A 79-year-old Caucasian man presented to the clinic with reduced appetite, unintentional weight loss of approximately 6 kg, and a history of fatty food intolerance. He denied any abdominal pain or febrile episodes, and physical examination was unremarkable. Biochemical investigations, however, revealed deranged liver-function tests with total bilirubin, 66 μmol/L; ALT, 122IU/L; ALP, 274IU/L; and gamma GT, 864IU/L.
An abdominal ultrasound showed a dilated common bile duct (CBD) with stones inside it. The gallbladder was not visualized, but strong echoes with acoustic shadowing were seen, suggesting a contracted gallbladder. A computed tomography (CT) scan revealed stenosis of the proximal CBD and dilated intra- and extra-hepatic bile ducts. Furthermore, it showed a small pseudocystic structure that was assumed to be a shrunken gallbladder.
The patient proceeded to endoscopic retrograde cholangiopancreatography (ERCP), which showed multiple stones (the largest measuring 1.5 cm) in the CBD, which could not be removed, and so a stent was inserted (see Figure ). The gallbladder was not visualized, but this was thought to be the result of insufficient contrast. A repeated ERCP a few months later was reported as having cleared the CBD of all stones; the stent was removed, but the gallbladder had still not been visualized. As he had developed some intermittent right upper quadrant pain over this time, he was listed for a laparoscopic cholecystectomy for presumed choledocholithiasis.
At laparoscopy, a small fibrous remnant was seen in the gallbladder fossa, but the gallbladder could not be found despite an extensive search of all possible ectopic sites. Conversion to an open procedure and on-table cholangiogram revealed a dilated CBD and confirmed gallbladder agenesis (see Figure ). The CBD was explored, numerous stones removed, and a T-tube was inserted.
Post-operatively, he made an uneventful recovery, and remains symptom free.


The patient was a right-handed, 48-year-old man who suffered a severe closed head injury after falling from a ladder 33 days prior to the first MRI examination. His Glasgow Coma Scale score at the time of admission was 5 (E:1; V:1; M:3). Initial computed tomography (CT) revealed a large right parenchymal insular hematoma with subdural collection, right subarachnoid hemorrhage, intraventricular hemorrhage and a right-to-left shift, as well as subfalcial and infratentorial herniation affecting the brainstem. A right craniotomy was performed and the insular hematoma was evacuated. Post-surgical and control CT revealed an important reduction of the mass-effect without midline shift or herniation signs. Sedation was progressively withdrawn and the patient remained in a comatose state. An EEG examination showed a diffuse bilateral pattern of slow-wave activity and no response to stimuli.
At the time of the first MRI evaluation (33 days post-TBI) the patient presented with spontaneous ocular opening and flexion withdrawal in the upper extremities. The patient's score on the Disability Rating Scale [] was 24, with a level 2 (generalised response) score on the Level of Cognitive Functioning Scale []. There was no evidence of visual fixation, visual pursuit, or of response to command, indicative of the VS []. In the months that followed, the patient made a progressive neurological recovery. At the time of discharge (three months post-ictus), he demonstrated evidence of visual pursuit and reproducible responses to simple commands, indicative of a recovery to a minimally conscious state (MCS) at this time [].
Seven months from discharge (ten months post-ictus), the patient was re-admitted to hospital, at which point he and his relatives were approached for a re-evaluation. The patient was aware although disoriented in time. A brief bed-side neuropsychological assessment assessed the patient's language and praxis using subtests of the Spanish version of the Boston Diagnosis Aphasia Examination: conservational and expository speech, auditory comprehension, oral expression and praxis []. Visual neglect was assessed with Albert's test []. The patient showed a recovery of language comprehension and repetition skills, although his spontaneous speech and phonetic and semantic fluencies were still impaired. Although his speech was dysprosodic and dysarthric, the recovery of the patient's receptive language abilities allowed reliable communication. Normal ideomotor praxis was observed with the right hand, although bilateral neglect with a left-sided predominance, along with a major attentional impairment precluded the assessment of other complex cognitive functions.
Twelve months after the TBI, the patient was admitted to a neurorehabilitation center where he underwent a second MRI examination and a comprehensive neuropsychological assessment including the Test Barcelona-Revised, [] Digit span and Letter-Number Sequencing (LNS) subtests of the WAIS-III, [] Rey's Auditory Verbal Learning Test (RAVLT), [] and a phonetic verbal fluency task []. Although he was orientated in person, place and time, he showed impairment in attention, working memory and visuo-perceptive functioning. All language functions (repetition, confrontation naming and comprehension tasks) were in the normal range. A second neuropsychological assessment performed two months later (14-months post-ictus) showed a significant improvement in all impaired functions including, most markedly, declarative memory (Table ).


Our patient was a 37-year-old Japanese woman with a 12-year history of AN. She had been hospitalized frequently in the gastrointestinal unit for nutritional treatment, but she continuously rejected hospitalization in a psychiatric unit probably due to her denial of the illness, despite her frequent, self-induced vomiting. She had been admitted to the hospital three times previously because of general fatigue combined with acute liver failure. At these admissions, her Glasgow coma scale (GCS) was in the normal range of 12 to 15. She was strongly recommended to consult a psychiatrist, but turned down the advice because of denial of her AN. Therefore, she was only prescribed ursodeoxycholic acid, multivitamin, and an enteral nutritional supplement. She had never previously received any psychotherapy. Furthermore, she had no history of alcohol abuse, anti-depressant drug intake, narcotic drug abuse or suicide attempt.
On the three prior admissions, physical examination had revealed severe emaciation, with a weight of 29.0 kg and a height of 1.52 m (body mass index (BMI) = 12.6). Her body temperature was below 36°C, her blood pressure was around 85/50 mm/Hg, with a regular heart rate of around 80 beats per minute.
At the time of her fourth admission, she was in a deep coma with a GCS score of 3. Arterial blood gas analysis revealed an arterial oxygen concentration of 97% in room air. The electrocardiogram showed sinus rhythm and a heart rate of 88 beats per minute. She had a weak, but positive papillary response without papillary mydriasis or miosis. Her body temperature was 35.6°C. There were no signs of respiratory or cardiac disease. Her blood sugar level was 68 at the time of admission, in the range of her usual level of 50 to 70. Computed tomography (CT), magnetic resonance imaging, and magnetic resonance angiography of the head showed no abnormality.
Aspartate aminotransferase was 3194 IU/L (reference range, 7 to 38 IU/L); alanine aminotransferase, 3540 IU/L (4 to 44 IU/L); alkaline phosphatase, 2388 IU/L (100 to 320 IU/L); γ-glutamyl transpeptidase, 342 IU/L (2 to 40 IU/L); NH3, 51 μg/dL (40 to 80 μg/dL). The ratio of branched-chain amino acids versus aromatic amino acids (BCAA/AAA) was 3.8 (2.5 to 3.5); albumin was 3.6 g/dL (3.8 to 5.3 g/dL); total bilirubin, 1.7 mg/dL (0.2 to 1.0 mg/dL); total cholesterol, 117 mg/dL (130 to 220 mg/dL); prothrombin activity, 49.8% (80 to 120%); hepaplastin test, 50.1% (70 to 130%); Type IV 7S-collagen, 4.9 ng/mL (< 6.0 ng/mL); HbA1c, 4.0% (4.3 to 5.8%); blood urea nitrogen, 23.6 mg/dL (8.0 to 20.0 mg/dL); creatinine, 0.69 mg/dL (0.3 to 0.8 mg/dL); white blood cells, 4070/mL (3800 to 9300/mL); hemoglobin, 12.1 g/dL (11.5 to 15.0 g/dL); hematocrit, 34.5% (33.5 to 44.5%); Fe, 123 μg/dL (48 to 154 μg/dL); Cu, 78 μg/dL (66 to 130 μg/dL); Zn, 92 μg/dL (59 to 135 μg/dL); platelet count, 12.7 × 104/μL (13 to 37 × 104/μL); and total protein, 5.0 g/dL (6.5 to 8.2 g/dL). Anti-nuclear and anti-mitochondrial antibodies were negative. Serologic tests for hepatotropic viruses (hepatitis A, B, and C viruses, cytomegalovirus, and Epstein-Barr virus) and the urinary toxicology screen (alcohol, cannabis, cocaine, paracetamol, amphetamines, benzodiazepines, methadone, opiates) were negative. Ultrasound showed a mild fatty liver, but the CT score (Hounsfield units) of the liver was slightly higher than that of the spleen (data not shown).
The NH3 and BCAA/AAA levels remained normal during our patient's coma and afterward, and the blood sugar remained close to her usual level (Table ). TPN and enteral tube feeding were administered on the day of admission. Her consciousness gradually normalized at day 10, which was paralleled with an improvement of her severe liver dysfunction (Table ). Comparing the broad spectrum of laboratory clinical parameters with her GCS level, only serum transaminases showed a strong inverse correlation. Of note, blood sugar, plasma NH3, and the BCAA/AAA ratio were not correlated with her consciousness. A liver biopsy was performed after the recovery of her liver function at day 14. Ballooning of hepatocytes, necroinflammatory changes, and macrovesicular steatosis were observed in hematoxylin-eosin-stained sections (Figure ), but both iron and copper staining were negative (data not shown). No etiology of the deep coma, other than acute malnutrition-induced liver injury, was detected.


A 21-month-old female with recent history of two febrile seizures (30 minutes apart) presented for an outpatient MR imaging study of the brain to complete the neurological evaluation. The infant was an otherwise healthy, full-term baby, and with an unremarkable medical history and review of systems. Upon presentation, the patient was not taking any medications although her mother carried Diastat (Acudial. Diazepam, Valeant Pharmaceuticals. Costa Mesa, CA) in the event the seizure recurred. The 9.9 kg infant presented in a calm, nonagitated state with a heart rate (HR) of 110 beats/minute, respiratory rate (RR) of 20 breaths/minute, and noninvasive brachial blood pressure (NIBP) measurement of 85/60 with a mean arterial pressure (MAP) of 68 and a room air oxygen saturation of 100%.
After careful review of the infant, discussion with the mother, and a physical examination, the dexmedetomidine was ordered per protocol by the pediatric nurse practitioner under the supervision of an anesthesiologist. Written, informed consent was obtained from the mother for the dexmedetomidine sedation. A 24-gauge intravenous catheter was initiated and dexmedetomidine was ordered per protocol, specifying a bolus of 2 mcg/kg over 10 minutes and a subsequent infusion of 1 mcg/kg/hr. The bolus was administered at the ordered rate of 2 mcg/kg over a period of 10 minutes using an Iradimed 3850 mRidium MR IV pump (Iradimed Corp, Winter Park, Fla). Vital signs (NIBP, MAP, 3-lead EKG, pulse oximeter, and HR) were monitored continuously using an Invivo Precess monitor (Invivo Corp, Orlando, and Fla) and documented every five minutes. The patient achieved successful sedation conditions (Ramsay Sedation Score = 4) by the completion of the bolus. Upon completion of this 10-minute bolus, NIBP was 118/79 (MAP 92), HR 77 (normal sinus), RR 17, and oxygen saturation of 97% on 2 liters/min oxygen via nasal canula (see ). Sedation guidelines at our institution specify supplemental oxygen delivery throughout the sedation and recovery period until discharge criteria are met. Following the bolus, the infusion pump initiated delivery of the dexmedetomidine infusion at the rate that had been programmed in at the initiation of the sedation. The dexmedetomidine infusion was continued throughout the sedation until the MRI scan was complete. The patient remained hemodynamically stable throughout Vital signs were documented at five-minute intervals per our hospital standard for sedation, following initiation of this dexmedetomidine infusion ().
At the termination of the study, the radiology nurse noted that the dexmedetomidine remaining in the syringe was less than anticipated and medication reconciliation was immediately initiated with a second nurse. Reconciliation revealed that the 9.9-kg infant had received 196 mcg more dexmedetomidine than had been ordered. Review of the intravenous infusion pump revealed that the pump had been misprogrammed to deliver the infusion at a rate of mcg/kg/min rather than mcg/kg/hr. Thus, instead of delivering the usual 1 mcg/kg/hour as ordered, the infusion pump delivered the dose at 1 mcg/kg/minute (equivalent to 60 mcg/kg/hr). Because this error was not identified during the mandatory institutional nursing “double check” when the infusion pump was programmed, the error was not identified, and the dexmedetomidine infusion was continued for the 20 minutes.
Following the imaging study, the patient was transferred uneventfully to the radiology recovery room at which time monitoring was continued. The patient arrived in the recovery room deeply sedated with an RSS 4. Per recovery room policy, NIBP, MAP, EKG, HR, O2 Sat, and RR are documented every 5 minutes until modified Aldrete discharge criteria are met []. A minimum Aldrete score for discharge from the recovery room is 9. Although documented every 5 minutes, pulse oximetry is monitored continuously. Throughout the recovery room course, all physiologic parameters remained within age-adjusted normal values. 20 minutes after the discontinuation of the dexmedetomidine, the infant had achieved a modified Aldrete score of 9 and maintained an Aldrete of 9-10 throughout the remainder of the recovery room stay. No cardiac arrhythmias were noted at any time, both during the sedation as well as in the recovery room period. As soon as the error in dosing was identified, Risk Management was notified and the parents were debriefed and all questions answered by the Risk Management team as well as the supervising anesthesiologist. The parents were informed that the intravenous infusion pump had been misprogrammed and that the child had received an infusion of 60 times that which was ordered and intended. The effects of such a high dosage of dexmedetomidine delivery to an infant had not to date been described nor documented. In adults, the distribution half-life (t1/2) is approximately 6 minutes and the terminal elimination half-life (t1/2) is approximately 2 hours [].
The infant returned to baseline neurological status, an Aldrete Score of 10, after a 2-hour recovery room period. Although the half-life of dexmedetomidine is relatively short, and the child met discharge criteria with a modified Aldrete Score of 10 within hours of discontinuing the dexmedetomidine, the physicians chose to admit her to the hospital for overnight, continuous cardiorespiratory monitoring. Subsequently, the patient was admitted to the intensive care unit (ICU) for overnight monitoring of EKG as well as pulse oximetry, blood pressure, and neurological status. The child remained hemodynamically and neurologically stable in the ICU throughout the night. There were no arrhythmias, no change in neurologic status nor any deviation in heart rate or blood pressure outside of age-adjusted anticipated normal values. The next morning, after reassessment by neurology and the intensive care unit service, the infant was discharged home with no subsequent sequela.


An 1 year old male with no family history of RB had right leukocoria at presentation. The first examination revealed a solid mass which occupied the vitreous cavity, and caused a great inferior retinal detachment. There was no evidence of vitreous seeding. Intraocular pressure (IOP) elevation was detected in the right eye. The right eye was enucleated and substituted by an hidrossiapatite implant. Histopathologic examination revealed an elevated grade of differentiation of the tumor without infiltration of the optic disc. In the left eye, we observed a little tumor near the inferior vascular arch, at 1 optic disc distance from the fovea, and another little tumor near the superior vascular arch.
Genetic analysis (FISH fluorescence In Situ Hybridization Retinoblastoma DNA Probe 13q14) demonstrated the absence of RB1 gene delection.
The left eye was treated with chemotherapy associated with focal laser treatment. The AIEOP/RTB-92 Treatment Protocol (4 cycles regimen consisting of Carboplatin (165 mg/kg/die) and VP16 (50 mg/kg/die for 2 days)) was applied. An examination at the end of chemotherapy with B-scan ecography confirmed a partial reduction of both tumor size (from 5,5 mm to less then 4 mm) and thickness (from 2 mm to 1 mm). At the end of chemotherapy, a laser treatment around the tumor mass in the left eye was applied using 450-600 mW, with a spot of 600μ. Subsequently, fundus examination revealed the presence of a tumor in a phase of "cottage cheese" and a ipopigmented atrophic area near the treated areas. Patient was then evaluated every 3 months for the the first year, and every six months starting from the second year. No signs of disease were detected. At the 5 years follow-up, we observed a very aggressive recurrence in a new site at the B-scan ecography (Figure ). Fundus examination of the left eye revealed the presence of a new great tumor mass (9,77 mm height and 12 mm at the base) in the superior quadrant, with partial detachment of the surrounding retina (Figure ). This recurrence was treated with chemotherapy (6 cycles of IVad: VCR 1,50 mg/push in 15 ml Sol. Gluc. 5% in 10' Adriblastin 15 mg in 100 ml SF; MESNA 800 mg in 20 ml SF/push; Ifosfamide 2000 mg in 1 vial/20 ml; with preidratation and idratation after the infusion of the chemotherapy) and, subsequently, with cryotherapy. After cryotherapy, we observed a reduction in the tumor size, paralleled by an increased retinal detachment and presence of fibrin in the anterior chamber, that impaired any further examination of the fundus. 30 days after, when the fibrin clot in the anterior chamber was reduced, we observed numerous little new tumors, widespread in the retinal tissue. Given the intraocular dissemination of the tumor, the left eye was enucleated.


An 8 months old female with no family history of RB had bilateral leukocoria at presentation. The first examination revealed three masses of medium dimension at 700 μ distance from the fovea, with retinal detachment in the right eye and a mass (whose dimensions were 1.01 × 1.03 × 1.22 cm) in the left eye. The mass occupied 50% of the vitreous cavity and was associated with retinal detachment and vitreal seeding (Figure ). Nuclear Magnetic Resonance (NMR) showed a large calcified mass with no evidence of invasion of the optic nerve. No metastases were detectable.
The left eye was enucleated according to the AIEOP/RB 05 Treatment Protocol. The histological examination revealed an elevated grade of differentiation of the tumor without infiltration of the optic disc. RB1 gene analysis on peripheral blood lymphocytes demonstrated deletion of exon 24. At the end of the third cycle of chemotherapy the tumors in the right eye were partially reduced (50% of tumor size) and cryotherapy and laser therapy were utilized. Patient was evaluated every 3 months for the the first year, and every six months starting from the second year. Two years later we observed a new aggressive recurrence in the right eye but, unlike Case 1, we decided to treat this patient according to the more recent aquisitions in literature []. Therefore, the patient was assigned to receive tandem HDCT/ASCR (high-dose chemotherapy/autologous stem cell rescue). The right eye at the moment (4 months from the end of therapy) shows a modest reduction of dimension of recurrence, between still accompained by a subtotal retinal detachment.
In both RB samples we studied the VEGF-A pathway, determining the expression level of the vegf-a, VEGF-A receptors (flt-1 and kdr) and hif1-α transcripts. The RQ-PCR technique was applied and results were compared with the expression level of the corresponding normal retina. The vegf-a transcript was over-expressed in both cases (3.8 fold increase in case 1; 51.9 fold in case 2). Both flt-1 and kdr were expressed in both RB samples at levels higher than normal retina (flt1: 2.8 folds in case 1, 8.1 folds in case 2, median value 5.5; kdr: 5.5 folds in case 1, 2.1 folds in case 2, median value 3.8). Finally, the hif1-a transcript was over-expressed in both samples (11 fold in case 1), with higher levels in case 2 (76 fold). Both isoforms of the herg1 gene (herg1a and herg1b) were over-expressed compared to the corresponding normal retina. The full length isoform herg1a was expressed at higher levels compared to the herg1b transcript (herg1a: 17.1 folds in case 1, 150 folds in case 2, median value 83.6; herg1b: 4.3 folds in case 1, 15 folds in case 2, median value 9.7). Finally, both isoforms were expressed at higher levels in case 2 (Figure ).
We then evaluated the levels of VEGF-A in the vitreous fluid obtained during the enucleation of both cases. The levels of VEGF-A were 854 ± 83 pg/ml in case 1 and 1120 ± 98 pg/ml in case 2, respectively (Figure ). The median value of VEGF-A was markedly higher in the RB samples (987 pg/ml) when compared to the values of the control group, reported in [], which refers to aged-paired patients (see Table Two reported in []: 59 pg/ml; range 38-135).
Finally, we studied the distribution of the VEGF-A and hERG1 proteins in the two tumor samples by immunohistochemistry. A strong immunopositivity for VEGF-A was detected in both the RB samples analyzed (see Figure ): 68 ± 7% and 83 ± 6% of cells were positive for VEGF-A staining, in case 1 and 2, respectively. According to the method reported in [] most of the cells showed a strong intensity score for both samples (see insets of the VEGF-A panels in Figure ). Consistently the microvessel density (MVD), evaluated by CD34 staining, was high, with a significant higher MVD value in case 2 compared to case 1 (p = 0.037, t Student test)(Figure ). hERG1 expression was evaluated by staining with a monoclonal anti hERG1 antibody (clone A12) which exhibits an high specificity although with a less intense staining (Lastraioli E, manuscript in preparation). 82 ± 4% (in case 1) and 91 ± 7% (in case 2) tumor cells were positive for hERG1, most of them with a moderate intensity score.
Finally, a quantitative analysis of proliferation and apoptosis was performed. The proliferative index (PI) was 54 ± 7% in case 1, and 78 ± 8 in case 2. Similar levels of the apoptic index (AI) were found in both samples, with values comparable to those described in the literature (less than 3%) [](Figure ).


A 25-year-old Caucasian gentleman presented to our clinic with a 12 month history of worsening dysaesthesia and paraesthesia affecting the thumb and index finger of his right dominant hand. There were no deficits in motor function affecting the right hand. The patient reported no medical antecedents, apart from an apparent ganglion of his right wrist.
Physical examination revealed a soft, non-tender, subcutaneous 2 × 1 cm mass over the volar aspect of the right wrist with no atrophy of the thenar eminence. Altered sensation in the distribution of the median nerve of the right hand was detected by static two point discrimination and the use of the Semmes-Weinstein monofilament test. Tinel's sign and Phalen's test were both positive. Somatic examination did not reveal any signs of neurofibromatosis or lymph node tumefaction.
Nerve conduction studies inferred a reduction in median nerve conduction at the wrist. Radiographs of the hand and wrist did not show any ectopic calcifications or bony abnormalities. The MRI scan revealed an 18.5 × 9.7 mm volar mass within the carpal tunnel which was initially thought to be a ganglion cyst compressing the median nerve (Figures and ). On the basis of this surgical exploration and decompression was recommended.
Under general anaesthetic, a longitudinal incision was made at the wrist. After dividing the transverse carpal ligament a grossly enlarged median nerve was observed. To facilitate exploration, the incision was extended up to the middle of the forearm and distally into the palm. The nerve was yellow and pink with longitudinal fatty streaks within the nerve fibres. These fatty streaks extended from the distal third of the forearm to the distal edge of the carpal tunnel (Figure ). Excision of the adipose tissue between the nerve fascicles was impossible due to the risk of causing neuronal injury. A biopsy of the nerve was taken and the carpal tunnel was successfully decompressed.
Histological examination revealed non-neoplastic fibrofatty elements infiltrating the perineurium with mature fibrous and adipose tissue separating nerve fascicles. There was no neural hypertrophy. A histological diagnosis of LFH was made. The postoperative period was unremarkable and the patient experienced a complete resolution of his symptoms. At the 6 month follow-up appointment he remained asymptomatic.


A 68-year-old male presented to our surgical outpatient department with a huge lump at the back of his neck for the last 11 years. There was an ulcer at the top of the lump for the last 9 months. Episodic bleeding was also reported from the ulcer for the last 6 months.
On local examination of the neck, there was a 22 cm × 12 cm-sized swelling at the posterior side of the neck
[]. It was nontender and soft to firm in consistency. There was a 6 cm × 5.5 cm decubitus ulcer at the top of the lump. Dilated veins were present in the skin around the decubitus ulcer. There was no regional lymphadenopathy.
Fine needle aspiration revealed mature lipocytes indicative of lipomatous lesion. Contrast Enhanced Computed Tomography Scan of the neck revealed a giant subcutaneous lipoma at the posterior triangle without septations. There was no communication with the spinal cord.
After intubation with general anaesthesia, the patient was positioned in a prone position. An elliptical transverse incision of 12 cm was given at around the base of the lump. The superior and inferior skin flaps were raised []. Separation of lipoma from the surrounding tissues was easy and was performed with sharp and blunt dissections. Five to six large feeding vessels were also ligated to isolate the lipoma. The redundant skin was removed and the upper and lower skin flaps were stitched together with Silk-3-0 after securing haemostasis and placing a suction drain. The resected mass was 2.2 kg in weight and 22 cm × 12 cm in diameter []. The postoperative period was uneventful. The drain was removed after 3 days and the patient was discharged on the 9th postoperative day.
Histopathological analysis of the resected mass revealed mature, proliferative lipocytes with no cellular atypia, and it was diagnosed as benign giant lipoma.


A 76-year-old gentleman with a history of obstructive airways disease and obstructive sleep apnea was found to be hypoxic during a routine colonoscopy procedure. To evaluate his lungs and hypoxia further, he was referred for a CT pulmonary angiogram (CTPA). This was performed on a third generation multislice scanner (GE LightSpeed RT, GE Medical Systems, Milwaukee, WI) which demonstrated anomalous drainage of a left superior vena cava (LSVC) into the left atrium (LA) with features of pulmonary arterial hypertension and a possible atrial septal defect (ASD). Transthoracic echocardiography (TTE) confirmed pulmonary hypertension, but was unable to demonstrate either an ASD or the anomalous drainage due to suboptimal views in this rather large patient. Cardiac catheter study with oxygen saturations performed on the same day demonstrated a shunt fraction of 1. Subsequent cardiovascular magnetic resonance (CMR) imaging was requested to further delineate the anatomy and physiology. This was performed on a 1.5-T system (Avanto, Siemens, Germany).
Transaxial multislice scout images [] and multiple steady-state free precession cine images in left ventricular long- and short-axis planes were acquired. A coronal cine was acquired to show the LSVC relative to the LA []. In view of the suspected ASD, a contiguous stack of atrial short-axis cine images was also acquired, parallel to the ventricular short axis [], and breath-hold phase-contrast velocity mapping was performed through planes transecting the aorta with a velocity encoding (VENC) of 150 as well as transecting the main pulmonary artery (VENC 150) and in a plane aligned with flow through the suspected interatrial communication [Figure and ].
The images and velocity maps showed evidence of a completely unroofed coronary sinus. Given the fact that there was a left as well as a right SVC, visibility of its continuation into a dilated coronary sinus would have been expected in the three- or four-chamber long-axis cine images, but this was not the case []. Bidirectional flow through the associated interatrial communication, which was about 15 mm in diameter, was visible in the atrial short-axis cine images and the corresponding velocity acquisition [Figure and ]. The through-plane velocity mapping measured 4.4 l/min in the pulmonary trunk and 6.9 l/min in the ascending aorta, indicative of a net right-to-left shunt with Qp : Qs = 0.6 : 1. The right ventricle was dilated and moderately hypertrophied, with a low ejection fraction of 25%, and there was systolic flattening of the interventricular septum. Biventricular volume measurements showed a larger left ventricular stroke volume than right ventricular stroke volume, which was in accordance with the presence of a right-to-left shunt. The pulmonary arteries were dilated (main PA = 41 mm) and showed limited systolic expansion on cine images. The combined findings were consistent with pulmonary arterial hypertension.


A 13-year-old girl came with complaints of progressively increasing abnormal movements, intellectual decline, and slowing of voluntary movement. She was not able to continue her studies because of her complaints. She was the product of a consanguineous marriage and had been born at full term. There was history of normal development till the age of 8 years. A history of perinatal hypoxic insult or birth trauma was absent. There was no other relevant family history.
Neurological examination revealed a generalized increase in the tone in all four limbs, with dystonic arching of the trunk; mild hyperreflexia, with extensor plantar responses; choreoathetotic movements in the upper extremities; and tremors in the tongue, with speech deterioration. On ocular examination, nystagmus was present on lateral gaze. Ophthalmic evaluation was normal. No evidence of a Kayser-Fleischer ring was seen on slit-lamp examination. Serum electrolytes, iron, copper, and ceruloplasmin levels were within normal limits. Amino acid chromatographic analysis was normal. A few acanthocytes were seen in the blood smear.
MRI examination was performed on a 1.5T scanner (GE Medical Systems, Milwaukee, USA). MRI showed marked hypointensity within both globus pallidi, with a small area of central hyperintensity (eye-of-the-tiger sign) on T2W images [Figure ]. Similar hypointense signals were also seen on the FLAIR images []. The marked hypointensity in the globus pallidi was better appreciated on T2W gradient-recalled-echo (GRE) images due to the susceptibility effect, suggesting iron deposition []. Similar T2-hypointense lesions were seen in the substantia nigra []. Mild thinning of the corpus callosum was noted in the region of the body. Based on the clinical assessment and the typical MRI findings, we arrived at the diagnosis of PKAN.
To evaluate the neuroaxonal degeneration quantitatively, proton MRS was performed using multivoxel chemical-shift imaging with spin-echo, point resolved spectroscopy (PRESS). The imaging parameters were as follows: TR - 4000 ms; TE - 86.6 and 35 ms; slab thickness - 10 mm; voxel size - 20 × 20 × 20; number of acquisitions - 2, and scan time - 6.0 min. Automated software was used for the calibration of the spectrum. Spectra were taken with the ROI (region of interest) circle placed in the right globus pallidus [], which showed the following values; N-acetylaspartate(NAA)/creatine(Cr): 1.14 and myoinositol(mI)/Cr: 0.73. The study revealed a decreased NAA peak [] and a reduced NAA/Cr ratio [], suggesting neuroaxonal loss. An increased myoinositol peak (thin white arrow) and mI/Cr ratio were seen on MRS done at a TE of 35 ms, suggestive of glial proliferation [].


A 29-year-old Asian man presented to our hospital with upper and lower extremity weakness, significant weight gain (20 kg over 18 months), dyspnea, insomnia, early-morning awakening, psychiatric symptoms (illusions, impaired concentration and memory, inappropriate laughter and crying attacks), and erectile dysfunction. Physical examination revealed the typical clinical features of Cushing's syndrome: hypotension, moon face, buffalo hump, multiple purple striae on the flanks, proximal myopathy and oral candidiasis. He was admitted to our hospital with an initial diagnosis of hypercortisolism. Biochemical test results confirmed the diagnosis and revealed that he had elevated serum cortisol (8 a.m.) and ACTH levels on multiple samplings. Dexamethasone suppression test results were positive on two consecutive samplings. His urine cortisol level was elevated, but his vanillylmandelic acid and metanephrine levels were normal. Other laboratory tests were noncontributory to the diagnosis.
An MRI scan of his brain found no pituitary defects, but a spiral abdominal CT scan revealed bilateral adrenal hyperplasia. The clinical and imaging findings raised suspicion of an ACTH-producing tumor. A bronchoscopy and alveolar lavage was performed to investigate the patient's lungs, but no bronchial lesion was found.
Technetium-99m-labeled octreotide acetate scintigraphy was performed in the whole body planar (Figures and ) and single photon emission CT mode (Figure ), 3 hours after the injection of 555MBq (15mCi) technetium-99m-labeled octreotide acetate. The scan demonstrated a focal uptake in the lower lobe of the patient's right lung, just above his diaphragm, which was highly suggestive of an ACTH-producing bronchial tumor.
Corresponding transverse images from a chest CT scan showed a well-defined mass about 22 mm in diameter in the lower lobe of the patient's right lung (Figure ).
A transthoracic biopsy was performed and histopathological evaluation established the diagnosis of a carcinoid tumor of the ectopic ACTH-producing type. After removal of the mass, the patient's condition improved significantly. His clinical symptoms diminished and the results of biochemical tests returned to normal ranges.


A 5-year-old girl presented with complains of abdominal swelling and low grade fever since few days. No relevant past history of bowel or urinary complains was present. There was also no significant family history of cancer. Physical examination revealed distended abdomen with a palpable lump in the right hypochondrium. Her temperature was mildly raised, blood pressure was 110/72 mm Hg and rest of the vitals were unremarkable.
Ultrasonography of abdomen revealed a large heterogenous mass of size approximately 11×10.6×9.26 cm in the right suprarenal region, with calcifications, large necrotic and hemorrhagic areas within and indistinct fat planes from the superior pole of right kidney. Left kidney was not seen in left renal fossa or anywhere else in abdomen, suggesting possible congenital absence. No focal lesion was observed in liver.
Contrast enhanced CT scan of abdomen confirmed the above findings [Figures and ]. In addition, the lesion was abutting right lobe of the liver causing mass effect and displacing the right branch of portal vein and right hepatic vein [Figures and ]. Inferiorly, the supero-medial pole of the right kidney had indistinct fat plane with mass lesion. However the claw sign and organ embedded sign were negative, suggestive of extra renal origin []. Medially, the lesion was extending in the midline abutting the caudate lobe, causing compression and displacement of inferior vena cava []. It was abutting the renal vessels without any evidence of invasion/thrombosis. Multiple, moderately enhancing round to oval shaped, randomly distributed lesions were observed, involving bilateral lung parenchyma [], suggestive of bilateral pulmonary metastatic deposits. There was no evidence of bone marrow/bone metastasis.
Ultrasound guided biopsy revealed ACC. The tru-cut biopsy cores showed areas with patternless sheets of cell interrupted by a fine sinusoidal pattern and broad trabeculae. Wide expanses of necrosis were seen. [] The individual cells had predominantly eosinophilic cytoplasm. Less than 25% cells were clear cells. Significant nuclear atypia and hyperchromatism were noted. Infrequent but definitive atypical mitotic figures were seen. [] Six out of 9 Weiss criteria were fulfilled. The histologic score as per Van Slooten et al. was 21.
Routine laboratory investigations were unremarkable. Although there were no clinically evident signs of virilization or Cushing’s syndrome, plasma cortisol urinary levels of 17-ketosteroids and 17-hydroxycoticosteroid were measured and found to be within normal range.
The patient was grouped as stage IV (according to TNM classification) and was planned for palliative chemotherapy. As it was an advanced stage disease with metastasis and considering the remote chances of complete cure and the costly chemotherapy drugs which were not affordable by the parents, they decided to start ayurvedic treatment. The patient survived for 2 months after diagnosis.


A 17-year-old male high school hand-ball player noticed an approximately 1-cm mass in the first web space of his dominant right hand 1 month before presentation at our hospital. He sought medical attention because the mass became rapidly and painfully enlarged, doubling during the 4-week interval from first observation. He belonged to a high school hand-ball club and played every day. He had not suffered any infection or major trauma, and there was no relevant family history. On physical examination at our hospital, a painful mass measuring 2 cm in diameter was observed in the first web space. The mass was mobile, elastic-hard, and tender to palpation with a smooth surface and clear margin. No local heat and redness on the mass were present. Neurovascular examinations including Tinel's sign were normal. His hematological profile was unremarkable. Radiographs of the right hand showed an increased soft tissue shadow in the first web space (). On computed tomography, the mass, 20 mm in diameter, showed a lower density than muscle (CT value was 30 to 50 H.U.), with a comparatively clear margin (). There was no invasion or calcification. Magnetic resonance imaging (MRI) confirmed a smooth surface mass measuring 20 × 20 × 16 mm in the first web space of the patient's right hand. The mass had low intensity on T1-weighted images () and was heterogeneously hyperintense on T2-weighted images (). After intravenous injection of the gadolinium—diethylenetriamine penta-acetic acid—the mass was enhanced heterogeneously on T1-weighted images (). Thallium-201 scintigraphy showed high uptake at the early phase and no wash-out at the delayed phase ().
Eleven days after the first visit, the mass was even more enlarged measuring 25 mm in diameter. We performed an excision biopsy (). A well circumscribed white homogeneous mass was present subcutaneously and adhered to the interosseous muscle fascia. Intraoperative examination by frozen section showed a low-grade spindle-cell sarcoma. Histological examination by permanent section showed vascular hyperplasia and infiltration of inflammatory cells (). The spindle cells formed S- or C-shaped fascicles in the highly cellular area (). In the poor cellular area, plump and spindle cells were present between the hyalinization of fibrous stroma (). There was little variation in the size and shape of the nuclei. Mitotic activity ranged from two to three mitotic figures per ten high-power fields, but atypical mitoses could hardly be detected (). Immunohistochemical studies showed alpha-SMA and HHF-35, but no desmin; CA5.2, S-100, CD34, and c-kit were positive. MIB-1 labeling index measured 5%–10%. P53-positive cells were about 50%. From these findings, the final diagnosis of nodular fasciitis was made. The patient was able to restart hand-ball 2 months after the operation. There has been no local recurrence at the recent follow-up 2 years after operation.


A 90-year-old male with a history of diabetes mellitus, hypertension, IHD, and right-sided hemiplegia presented to a urologist with recurrent episodes of retention of urine due to prostatic hyperplasia. Considering his medical co-morbidity, a prostatic stent (Urolume) was placed instead of surgery. Approximately 9 months later, he presented to us with severe dysuria, hematuria and poor stream, and urinary tract infection since the last 3 months.
On admission, his physical examination was normal except for right hemiparesis. The prostate had a benign feel on rectal examination. Urinalysis showed pyuria and microscopic hematuria. A urine culture was positive for E. coli. An ultrasonography revealed normal kidneys with approximately 75 gms of prostate. An echogenecity of the Urolume stent was visible in the bladder. Hence, removal of stent was planned.
A urethrocystoscopy showed a normal urethra with big median and lateral lobes of prostate. The Urolume stent had migrated into the bladder lying in a pool of pus flakes. We tried to remove it using alligator forceps but the stent could not be withdrawn into the sheath of cystoscope. Hence, the stent was cut into small pieces using a Holmium: YAG laser using 550 micron fiber with a power setting of 1.2 J and 20 HZ. The stent fragments were removed by withdrawing them into the sheath using grasping forceps. Subsequently, the enlarged prostate was enucleated by Holmium laser. The post-operative period was uneventful and the patient could pass urine satisfactorily after removal of the catheter on the second day after the operation.


A 6-month-old male infant presented to our hospital with cardiac murmur and progressive cyanosis of one day. He was born at term after a normal pregnancy and without prenatal complications. His family history was unremarkable and specifically negative for cardiopulmonary disorders. He had dusky color, but was relatively well and thriving. His mother related a history of orthodeoxia with an increase in cyanosis when her infant is embraced and decrease in cyanosis when lying on the back.
Cardiovascular examination revealed bounding peripheral pulses in both upper and lower limbs, normal first and second heart sounds and a continuous machinery murmur, which were heard at the left upper sternal border space, suspicious of patent ductus arteriosus (PDA). Chest examination revealed normal vesicular breathing, equal on both sides of the chest. The examination of abdomen, central nervous system, skin and mucosa did not reveal any abnormality.
Investigations showed arterial blood gases (room air) to have pH of 7.23, PCO
2
 37mmHg, HCO
3
 17mmol/L, PO
2
 34 mm Hg and O
2
 saturation 61%. Chest X-ray showed normal cardiac size and clear fields in both lungs. Electrocardiogram showed normal sinus rhythm, normal QRS axis, and no right ventricular hypertrophy. Other laboratory findings showed hemoglobin levels of 11.8 g/dl with normal methemoglobin of 1.2%.
Echocardiography confirmed the PDA in our patient, but was not a rationale for his diagnosed cyanosis. Our patient underwent video assisted thoracoscopic surgery (VATS) for closure of PDA. To our expectation, as the PDA closure did not resolve the progressive cyanosis and early clubbing, contrast echocardiography study was performed, suggesting the presence of PAVM with significant right to left shunt, as evidenced by rapid filling of the left atrium with dissolved bubbles. Cardiac catheterization performed which revealing the pulmonary arteriovenous malformation in postero-basal segment of lower zone of the left lung (
), with normal pulmonary artery pressure of 15/5mmHg (mean 10). Oxygen saturation of the aorta was 64%, which made him a candidate for surgical versus catheter-based intervention. Percutaneous transcatheter embolization of PAVM with spring coils was performed in this patient with significant improvement in systemic oxygenation and excellent result in 5 years follow up.


A 10-year-old girl was referred to the department of pediatric cardiology with severe cyanosis. There was a history of bluish discoloration of fingers and lips for the past 5 years. There was no history of breathlessness. On examination, the patient was thin built; peripheral pulses were normal, blood pressure 95/72 mmHg, with normal S1 and S2. There was central cyanosis in lips and nails and grade 2 clubbing was present. There was no evidence of mucocutanious telangiectasis. Examination of cardiopulmonary and nervous system was essentially normal. Hemoglobin was 16.8g/dl with normal methemoglobin of 2%. Chest X-ray and pulmonary function tests (PFTs) were reported to be normal by pulmonologists. Arterial blood gas analysis at room air showed pH 7.20, PO
2
 58.8 mmHg, PCO
2
 40.3 mmHg and oxygen saturation of 88%.
Contrast echocardiographic study was performed, but did not reveal any significant anomaly. Cardiac catheterization was performed and injection of contrast media in the main pulmonary artery showed immediate opacification of the left atrium and small haziness in the left lung. Selective injection of contrast media in left pulmonary artery demonstrated the PAVM in superior lingula of the left lung (
). Successful transcatheter embolization was attempted in the patient and resulted in resolution of cyanosis during 6 moths follow up.


A 39-year-old, previously healthy woman presented to the emergency unit with a 3-day history of myalgia, chills, high fever and a sore throat as well as vomitus, diarrhea and headaches. She was febrile, tachypnoeic with a respiratory rate of 40 per minute and showed an oxygen saturation of 92% despite receiving 10 litres of oxygen via face mask. She was tachycardic and hypotensive, indicating a septic shock. A patchy exanthema was visible on her trunk, and rales were noted upon bilateral auscultation. Her laboratory findings revealed a leucopenia of 2.9 ×10-9/l, a CRP of 507 mg/l and a procalcitonin of 139 ng/ml (Table ). A chest x-ray showed diffuse infiltrates bilaterally.
Blood cultures and expectorate were taken, and antibiotic therapy was initiated with intravenous ceftriaxone 2 g/day. Due to further respiratory deterioration, the patient was intubated. Aggressive volume replacement and high dose vasopressors were needed to stabilize the hypotensive shock. Haemofiltration was started for acute renal failure. After a diagnostic bronchoscopy with bronchoalveolar lavage on the day of admission, the antibiotic therapy was empirically changed to piperacillin/tazobactam 4.5 g 3 times daily and clarythromycin 500 mg twice daily. Oseltamivir 75 mg twice daily was added for 5 days, as the history was suspicious for influenza disease with bacterial superinfection. Due to progressive, refractory hypercapnia and respiratory acidosis, an interventional lung assist device (iLA membrane ventilator, Nova Lung®, Talheim, Germany) was installed the same day.3
Group A streptococci grew in four (of four drawn) blood cultures as well as in the bronchoalveolar lavage fluid. Taking the clinical picture into account, a streptococcal toxic shock syndrome was diagnosed, and the antibiotic therapy was changed to high-dose penicillin (5 Mio U four times a day). Clindamycin 900 mg three times daily was added to prevent toxin production for 3 days. A 5 day immunoglobulin therapy (Redimmune® 0.4 g/kg/d) was started. The bronchoalveolar lavage was repeated 5 days after admission to look for viral pathogens and confirmed the presence of Influenza B by real-time polymerase chain reaction (RT-PCR4) with 40,565 copies/ml (Table ).
Over the following days, a nosocomial, ventilator-associated pneumonia was diagnosed and the antibiotic therapy was empirically changed to imipenem 500 mg four times a day. Methicillin susceptible Staphylococcus aureus was detected by culture of respiratory samples. Following clinical improvement, the lung assist device was removed after 11 days and the antibiotic therapy was stopped after 14 days. A third episode of respiratory deterioration occurred 2 weeks after admission. No pathogen could be isolated from bronchoalveolar lavage. The recurrence of severe respiratory acidosis due to refractory hypercarbia required another lung assist device, and a dilatational tracheotomy was performed.
After 24 days of intensive care, the patient's condition had finally improved enough for the lung assist device to be removed; the patient was weaned successfully and decanulated on day 35. The patient was transferred to a rehabilitation facility with mild critical illness polyneuropathy. She showed no neurological deficits otherwise.


A previously healthy 27-year-old woman presented with complaints of an influenza-like infection with sore throat, fever and dry coughs since three days. The night before admission her sore throat became worse, fever climbed to 39°C and she complained of headaches. On admission, she was clinically diagnosed as having influenza, received intravenous fluids, and her discharge was planned thereafter. Within 8 hours post admission, her general condition deteriorated rapidly, showing progressive hypotension and respiratory failure. She was alert but in a bad general condition, fulfilling all criteria of a septic shock. Her laboratory findings on admission showed a leukocyte count of 12 ×10-9/l, a CRP of 103 mg/l and a procalcitonin level of 34 ng/ml (Table ). A chest x-ray showed infiltrations in the right lung consistent with pneumonia. After blood cultures were drawn, an antibiotic therapy with amoxicillin/clavulanic acid 2.2 g three times daily and clarithromycin 500 mg twice daily was initiated and crystalloids and high dose catecholamines had to be applied. Due to respiratory failure, the patient was intubated. On the next morning, a marked pleural effusion with 1000 ml of exudative fluid was drained. Despite all these measures, her respiratory situation became worse with refractory hypoxaemia and increasing ventilation pressures, compelling us to install an interventional lung assist device. After this, her situation stabilized markedly. To this point, no other organ failures had occurred.
Streptococcus pyogenes grew in all four blood cultures drawn at admission and from the bronchoalveolar lavage. RT-PCR of the bronchoalveolar lavage for Influenza B was positive with 3,568 copies/ml and suggested a primary influenza infection with bacterial super infection (Table ). Antibiotic treatment was changed to high-dose penicillin (5 Mio U four times a day) and oseltamivir 75 mg twice daily was added (on day 5 after admission) for 5 days. On day seven, ventilator associated pneumonia was suspected and bronchoalveolar lavage repeated. No causative agent was identified, and another Influenza RT-PCR was negative for Influenza B.
Fifteen days after admission, a sudden respiratory deterioration occurred with pulmonary hemorrhage, compounding mechanical ventilation markedly. A bronchoscopy showed crustal lesions in the trachea but no active bleeding. On suspicion of pneumothoraces, thoracic drainages were inserted bilaterally. A malfunction of the interventional lung assist device made a change of the device necessary. The invasive procedure was poorly tolerated, and temporary mechanical resuscitation had to be performed. Thereafter, the patient remained dependant on high dose catecholamines and showed persistent signs of cerebral hypoxaemia. The patient died 18 days after admission. Autopsy was declined by the family for religious reasons.


A 61-year-old previously healthy woman was admitted to hospital after returning from a one week of holiday in Morocco. She complained of headaches, a sore throat, no appetite and general weakness since 9 days, and mentioned having diarrhoea since 6 days. She was started on amoxicillin 750 mg three times daily by a family doctor without effect. The day she presented to our emergency unit she was febrile with 38°C and tachypnoeic. Auscultation detected rales bilaterally and bilateral infiltrates were found on a chest x-ray. Her laboratory findings showed a leukocyte count of 14 ×10-9/l and a CRP of 195 mg/l initially (Table ). An empiric antibiotic therapy with ceftriaxone 2 g/day and clarythromycin 500 mg twice daily was initiated, and the septic patient was transferred to the intensive care unit. The pneumococcal antigen was positive in the urine sampled on admission. Blood cultures remained negative, which may be explained by the antibiotic pretreatment. A direct influenza antigen test was negative initially.
Due to respiratory failure, the patient was intubated on day 4 despite non-invasive ventilation. A spontaneous right-sided pneumothorax was treated with a thoracic drainage. Over the next few hours, her respiratory situation deteriorated further showing a progressive hypoxaemia and a reduced lung compliance requiring an interventional lung assist device. With the suspicion of penicillin-resistant pneumococci in this patient returning from Morocco, the antibiotic therapy was changed to vancomycin 1 g twice daily. A second urine test for pneumococcal antigen was again positive. No other bacteria was detected in a bronchial alveolar lavage five days after admission, but RT-PCR from a bronchial alveolar lavage on day 5 revealed Influenza B with 12,231 copies/ml (Table ). Oseltamivir 75 mg twice daily was added (on day 9 after admission) for 5 days, and the RT-PCR repeated thereafter remained positive with 13,363 copies/ml for Influenza B. Seven days after admission, the pulmonary situation had stabilized enough that the interventional lung assist device could be safely removed. One day later she was extubated. The patient was transferred to a rehabilitation facility 26 days after admission.
This is the first report of Influenza B infections accompanied by severe bacterial superinfection in three healthy women.
Fatal co-infections of influenza virus with Streptococcus pneumoniae have been described in mice since the early eighties [], and have been reported following in clinical outbreaks []. In addition to S. pneumoniae, the most common co-infecting bacteria are Staphylococcus aureus, Haemophilus influenzae, Streptococcus pyogenes and Mycoplasma pneumoniae. Pathophysiologically various data from mice models suggests that influenza virus infection damages the epithelium of the bronchi and lungs, allowing microaspirated bacterial pathogens to establish infection []. On the other hand, influenza alters the local immunity[] and the inflammatory response [-] facilitating the outgrowth of bacteria. Underlying immunosuppression might lead to more severe and prolonged disease []. Our patients presented with no evidence of underlying disease or other known risk factors for dual infection such as age (< one year, > 65 years), pregnancy or comorbidity [].
From an epidemiological viewpoint, the circulating strain is known to contribute to mortality with H3N2 subtype causing more influenza-associated deaths than H1N1 or influenza B viruses []. Influenza B leads to fewer co-infections [] and milder disease compared to Influenza A [,]. In Switzerland, the seasonal influenza epidemics of 2007/2008 were characterized by an initial increase in Influenza A H3N2 cases, whereas in the later months, when our patients presented, infections with Influenza B prevailed [] (Figure ). The predominant circulating B strain in Switzerland was B/Jiangsu/10/03. This strain was not matched by a corresponding strain in the B component of the vaccine and was not present in the previous year (Figure ), therefore, it is possible that there is no immunologic memory for this infection, perhaps contributing to the severity of infection []. The first and second patient had young children suffering from influenza-like symptoms and most probably transmitted the virus to their mother. In general, we do not think that there is an association between sex and disease severity. While pregnancy was associated with poor outcome in the H1N1 pandemics, none of our patients was pregnant.
Diagnosis in our cases was based on real-time quantitative PCR, according to a previously published protocol detecting Influenza A and B in separate reactions with a limit of detection of 2.5 log10 copies/ml []. This method is known to be very sensitive and specific compared to antigen testing, which is a more rapid but less sensitive test, and was negative in one of our three patients. Severe cases of Influenza B have probably always occurred. But as most routine laboratories did no specific testing for Influenza B, this could not be documented until recently. Interestingly, a recent study on the effect of oseltamivir in patients hospitalized with Influenza infection included 215 patients with influenza B, of which 13 died, confirming our data on serious courses of Influenza B infections [].
All three patients received oseltamivir, a highly selective neuraminidase-inhibitor. This agent is effective against Influenza A and B. Although clinical data are limited in Influenza B, some evidence exists that oseltamivir might have less effect on Influenza B, therefore, higher dosing may be necessary []. Patient 1 received an early empiric treatment with oseltamivir, as her history was indicative for primary influenza infection and bacterial superinfection. In the other two patients, antiviral treatment was started only later, on day 5 in the second and at day 9 in the third patient. Given the fact that antiviral treatment is efficacious especially in the early course, empirical use of oseltamivir in severe pneumonia during an influenza season might be advisable, even though PCR-testing is a highly sensitive and reasonably rapid. With regard to oseltamivir's properties and the fact that septic patients are immunocompromised per se, the early use of neuraminidase-inhibitors seems warranted. In fact, our case series favours the approach of empirical combined antiviral-antibiotic treatment, a discussion that evolved during the H1N1 pandemics. However, there is no evidence that the use of oseltamivir yields any benefit after 48 hours of infection.
Supportive care measures for septic shock and respiratory failure were taken immediately. Minimal inhibitory concentrations for penicillin were low in the two cases with S. pyogenes indicating full susceptibility towards the administered empirical betalactam antibiotic. To prevent toxin production, clindamycin was added. S. pyogenes is known to cause fulminant toxic shock syndromes, for which both patients qualify, occurring also in young and previously healthy persons []. In the third case, no resistance testing was available due to diagnosis of S. pneumoniae by means of a non-cultural assay only. A penicillin-resistant strain might have been possible in the patient returning from Morocco, where the local epidemiology shows up to 40% penicillin-resistant pneumococci [] and, therefore, may have contributed to delay in a correct antibiotic treatment.
The application of an interventional lung assist enables a safe application of lung protective ventilation [] together with a therapy of respiratory acidosis in patients where all other intensive care measures have failed to provide adequate ventilation and decarboxylation. Although uncommonly used on our ward, the lung assist device allowed us to combine very low tidal volumes (3-6 ml/kg) and high PEEP levels with lower plateau pressures and, thus, the avoided barotraumas reported in these patients. Especially during acute lung injury due to pneumonia, the application of low tidal ventilation may prevent secondary injury from mechanical ventilation to the uninfected parts of the lung.


A 55-year-old lady underwent an elective laparoscopic Nissen fundoplication. On the second postoperative day she deteriorated with persistent vomiting, pyrexia, and low oxygen saturations and was promptly admitted to the intensive care unit (ICU). Oesophago-gastro-duodenoscopy (OGD) was performed with dilatation of a tight gastro-oesophageal junction (GOJ).
She then continued to be hypoxic and hypotensive and a chest X-ray revealed a right sided pneumothorax which was managed with a right chest drain. Serial arterial blood gas samples did not reveal any acid-base disturbances at this stage. A gastrografin swallow ruled out any perforation in the oesophagus and was otherwise nonspecific. After a short period of noninvasive ventilation, she remained hypoxic and was consequently intubated. A second chest drain was inserted on the left side due to worsening bilateral pleural effusions on a subsequent chest X-ray.
A repeat OGD revealed a normal GOJ with a rotated and distended stomach but there was no evidence of gastric outlet obstruction. As the patient continued to deteriorate, a computerised tomography (CT) scan was done () which confirmed herniation of the stomach into a congenital postdiaphragmatic space. At exploratory laparotomy, the anterior wall of the stomach was noted to be herniating into a congenital space behind the diaphragm pushing the liver and the inferior vena cava anteriorly. There was no evidence of a gastric volvulus. Following the retrieval of the herniating segment, the wrap was noted to be congested with an area of ischaemia which recovered promptly following reduction and no resection was necessary. The wrap was undone revealing a defect in the anterior wall of the stomach. The margins of the defect, which were of doubtful viability, were trimmed and the defect was closed with nonabsorbable sutures and covered with omentum. The stomach was then fixed to the anterior abdominal wall with nonabsorbable sutures. No defect was noted in the diaphragm where three hiatal interrupted nonabsorbable sutures, which were placed during the previous fundoplication and were noted with a normal length of abdominal oesophagus.
The patient made a swift recovery and was discharged from the ICU. Both drains were eventually removed prior to her discharge from the hospital on the seventh postoperative day. The patient remained asymptomatic with no reflux symptoms after a period of 2 years of followup leading to her discharge from our care.


A 48-year-old male admitted to our hospital suffering from a bulky mass on his left buttock and a foul odor. The mass on his left buttock was 20 × 10 × 4 cm in diameter, and its surface was cauliflower-shaped with profuse exudates and ulceration (). The skin around the cauliflower-shaped mass colored brownish to purplish and partly had ulcers likely penetrating to the mass. The patient noticed a part of an elongated oval nodule in childhood and its rapid growth in his late thirties. He stated that the nodule had grown to huge.
Computed tomography (CT) and magnetic resonance imaging (MRI) () showed that the mass invaded into coccygeal bone and the posterior wall of the rectum. While bilateral inguinal lymph nodes were slightly swollen, no apparent visceral metastasis was detected.
Histologically, under low magnification, the tumor cells grew upward with keratinization and downward intermingling hypertrophic scar. Under high magnification, the tumor included papilliform-acanthosis, a number of cancer pearls and individual cell keratinization (). Atypical epidermis contained numerous mitoses and moderate to severe cell atypia. Two of bilateral inguinal lymph nodes were also biopsied as sentinel lymph nodes, and no histopathological metastasis was detected. These findings led the diagnosis of well-differentiated squamous cell carcinoma (SCC) T4N0M0 according to AJCC staging.
We selected initial treatments with combination of cisplatin, fluorouracil (5-FU), and pepleomycin and concurrent 50 gray of irradiation because the tumor margin was not as demarcated as an ensured excision. As a consequence of the treatments, the tumor became necrotic and crumbled, and finally, it markedly shrank to a 7 × 3.5 cm ulcer. After chemoradiation therapy, a washy red smooth nodule was noticed from the beneath of tumor. It was adjneent to the ulceration (). The ulcer was considered to be located at the site where the SCC originated from. The patient stated that it was the nodule that he noticed in childhood and a part of which became huge.
Since there is no evidence of metastasis during the above treatments, we decided to perform a radical operative procedure to achieve complete control of the tumor. According to the first extent of tumor, it was excised together with sacrococcyx and rectum. The perineum was reconstructed with a pedicled musclocutaneous flap using the right abdominal rectus muscle, and sigmoid colostoma was created (Figures and ).
Macroscopically, the division surface of tumor showed a white fibrous tissue. Surprisingly, histopathological findings showed no remaining tissue of SCC but showed only fibrous. The fibrosis reached to rectum, and tissues was also seen around the trabecula of the sacrococcyx, suggesting previous invasion of the tumor. The nodule located behind the huge SCC showed papilliform structure which invaginated to the dermis and the wall was consisted with two layers of epithelial cells. The outer layer of the epithelium was composed of cuboidal cells and the inner layer of columnar cells. Many plasma cells infiltrated to the interstitial area (Figures and ). According to these findings the nodule was diagnosed as SP.
Three courses of chemotherapy with the same regimen as a neoadjuvant one were performed after surgery. Fifteen months after the operation, the patient is well and working without any local recurrence and metastasis.


A 73 year old lady attended ophthalmic outpatients in 1993 describing photopsia in her left pseudophakic eye. Snellen visual acuity was right eye 6/9, left eye 6/18. Dilated fundoscopy demonstrated an elevated, pigmented mass in the superotemporal quadrant of her left eye approximately 3 disc diameters from the optic nerve head. The clinical appearance was consistent with malignant melanoma of the choroid and ultrasound (U.S.) B scan demonstrated a lesion of 14 mm width and 7 mm height. Haematological markers, chest radiography and liver ultrasound were all normal. Following referral to a national centre for ocular oncology, a course of proton beam radiotherapy was instituted and the patient attended for regular follow up. Tumour dimensions were repeatedly assessed and found to be stable and regular clinical review until June 2005 demonstrated no change in the fundal mass, or anterior extension in relation to scleral markers inserted at the time of proton beam radiotherapy. No scleral extension was evidenced on thorough orbital ultrasound, nor was there any evidence of systemic involvement.
In July 2005, the patient now aged 85, presented with sudden, non-axial, painless proptosis of the right globe associated with upper lid ptosis. There was 2 mm proptosis and medial and inferior displacement of 2 mm and 8 mm respectively (). There was associated oedema and erythema of the soft tissue but no significant pain or heat on palpation. Orbital CT displayed a well-defined, non-enhancing, rounded mass lesion in the superolateral right orbit extending medially and inferiorly into the intraconal space displacing the superior rectus and optic nerve (). Histology of the biopsied lesion showed a necrotic malignant melanoma composed of epithelioid cells containing large nuclei with prominent nucleoli and a moderate amount of pink cytoplasm. Focally the cells contained melanin pigment. These expressed the melanocytic markers melan A and HMB 45 on immunohistochemistry but were non-reactive to S100 and CAM 5.2. ().
Systemic evaluation with CT scan of chest, abdomen and pelvis showed multiple metastatic deposits in the liver and at least one in either lung base. Referral to the regional oncology unit ensued and palliative chemotherapy of oral Temozolomide was commenced. Additional treatment with oral dexamethasone 4 mg daily greatly improved the right orbital tissue swelling and globe proptosis and the patient remained comfortable for 6 months. She died one year following this presentation.


A 3-day-old female child, (full-term, normal delivery) weighing 2.75 kg at birth with stable vitals, moderate abdominal distension and history of not being able to pass meconium was referred to us. Examination showed normal anal opening but red rubber catheter could not be passed beyond 3 cm of anal verge. Spine and external genitalia were normal. X-ray (invertogram with red rubber catheter passed through anal opening) showed catheter abutting the air column in the bowel) [].
With anal retractors anal canal was opened but upper-end of membrane could not be seen.
An endoscopy was done with a 7.5F cystoscope. About 3 cm from the anal verge, the anal canal ended blindly and a membrane was visualized at the blind end. We incised the outer mucosal membrane, i.e. of the distal pouch with a bugbee, but the vision was lost due to technical reasons. Then anal retractors were used, and after the incision on the mucosa of the distal pouch, the inner mucosal membrane was seen bulging significantly due to meconium within it [].
Transanal membranotomy was done. Postoperatively, on day one, dye study was done and feeds were started. Patient was discharged on day 4.


A 4-year-old girl was referred to the ophthalmology department of Jimma University Specialized Hospital, Ethiopia for the evaluation of a 'small left eye' since birth.
The perinatal history of the child was uneventful. Family history was negative for similar problems. History of miscarriages was also denied. Her growth and developmental achievements were satisfactory.
On examination, both eyes had simple infectious conjunctivitis. The right eye was within the normal limits apart from the conjunctival inflammation. The left eye was microophthalmic with typical inferior iris coloboma and cataractous lens (Figure ).
Cutaneous examination showed multiple hypopigmented, slightly depressed and ill-defined macules over the left side of face (Figure ). Similar skin lesions with reticular pattern were present on the side of chest, abdomen, buttock, and upper and lower limbs on the left side only (Figure ).
We noted complete syndactyly between the third and fourth fingers of the left hand, with typical lobster claw deformity (Figure ). The left foot showed oligodactyly and syndactyly (Figure ). The nails of the left hand and foot were dysplastic. The right hand and foot were within the normal limits.
There appeared to be facial asymmetry with left hemiatrophy, and low-set protruding left ear and pointed chin. The scalp hair was sparse; and the deciduous teeth were irregularly spaced (not shown in the pictures).
The simple bacterial conjunctivitis was treated with topical chloramphenicol eye drops. We arranged appointment for regular follow up.


A 72-year-old Caucasian woman was admitted to our surgical department having experienced diffuse abdominal pain and discomfort, nausea and vomiting for the previous two days. Her medical history included diabetes mellitus and arterial hypertension, for which she was on medication. She had no relevant family history and did not smoke or drink alcohol.
On physical examination, she showed signs of acute abdomen and a palpable painful mass in her left abdomen was noted. She was tachycardic and laboratory tests showed a white blood cell count of 13,000 cells per cubic millimeter. Her chest and abdominal radiographs did not reveal any abnormalities.
An ultrasonography (US) examination demonstrated a complex cystic mass with internal septa, without increased vascularity. The source organ could not be identified (Figure ).
Computed tomography (CT) examination demonstrated a large 18.7 × 13.2 × 22cm, intra-peritoneal hypodense mass in her upper left abdomen, lying between the great curvature of the stomach, the spleen and the tail of the pancreas, and extending caudally to the upper margin of the pelvis, causing pressure over the adjacent organs. The mass demonstrated no internal septa and no enhancement after the intravenous administration of a contrast medium. No abnormal lymphadenopathy was present (Figure , Figure and Figure ).
She underwent an urgent operation and the multicystic mass was found to occupy her entire left abdomen, adherent to the spleen. A complete resection of the lesion and splenectomy were performed. She had an uneventful post-operative recovery and a post-splenectomy prophylaxis was used.
Gross examination of the specimen showed a large gelatinous cystic mass containing multiple smaller cystic spaces. Her immunohistochemical stains were positive for calretinin and cytokeratins, confirming the mesothelial origin of the mass. The final diagnosis was benign multicystic peritoneal mesothelioma. (Figures and ).
Six months post-operatively, she had experienced no recurrence and was free of symptoms.


A 54-year-old Malay man presented with a week history of unprovoked, recurrent epistaxis. Bleeding was more evident from the right side and resolves after 5-10 min of anterior nares compression. He had not experienced similar incidence, and there were no other pre-existing nasal symptoms, such as nasal congestion, rhinorrhea, anosmia, sneezing, itchiness, or any other aerodigestive symptoms. On further questioning, he is an active smoker but had no history of trauma, bleeding tendencies, and intake of blood-thinning medications or supplements. Upon further assessment, he was hemodynamically stable with no active bleeding. He had no evident nasal deformity, whereas nasoendoscopy showed blood clots along the middle meatus bilaterally with left septal spur, but no bleeding point or lesions within nasal cavity.
Posteriorly, we noted a small single hemorrhagic cystic-like lesion at the anterior surface of the right torus tubarius that was nonpulsatile and no active bleeding, whereas the fossa of Rosen Mueller was clear (). Bilateral ear and neck examinations were unremarkable. Blood parameters had no coagulopathies, and he was discharged with well status. Contrast-enhanced computed tomography (CECT) of the paranasal sinus (PNS) was performed at the outpatient setting, and no sinonasal, nasopharyngeal, or right tubarial lesions were detected (). Upon follow-up, the cystic lesion was persistent, but no active epistaxis was observed. In view of the findings, a biopsy was taken under endoscopic guidance using punch biopsy forceps in the clinic under local anesthesia. Only minimal bleeding occurred after biopsy, which resolved with ice compression and nasal packing using Moffet’s solution. With the limited surgical field and fear of over excision of the normal tubarial tissue which may cause epistaxis or scarring later, only a small yet sufficient tissue was obtained without attempting to manipulate further. Symptoms resolved the following month with no additional ear complaints, and only a small residual lesion was seen on endoscopy (). The final histopathology examination revealed the lesion as an OPC (,). As the lesion was not causing any disturbances, he was not kept for further evaluation or surgical intervention.


The reporting of this study conforms to the CARE guidelines.
A 32-year-old man who had been involved in a traffic accident was admitted to a community hospital immediately after injury. He had severe left knee pain with a visual analog scale score of 8 points. The general practitioner found no obvious abnormalities on the initial X-ray image. Analgesics were administered, but the patient was still unable to walk for the next several days. Therefore, he presented to us 7 days after injury with persistent left knee pain and disability of weight bearing. The patient had no history of drug allergy, other major diseases, or hospitalization. He denied any significant family history of disease. In addition, no history of trauma to his left knee was noted. Physical examination revealed significant swelling, extensive tenderness, and limited range of motion of his left knee. The anterior drawer test and Lachman test were positive during the examination. Anteroposterior and lateral radiographs showed an occult medial Hoffa fracture (). Computed tomography (CT) demonstrated a displaced medial Hoffa fracture of Letenneur type III
 (). Magnetic resonance imaging (MRI) revealed a medial Hoffa fracture associated with an ACL tear (). Therefore, the patient was diagnosed with a medial Hoffa fracture combined with an ipsilateral ACL tear.
The patient was placed in the supine position under spinal anesthesia. Using standard anteromedial and anterolateral portals, a complete diagnostic arthroscopic examination was performed to identify associated intra-articular injuries with the aid of a tourniquet. After removal of the hematoma, a displaced coronal fracture of the medial femoral condyle with a concomitant proximal ACL tear was confirmed. The posterior cruciate ligament and the meniscus were intact.
By flexing the knee to 120°, the fracture was fully reduced with the aid of a curved probe. A percutaneous K-wire was percutaneously inserted in the anterior-to-posterior direction to temporarily stabilize the fracture. Under arthroscopic control, two additional percutaneous guide pins were inserted parallelly into the medial aspect of the suprapatellar bursa, perpendicular to the fracture line (). These pins were carefully placed to avoid penetration into the medial femoral condyle cartilage. The final fracture reduction and position of the guide pins were verified by C-arm imaging (). Following placement of the guide pins, two 4.5-mm cannulated cancellous lag screws (Depuy Synthes, West Chester, PA, USA) were placed to achieve appropriate compression (). During arthroscopy, stable fixation was observed and the length and size of the screws were confirmed to be correct. ACL reconstruction was then performed using a hamstring tendon autograft with preservation of the tibial insertions. An additional anteromedial portal was used for preparation of the femoral tunnel to allow improved access to the anatomical footprint. An Endobutton device (Smith & Nephew, Andover, MA, USA) was used for femoral fixation, and a polyether ether ketone interference screw (BIOSURE SYNC; Smith & Nephew) was used for tibial fixation (Video 1).
The patient underwent X-ray and CT examinations postoperatively (). A long-leg knee brace was used for 3 weeks. Physiotherapy including active and passive knee movement was then performed. Weight-bearing activity was limited for 6 weeks. After successful fracture union was observed radiographically, the patient was allowed to walk without support at 3 months postoperatively with no pain and full range of motion of the knee. At the 1-year follow-up, the patient returned to daily activity and sports with favorable knee function (). He developed no short- or long-term complications and was fairly satisfied with his knee status and clinical outcomes. We did not remove the screws; we planned to remove the screws only if the patient developed hardware-related complications during follow-up, such as persistent pain or infection.
The procedures performed in this study were approved by the ethics committee of the Third Hospital of Wenzhou Medical University in accordance with the Helsinki declaration. Written informed consent was obtained from the patient.


The patient was a 48-year-old man with hypertension that was inconsistently controlled with medication. He visited our clinic because of a growing mass on the volar side of his left wrist that was present for 1 year. He denied a history of trauma or a remarkable family history. According to the patient, the mass had increased in size but was not associated with pain or numbness. There was no skin discolouration, and the neurovascular examination was normal. Full range of motion of the left wrist (flexion, extension, supination and pronation) and fingers (flexion and extension) was intact. Radiographs of the left wrist showed a calcified, ill-defined tumour on the volar side of the distal ulna ( and ), and magnetic resonance imaging (MRI) revealed an ossified mass (3.0 × 1.8 × 1.3 cm) on the volar ulnar side, with multiple calcified areas. However, the ulnar artery and nerve were not involved with the tumour, and there was no bony connection to the distal ulna ( and ).
Because of cosmetic concerns and flexion limitation associated with the enlarged mass, the patient consented to surgical resection for excisional biopsy. A volar approach was adopted, and a pseudo-capsule surrounding the mass was found (). We excised the mass completely and cautiously, leaving no gross remnant at the lesion site (). We used 95% alcohol to flush the surgical field, and the wound was closed. The postoperative condition was good, and there were no motor or sensory impairments. Pathological reports showed mature osseous tissue mixed with adipose tissue and the presence of a cartilage cap. The chondroid tissue showed mild nuclear atypia, and BPOP (Nora’s lesion) was observed ( and ). Immunohistochemistry for mouse double minute 2 (MDM2) and cyclin-dependent kinase (CDK)4 proteins was not performed because parosteal osteosarcoma was included in the differential diagnosis, and this tumour is usually negative for these markers. Radiography and MRI 1 year after surgery revealed no recurrent tumour or calcification in the left wrist ( and ). The range of motion was complete, and the patient was satisfied with the results of the operation. The reporting of this study conforms to the CARE guidelines.


A 56-year-old male presented with an acute-onset headache. The Hunt-Hess grade was 3. Computed tomography (CT) revealed subarachnoid hemorrhage (modified CT Fisher grade 3) with blood predominantly in the left cerebellopontine and cerebellomedullary cisterns. Digital subtraction angiography, including 3D rotational angiography, revealed the presence of a wide-necked proximal posterior inferior cerebellar artery (PICA) aneurysm on the left side (). The V4 segment of the left vertebral artery (VA) at the level of PICA origin revealed a fenestration ().
The patient was prepared for endovascular coiling. A 6F long sheath and a DAC 057 catheter (Stryker Neurovascular, Fremont, CA, USA) were placed in the left VA through right femoral artery access. The balloon-remodeling technique was chosen after initial attempts at simple coiling resulted in repeated prolapse of the coils into the PICA origin. Various balloon configurations in relation to the V4 segment of vertebral artery fenestration were considered (–). After attempts to navigate the balloon into PICA from the ipsilateral VA failed, as illustrated in , the plan was revised to place the balloon horizontally across the neck of the aneurysm (as in ). The right brachial artery was accessed, and a 5F Envoy catheter (Cordis Neurovascular, Miami Lakes, FL, USA) was placed in the right VA. A Transform 4×10 compliant balloon (Stryker Neurovascular) with Synchro 2 soft wire (Stryker Neurovascular) was advanced into the right VA and retrogradely into the left VA. After multiple failed attempts to navigate the balloon into the left PICA, a 4×10 Apollo balloon dilatation catheter (Brosmed Medical, Dongguan, China) was advanced antegradely in the left VA just below the level of PICA origin. The inflated Apollo balloon helped to deflect the Transform balloon into the PICA (, ). The left VA Apollo balloon was then removed, and a Headway 17 microcatheter (Microvention Terumo, Aliso Viejo, CA, USA) was advanced into the aneurysm. Coiling of the aneurysm was done using the BRT to achieve Grade 1 occlusion () of the aneurysm (Consensus Grading scale, Meyers et al. []). The patient was discharged 2 weeks later with no residual neurological deficits. A follow-up MR angiogram at 6 months revealed no aneurysm residue.


A 60-year-old female presented with an acute-onset headache and an episode of loss of consciousness. Her HuntHess grade was 2. CT revealed subarachnoid hemorrhage (modified CT Fisher grade 2). Cerebral angiography revealed a wide-necked anterior communicating artery (ACoA) aneurysm (, ). The A1 segment of the right anterior cerebral artery (ACA) was relatively hypoplastic and revealed a fenestration. Both limbs of the fenestration appeared to be of equal diameter (). The diameters of the fenestration limbs were measuring 0.92 to 0.95 mm each. Angiography also revealed 2 unruptured middle cerebral artery (MCA) bifurcation aneurysms (). The patient was on long-term steroids for nephrotic syndrome.
The patient was prepared for coiling. As the wide-necked aneurysm morphology made it unsuitable for simple coiling we planned a BRT. The following strategies were considered: 1) navigating a balloon via the ipsilateral (left) internal carotid artery (ICA) into either the ipsilateral or contralateral A2 segment, 2) parallel balloon placement using a transcirculation approach with a balloon through the right A1 segment and coiling microcatheter through left A1 segment, and 3) using a double-balloon strategy with balloons navigated through ipsilateral ICA. We felt that the first strategy would not provide complete neck protection as the aneurysm neck incorporated both the ACoA and the proximal left A2 segment. The second strategy of parallel balloon placement, which involved placement of a balloon from the right A1 segment into the left A2 segment across the ACoA aneurysm neck, seemed to provide more complete neck coverage. Although the right A1 fenestration raised concerns regarding the ease of navigation of the balloon we decided to proceed with the transcirculation technique. The plan was to switch to the double-balloon strategy if the transcirculation technique failed. A 6F long sheath and DAC 057 were placed in the right ICA and a Transform 4×10 compliant balloon with a Synchro 2 soft wire was advanced into the right ICA. The acute angle of origin of the right A1 segment () made navigation of the balloon into the ACA impossible. The Transform balloon and the DAC 057 were removed. The Transform 4×10 balloon was then advanced through the 6F long sheath and placed in the right proximal M1 segment, and a second Transform 4×10 compliant balloon was navigated into the right ICA. With the M1 segment balloon inflated, the second balloon catheter was navigated into the right A1 segment and further through the ACoA into the left A2 segment (). Spasm in the fenestration limb through which the balloon had passed created hurdles in balloon advancement. The spasm was relieved by infusion of nimodipine in the flush line connected to the long sheath. The balloon was positioned horizontally across the neck of the aneurysm (). Through a 6F Envoy catheter placed in the left ICA, an Echelon-10 microcatheter (Medtronic, Irvine, CA, USA) was advanced into the aneurysm, and coiling of the aneurysm was done using the BRT. Final angiograms revealed Grade 0 occlusion of the aneurysm (Consensus grading scale) (, ).
The patient recovered from the procedure without any complications but eventually succumbed 12 days later to complications of sepsis contributed by her immunocompromised status (nephrotic syndrome and long-term steroid use).


A 28-year-old woman was admitted to our hospital due to unstable gait caused by pain in the right hallux, for which she was unable to stretch for over three months.
The patient had undergone “resection of ameloblastoma at the right mandible, mandibulectomy, and autogenous right fibula grafting” at another hospital on November 20, 2017. The right lower limb of the patient was wrapped with gauze and fixed with a splint for over 20 d. The patient reported within 20 d that the wrapping was too tight, without any evident pain. The patient started walking with the assistance of a single crutch after the splint was removed, and could walk unaided one month after the operation. However, the hallux toe, as well as the second and third toes of the right foot could not be stretched, with pain in all the toes. Therefore, the gait of the patient was unstable, and she was susceptible to fall. Going up and downstairs was very difficult for her.
The patient had no significant medical history, psychiatric history, or history of substance abuse. On admission, the physical examination showed that the patient had clear consciousness, and her mental status was normal.
A healed scar of approximately 15 cm in length was found at the jaw. Another healed scar of approximately 28 cm in length was found at the posterolateral side of the right calf. No evident swelling was found in the right calf and right foot, and no evident pressing pain was found around the right ankle. The motion of the right ankle was restricted, and the parameters were as follows: Dorsal flexion was about 0°; plantar flexion was about 45°, which was generally normal; the right hallux toe was slightly valgus; the second and third toe joints were flexed and could not be stretched when the ankle joint was in neutral position. However, these two toe joints could be stretched during plantar flexion of the ankle. The circumference of the calf was 21.5 cm and 22.5 cm at 10 cm above the right and left ankles, respectively. In addition, the circumference of the calf was 29.5 cm and 31.5 cm at 20 cm above the right and left ankles, respectively. Hypoesthesia of the skin was found at the lateral side of the right calf and right foot-back. Fluctuation of the dorsal artery was normal (Figure ).
X-ray of the right ankle and right knee performed on March 30, 2018 (Figure ) showed discontinuity of the proximal and distal ends of the right fibula, which could be the changes following fibula osteotomy. Electromyography (EMG) performed on April 1, 2018 showed damages of the sural sensory nerves. Color ultrasound examination suggested the possibility of flexor hallucis longus injuries. Magnetic resonance imaging of the right calf on April 12, 2018 (Figure ) showed discontinuity of the bony substance of the right fibula, which was postoperative change; abnormal signal of the right flexor hallucis longus that suggested injuries; and tenosynovitis of the right flexor hallucis longus.


A 48-year-old man was admitted to our hospital with 1-year history of pancreatic and liver tumors. The tumors were accidentally found when the patient went to a local hospital after a sudden incidence of fainting. It is noteworthy that he reported of recurrent incidences of hypoglycemia, however, there was no history of any endocrine disease.
His medical history showed that he had been treated eight times for metastatic liver tumor by transcatheter arterial chemoembolization and once by radioactive seed implantation. Five years before presentation, he had undergone an excision of a tumor of the right pterygopalatine fossa.
Physical examination showed no other positive findings except that the liver was enlarged and palpable.
Laboratory investigations showed an abnormal hemogram, including hemoglobin of 123 g/L (reference range: 131-172 g/L), neutrophils 72.8% (reference range: 50%-70%) and lymphocytes 10.8% (reference range: 20%-40%). The results of liver and kidney function were normal. The levels of serum tumor markers (CEA, CA 19-9, CA 12-5, and AFP) were all within normal limits.
Computed tomography (CT) imaging of the abdomen showed a 4.7 cm well-defined mass located in the lower posterior part of the body of the pancreas (Figure ). Non-uniform enhancement was observed from the arterial to portal venous phase. Meanwhile, multiple nodules and masses of various sizes were seen in the liver (Figure ). The largest one was located in the segment VIII of the liver with a diameter of about 15.9 cm. No obvious dilatation of intrahepatic and extrahepatic bile ducts was observed.
Pancreatic magnetic resonance (MR) imagining also confirmed a hypervascular tumor located in the body of the pancreas and multiple tumors located in the liver. Those tumors were hypointense on T1-weighted MR images and hyperintense on T2-weighted MR images.
For a complete preoperative evaluation, fluorine-18 fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) was performed. Images from the PET/CT revealed that both the pancreatic and the metastatic liver lesions had an increased uptake of the tracer FDG. Besides, the thoracic and lumbar vertebrae, humerus, femur, scapulae, ribs, sacrum, and pelvis also showed heterogeneous FDG uptake (Figure ).
A liver biopsy guided by B-mode ultrasound confirmed that the tumor was an SFT/ hemangiopericytoma (Grade 2).


A 67-year-old woman with autoimmune hepatitis cirrhosis was admitted for liver transplantation. At 13 d post-surgery, the patient developed fever of 38.4 °C. The next day, she complained of fatigue, nausea, and vomiting after eating.
This patient presented with myalgia, arthritis and arthralgias, photosensitivity, oral ulcers, keratoconjunctivitis sicca, and positive antinuclear antibody test 11 years ago, and she was diagnosed with systemic lupus erythematosus (SLE) then. Her arthralgias were improving with treatment, but right upper quadrant abdominal pain was worsening. Abdominal ultrasound showed evidence of cirrhosis 5 years ago, and she started to have recurrent hematemesis and melena 1 year ago. She underwent gastroscopic esophageal varies ligation 1 year ago as well as surgical aortic valve replacement due to severe aortic valve stenosis. She was diagnosed with SLE related autoimmune hepatitis with decompensated liver cirrhosis.
Her admission diagnosis was SLE related autoimmune hepatitis with decompensated liver cirrhosis, the Model for End-stage Liver Disease score was 38[]. An appropriate donation after circulatory death was available on the day of admission, and the patient underwent orthotopic liver transplantation. The surgery time was 6 h 25 min, with intraoperative blood loss of 500 mL and transfusion of 400 mL of red blood cells. The patient was removed from the ventilator upon transfer to the intensive care unit (ICU) after surgery. Following surgery, cefepime was administered to prevent infection, along with methylprednisolone and tacrolimus to prevent graft rejection. Epstein–Barr virus test and 1, 3-β-D-Glucan Assay were positive, and Enterococcus faecalis was cultured from intraoperative donor liver lavage fluid and postoperative drainage fluid. As a result, ganciclovir, vancomycin, and micafungin were successively added to the treatment regimen to prevent infection, and liver function improved. Doppler ultrasonography on postoperative day 4 indicated the transplanted liver was normal in shape and size, with an anterior to posterior diameter of about 8.4 cm in the right lobe, homogeneous parenchyma echo, non-dilated internal and external bile ducts, and normal liver blood flow. The patient was transferred to a general ward 4 d after the operation.
By the afternoon of postoperative day 13, the patient’s body temperature increased to 38.9 °C, and she showed lethargy and weakness. From postoperative days 15 to 19, the patient showed persistent fever, with a temperature up to 42 °C. She developed progressive consciousness disorder, which gradually developed into shallow coma with no response to pain stimulation, neck resistance (+), continuous limb and trunk tremors, and higher limb muscle tension. The patient was returned to the ICU on day 19 post-surgery. At this point, the patient had a Glasgow coma scale score of 6, indicative of deep coma with intermittent convulsions, and a temperature of 38.6 °C.
On day 13 post-surgery, routine blood examination showed the following results: white blood cell (WBC) count, 11.67 × 109/L; granulocyte percentage, 90.8%; hemoglobin, 87 g/L; and platelet count, 58 × 109/L. Blood gas results were: pH, 7.42; PCO2, 33 mmHg; PO2, 66 mmHg; and base excess (BE), −3.2. Biochemical test results were: aspartate aminotransferase, 17 U/h; alanine aminotransferase, 8 U/h; albumin, 36 g/L; total bilirubin, 13.93 μmol/L (3.4–17.1 μmol/L); creatinine, 62 μmol/L; lactic acid, 0.5 mmol/L; K+, 4.01 mmol/L; and Na+, 129 mmol/L.
On the day of return to the ICU, blood test results were: WBC count, 7.45 × 109/L; granulocyte percentage, 85.4%; hemoglobin, 74 g/L; and platelet count, 88 × 109/L. Blood gas results were: pH, 7.45; PCO2, 32 mmHg; PO2, 158 mmHg; and BE, −1.9. Biochemical test results were: Aspartate aminotransferase (AST), 14 U/h; alanine aminotransferase, 4 U/h; albumin, 36 g/L; total bilirubin, 12.06 μmol/L; creatinine, 87 μmol/L; lactic acid, 0.5 mmol/L; K+, 4.51 mmol/L; and Na+, 131 mmol/L.
On day 13 post-surgery, chest computed tomography revealed lung bronchiectasis with infection and pleural effusion. Head computed tomography showed lacunar infarction. Head magnetic resonance imaging revealed abnormal signals in the bilateral thalamus and caudate nucleus, bilateral temporal sulcus gyrus, and parahippocampal gyrus, along with infectious lesions, leading us to suspect JEV infection (Figure ).
On the day after return to the ICU, lumbar puncture revealed that cerebrospinal fluid pressure was 70 mmH2O; cerebrospinal fluid white blood cell count, 140 × 106/L (0–8 × 106/L); total albumin 244.67 mg/dL (15–45 mg/dL); and glucose, 4.96 mmol/L (2.24–3.92 mmol/L).
Serum and cerebrospinal fluid samples taken on day 20 were positive for JEV antibodies. And autoimmune encephalitis panel tests were negative in serum and cerebrospinal fluid, including N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma inactivated 1 (LGI1), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor 1 (AMPAR1), AMPAR2, gamma-aminobutyric acid B receptor (GABA-B receptor), contactin-associated protein-like 2 (Caspr2).