity between the RA and the SVC (Fig. b). The SVC was divided above the confluence of the RUPV, and the cardiac side of the SVC stump was sutured closed. A new RUPV outflow route to the left atrium (LA) was subsequently constructed through the surgically created ASD. A cavoatrial pathway between the cranial side of the SVC stump and the right atrial appendage (RAA) was reconstructed using a ringed extended polytetrafluoroethylene (EPTFE) prosthesis with a diameter of 16 mm.
The patient tolerated all procedures well and was discharged with normal sinus rhythm after recovery from temporary sinus bradycardia. Anticoagulation therapy with oral warfarin was postoperatively prescribed for 3 months, which is standard procedure after bioprosthetic valve replacement. Postoperative echocardiography showed flow from RUPV to the LA through the new ASD without a significant pressure gradient or congestion. Postoperative contrast-enhanced CT showed that the RUPV flowed into the LA through the new pathway without intracardiovascular thrombus (Fig. ). The patient remains free of dyspnea, arrhythmia, and thrombotic events, and echocardiography at 4-year follow-up did not reveal evidence of stenosis or obstruction of the reconstructed pathway.
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pmc-6260670-1
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Case 1: ‘An 18-year old girl with a boyfriend would like to start contraception . What would you advise her?’ Only 22% would recommend the hormonal IUD, and the other LARCs received even lower scores ().
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pmc-6260670-2
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Case 2: ‘A 22-year old women with one child would like to postpone a second pregnancy. In the meantime she would like to use a contraceptive method that she can stop when she would be ready for a second pregnancy. She thinks this might be after 3 years. What would you advise?’ For this case, LARCs would be an option: 55.9% would recommend hormonal IUD, 27.9% a cupper IUD and 16.2% an implant.
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pmc-6260670-3
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Case 3: ‘An 18-year old women would like to continue contraceptive use, but she often forgets to take the combination pill which she is currently using. What would you advise?’ Adherence to pill taking is problematic in this case, so one would expect a vast majority of respondents indicating methods that are less dependent on adherence. And indeed, injectables, vaginal ring and hormonal IUD score more than 50% here. It is however remarkable that almost 30% would recommend to continue the pill.
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pmc-6260671-1
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A 38-year-old nulliparous woman presented at our emergency unit with right lower abdominal pain. Biochemically there were no changes worth mentioning. She had a negative pregnancy test, normal C-reactive protein (CRP) and no other signs of infection.
Abdominal ultrasound showed a non-echogenic cyst in the right flank. Computed tomography (CT) scan of the abdomen with intravenous and oral contrast confirmed a right lower abdominal cystic formation with a diameter of approximately 38 by 33 millimetres () and a density of 24 Hounsfield units (HU) (). Imaging also revealed a narrow approximation of the cyst with the colon ascendens and the psoas muscle as well as a very discrete infiltration of the perilesional fat tissue. The differential diagnosis of enteric duplication cyst, mesenterial cyst, peritoneal inclusion cyst and appendiceal mucocele was established. Gynecological examination showed a normal cervix in speculo. Bimanual vaginal examination reported a mobile uterus with normal adnexa. Vaginal ultrasound showed an intra uterine device in the uterine cavity and a normal left ovary. The right adnex could not be visualized. To further investigate the origin of the abdominal pain, the patient was hospitalized and planned for an explorative laparoscopy a few days after the first symptoms appeared.
During laparoscopy, the appendix was found to be normal, without mucocele. The cecum was located deep in the right fossa. Exploration of the internal genitals showed a unicornuate uterus and a normal left fallopian tube and ovary (). The right fallopian tube and ovary could not be visualized in the right pelvis but were found to be infrahepatically. Both the ovary and fallopian tube were positioned upon the psoas muscle, which was also crossed over by the right ureter. () Over the course of the left round ligament we observed a firm spherical formation, most likely a rudimentary part of the unicornuate uterus (). In summary, we concluded that this was an anatomical anomaly of the right mullerian system, known as an infrahepatic undescended ovary with adjacent fimbrial ending of the fallopian tube. The cystic formation seen on abdominal CT could not be visualized during laparoscopy. We believe it may have been a temporary follicular cyst of the smaldescended ovary.
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pmc-6260675-1
|
A 48 year old male with a past medical history of pituitary abnormality with central diabetes insipidus and hypogonadotrophic hypogonadism for eight years treated with desmopressin (DDAVP) and testosterone. He presented with shortness of breath, exertional dyspnea, and a four month history of weight loss and drenching night sweats. Computed tomography scan of the chest revealed a large mediastinal mass, and positron emission topography-computed tomography (PET/CT) scan demonstrated extensive, 2-[18F] fluoro-2-deoxy-D-glucose (FDG)-avid mediastinal and abdominal lymphadenopathy. A subsequent mediastinal lymph node biopsy showed Burkitt lymphoma with t(8;14), and a staging bone marrow was negative for lymphoma. Burkitt lymphoma was staged as IVB. The patient received four cycles of R-CODOX/M/IVAC (Rituximab, Cyclophosphamide, Oncovin [Vincristine], Doxorubicin, Ifosfamide, Vepesid [etoposide], and Ara-C [Cytarabine] with methotrexate held due to pleural effusions and ifosfamide deleted in cycles 2 and 4 due to neurotoxicity. He also received involved field radiotherapy of 40 Gy in 20 fractions to residual mediastinal/subcarinal/pleural disease. During this time he was diagnosed with central hypothyroidism and began treatment with levothyroxine.
After completing the chemotherapeutic regimen, the patient experienced waxing and waning pain in the lower back and knees as well as bilateral leg weakness. Magnetic resonance imaging (MRI) scan of the knee showed infiltrative lesions within the distal femoral metaphysis/diaphysis and proximal tibial diaphysis; however, PET/CT scan was negative for PET-avid bone disease. A bone biopsy was suboptimal with nonspecific findings. Bilateral iliac crest biopsies revealed mildly hypercellular bone marrow with a small non-paratrabecular lymphohistiocytic aggregate. Over the next six months he showed progressive failure to thrive with new onset dysphagia. Neurologic and endocrine evaluations remained negative. MRI scans of the thoracic and lumbar spines showed progressive bone disease and extensive lung and pleural disease. The patient underwent bone marrow and pleural/lung biopsies.
The bone marrow biopsy showed normocellular bone marrow with preserved trilineage hematopoiesis and a few variably-sized, atypical histiocytic aggregates (Fig. ). The histiocytes were epithelioid to spindled in appearance with a subset of foamy histiocytes. A rare multinucleated histiocyte was noted. The histiocytes were positive for CD68, CD163, factor XIIIa, and BRAFV600E (clone VE1) and negative for CD1a, CD21, and CD23. There was no evidence of Burkitt lymphoma.
The pleural/lung lesion biopsy contained a small amount of tissue with a nonspecific fibrohistiocytic infiltrate. Genomic DNA was extracted and polymerase chain reaction (PCR) was performed for exon 15 of BRAF gene. The product was analyzed by pyrosequencing for mutations in codons 600 and 601 of BRAF with an analytic sensitivity of 5% for mutated allele. The BRAF p.V600E mutation (c.1799 T > A) was detected.
The histologic findings in combination with histiocyte phenotype, presence of BRAF mutation, and clinical and radiologic data supported a diagnosis of ECD.
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pmc-6260677-1
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A 74-year-old Japanese woman was referred to our hospital with a history of several days of jaundice. She had no past medical history and no family history. On examination, her skin was jaundiced, and her abdomen was flat and soft. Her laboratory data revealed obstructive jaundice and cholangitis, and tumor marker levels were elevated with carcinoembryonic antigen at 3.6 and carbohydrate antigen 19-9 at 4573.9. Computed tomography (CT) revealed an enhancing mass in the biliary duct hilum and dilation of the intrahepatic bile ducts, and there was no evidence of lymph node metastasis or distant metastasis. Endoscopic retrograde cholangiopancreatography revealed a luminal filling defect in the biliary hilum (Fig. ). We diagnosed the tumor as a Bismuth type 1 cholangiocarcinoma, and performed endoscopic retrograde biliary drainage. Biopsies of the tumor revealed adenocarcinoma. The estimated volume of the postoperative liver remnant was less than 35%. Therefore, percutaneous transhepatic portal vein embolization (PTPE) of the right branch of her portal vein was performed (Fig. ). Twenty-one days later, the volume of the left lobe of her liver increased, and we performed right hepatectomy, caudate lobectomy, extrahepatic bile duct resection, and lymph node dissection. Node dissection included resection of hilar and pericholedochal nodes in the hepatoduodenal ligament, common hepatic artery nodes, and those at the celiac trunk, and posterior and anterior pancreaticoduodenal nodes.
Histological examination of the tumor showed moderately differentiated tubular adenocarcinoma without regional lymph node metastasis, a pathological stage II tumor according to the Union for International Cancer Control classification of malignant tumors, 7th edition (Fig. ). Postoperative blood laboratory tests showed that liver enzymes were slightly elevated, but that total bilirubin was within normal limits. Resumption of diet started on postoperative day (POD) 3. Although the fluid in the abdominal drain had been serous until POD 4, the appearance of the fluid became milky on POD 5, and the amount of the drainage increased up to 1 L/day. CT showed a large amount of ascites with a small right pleural effusion. We placed our patient on total parenteral nutrition (TPN), and the ascites gradually decreased and became serous again. The abdominal drain was removed on POD 27, and an oral diet was restarted. She experienced sudden dyspnea on POD 42, and CT showed a massive right pleural effusion and a small amount of ascites (Fig. ). We performed a thoracentesis and placed a chest tube. One liter of chylous effusion was drained, and the triglyceride concentration of the pleural fluid was 1026 mg/dL. With a diagnosis of chylothorax, she was started again on TPN and given subcutaneous octreotide injections. Although the chest drained approximately 2 L/day for several days, both the output and the pleural effusion decreased on chest X-ray. On POD 46, the pleural effusion nearly completely resolved and the chest tube was removed. From then on the octreotide was stopped. Her oral diet was resumed on POD 57, and subsequent CT revealed recurrence of the massive right pleural effusion and a small amount of ascites, leading us once again to make our patient take nil by mouth (Fig. ).
Given that conservative management was ineffective in definitively treating the chyle leak, we performed lymphangiography through the inguinal lymph nodes with Lipiodol (ethiodized oil) on POD 62 to identify the location of the chyle leak and to develop a therapeutic strategy. Lymphangiography and post-procedure CT revealed that there was extravasation of the Lipiodol (ethiodized oil) near the right mediodorsal pleural space along the diaphragm, with an accumulation of Lipiodol (ethiodized oil) located near the staple line of the stump of the right hepatic vein; the exact location of the leak was not identified (Fig. ). CT also showed that there was neither obstruction nor dilation of the thoracic duct (Fig. ). An abdominal source of the chyle leak was not demonstrated. A chest tube was placed after the lymphangiography. The tube drained less than 500 mL/day for a week, and the pleural effusion resolved 10 days after the lymphangiography. She was discharged on POD 72. Two and a half years after the original surgery, she is doing well without evidence of recurrence of either chylothorax or cancer.
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pmc-6260701-1
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A nine-month-old Chinese girl presented with one-week history of pallor at a referral hospital where she received a red blood cell transfusion for severe anemia (Hb 3.4 g/dL) and started to treat for iron-deficiency anemia (IDA) after microcytosis (mean corpuscular volume 74.6 fl), hypochromia (mean cell Hb 21.5 pg), and low serum iron concentration (1.28umol/L) were confirmed. On discharge after 1 week of treatment, anemia was corrected (Hb 12.4 g/dL). However, recurrent anemia was observed over a six-month period, even another red blood cell transfusion was given in this period. Positive fecal occult blood test results were intermittent. A chest computed tomography (CT) scan showed the increase of patch density in the left lower lobe (Fig. a) and right upper lobe (Fig. b) of the lung. Although she had no history of repetitive haemoptysis, chronic cough and dyspnoea, idiopathic pulmonary haemosiderosis (IPH) was entertained and the IDA therapy was discontinued.
Patient was referred to our hospital for further management. Flexible bronchoscopy was performed, but bronchoalveolar lavage examination of blood-stained fluid and hemosiderin-laden macrophages from involved areas was negative. Review of the chest CT scan showed no extensive ground glass opacities and reticular shadows. Therefore, diffuse alveolar haemorrhage was ruled out. Review of the patient’s history found an episode of intermittent melena 1 month after the IDA treatment, and that was considered to be the side effect of the drug by the outpatient doctor. No related family genetic history. Physical exam demonstrated a girl of normal appearance consistent with her ethnicity except pallor. The diagnostic approach for gastrointestinal bleeding was started. However, the patient underwent both upper and lower endoscopy with negative findings in all of the endoscopic examinations. Plain and enhanced CT of abdomen and the technetium-99 m–labeled red blood cell scans were performed. Again, they were all negative. Her symptoms persisted and one red blood cell transfusion was needed each week.
The department of general surgery was involved in the management and a decision to do surgical exploration with laparoscopy was taken. A 3 cm lesion with dense blistered protrusions on the surface was found within the wall of jejunum (Fig. ), acting as a lead point, so a jejunal segment was resected and an end to end jejunojejunostomy was performed. Pathological examination indicated a vascular malformations (VM) (Fig. ). Postoperative period was uneventful and she was discharged home with no complications. There was no recurrence during follow-ups.
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pmc-6260715-1
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A 38-year-old female patient presented with abdominal pain and right upper quadrant mass for 10 days. She had a history of pregnancy and caesarean section in the lower abdominal region 10 years before, but there was no history of trauma and upper abdominal surgery. Clinical examination showed a huge mass about 15 × 10 cm over right upper abdomen. There was moderate tenderness in the area of the mass, and the abdominal skin was intact and smooth except the lower abdominal surgical scar. Abdominal wall ultrasonography showed mixed echo-mass under the muscle layer of right upper abdominal wall. Contrast-enhanced computed tomography (CECT) of abdomen was performed. The images revealed the dilated small intestine between the swelling ventral muscles in the right upper abdominal wall which suggested a ventral hernia in the right upper abdominal wall (Fig. ). There was no history of right upper abdominal surgery or trauma, therefore the surgeons considered it was a spontaneous hernia. The patient had much more severe abdominal pain and began to present hematochezia during her hospitalization. Two hours later, she underwent emergency surgery. The intraoperative finding was an atypical ventral hernia, and the ileum loop was incarcerated with necrosis. The hernia sac was located between the semilunar line and the edge of the rectus abdominis muscles in the right upper abdominal wall (Fig. ), which confirmed it was a Spigelian hernia. The defect size was 2.0 × 1.5 cm. The necrotic ileum segment was resected and end-to-end anastomosis was performed. Considering the small defect and the necrotic ileum, a simple herniorrhaphy was made by suturing the internal oblique and transverse muscles to the rectus sheath. The postoperative course was uneventful, the patient recovered well and was discharged after 1 week of hospitalization. The patient had a favorable outcome for 1 year without recurrence.
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pmc-6260726-1
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A 33-year-old woman of African background with three symptomatic intramural uterine fibroids in the anterior wall which caused deformation of the uterine cavity with two years of unsuccessful pregnancy attempts. Patient had previously underwent a laparoscopic enucleation of uterine fibroid in the posterior wall. The fibroids were classified as Funaki type I based on T2W images, and contrast-enhanced T1W images were assessed. Fibroid sizes were 5.0 × 4.6 × 4.8 cm3, 2.5 × 1.3 × 2.0 cm3, and 2.8 × 1.9 × 2.4 cm3 (Fig. ).
As demonstrated in Fig. , filling the urinary bladder to the point of discomfort did not achieve the desired results, and despite tilting and shaping of the ultrasound beam, sufficient coverage of the target could not be achieved (Fig. ).
After application of the wedged gel pad, the bowels were repositioned so that the two target fibroids could be safely sonicated (Fig. ).
Before therapy, good acoustic coupling between the surfaces was ensured with T1W sequence for detecting any air bubbles in the sonication path. The largest fibroid was treated first followed by the smaller fibroid. Good temperature rise was observed, average maximum temperature per sonication was 76.8∘C (63.8−115.1∘C), in both fibroids. The patient experienced mild pain during the treatment and opioid pain medication (fentanyl, 0.5 μg) was administered. The patient reported the gel pad was getting hot during the sonications but no adverse effects were observed due to the treatment and gel pad usage. This highlighted the importance of increased cooling times between sonications. The total treatment time from first to last sonication (19 sonications) was 178 min and average treatment power and energy per sonication were 184 W and 5.3 kJ, respectively. Immediately after the treatment contrast-enhanced T1W images were acquired, indicating non-perfused volume ratios (NPVs) of 86% and 39% (Fig. ). There were no areas of abnormal enhancement within the subcutaneous tissue, and skin was normal after treatment. The volume and shrinkage of the fibroids at 3 months follow-up were 29 mL and 49%, 5 mL and 2.5% respectively (Fig. ).
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pmc-6260726-2
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A 40-year-old woman presented with symptomatic intramural uterine fibroid in posterior wall. The presented symptom was severe menorrhea. The fibroid was classified as Funaki type II based on T2W images, and contrast-enhanced T1W images were assessed. Fibroid size was 5.7 × 5.0 × 5.0 cm3 (Fig. ).
As demonstrated in Fig. , the filling of the urinary bladder to the point of discomfort, again did not achieve the desired results and despite tilting and shaping of the ultrasound beam, sufficient coverage of the target could not be achieved (Fig. ).
After application of the gel pad, the bowels were repositioned so that the uterine fibroid could be targeted safely (Fig. ).
Before therapy, good acoustic coupling between surfaces was confirmed with T1W sequence to detect any air bubbles in the sonication path. Despite high power (300 W), a poor temperature rise was observed, average maximum temperature per sonication was 56.4∘C (47.3−72.4∘C) in the fibroid. The patient experienced severe pain during the treatment and opioid pain medication (fentanyl, 0.5 μg) was administered twice during the treatment. The patient reported a sensation of heat on the skin surface during the sonications and after the treatment the skin looked visually irritated due to the treatment and the gel pad usage. The total treatment time from first to last sonication (9 sonications) was 155 minutes and average treatment power and energy per sonication were 284 W and 6.8 kJ, respectively. Immediately after the treatment, contrast enhanced T1W images were acquired, showing non-perfused volume ratio (NPV) of only 3% (Fig. ). For this reason more detail analysis was done after the treatment. The conclusion is that the poor treatment result was most likely caused by high perfusion in the fibroid which prevents the tissue from reaching the thermal ablation temperature. This conclusion was reached based on dynamic contrast enhanced images that were obtained during the screening MRI. There were no areas of abnormal enhancement within the subcutaneous tissue, however there was reddening of the skin which disappeared in few hours. This could be caused by the gel pad as it is functioning as an insulator and the cooling effect of direct skin cooling device does not reach the skin as effectively as without the gel pad. This results in higher tissue temperatures which may cause irritation to the skin or even skin burns if cooling times are not sufficient.Since this is unusual shape of the gel pad in not taken into account by the software, maybe we should use slightly longer cooling times in these cases. Due to poor treatment result patient was settled on hysterectomy.
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pmc-6260759-1
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An 81-year-old female patient, Mrs. NM, presented to casualty with a 3-year history of melaena, haematemesis and lethargy. Previous medical history recorded 8 uneventful deliveries and multiple tooth extractions which were not associated with excessive bleeding. There was also no family history of pathological bleeding. On clinical examination, the patient had signs of cardiac failure but was haemodynamically stable. She had diffuse cutaneous ecchymoses and significant pallor. There was no clinical evidence of haemarthrosis. There was no organomegaly or lymphadenopathy and the neurological system was grossly intact. Laboratory testing revealed a severe normocytic, normochromic anaemia. Her renal function was normal. Her albumin was mildly reduced but liver enzyme levels were not elevated. Coagulation testing revealed a prolonged activated partial thromboplastin time and a mildly prolonged prothrombin time which both corrected on mixing studies (data not shown). The Factor VIII levels and von Willebrand Factor antigen and activity (Ristocetin co-factor) levels were markedly reduced. Platelet aggregation studies showed a markedly reduced response to all platelet agonists including high dose ristocetin. Platelet function analyser studies demonstrated prolonged closure to both collagen/epinephrine and collagen/ADP (Table ).
Gastroscopy revealed mild duodenal angiodysplasia with active bleeding. The patient received multiple blood and coagulation factor transfusions, anti-fibrinolytic agents and proton-pump inhibitors to control the bleeding. Monthly cycles of intravenous immunoglobulin (Polygam) of 40 g daily for 3 days were commenced and 3 cycles were administered. This regimen resulted in a clinical response with cessation of the bleeding and partial transient recovery of the vWF and Factor VIII levels (Fig. ). Ristocetin response on platelet aggregometry demonstrated partial improvement. Following clinical stabilization, the patient was discharged to outpatient follow-up and subsequently relocated to a rural treatment facility.
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pmc-6260776-1
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Case 1 is a 51-year-old man of North African origin with a history of hypertension who had been diagnosed as having a right kidney tumor associated with one bulky pleural metastasis and some smaller metastatic lesions of the lung; Case 1 is summarized in Fig. . No bone or brain metastases were observed at initial work up; he was classified in the poor risk group according to the International Metastatic RCC Database Consortium (IMDC) []. A radical nephrectomy was performed in July 2012. A pathological report indicated a renal cell carcinoma (RCC) of 16 cm with a clear cell component and some degree of a more aggressive cellular component, giving a Fuhrman grade of 4, pT3a pN0 M1 according to the Union for International Cancer Control (UICC) classification. Sunitinib, 50 mg/day, then reduced to 37.5 mg due to side effects, was administered during 6 months. Because of the painful progression of the pleural metastasis in the upper part of his left lung, radiation therapy was delivered to this tumor. Systemic treatment was further modified for the approved second-line treatment everolimus. This latter treatment induced a significant tumor response in most metastatic sites for 15 months before re-progression. In March 2014, our patient complained of persistent headaches and brain magnetic resonance imaging (MRI) identified a single right frontal metastasis. Stereotactic radiotherapy was performed and a treatment with axitinib, a second-line TKI directed against VEGFrs, was started. Axitinib induced significant tumor shrinkage in the pleural and lung metastases; the brain metastasis was much improved because a brain MRI was considered almost normal. Axitinib was maintained for 18 months, but had to be completed because of a severe episode of angina pectoris. A coronary stent that required dual anti-platelet therapy for 6 months was indicated. Due to an increased hemorrhagic risk with this treatment together with a VEGFr inhibitor, axitinib was not resumed; nivolumab, a programmed death-1 (PD1) directed antibody recently approved for mRCC treatment, was administered as part of a compassionate program. Our patient was admitted with seizures and vertigo 4 months after immunotherapy initiation. A brain MRI evidenced the enlargement, with some hemorrhagic traits, of the previously treated metastasis. Further to therapeutic control of his neurological symptoms, a thoracic and abdominal computed tomography (CT) scan showed the progression of the disease at all metastatic sites. He then entered a genetic profiling experimental program (NCT 01774409) and sequencing of the primary tumor was performed. Due to the long duration of this analysis, cabozantinib that had been recently made available was administered after our patient gave consent. All neurological symptoms disappeared and his performance status (PS) score improved to 0 after 2 weeks of treatment with cabozantinib. A brain MRI and thoracoabdominal CT scan at 8 weeks indicated a significant shrinkage of the metastatic lesions including the brain metastasis (Fig. ). Treatment was rather well tolerated but had to be reduced to 40 mg per day instead of 60 mg, due to fatigue, stomatitis, and loss of weight. Cabozantinib is ongoing with a reduced dose of 40 mg/day with a maintained efficacy over 8 months. Meanwhile, results of the tumor sequencing indicated the expression of mutation of the MET gene.
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