A 57-year-old woman was admitted with jaundice all over the body in the last two years. The jaundice was intermittent and progressive. This complaint was accompanied by weight loss of around 20 kg in the last one year. There was no nausea, vomiting, abdominal pain, itching on the body, and fever. The patient reported dark urine, and pale stools manifested in the last six months. There was a history bilioenteric bypass hepaticojejunostomy Roux-en-Y due to common bile duct cyst, two years previously. There was no history of chronic disease. There was no family history of jaundice. Vital sign within normal limits.
Physical examination revealed a lack of nutritional status, the presence of anemic conjunctiva, scleral icterus, jaundice, and surgical scars in the abdomen (). On rectal toucher examination, a pale stool was found. From laboratory tests found a decrease in hemoglobin (7.6 mg/dl), negative results for serum HBsAg tests, and an increase in liver enzymes (AST 177 U/L and ALT 85 U/L), serum direct bilirubin 18.66 mg/dl, and total serum bilirubin level 20.15 mg/dl. Chest X-ray examination within normal limits. On non-contrast MRCP showed dilated right and left intrahepatic duct containing multiple stones, and stricture of bilioenteric anastomosis (). The patient was diagnosed with obstructive jaundice due to stricture of bilioenteric anastomosis after bilioenteric bypass hepaticojejunostomy Roux-en-Y with hepatolithiasis type II LR according to the Takada classification.
Exploratory laparotomy bilioenteric anastomosis reconstruction was then we decided to do (). During surgery, a bilateral subcostal incision (Mercedes incision) and adhesiolysis was performed. We found bilateral hepatic duct dilatation, we make an incision and remove multiple stones. And then, we performed a choledochoscope and confirmed the total occlusion of the tract to the distal common hepatic duct. We did reconstruction Roux-en-Y hepaticojejunostomy with stenting from the left hepatic duct to the right flank.
The postoperative management includes oral feeding administered via nasogastric tube after 48 h of aspiration, H2 blockers, and antibiotics drug, monitoring of complete blood count, and hepatic function for the first four postoperative days. After discharge, clinical conditions and liver function are monitored at 1, 3, and 6 months from surgery. Liver morphological findings were assessed by assessed radiography such as abdominal ultrasonography (US) and MRCP. During the follow-up period, our patients were disease-free.


A 4-year old male neutered Bull terrier weighing 27.6 kg (body condition score 4 of 9) was presented with a 3-months’ history of generalized weakness and pyrexia (body temperature reached up to 42 °C the day before presentation), and a transient lameness (grade 2/4) noted on the left hind limb at the time of initial onset of the dog’s clinical signs. A thorough patient history revealed that the dog lived in the neighborhood of a farm housing pigs. No other relevant details could be extracted from the patient history for guiding the further diagnostic evaluation of the dog. The dog did not have a travel history outside the country of origin (Germany).
On physical examination, the dog was found to be febrile (body temperature 39.5 °C, normal body temperature in an adult dog: 38.0 – 39.0 °C). Cardiac auscultation revealed a grade 3/6 left-sided systolic heart murmur with the same intensity at the left heart base and apex and an irregularly irregular heart rhythm. No other abnormalities were noted during the physical examination. A full neurological and orthopedic examination of the dog was also within normal limits.
The minimum database comprised of a complete blood cell count and serum biochemistry revealed leukocytosis and neutrophilia, as a result of inflammation. There was a mild increase in total proteins and creatinine in blood chemistry, likely resulting from dehydration.
Urinalysis showed a specific weight of 1.016 and microscopic evidence of bacteria. Urine culture was negative. Diagnostic imaging consisting of abdominal ultrasonography and thoracic radiographs was performed to further evaluate the dog for the source of a possible infection. Abdominal ultrasonography revealed no abnormalities. Thoracic radiographs (two orthogonal views) were suspicious for an underlying compensated cardiomyopathy due to an enlarged vertebral heart score (VHS) of 11.5 (reference interval: 8.5–10.5) (Fig. ).
Arthrocentesis or joint radiographs were not performed because swelling of any joint was not noted during the physical examination, and the orthopedic examination of the dog was also unremarkable. While a monoarthropathy (e.g., infectious or traumatic monoarthritis) rarely occurs without an obvious swelling of the affected joint, mild inflammation involving multiple joints (e.g., primary or secondary immune-mediated polyarthritis) cannot be definitively excluded without synovial fluid analysis and cytological examination []. Additional spinal radiographs were not performed because there were no abnormal findings during the neurological and orthopedic examinations.
Further diagnostic evaluation of the dog included molecular diagnostic tests for infectious diseases (Babesia spp. PCR, Anaplasma spp. PCR, Hepatozoon canis PCR, and Ehrlichia spp. PCR) that are compatible with the clinical presentation of the dog. These tests were all negative. Also, serological evaluation for Brucella canis and a whole blood PCR for Bartonella spp. were performed, both of which were also negative.
Further evaluation of the dog to determine the source of chronic pyrexia, the heart murmur noted during cardiac auscultation, and the findings on thoracic radiographs consisted of transthoracic echocardiography and a blood culture with aerobic and anaerobic incubation. A blood culture system with a culture medium that is suitable to promote the growth of aerobic, anaerobic, and micro-aerophilic organisms (Oxoid BC0100 tryptone soya broth, gelatine peptone; Oxoid Limited, Basingstoke, Hampshire, UK) was used. Collection of blood for bacterial culture was performed longitudinally (2 h apart) and from multiple sites (three separate venipunctures: one from a cephalic vein, one from a jugular vein and one from a saphenous vein) according to current consensus, yielding six blood samples (2 ml each) altogether. Blood samples obtained from one of the 3 different venipuncture sites (3 × 2 mL) were first combined, then the total volume was added to the bottle of culture medium (2 × 3 mL). The blood samples were collected under strictly aseptic conditions using sterile disposable syringes.
Transthoracic echocardiography displayed characteristic mobile vegetations of the aortic and mitral valves (Fig. ), with structurally altered and irregularly thickened valve leaflets (especially the aortic non-coronary cusp and the anterior mitral valve leaflet), severe aortic stenosis (max. pressure gradient: 93 mmHg), moderate aortic regurgitation (max. pressure gradient: 142 mmHg), and moderate to severe mitral regurgitation (max. pressure gradient: 212 mmHg). Pulmonic and tricuspid valves appeared structurally normal with normal pulmonary flow, but mild tricuspid regurgitation was noted. There was mild left ventricular concentric hypertrophy with normal end-diastolic and systolic dimensions. The left atrium-to-aortic root ratio (LA/Ao) was within normal limits (LA/Ao: 1.20). Ventricular extrasystoles were noted on the electrocardiogram. A presumptive diagnosis of infective endocarditis was made based on the echocardiographic findings.
Medical treatment was initiated on the day of presentation and diagnostic evaluation (day 1), while the results of aerobic and anaerobic blood culture were still pending, with enrofloxacin 5 mg/kg PO q24h (Baytril®; Bayer, Leverkusen, Germany), amoxicillin/clavulanic acid 18 mg/kg PO q12h (Synulox®; Zoetis, Parsippany, NJ, USA), sotalol 0.7 mg/kg PO q12h (Sotalol; Ratiopharm, Ulm, Germany) and clopidogrel 1.3 mg/kg PO q24h. (Plavix®; Sanofi-Aventis, Frankfurt, Germany). The dose of sotalol was reduced to 0.3 mg/kg PO q12h on day 3 because the dog developed tachycardia (noted on palpation of the chest by the owners) after sotalol administration for the first 3 consecutive doses.
One week later (day 7), the blood culture result was reported and revealed a positive result with the isolation of a gram-positive organism that was subsequently identified as E. rhusiopathiae. Antimicrobial susceptibility testing showed that this bacterial organism was susceptible to penicillin, amoxicillin/clavulanic acid, cephalosporine, lincomycin, fusidic acid, chloramphenicol, and metronidazole. These results led to the replacement of enrofloxacin by metronidazole 40 mg/kg PO q8h (Metrobactin® CP-Pharma, Burgdorf, Germany), whereas amoxicillin/clavulanic acid, clopidogrel and sotalol were continued as initiated. The dog’s rectal temperature decreased to normal within 72 h after switching to metronidazole, and the dog’s overall condition returned to normal.
A follow-up evaluation including echocardiography two weeks later (day 21) showed a slightly reduced aortic stenosis (pressure gradient: 82 mmHg), but the aortic and mitral regurgitation, as well as the aortic valve vegetative lesions, remained unchanged. Also, there was a severe left atrial dilation (LA/Ao: 2.03). Thus, benazepril at 0.2 mg/kg PO q24h was added to the dog’s treatment plan. Pimobendan (Vetmedin®; Boehringer, Ingelheim am Rhein, Germany) was withheld as a treatment option due to the marked aortic stenosis.
Recheck echocardiography was performed approximately every 2 weeks, and the treatment plan (as described above) was continued for approximately 3 months when the next recheck was performed (day 91). On echocardiography, aortic and mitral valve vegetations were reduced, and the left ventricular dimensions and systolic function had remained stable. However, the anterior mitral valve leaflet appeared notably immobile during diastole compared to the posterior leaflet. There was no mitral stenosis, and mitral inflow pattern was within normal limits. The left atrium remained markedly dilated.
A recheck echocardiographic evaluation of the dog two months later (day 147, delayed by 5 weeks due to owner constraints) showed the left atrial dimensions to be unchanged, but the anterior mitral valve leaflet be slightly more mobile (but not normal). Systolic dysfunction was mildly progressive, and aortic regurgitation had progressed from moderate to severe. Owner compliance was considered to be good, with reliable medication administration. Metronidazole and sotalol were discontinued, and furosemide 1.0 mg/kg PO q12h (Dimazon®; MSD, Kenilworth, NJ, USA) was added.
The dog was then lost to direct follow-up because of the emigration of the owners shortly after the last recheck examination of the dog approximately 16 weeks after initiation of medical treatment. Drug administration was continued, but no further follow-up echocardiograms were performed due to owner constraints.
The dog remained in good general and physical condition and showed no signs of disease recurrence at home (i.e., absence of pyrexia, lameness, and signs of weakness) until approximately 27 months after the initial presentation and diagnosis of infective endocarditis. At that time, the dog started to show spontaneous coughing and a moderately reduced general condition. The local veterinarian suspected pneumonia, and the owner elected humane euthanasia of the dog at the referring veterinarian due to the poor general health. A necropsy was not consented to by the owner.


A 65-year-old Caucasian male with past medical history significant for hepatitis and HIV complicated by multiple pneumocystis pneumonia (PCP) infections [not on highly active antiretroviral therapy (HAART)] initially presented to the emergency room for evaluation for severe depression after his cat passed away. The patient stated that he had a sense of depression for three weeks prior to his admission. He conveyed that during that three-week stretch, he experienced decreased appetite, unintentional weight loss of roughly 20 pounds, and suicidal ideation. Furthermore, during that time frame, he also complained of progressively worsening shortness of breath associated with a cough productive of yellow-whitish sputum, diffuse body aches, weakness, and bilateral lower extremity edema. The patient denied coming into contact with individuals who were ill and denied exposure to the COVID-19 virus. However, he did admit to living in a boarding home where he shared public areas, such as bathrooms, with other tenants. The patient revealed that he had a significant history of substance abuse spanning the last 10 years. He admitted to the use of Marijuana and Cocaine, with his last use being 10 days prior to his admission. The patient's urine drug screen (UDS) was positive for cannabinoids. Moreover, he denied the use of IV drug use.
Upon evaluation in the emergency room, the patient was found to be afebrile with an oral temperature of 36.4 C, normotensive with a blood pressure of 114/90, saturating at 94% on room air, and with a heart rate of 100 bpm. Electrocardiogram (EKG) shows normal sinus rhythm. His complete blood count (CBC) revealed no leukocytosis with a white blood cell (WBC) count of 8.7 K/uL (4.8-10.8 K/uL). Troponin was negative. The patient's chest X-ray revealed bilateral reticular opacities (left significantly greater than right) with small bilateral pleural effusions. After review of his comprehensive metabolic panel (CMP), the patient was noted to have an elevated creatinine of 2.28 mg/dL (0.7-1.2 mg/dL). Arterial blood gas (ABG) studies revealed a partial pressure of oxygen (PO2) of 46 mmHg (>80 mmHg) and a partial pressure of carbon dioxide (PCO2) of 23.6 mmHg (35-45 mmHg).
Upon admission to the hospital, the patient was noted to have acute kidney injury (AKI) with a creatinine 2.28 mg/dL (0.7-1.2 mg/dL). Further evaluation via ultrasound revealed a left renal cyst. Ultimately, the patient's creatinine mildly improved during the course of his treatment to 1.97 mg/dL. After undergoing a more thorough work-up, the patient was found to be positive for COVID-19. He also had a transthoracic echocardiogram, which showed a left ventricular ejection fraction (LVEF) of 30-35%, ischemic cardiomyopathy, and muscular ventricular septal defects with left to right shunting and severely elevated pulmonary artery systolic pressure as seen in Video .
Over time, the patient's bilirubin and aspartate transaminase/alanine transaminase (AST/ALT) continued to rise, peaking at an AST of 2,365 U/L (15-41 U/L) and an ALT of 1167 U/L (17-63 U/L). The patient's international normalised ratio (INR) also became elevated reaching a maximum of 7.0 with a prothrombin time (PT) of 37.8 seconds (12.6-14.6 sec). Evidently, the patient was in fulminant hepatic failure. Furthermore, he was also in renal failure with a blood urea nitrogen (BUN) > 130 mg/dL (8-20 mg/dL). Also, the patient was found to have an elevated D-dimer of > 5,000 ng/ml (0-230 ng/ml). However, the patient was unable to receive anticoagulation due to his elevated INR. On physical examination, he had multiple petechiae and ecchymoses throughout his body, was severely jaundiced, but was awake, alert and oriented to date, place and person (AAOx3). No cardiac murmur was appreciated on examination. An extensive conversation was held with the patient with regards to goals of care in which patient made himself do-not-resuscitate/do-not-intubate (DNR/DNI). Unfortunately, his health continued to deteriorate. Both cardiology and nephrology were on board as he was treated symptomatically.
During hospital course, the patient became dyspneic and was subsequently started on 2 mg of IV morphine for comfort as per family discussion. The next morning, the patient was found unresponsive with dilated pupils and no brain stem reflexes and was pronounced dead.


A 35-year-old woman developed grip myotonia at age 27. She had no underlying diseases or family history of relevant conditions, including DM1. Her symptoms gradually worsened, and she visited a tertiary hospital at age 32. A neurological examination revealed no focal neurological deficits, including weakness or muscle atrophy. Myotonia was observed only when gripping with both hands. Her laboratory tests were normal. Needle EMG showed increased insertional activity and a myotonic response with positive sharp waves and fibrillation potential. Genetic testing revealed DM1 (CTG)n with repeat lengths of 150. She was finally diagnosed with adult-onset DM1. She was started on carbamazepine (CBZ) 200 mg/d. Immediately after medication administration, her grip myotonia improved without discomfort. However, the symptoms returned after 6 hours. Despite CBZ, her hand grip relaxation time (RT) gradually increased over 1 year. The CBZ dose was increased to 400 mg/d; later, due to lethargy, it was changed to phenytoin 100 mg/d. Nevertheless, her symptoms were consequently aggravated, and she eventually stopped taking all medicine. Five months later, she visited our clinic with a chief complaint of bilateral grip myotonia (Fig. ).
Her baseline RT after a maximal voluntary isometric contraction was 59 seconds. Figure shows the time-course of the patient's condition. EA treatment was performed 29 times over 3 months. After insertion of acupuncture needles into the acupuncture point TE9 and 2 cm above TE9 at the extensor digitorum communis in the forearm, 10-Hz electrical stimulation was applied, with visible muscle twitching, for 10 minutes. The EA procedure is described in the STandards for Reporting Interventions in Clinical Trials of Acupuncture (STRICTA) checklist[ (Supplement 2).
Initially, she could repeatedly grasp and open her hands without grip myotonia immediately after EA treatment, although her symptoms generally returned within an hour. However, with additional acupuncture sessions, her symptom relief was prolonged. After the 24th treatment session, she maintained a normal state with good symptom control for up to 7 days. At this point, her treatment frequency was decreased to once a week.
Improved RT and symptoms were well maintained (Fig. ). Her grip RT decreased from 59 seconds (1st session) before treatment to within 2 seconds after 29 treatment sessions over 3 months. A video clip of her hand motion is provided as Video-1. During EA treatment, she did not use any myotonia-related medication or engage in any other rehabilitation.
The Korean version of the Michigan Hand Outcomes Questionnaire (K-MHQ) is a hand-specific outcome instrument which assesses overall function, activities of daily living (ADLs), pain, work performance, aesthetics, and patient satisfaction.[ The K-MHQ was administered at monthly intervals to measure functional hand improvements. Over 3 months, there was an improvement in the K-MHQ total score (from 66.6 to 75.9), function score (from 70 to 90), satisfaction score (from 75 to 91.7), and ADL score (from 59.7 to 73.6) (Table ).
The Measure Yourself Medical Outcome Profile 2 (MYMOP2) is a patient-reported outcome measure where patients report any specific symptoms, activity limitations, and emotional states.[ The MYMOP2 can be utilized to assess physical, emotional, and social variables using a brief and simple questionnaire. Results are reported according to a seven-point Likert scale (from 0 to 6), and average scores are used. The most uncomfortable symptoms were described as “a symptom of not spreading well after holding her hand tight,” and her most uncomfortable activity was described as “uncomfortable when locking the buttons on clothes.” After treatment, her MYMOP2 scores for her 2 chief complaints decreased from 4.33 to 2 (Fig. ).
There was no deterioration in EMG after EA treatment. Over the 3 months of treatment, no adverse effects were observed except for bruises, <2 cm in diameter, located at the EA treatment site. These bruises were monitored closely and improved within 1 week.


A 36-year-old man presented with cough for three days. Examination revealed incidental findings of an asymmetric chest with hypoplastic and flattened left anterior chest wall. There was an absence of the pectoralis major, axillary fold, and axillary hair on the left side (Figure ). The nipple on the left side was smaller and higher. Movements at the left shoulder were full. The patient also had short and webbed fingers (brachysyndactyly) in the left hand (Figure ). These deformities had been present since birth. Heart, breath, and bowel sounds were normal. The examination of the neck and back was unremarkable.
X-ray of the chest showed hyperlucency on the left side, with normal lung parenchyma, heart shadow, and ribs (Figure ). Computed tomography (CT) scan of the chest showed an absent left pectoralis major. X-ray of the left hand showed hypoplasia of the proximal phalanx, aplasia of the middle and distal phalanges of the second digit, and aplasia of the middle phalanges of the third and fourth digits (Figure ). Ultrasonography (USG) of the abdomen and pelvis was normal. All laboratory investigations were normal.
A diagnosis of left-sided Poland syndrome with associated brachysyndactyly was made. Options of available reconstructive procedures, along with the risks and benefits, were explained to the patient. He opted against it, as he had limited and manageable functional limitations and was not concerned about cosmesis.


A 55-year-old male with a past medical history of squamous cell carcinoma of the tongue, chronic kidney disease, gastroesophageal reflux disease, and severe alcohol use disorder presented to our institution for the evaluation of abnormal liver biochemistries and painless jaundice. The patient initially presented to the ED after suffering a fall at his home. Initial evaluation revealed diffuse jaundice. As such, lab work was obtained, which was significant for total bilirubin of 23.7 mg/dL (reference range: 0.1-1.2 mg/dL), direct bilirubin of 21 mg/dL (reference range: 0.1-0.3 mg/dL), alkaline phosphatase of 1,480 U/L (reference range: 44-147 IU/L), aspartate aminotransferase of 320 U/L (reference range: 5-40 IU/L), alanine aminotransferase of 180 U/L (reference range: 7-56 IU/L), white blood cell count of 40 x 109/L (reference range: 4.5-11.0 × 109/L), potassium of 5.4 mmol (reference range: 3.5-5 mmol/L), blood urea nitrogen of 51 mg/dL (reference range: 2.5-7.1 mmol/L), and creatinine of 5 mg/dL (reference range: 0.7-1.2 mg/dL). C-reactive protein was also elevated at 35 mg/dL (reference range: 0.1-1 mg/dL); however, lactic acid and procalcitonin were within normal limits at 2.3 mmol/L (reference range: 0.5-2.3 mmol/L) and 0.47 ng/mL (reference range: 0.10-0.49 ng/mL), respectively. Coagulation studies revealed an international normalized ratio of 1.2 and prothrombin time of 12 seconds (reference range: 11-13.5 seconds). The patient denied abdominal pain, fever, or chills, and vital signs were within normal limits. CT of the abdomen showed an enlarged liver and diffuse hepatic steatosis (Figure ).
On day one of admission, the patient was started on broad-spectrum antibiotics with cefepime and vancomycin. The etiology of his leukocytosis was unclear as extensive infectious workup was negative. Multiple sets of blood cultures did not show any growth, and there was no evidence of any abnormal collection on imaging. Urine analysis and X-ray chest were unremarkable. Despite the initiation of antibiotics, there was no improvement in white blood cell count, which peaked at 42 x 109/L on the fifth day of admission. The gastroenterology service was consulted, who determined that the most likely etiology was alcoholic hepatitis in the setting of underlying cirrhosis. Given the negative infectious, metabolic, and autoimmune workup, the patient underwent endoscopic ultrasound (EUS)-guided liver biopsy, which showed diffuse fibrosis and neutrophilic infiltration (Figures , ). The patient had an elevated Maddrey’s discriminant function score of 34, with an MELD (model for end-stage liver disease) score of 24.
The patient was subsequently started on prednisolone for the treatment of alcoholic hepatitis, with only mild improvement in liver function tests. Total bilirubin peaked at 24.9 mg/dL and was at its lowest on the second day of prednisolone treatment at 20.3 mg/dL. In light of abnormal liver biochemistries, he underwent magnetic resonance cholangiopancreatography (MRCP), which was negative for biliary obstruction. Esophagogastroduodenoscopy (EGD) was significant for candida esophagitis, along with portal hypertensive gastropathy (Figures , ). As such, steroids were discontinued. The patient was not a candidate for fluconazole given his liver injury and was started on micafungin which was later discontinued after learning of its potential hepatotoxicity. Because of the unusual presentation with such profound leukocytosis without evidence of sepsis, the hematology service was consulted to rule our possible hematologic malignancy. Bone marrow biopsy showed no evidence of myeloproliferative state. Janus kinase 2 (JAK 2) testing was negative, ruling out a myeloproliferative disorder. Unfortunately, the patient passed away on the 13th day of admission. Request for autopsy was declined; however, it was presumed that the patient expired as a result of alcoholic hepatitis.


A 9-year-old boy from the district of Kilinochchi, in the Northern Province of Sri Lanka, was transferred to the Sirimavo Bandaranayake Specialized Children’s Hospital (SBSCH), Peradeniya from the District General Hospital, Kilinochchi on 4 January 2020. This child was visually impaired due to bilateral glaucoma. After admission to the SBSCH, the patient’s mother gave a history of fever with diarrhea, which progressed from watery to bloody diarrhea 1 week prior to the transfer.
This patient was misdiagnosed at the District General Hospital, Kilinochchi and treated for paralytic ileus associated with electrolyte imbalance for 3 days, and he was on intravenous cefotaxime.
On admission to the surgical intensive care unit of the SBSCH, the child was tachycardic, hypotensive, and hypothermic. He had a heart rate of 190/min, and his blood pressure was unrecordable. He had bloody diarrhea with abdominal distension and tenderness. The nasogastric tube in site had large volumes of a dark green bilious aspiration. Initial blood investigations revealed elevated inflammatory markers indicative of severe sepsis. The X-ray showed a dilated colon (Fig. a). The patient was resuscitated with intravenous fluid boluses and fresh frozen plasma. Noradrenalin was added as an ionotrope, and antibiotics were changed to meropenem and teicoplanin.
Surgery was performed at 6 am the next day, 8 h after he was admitted to SBSCH. Thick turbid ascetic fluid was found with multiple transmural ulcers affecting the entire colon (Fig. b). There were multiple concealed perforations at the hepatic and splenic flexures. The small bowel was not involved. Part of the sigmoid colon was preserved for future anastomosis. Terminal ileostomy was performed, and a mucous fistula from the distal sigmoid colon was created. Intravenous metronidazole was added post-operatively.
The patient initially improved but later deteriorated showing signs of ongoing peritonitis. Therefore, a re-exploration was performed to excise the remaining part of the colon as it was found to be necrotic and Hartman’s fistula was created. Specimens were sent for histological and microbiological investigations including bacterial and amebic culture. Histopathology of the colon and the caecum revealed extensive benign ulceration in colonic mucosa extending to the full thickness. Typical flask-shaped amebic ulcers were seen (Fig. a). Periodic Acid Schiff-Martius (PAS-Martius) Yellow 40 stain highlighted amebae with erythrophagocytosis within the necrotic debris of the ulcers (Fig. b). Smears and cultures were negative for amebae. The PCR that was conducted with primers specific for E. histolytica to confirm the diagnosis was positive for E. histolytica on both the ulcerated colon segments and peritoneal washings.
The post-operative course in the surgical intensive care unit was complicated by septicemia. The patient was treated with ionotropes, and ventilatory care was given for 2 weeks. Intravenous antibiotics as a combination of metronidazole, meropenem, teicoplanin, ciprofloxacin, and colestin were given. Partial parenteral nutrition was added to reduce nutritional stress as oral feeding was not possible.
The child gradually improved and 3 weeks later was moved from the surgical intensive care unit to the high dependency unit. A month after admission to the SBSCH, the patient showed good recovery and was feeding well. He was transferred to the surgical ward and subsequently discharged home on a normal diet.


An 82-year-old woman presented to the Emergency Department (ED) with progressive shortness of breath that had started three days prior and was associated with bilateral swelling of the lower extremities. The patient also complained of orthopnea but did not notice any cough, fever, or chest pain. The patient denied any alcohol, tobacco, or illicit drug use but had a significant past medical history of type II diabetes mellitus, hypertension, hyperlipidemia, diastolic heart failure, peripheral arterial disease, hypothyroidism, and stage 3B chronic kidney disease (CKD). Home medications included amlodipine, benazepril, hydralazine, hydrochlorothiazide, aspirin, clopidogrel, cilostazol, atorvastatin, and basaglar insulin. At presentation, the patient had a blood pressure of 176/82 mmHg, a pulse of 78 bpm, a temperature of 98°F, a respiratory rate of 28 breaths/min, and an oxygen saturation in room air of 92%. Physical examination revealed reduced bilateral breathing sounds in the lung bases, distant heart sounds with a normal S1 and S2, no jugular venous distension (JVD), and bilateral pitting edema of the lower extremities up to the mid-calf.
Initial testing showed: normocytic anemia with a hemoglobin (Hb) of 9 g/dL, CKD with a creatinine (Cr) of 1.86 mg/dL and a glomerular filtration rate (GFR) of 31, an elevated NT-pro B-type natriuretic peptide (PBNP) of 3760 pg/dL, an elevated D-dimer 3110 DDU, and negative cardiac enzymes. Chest X-ray showed cardiomegaly and bilateral pleural effusions [Figure ].
Electrocardiogram (EKG) at presentation showed low voltage QRS complexes in most leads, with nonspecific t-wave abnormalities, and the heart rate was 76 bpm in normal sinus rhythm [Figure ].
Computed Tomography (CT) of the chest was done that showed cardiomegaly with pericardial effusion and bilateral pleural effusion [Figure ].
The patient was started on furosemide for diuresis in the face of acute exacerbation of heart failure. She received one day of therapeutic anticoagulation in view of her high Wells score and D-dimer levels indicative of possible Venous thrombo-embolism. A pulmonary embolism was ruled out with a ventilation-perfusion scan. Lower extremity duplex showed no deep vein thrombosis.
An official echocardiogram was performed and showed moderate pericardial effusion (13 mm) with EF 50-55% and diastolic dysfunction [Figure ].
The previous echocardiogram from three months prior showed preserved left ventricular ejection fraction with grade 1 diastolic dysfunction and a trace pericardial effusion. It was decided to continue to monitor the effusion with serial echocardiograms as there was no clinical evidence of tamponade physiology.
The patient's respiratory status mildly improved with continued diuresis. A repeat echocardiogram on the sixth day of hospitalization showed a persistent moderate pericardial effusion (measuring 13 mm) [Figure ]. The patient remained hemodynamically stable with no evidence of tamponade physiology.
After discussing options with the patient she agreed to a therapeutic as well as diagnostic pericardiocentesis. Straw-colored pericardial fluid (620 ml) was drained and a pericardial drainage catheter was left in-situ. Pericardial fluid analysis was suggestive of transudative fluid with albumin 2.7 g/dL pH 8, lactate dehydrogenase (LDH) 179 units, cytology was negative for malignant cells, and macrophages were present. Gram stains and cultures were unremarkable.
The patient's thyroid stimulating hormone (TSH) and thyroxine (T3/T4) levels were within normal limits; her home dose of levothyroxine was continued and hypothyroidism seemed unlikely to be the precipitating factor for her pericardial effusion.
Due to the high output from the pericardial catheter, further investigation was warranted to delineate the etiology of the effusion. Autoimmune diseases screening was done and results are shown in Table .
A diagnosis of pericardial effusion secondary to HILS was made and hydralazine was stopped indefinitely. A repeat echocardiogram a week after stopping the medication showed no pericardial effusion and normal left ventricular ejection fraction. Over the course of her hospital stay the patient developed a left middle cerebral artery ischemic infarct and was discharged to a rehabilitation facility.


A 52-year-old African American male with a history of chronic hepatitis B noncompliant with tenofovir presented with worsening intermittent right upper quadrant abdominal pain for the last two years. Given the increase in the intensity of the pain, he presented for evaluation. The patient denied smoking and illicit drug use and had not consumed alcohol for over 10 years. The progressive nature of the pain prompted further evaluation by his hepatologist. No acute findings were found on examination or laboratory workup. An ultrasound of the liver demonstrated three suspicious lesions. Given these findings, magnetic resonance imaging (MRI) of the abdomen with the liver protocol was obtained, which demonstrated three lesions in the liver; all were LI-RADS 3 (Liver Imaging Reporting and Data System). At baseline, this patient had an ECOG (Eastern Cooperative Oncology Group) performance status of 0. Follow-up MRIs over the next two years portrayed similar-sized lesions and overall stable findings. At the two-year mark, a follow-up MRI demonstrated that the lesion in segment 6 of the right hepatic lobe had increased in size to (1.3x2.2x2 cm) 2.2 cm. This lesion demonstrated arterial enhancement with washout and was categorized as an LI-RADS 5 lesion. He underwent an unsuccessful transjugular liver biopsy. The alpha-fetoprotein (AFP) at the time was 5.7. He was referred to medical oncology at this point. Given the high clinical and radiological suspicion of malignancy, the patient underwent a liver resection to excise the tumor segments. Two hepatic tumors were found and resected, one in segment 6 of the right lobe and another in segment 4b of the left lobe. The lesion in segment 6 was characteristic of both HCC and CC and was thus was termed biphenotypic cHCC-CC. The lesion in segment 4 lobe was moderately differentiated HCC.
The patient recovered well from surgery. Follow-up imaging did not demonstrate evidence of disease. The patient was placed on three to four monthly surveillance visits and scans. Six months later, his lab work demonstrated elevated AFP. MRI of the abdomen showed a liver lesion in the right lobe segment 6/7 measuring 3.6x3.6x3.3 cm with some vascular flow. Computed tomography (CT) of the chest did not demonstrate metastatic disease to the lungs. The patient then underwent a right hepatic lobectomy with a portal lymphadenectomy. The mass resected was HCC, and one out of the three lymph nodes resected was positive for cholangiocarcinoma. He was commenced on chemotherapy with 5-FU and oxaliplatin. After eight cycles of therapy, he developed abdominal pain, which prompted a CT scan of the abdomen. This demonstrated stable disease. Due to the persistent symptoms, a positron emission tomography-computed tomography (PET-CT) was done, which, on the contrary, demonstrated worsening disease. At this point, the option of looking for a clinical trial was explored with the patient. He declined. Given the rising AFP, it was thought that the dominant component is likely the HCC and hence the patient was commenced on nivolumab. He showed some clinical improvement and a stable AFP. After five cycles of therapy, he demonstrated a rising AFP. PET-CT demonstrated significant disease progression. Since he retained an excellent performance status (ECOG 0), we subsequently proceeded with gemcitabine and cisplatin. We attempted to treat both components, HCC and CC, of the disease. Hence, in the setting of disease progression and lack of treatment guidelines, this patient was also commenced concurrently on sorafenib with gemcitabine and cisplatin. Again, he demonstrated clinical stability in the setting of a stable but elevated AFP. He tolerated this therapy very well with a mild hand-foot syndrome, which very quickly responded to salicylic acid 6% cream. A CT scan after three cycles of therapy, along with continued sorafenib treatment, demonstrated stable disease and a slight reduction in the AFP; hence, the current regimen was continued. Unfortunately, at the end of six cycles, imaging demonstrated disease progression and rising AFP. The patient declined a suggestion to explore a clinical trial at a local university hospital.
While he received gemcitabine, cisplatin, and sorafenib, next-generation sequencing (NGS) was performed on his tumor. The results demonstrated a CCND1 genomic alteration, as well as stable microsatellite status and intermediate tumor mutation burden. While there is no Food and Drug Administration (FDA)-approved therapy for a CCND1 genomic amplification in biphenotypic cHCC-CC, Palbociclib is indeed FDA-approved therapy for a CCND1 genomic amplification in hormone-positive, advanced or metastatic breast cancer. After discussing with the patient information available from NGS and lack of data to provide evidence of success with palbociclib in the setting of his disease, the patient agreed to pursue this last option of therapy. He was commenced on palbociclib, which portrayed evidence of decreasing AFPs. The patient continued to maintain a good quality of life for two months that he was on Ibrance. He continued to work full time. Blood counts and other necessary parameters were monitored and remained within acceptable ranges. Unfortunately, two months into being on Ibrance, his disease progressed. During this time, the patient developed bilateral pneumonia and died within a week of his infectious diagnosis from sepsis in the setting of an immunocompromised state.


A 34-year-old male patient with no prior medical history presented at the Emergency Department reporting ingestion of several metallic objects one hour before. The clinical examination was unremarkable, and the patient was in overall excellent condition. X-ray studies revealed at least 4 metallic objects in various parts of the GI tract ().
Considering the timing of ingestion and the type of the ingested materials, endoscopic extraction was decided. Esophagogastroduodenoscopy (EGD) was performed approximately 2 hours after the ingestion. Four objects were visualized at the time of endoscopy (one screw in the first part of the duodenum, one elongated pin impacted in the antrum wall, one coin, and one small metal scrap in the stomach). The coin was easily removed from the stomach using a Roth Net. An overtube was then introduced in order to safely retrieve the remaining 2 larger sharp objects. None of the remaining objects could fit in the overtube, and the patient's tolerance to the procedure was poor despite maximal sedation. No other protective devices such as rubber hoods were available at the time of endoscopy. Therefore, any further efforts to extract the objects were abandoned, and the patient was returned to the surgical ward for monitoring. Recognizing that complication rates could be high in this patient and that referral to another better equipped facility would require time, such that it would expose the patient to the risks of delayed intervention, we improvised a handmade rubber hood, constructed from a plastic transfusion pressure infusor (). This material was chosen because it was flexible enough to invert back on itself in the cardia upon withdrawal of the scope, yet thick enough to withstand puncturing. The hood was fastened with silk tape to the distal end of a 9.8 mm gastroscope (GIF-Q165 Olympus Corporation, Tokyo, Japan), in a coned fashion (to facilitate the entrance of the foreign body into the bell of the hood), and then inverted (Figures and ). A test was carried out before scope insertion to make sure that the foreign body fits in the hood ().
The patient was called for the second EGD three hours after the first one. The two larger foreign bodies were still in the stomach, whilst the small metal scrap had migrated distally. With the help of mild sedation (3 mg of midazolam), the pin and the screw were captured using rat-tooth forceps, pulled into the hood, and extracted (). The hood inverted back on itself as expected, with no tissue injury upon second endoscopic look. The procedure lasted only 15 minutes with excellent tolerance from the patient.
The patient was returned to the ward for monitoring because two sharp objects were still in his small bowel. Repeat X-ray studies the following days confirmed successful discharge of the foreign bodies per rectum without any complications.


A 68-year-old Hispanic man with ESRD initially presented to an outside urgent care clinic with a 3-day history of a painful rash over the right thorax. His examination revealed a dermatomal, vesicular eruption at the T9 dermatome consistent with zoster reactivation. He was started on oral acyclovir 800 mg five times daily. The recommended renal dosing for a patient receiving dialysis would have been 200 mg twice daily. Over the following 4 days, his family noted progressively worsening confusion. On the day of admission, the patient’s son called emergency medical services due to the patient’s confusion, agitation, and auditory hallucinations. Prior to this presentation, the patient had lived independently and was able to complete all activities of daily living without assistance. His past medical history included ESRD, hypertension, type 2 diabetes mellitus, anemia due to chronic renal disease, and secondary hyperparathyroidism. Prior to arrival at our hospital, his medications included amlodipine 5 mg oral once daily, aspirin 81 mg oral once daily, metoprolol succinate 150 mg oral once daily, and 20 units of insulin NPH-regular 70–30% twice daily. Oral acyclovir 800 mg five times daily was the only new medication, started 4 days prior to arrival. He received outpatient dialysis three times weekly. Per the report of his family, he did not smoke or drink alcohol. His family was unaware of any family history of neurologic disorders. He was unemployed and had no recent travel exposures. He had not been previously vaccinated against VZV.
Upon physical examination, he was agitated and oriented only to self. His vital signs upon presentation included a body temperature of 37.3 °C, heart rate of 100 beats per minute, blood pressure of 177/90 mmHg, respirations of 20 breaths per minute, and oxygen saturation of 93% on 2 L by nasal cannula. Examination of the patient’s head and neck revealed no signs of trauma. His pupils were equal, round, and reactive to light. His oropharyngeal examination revealed dry mucous membranes but no lesions or ulcers. His pulmonary examination result was clear to auscultation bilaterally. His cardiovascular examination revealed normal heart sounds without murmurs, rubs, or gallops. His abdomen was nontender and without signs of guarding. His extremities were warm, and no lower extremity edema was present. His musculoskeletal examination revealed normal range of motion and no joint erythema or effusions. His skin examination revealed a right-sided rash with erythematous papules with vesicles along the T9 dermatome (see Fig. ). Upon neurologic examination, he was unable to follow commands for testing cranial nerves, strength, or coordination. His speech was dysarthric but not aphasic. He moved all extremities spontaneously and withdrew to pain. His reflexes were 3+ bilaterally in the upper and lower extremities with no clonus and a negative Babinski sign. His muscle tone was normal.
His laboratory studies were notable for a serum glucose level of 170 mg/dl, sodium level of 133 mEq/L, blood urea nitrogen level of 43 mg/dl, creatinine level of 5.90 mg/dl, calcium level of 8.5 mg/dl, white blood cell count of 6.8 × 109 cells/L, hemoglobin level of 11.5 g/dl, and platelet count of 239 × 109 cells/L. His thyroid-stimulating hormone level was 2.95 mIU/L. The result of his urine drug screen was negative, and his ethanol level was <10 mg/dl. The result of his urinalysis was negative for leukocyte esterase or nitrites.
MRI of the patient’s brain did not reveal large-territory infarct or evidence of vasculopathy. His electroencephalogram demonstrated diffuse slowing and disorganization consistent with a toxic-metabolic encephalopathy, but he had no epileptic discharges. His chest x-ray did not reveal any pulmonary infiltrates or evidence of pneumonia. CSF studies showed a protein level of 85 mg/dl, glucose level of 76 mg/dl, no red blood cells, no white blood cells, and no organisms on Gram staining. His CSF PCR result was positive for VZV and negative for other common etiologies of bacterial or viral encephalitis.
The emergency department did not initially have access to his prior urgent care pharmacy records. Upon receiving the positive VZV CSF PCR result, he was started on intravenous acyclovir renally dosed at 5 mg/kg and was admitted to the medicine service for his acute encephalopathy. With common toxic and metabolic causes of encephalopathy ruled out in the emergency department, our primary differential diagnosis was between VZV encephalitis and AIN. Uremic encephalopathy was also included in our differential diagnosis. However, review of the outpatient dialysis records revealed adequately dosed dialysis, as evident by achieving target dialysis clearance parameters and no missed dialysis sessions, making this diagnosis unlikely. When the outside pharmacy records arrived, revealing inappropriate acyclovir dosing, AIN became the favored diagnosis. Acyclovir was stopped, and serum drug levels were sent to a reference laboratory. CSF titers for anti-VZV immunoglobulin M (IgM) and IgG were also pending at this time.
Nephrology was consulted, and the patient underwent two 4-hour dialysis sessions on consecutive days, after which his neurologic status improved significantly. His hospital course was not complicated by other evidence of infection, electrolyte abnormalities, or hypoglycemia that could have contributed to his acute encephalopathy. By hospital day 4, he had returned to his baseline neurologic status. On hospital day 5, he had a normal neurologic examination result and was discharged to home. At follow-up 6 months later, the patient had experienced no further episodes of confusion, dysarthria, or hallucinations and was living independently. The temporal onset of hallucinations, dysarthria, and confusion after initiating acyclovir therapy combined with the rapid improvement after discontinuation of acyclovir and initiating hemodialysis made AIN the likely diagnosis.


A 70-year-old male was prescribed telmisartan 40 mg a day to achieve the target blood pressure (BP), since his BP was not well controlled on amlodipine that he had been taking for the previous few months. He had no other significant past medical history. His serum creatinine and potassium, measured a few weeks earlier, were 1.3 mg/dL and 4.1 mmol/L, respectively. After starting telmisartan, he also took over-the-counter diclofenac 50 mg, up to three tablets a day, for his back pain. He presented to the casualty after a week on experiencing two syncopal episodes while sitting. They were not accompanied by chest pain, palpitations or breathlessness. There was no history of fever, diarrhea, vomiting, limb weakness or convulsions. On presentation, he had BP of 80/40 mmHg and pulse of 20 beats per minute. His electrocardiogram (ECG) showed junctional bradycardia (Figures , ).
The cardiology team was informed and preparations were made for temporary pacemaker placement. Meanwhile, a sample was sent for venous blood gas analysis for quick assessment of electrolytes. The potassium was found to be elevated to 6.6 mmol/L. The patient was administered intravenous calcium gluconate and dextrose with insulin, and nebulization was done with salbutamol. Within seconds, sinus rhythm was restored, albeit with peaked T waves (Figure ).
One session of hemodialysis was done, and the clinical course was uneventful thereafter. Telmisartan was substituted with hydrochlorthiazide. The serum creatinine and potassium values are mentioned in Table . No abnormality was found on urinalysis and ultrasound of the kidneys.


A healthy 17-year-old male was admitted to the emergency room due to left hemiparesis, which was caused by a ball hitting the right side of his head during a soccer game. The symptoms occurred suddenly 10 min after the impact, and he visited the nearest hospital before referral to our hospital. According to the neurological examination at the first hospital, hypesthesia was observed in the left face and arm of the patient. Weakness of his left upper and lower extremity (Medical Research Council scale, MRC grade 1) was also identified (National Institutes of Health Stroke Scale, NIHSS 9). At 1 h and 30 min after the onset of the neurological symptoms, no hemorrhage was found on brain computed tomography (CT). Therefore, intravenous tissue plasminogen activator (t-PA) was immediately administered and the patient was transferred to our hospital.
When the patient was admitted, his vital signs were stable. Neurological examination revealed that the hypesthesia of his left face and arm was similar to the condition at the time of onset, but his left hemiparesis had improved to MRC grade 4 (NIHSS 3). A blood test showed that the D-dimer slightly increased to 1.05 mg/L, but the results of a complete blood count, liver function test, and electrolyte test were normal. Brain magnetic resonance (MR) susceptibility-weighted image (SWI) revealed many small veins draining to the central vein in the area from the right posterior putamen to the periventricular white matter (Fig. ). Although not prominent, there was an increase in signal intensity around the DVA seen on diffusion weighted image (DWI) (Fig. ). We diagnosed the patient with ischemic stroke associated with DVA and administered an antiplatelet drug from the next day.
The patient was further investigated for risk factors associated with young age-related stroke. There were normal levels of autoantibodies and coagulation factors including antiphospholipid antibodies. No abnormality was found in a transthoracic echocardiography. His left hemiparesis had worsened to MRC grade 3 from MRC grade 4 on the second day of hospitalization. Subsequent brain MR DWI showed that the high signal intensity became more prominent and broader in the area of the DVA (Fig. ). Afterward, the patient's symptoms began to improve gradually, and the left hemiparesis completely resolved 3 weeks after the symptom onset.


A 79-year-old African American male with a past medical history of hypertension presented with a static slurred speech that manifested with sudden onset two days prior to his hospital visit in late January of 2020. Following the slurred speech the next day he had chills, however, did not record his body temperature at home. He initially considered his symptoms as a minor flu, however, later decided to seek medical care due to nonresolving speech impairment. On presentation, he was febrile with an oral temperature of 100oF. His blood pressure was 124/74 mmHg. He endorsed compliance of his antihypertensive medication. He did not give a history of tobacco abuse, shortness of breath, cough, or intermittent palpitation. Physical examination showed an irregular pulse at the rate of 96 beats/min, respiratory rate at 18 breaths/min, and fine crackles in bilateral lower lungs on auscultation. His blood oxygen saturation level was 95%. Neurological examination was unremarkable except mild dysarthria. From his dysarthria assessment it was inferred that he had a low pitched phonation, impaired prosody for the sentence “ I am hungry”, lack of hypo- or hyper-nasality, decreased rate of repetitions (repeated the word “buttercup” only four times in five seconds), impaired articulation of the word “PresbyEpiscopal” and letters “P, B, F”, but with normal articulation of the words “la-la-la”. The initial National Institute of Health Stroke Scale (NIHSS) was one.
The real-time reverse transcription polymerase chain reaction (RT-PCR) analysis of SARS-CoV-2 infection tested positive from the nasopharyngeal swab. Pertinent initial laboratory findings showed a white blood cell count of 9.3 (4.5-11 K/μL), lymphocyte count of 0.6 (1.2-3.4 K/μL), and giant platelet morphology with normal count. He had mild elevated creatinine 2.4 (0.7-1.5 mg/dL) and procalcitonin levels 0.19 (0.02-0.10 ng/mL). His interleukin-6 92 (0-6.3 pg/mL), ferritin 505 (15-150 ng/L), and D-dimer 1201 (0-230 ng/mL) levels were abnormal. His cardiac troponin levels were normal, but the transaminase levels were elevated. The electrocardiogram showed an AF with a rapid ventricular response (Figure ).
The CHA2DS2-VASc score was four (calculated from his risk factors: age, hypertension, and stroke). A pulmonary CT scan showed bilateral peripheral ground glass opacities without pulmonary embolism (Figure ), and a brain MRI showed a punctate left frontal lobe infarction (Figure ).
A magnetic resonance angiogram of the head and neck showed no significant stenosis and transthoracic echocardiogram showed normal systolic function, moderate left atrial enlargement without thrombus or atrial septal defect (Figure ).
The patient was treated with IV unfractionized heparin infusion at the rate of 15 units/kg/hour and metoprolol tartrate 25 mg two times a day. The COVID-19-associated pneumonia was treated with oral azithromycin and hydroxychloroquine and was closely monitored in the ICU setting. Still, after a week, the patient got an increased respiratory distress as a consequence of a cytokine storm and required mechanical ventilation. The laboratory findings during this time showed highly abnormal interleukin-6 330 (0-6.3 pg/mL) level. The patient was treated with IV steroids and tocilizumab, but his condition further deteriorated, complicated by a multi-system organ failure, including septic shock, acute respiratory distress syndrome, acute myocardial injury (NT-proBNP 936 pg/mL), acute kidney injury, and dyselectrolemia. Despite aggressive supportive measures that included hemodialysis, the patient died two and half weeks after his admission.


A 28-year-old man with an uneventful past medical history admitted to our department for chest pain and dyspnoea that started two days ago. He had six pack-year smoking history. He was not complaining of any other sign. The physical examination was in favour of the diagnosis of a right-sided pleural effusion. Chest X-ray showed an opacity of the lower part of the right hemithorax (Fig. -a). The pleural puncture was performed showing a clear pleural fluid with high lymphocytosis at 75% and protein level at 56 g/L. The search for tuberculosis bacillus in the pleural fluid and in sputum was negative. No tumour cells were found in the pleural fluid. Fibre optic bronchoscopy revealed an extrinsic compression on the right lower lobe bronchus. Abrams pleural biopsy demonstrated a non-specific inflammation. Chest computed tomography (CT) scan showed a right-sided pleural effusion without evidence of pleural or parenchymal lesion (Fig. ). The patient underwent a videothoracoscopy. Intraoperative findings revealed an anterior mediastinal thin-walled cystic structure ruptured into the pleural cavity. The resection of the latter was performed. Anatomopathological examination demonstrated a mesothelial cyst (Fig. ). One year after the intervention, the patient is asymptomatic without any anomaly at the chest X-ray (Fig. ) or control CT scan.


A 15-year-old boy, of South African descent, is presented with a long-standing history of social and communicative challenges dating back to early childhood, including difficulties in appropriate use of facial expressions, eye contact, and gestures to regulate social interaction (see Fig. for patient’s timeline). He has a history of difficulty in establishing and maintaining relationships, although he has been able to establish some friendships. His mother notes a history of selective mutism dating back to age 3. He has areas of fixated interests and some ritualized behaviors that on assessment were below the threshold for a diagnosis of obsessive-compulsive disorder. In 2016, he was formally diagnosed as having ASD by a specialized organization in British Columbia (BC), the Interior Health Children’s Assessment Network (IHCAN), with supporting evidence from Autism Diagnostic Interview – Revised (ADI-R) and the Autism Diagnostic Observation Schedule 2 (ADOS-2). He does well academically and there are no cognitive concerns. Sometimes he shows aggressive behaviors towards his mother and other relatives.
He was diagnosed as having epilepsy characterized by focal seizures at age 7 at an emergency department service in BC and was subsequently treated by his pediatrician and a pediatric neurologist at the Alberta Children’s Hospital (ACH). He was initially prescribed carbamazepine for seizures which was stopped in 2015 due to side effects (upset stomach), followed by clobazam (stopped in 2016 due to suicidal ideation) and valproic acid (VPA) (stopped in 2017 due to alopecia, tremor, and reflux). The latter also caused a significant weight gain of approximately 13 kg in 1 year, resulting in a calculated body mass index (BMI) with the Du Bois method of 25.5 kg/m2. He is currently on lamotrigine for seizures, lorazepam for breakthrough seizures, melatonin for insomnia, riboflavin, ranitidine, magnesium, and orally administered CBE 0.2 mL (4 mg of CBD and 0.1 mg twice a day). No therapy had been tried for behavioral symptoms, although his mother mentioned that VPA and lamotrigine were also prescribed for their effect on mood.
He is currently in psychotherapy at the Child and Youth Mental Health (CYMH) clinic in BC for his selective mutism and anxiety disorder diagnosed by psychiatrists in the same province. He has also had sleep difficulties since 2016. His perinatal history is unremarkable. His birth followed a full-term pregnancy and was uncomplicated except for a required caesarean section due to macrosomia (> 4000 g) and macrocephaly (his mother does not remember the measurement), and subsequent hospitalization for neonatal jaundice. No genetic syndrome was suspected; no genetic testing was ever done. He met expected neurodevelopmental milestones for his age. His mother and grandmother have a history of depression and anxiety. There is other familial history of eating disorders and alcoholism, but no history of genetic syndromes.
In mid-2017, his parents consulted a medical cannabis physician from Caleo Health to assess the suitability of cannabis-based medicines as adjunctive or replacement therapy for seizures. At the time, a physical examination and laboratory findings were normal. A neurologic examination was unremarkable; mental status – awake, alert, cooperative; cranial nerves – normal; motor – normal tone, bulk, strength, and reflexes in upper and lower extremities, proximal and distal, deep tendon reflexes 2+ symmetric. A skin examination was unremarkable, there were no hypopigmented macules, café-au-lait macules, neurofibromas, or axillae ephelides. A long-term (48-hour) electroencephalogram done in 2016 did not record any epileptogenic potentials; magnetic resonance imaging (MRI) showed no intracranial abnormalities and a computed tomography (CT) scan of his head was normal (2015). Laboratory results done mid-2017 were normal: complete blood count and differential, vitamin B12, creatinine, sodium, potassium, calcium, magnesium, total bilirubin, albumin, alkaline phosphatase, aspartate aminotransferase, gamma-glutamyl transferase, alanine aminotransferase, and triacylglycerol lipase. He had low ferritin (11 μg/L) but normal hemoglobin (159 g/L), due to starting a vegetarian diet, which was followed up by the family physician.
He had not had a clinical seizure in 6 months (last seizure in March 2017). Medications at the time of initiation of CBE were: lamotrigine 200 mg twice a day for seizures, lorazepam 1 mg sublingual (SL)/buccal as necessary, infrequently used for seizures, melatonin 6 mg for sleep initiation, riboflavin 400 mg administered orally once daily, magnesium 1 tablet administered orally once daily, and ranitidine 150 mg twice a day. When asked for symptom severity on a visual analog scale (VAS) (0 = least severe, 10 = most severe), his mother reported overall anxiety, social anxiety, aggressiveness, and irritability severity, at 10/10, 10/10, 6/10, and 9/10, respectively. On VAS to assess for talkativeness (0 = quiet, 10 = very talkative) in social situations, the mother reported 0/10. On VAS for concentration (0 = unfocused, 10 = very focused), 4/10 was reported. In regards to sleep, the mother stated he was sleeping approximately 5 to 6 hours, and was having trouble falling asleep. After the initial assessment, his parents gave consent to start therapy and CBE was prescribed (60 mL bottle of 1:20 CBE – 0.001% THC and 0.02% CBD), from CanniMed, with an olive oil carrier. His parents were instructed to administer 0.1 mL twice a day (2 mg CBD and 0.1 mg THC) and increase by 0.1 mL (2 mg CBD and 0.1 mg THC) per dose if no effects were shown to a maximum of 0.5 mL (10 mg CBD and 0.5 mg THC) per dose.
In December 2017, after 3 months of the CBE prescription, his mother increased the dose to 0.2 mL twice a day (4 mg CBD and 0.2 mg THC) as the family noted only mild improvements in anxiety symptoms. In August 2018, a medical cannabis follow-up was conducted. At 0.2 mL twice a day for almost 9 months, our patient’s family reported an improvement of 7 points for overall and social anxiety and irritability, and 6 points on aggressiveness on their respective VAS. Talkativeness improved by 4 points and focus by 2 points. In February 2020, another medical cannabis follow-up was conducted and positive effects were still evident at the same dose. When the mother was asked to complete the Children’s Sleep Habits Questionnaire (CSHQ) Abbreviated, she stated that he slept 7 hours and only had trouble falling asleep in his own bed as he resists going to bed. No side effects were reported (nausea/vomiting, diarrhea, headaches, euphoria, feeling high, anxiety, panic attacks, palpitations, somnolence during the day or drowsiness). Laboratory results remained normal and low ferritin was corrected. He began to initiate and reciprocate conversations with acquaintances he had previously been unable to speak to (for example, doctors, community members). He became more motivated and energetic, starting his own vegetarian diet and exercise programs, ultimately losing 6.4 kg after starting CBE for a calculated BMI of 21.33 kg/m2. He was able to start his first part-time job helping customers and interacting with them. He was instructed to fill out the self-administered Adult AQ which resulted in a normal score of 10 as shown in Table . His mother stated he now also has a girlfriend. Recently, his mother started weaning him off CBE to go on a trip and noted an immediate change. He became more irritable and aggressive.
In discussion with their neurologist, the family decided to wean lamotrigine while remaining on CBE (0.2 mL twice a day – 4 mg CBD and 0.2 mg THC). Unfortunately, there was a recurrence of seizures and lamotrigine was titrated back to the full 200 mg twice a day dose.
Currently, our patient remains on the same medication as mentioned above, as well as low dose of CBE. He has maintained the positive effect on his behavioral symptoms, anxiety, sleep, and social deficits on CBE 1:20 ratio, 0.2 mL twice a day (4 mg CBD and 0.2 mg THC) and no side effects have been reported.


A 51-year-old man was referred to our hospital, with a dumbbell-shaped nodule measuring 40 mm in the right upper lobe of the lung (Fig. ). He was a current smoker and also had hypertension, diabetes mellitus, and bronchial asthma. Imaging findings suggested the possibility of a lung cancer, so transbronchial biopsy was performed. However, definitive diagnosis was not able to be obtained from the biopsy specimens. Bacterial culture of bronchial lavage fluid also yielded negative findings, including for tuberculosis. After eight months of observation, computed tomography showed that the tumour had slightly increased in size. Surgery was therefore planned to resect the tumour and reach a definitive diagnosis. Because of the size of the tumour, a lobectomy of the lung was scheduled with the patient's consent.
The patient was placed in the left lateral decubitus position under general anaesthesia. We then made a 1.2-cm skin incision for insertion of a flexible thoracoscope and endoscopic autosuturing devices in the seventh intercostal space along the midaxillary line. We used a surgical scalpel only for cutting the dermis, and then gently separated muscles with forceps to access the thoracic cavity. Next, we made a 1.2-cm skin incision for the main port in the fourth intercostal space along the anterior axillary line for endoscopic forceps and a vessel-sealing device. In addition, a 5.5-mm skin incision in the third intercostal space along midaxillary line and a 3.5-mm skin incision in the fifth intercostal space along the posterior axillary line were placed to achieve effective visual overview of the surgical site.
The surgical procedure was basically the same as for conventional MVATS (Video , Supporting Information). We completed a typical right upper lobectomy and lymph node dissection. The lobectomy took approximately 3 h and there was a small amount of bleeding.
To remove the specimen, we created a 3-cm longitudinal incision 1 cm below the xiphisternal joint. We subsequently incised the linea alba, and bluntly reached the right chest cavity using the index finger. The specimen was placed inside a tissue bag and removed through the subxiphoid incision (Video , Supporting Information). An intercostal nerve block with ropivacaine hydrochloride hydrate was performed at the end of the surgery.
Intravenous patient-controlled analgesia (fentanyl citrate) was used for 18 h post-operatively instead of epidural analgesia. Numerical rating pain scale (NRS) at rest has consistently been 1/10 since 1 h post-operatively. The patient followed an uneventful post-operative course. The chest drain was removed 18 h post-operatively. He was discharged without any wound complications (NRS, 0/10).
The pathological diagnosis was tuberculoma, and antibacterial drugs were administered. The patient has not complained of any neuralgia or paraesthesia (NRS, 0/10) at the time of writing, six months post-operatively (Fig. ).


A 62-year-old non-smoker male patient presented to our hospital with a 6 months’ history of progressively enlarging mass along his back. It was first noticed 2 years ago upon palpation, with time it has gradually increased in size and became cosmetically noticeable. No history of pain, fevers, weight loss, or trauma.
Physical exam revealed a 10 cm soft, immobile, painless mass along the right paravertebral with no overlying discoloration of the skin. Computed Tomography (CT) Scan () revealed a 10 × 10 cm cystic lesion along the paravertebral muscles of the back. Given the patient’s exposure to cattle and dogs as well as prolonged ingestion of raw food, the lesion was considered to be most likely hydatid disease of the back muscles. The decision was made to proceed with surgical excision by Dr. Walid Faraj and Dr. Mohamad J. Khalife both of whom are Hepatopancreaticobiliary surgeons with vast experience with hydatid disease. The patient was started on Albendazole 400 mg twice daily for one week prior to surgery. Intraoperatively, a vertical incision was made along the lesion. Dissection was done reaching the mass with care to avoid spillage of contents. The mass was then injected with scolicidal agent cetramide 1.5% to prevent dissemination. The mass surrounding attachments were lysed. The mass was dissected off its attachments to the underlying paravertebral muscles at its roots completely. A multilocular cyst showing outer adhesions was excised as one entity. On gross examination, the lesion looked irregular and contained beige yellowish creamy material (). The wall of the lesion was elastic in nature. The inner aspect of the lesion was focally beige and granular. On microscopic examination, the sections showed fibrous cystic wall adhering to the surrounding connective tissue. Furthermore, the inner aspect was covered by large clusters of macrophages having microvacuolated cystoplasm and mixed with few mononuclear leukocytes (). The mass was diagnosed as a pseudocyst formed around liquefied soft tissue, surrounded by fibrous capsule and histiocytic reaction.


A 36-year-old man was referred from a peripheral hospital with a three-week history of progressive dyspnoea, fatigue, productive cough, and right-sided pleuritic chest pain. He had a background history of chronic obstructive pulmonary disease secondary to alpha-1 antitrypsin deficiency (PiZZ genotype), which was discovered the previous year when he had presented with a spontaneous pneumothorax and was found to have significant basal panacinar emphysema given his age. Despite the diagnosis, he never followed up with a respiratory physician and continued smoking. He had also managed to obtain access to a large amount of corticosteroids and had been taking 50 mg of prednisolone daily (1 mg/kg in the context of the patient weighing 48 kg) whilst continuing to smoke for a total of six months prior to his presentation.
His initial investigations revealed a white blood cell count of 18.5 × 109/L with neutrophilia (17.8 × 109/L) and lymphopaenia (0.5 × 109/L). C-reactive protein (CRP) was markedly elevated at 516 mg/L (reference range < 5 mg/L). His chest X-ray showed hyperinflated lungs with patchy consolidation in the right middle zone and perihilar region (Fig. ). Initially, he received empirical antibiotics for a severe community-acquired pneumonia (ceftriaxone and azithromycin) and stress-dose steroids. Preliminary sputum samples were sent for microscopy and culture.
Despite therapy, the patient continued to deteriorate over the next few days with worsening respiratory failure and increased consolidation on serial chest X-rays now also involving the right lower zone of his lungs. He was intubated, and on the same day his initial sputum sample was reported to have grown Gram-positive branching rods resembling Nocardia on Gram stain and modified Ziehl–Neelsen (Kinyoun) stain (Fig. ). The same organism was grown on multiple subsequent sputum samples and identified presumptively as Nocardia cyriacigeorgica by Bruker Biotyper Matrix-assisted laser desorption/ionization-time of flight (MALDI-TOF) instrument, MA, USA. It was then referred to a separate microbiology centre and identification was confirmed by polymerase chain reaction (PCR) and DNA sequencing of the SecA1 and 16S rRNA genes. A computed tomography (CT) of the thorax was done which showed a right lower lobe necrotizing pneumonia (Fig. ). Further CTs were done of his brain, abdomen, and pelvis which did not show any disseminated disease. He was continued on ceftriaxone 2 g daily and oral sulfamethoxazole/trimethoprim 800/160 mg twice daily, and one dose of intravenous amikacin 1 g was added to his antibiotic regimen. Ceftriaxone was subsequently changed to cilastin/imipenem 500/500 mg six-hourly after initially failing to improve.
After four days of ventilation, he was successfully extubated and slowly weaned off supplemental oxygen and stepped down from intensive care. His steroid dose was weaned down and his antibiotic regimen has been changed to a course of per oral (PO) sulfamethoxazole/trimethoprim 800/160 mg twice daily and intravenous ceftriaxone 2 g for four weeks followed by PO sulfamethoxazole/trimethoprim for another six months as eradication therapy. He has since been discharged back to a peripheral hospital for rehabilitation.


A 38-year-old female presented with a three-month history of progressive low back pain radiating to bilateral lower extremities with paraparesis and radiating shock-like pain with Valsalva. In addition, she reported gait instability, falling episodes, and bilateral foot drop (left worse than right) with muscle laxity. She denied any bowel or bladder incontinence. Physical exam demonstrated gait instability, impaired toe walking, impaired heel walking, impaired tandem gait, and an antalgic gait. The bilateral upper extremity strength was 5/5. Lower extremity exam demonstrated 4/5 psoas strength bilaterally, 3/5 quadriceps strength bilaterally, 2/5 (left), and 1/5 (right) tibialis anterior strength, 2/5 (left) 1/5 (right) extensor hallucis longus, 3/5 gastrocnemius strength bilaterally. All upper extremity deep tendon reflexes were 2/4 bilaterally. Patellar and ankle reflexes were 4/4 bilaterally. There were no changes in sensation among upper and lower extremities bilaterally. Babinski and Hoffman signs were absent. Finger to nose coordination was unremarkable.
Magnetic resonance imaging (MRI) demonstrated a large intradural intramedullary mass extending from T10-L1 (Figure ). The lesion was approximately 1.7 x 2.0 x 7.6 cm in size. Cranial imaging was negative for any lesions.
The patient underwent surgical resection, by which baseline motor evoked potentials (MEP) were recorded. Baseline MEPs were abnormal, consistent with the preoperative presence of paraparesis on admission. A vertical midline incision was made overlying the thoracolumbar junction, and a complete laminectomy from T10-L1 was performed. The spinal cord was abnormal, demonstrating an enlarged component, consistent with an intramedullary tumor. The surface of the spinal cord appeared discolored, and a midline myelotomy was performed. A large, firm, rubbery, pale mass that encompassed the overwhelming majority of the spinal cord was encountered, internally debunked, and sent for frozen pathology, which confirmed a World Health Organization (WHO) grade II diffuse astrocytoma. The tumor demonstrated extensive spread, and several areas were encountered that did not demonstrate a definable plane. The myelotomy was extended from the bottom of T10 down to L1. Serial MEPs were performed throughout the bulking process, which eventually indicated the loss of distal right lower extremity MEP and possible left lower extremity MEP loss. Given the clear intraoperative findings that this invasive tumor was without clear margins, and the evidence of MEP change, the surgical resection remained as a subtotal resection. The wound was then irrigated with copious amounts of antibiotic irrigation and hemostasis obtained. The dura was then closed in a watertight fashion and copiously irrigated with 3 liters of pulsatile antibiotic irrigation. During the closure of the case, there was an improvement in the left lower extremity MEPs and a questionable slight improvement in the right distal lower extremity MEPs. Cerebrospinal fluid (CSF) cytology was negative.
Following surgical resection, physical exam demonstrated 5/5 upper extremity muscle strength bilaterally. Lower extremity physical exam demonstrated 5/5 (left) and 4/5 (right) psoas strength, 5/5 (left) 3/5 (right) quadriceps strength, 0/5 dorsiflexion strength bilaterally, 1/5 (left) and 0/5 (right) extensor hallucis longus strength, and 0/5 (left) and 4/5 (right) gastrocnemius muscle strength. New-onset paresthesia was reported from L4 to distal right lower extremity, as well as from L1 to distal left lower extremity.
Due to social considerations, the patient began a seven-week course of chemoradiation two months postoperatively. This included six cycles of adjuvant temozolomide. Over the next two months, the patient reported progressive lower extremity weakness, dizziness, fatigue, poor appetite, and depressed mood. Repeat MRI of the spine revealed a new expansile cord lesion with heterogeneous T2 signal within the thoracic spinal cord spanning from T1 to T5 (Figure ). She was subsequently admitted and began intravenous dexamethasone. She also underwent brain MRI that revealed non-enhancing T2 Fluid-attenuated inversion recovery (FLAIR) hyperintense mass located in the mesial right temporal lobe, with diffuse involvement of the hippocampus, measuring 1.6 x 5.1 x 2.4 cm, with diffuse subependymoma involvement and compression of the temporal horn (Figure ). In addition, another area of non-enhancing FLAIR hyperintense signal located in the vermis of the cerebellum measuring 2.6 x 2.9 x 2 cm with compression of the anterior aspect of the fourth ventricle without evidence of hydrocephalus.
A stereotactic needle biopsy of the right temporal lobe was performed, with subsequent histopathology demonstrating WHO grade III anaplastic astrocytoma without microvascular proliferation or necrosis (Figure ). Moderately cellular astrocytic proliferation with two to three mitotic figures detected on hematoxylin and eosin stain was demonstrated. Immunohistochemical studies were positive for glial fibrillary acidic protein (GFAP) and oligodendrocyte transcription factor 2 (OLIG2), with P53 demonstrating <20% labeling. IDH-1R132H labeling was equivocal, and ATRX expression was retained. Phosphohistone H3 (PHH3) demonstrated mildly increased mitotic figures. Ki67 labeling index was 5%. H3K27M mutation was noted.
The patient survived for only eight months post-diagnosis.
Literature review
The PubMed database and all major neurosurgery journals were searched during June of 2020 using the keywords “glioma”, “astrocytoma”, "glioblastoma", “spine”, and “cerebral extension”, alone or in combination to obtain articles fitting the inclusion and exclusion criteria. The inclusion criteria were high-grade astrocytomas or glioblastomas involving the spine with secondary brain parenchyma metastasis.
To date, 96 cases of primary spinal gliomas with secondary intraparenchymal manifestation have been reported since 1908, including this case (Table ). These lesions occurred in patients ranging from six months to 65 years old, with the majority presenting under the age of 40 years (Mean: 20, 95% CI: 17-23.28 months). Among such reported cases, the long-term survival and outcome remain poor with a mean average survival of 24 months from diagnosis (95% CI: 13.07-33.99 months). There appears to be a slight predilection for this subtype among men over females (1.46:1).
The most common location for tumor involvement was the thoracic spine, which was in agreement in a previous survey published by Linsenmann et al. []. Varying levels of resections were attempted (both within the spine and brain), demonstrating no remarkable differences in outcomes.


A 71-year-old woman underwent SSPPD for an adenocarcinoma of the ampulla of Vater, classified as stage IIB (pT1bN1M0) according to the 8th edition of the Union For International Cancer Control Tumor–Node–Metastasis classification. She experienced postoperative pancreatic fistula (PF) (Clavien–Dindo grade IIIa) [], treated with drainage and antibiotics, and was discharged on day 21, postoperatively. At that time, laboratory data demonstrated that albumin and lymphocyte were 4.0 g/dL and 2050/µL, respectively, not suggesting malnutrition. After that, she had less oral intake than preoperative intake in addition to fasting for a month in total due to recurrent cholangitis. An adjuvant chemotherapy including gemcitabine and cisplatin were administered for 6 months. Post-chemotherapy, she was on regular follow-up every 6 months, without evidence of recurrence. She was treated twice with antibiotics for cholangitis at 12 and 31 months, respectively, post-surgery. She had lost 8 kg compared to preoperative body weight at the first cholangitis, whereas hypoalbuminemia (3.0 g/dL) and lymphopenia (820/µL) were present at the second cholangitis. She presented with vomiting 33 months after surgery. Additionally, her family complained of her disorientation. She was non-alcoholic, and had been able to eat until just before the onset of the symptom, except for the period of PF and cholangitis. On neurological examination, no specific findings were noted, including mental status despite her family complaint. She had not gained weight, since the onset of cholangitis. Laboratory data revealed hyponatremia (133 mEq/L), hypoalbuminemia (3.2 g/dL), and lymphopenia (810/µL), suggesting mild malnutrition. However, computed tomography (CT) and magnetic resonance imaging (MRI) of the head confirmed no specific findings. Although a neurologist and a psychiatrist examined closely, the exact cause of these syndromes remained unknown. The psychiatrist measured thiamine concentration to examine the cause of disorientation, whereas the laboratory measurement required 6–8 days. Thus, she underwent the treatment for hyponatremia until the results of thiamine value came. After 6 days, her level of consciousness worsened (Glasgow Coma Scale score, 3/15) []. Her comatose state made it difficult to evaluate other neurological findings. MRI of the head showed symmetrically abnormal hyperintense signal on fluid-attenuated inversion-recovery (FLAIR) and diffusion weighted image (DWI) in the frontal horn of lateral ventricle, the medial thalami bordering the third ventricle, the mammillary bodies, the periaqueductal region of the midbrain, and the bottom of the 4th ventricle (Fig. ). As the MRI findings suggested WE, administration of thiamine (150 mg daily, intravenously) was immediately initiated. After 2 days, her serum thiamine level was 6 µg/mL (reference range, 24–66 µg/mL). Accordingly, she was diagnosed with WE. Shortly after initiation of the treatment, blood thiamine value reached 246 µg/mL, with significant improvement of her confusional state. After a week of intravenous therapy, oral administration of thiamine 100 mg daily was started. However, as short-term memory loss and ataxia remained, 3 weeks after starting the therapy, she was transferred to a rehabilitation hospital with continuation of thiamine administration. After 3 months, the memory impairment continued although walking with support was restored.


A 68-year-old male with a background of rheumatoid arthritis (RA) presented to the Emergency Department (ED) in August 2017 with a six-week history of pain, erythema, and swelling over the dorsum of his right hand and wrist, with subjective fever and chills. The patient initially sustained small abrasions to the right dorsal hand, volar aspect of the index finger, and lateral aspect of the fifth metacarpophalangeal joint from fish spines while ocean fishing, with further injuries from a tree branch while gardening the next day. These sites became painful with necrotic foci and purulent discharge, and erythema and swelling began spreading to the rest of the hand and wrist over subsequent days. Ultrasound of the right wrist via the patient's local doctor showed features consistent with cellulitis and generalised tenosynovitis of the flexor tendons and the extensor compartment. However, multiple courses of oral antibiotics (erythromycin, cephalexin, and Flucloxacillin, in turn) did not provide any clinical improvement. The patient had previously been well, with his RA, diagnosed in 2015, well-controlled on weekly methotrexate (10 mg) and etanercept injections (50 mg) and daily prednisolone (7.5 mg) and hydrochloroquine (200 mg).
On examination, the patient had necrotic lesions over the sites of injury and extensive oedema over the right dorsum of the hand and wrist. He had negative Kanavel's signs for flexor tenosynovitis and nonirritable carpal, metacarpal, proximal, and distal interphalangeal joints. His inflammatory markers were elevated (CRP 80 mg/L, ESR 80 mm/hr, WCC 12.6 × 109/L, and neutrophils 10.69 × 109/L), but he was afebrile and had negative blood cultures. Furthermore, MRI of his right hand showed extensive synovitis and flexor group enhancement, but follow-up labelled white cell scan was negative for deep infection or osteomyelitis. Excisional biopsies of his necrotic lesions did not identify any organisms. The patient was given intravenous (IV) Flucloxacillin and oral doxycycline; however, there was no clinical improvement despite seven days of treatment in total since initial clinical presentation. An atypical organism as the underlying aetiology was suspected given the patient's immunocompromised state and exposure to commercially available fertiliser product, and so, the patient's RA medications were ceased, and right wrist synovectomy with biopsy was organised to identify the organism.
Open synovectomy of the right wrist with biopsy was done in January 2018, which showed extensive arthritis of the distal radioulnar joint without any evidence of organisms on microbiology, culture, and sensitivities (MCS); myriad samples were taken intraoperatively. Follow-up X-ray and MRI of the right hand in April 2018 showed changes favouring acute or chronic rheumatoid arthritis, without joint effusion or drainable collections, while the patient continued to have pain and swelling over the dorsal and increasingly over the volar wrist with associated reduced range of motion (). Diagnosis of an acute rheumatoid arthritis “flare” was established given the recently ceased RA medications and negative microbiology results, and so, the patient was booked for a right wrist fusion with iliac crest graft for definitive management of his pain.
However, when the patient presented for the procedure in November 2018, a fluctuant collection under the previous synovectomy scar was noted. Upon incision, purulent material was discharged from the joint space, with extensive synovitis and destroyed distal radioulnar joint noted in the operation report. Surgical debridement and irrigation were performed instead, with multiple tissue and synovium samples sent for microbiology, culture, and sensitivities (MCS).
Microbiology was positive for Enterobacter cloacae and Lomentospora prolificans. The identified E. cloacae was resistant to co-trimoxazole and sensitive to gentamicin, ciprofloxacin, and Meropenem. Minimum inhibitory concentration (MIC) for L. prolificans was performed via the broth dilution method at a specialized mycology unit interstate, which showed MIC of >8 mg/L to Amphotericin, >256 mg/L to fluconazole, >16 mg/L to itraconazole, and >8 mg/L to voriconazole. During this clinical presentation, the patient's CRP was 14 mg/L and WCC 8.0 × 109/L. Following surgery, the patient was started on IV Meropenem (1 g TDS) with oral voriconazole (250 mg BD) and Terbinafine (250 mg BD), as per the recommendations from the infectious disease (ID) team. The trough voriconazole level was monitored weekly to ensure that the given dose was within the therapeutic range for the patient.
Surgical debridement and irrigation were repeated every three to five days to regain source control. There was extensive damage from the disease process requiring extensor tendon synovectomy and eventual proximal and distal row carpectomy. The skin overlying the wrist had necrotic, sloughed edges that required progressive debridement, resulting in a large dorsal wrist wound with exposed, residual extensor tendons (). This was deemed a nongraftable surface by the plastic surgical team until the infection was resolved, despite the prolonged time course. Due to the extent of bony and soft tissue damage and the possibility of upper limb amputation at a suitable level, should the infection not be controlled locally, advice was sought from local orthopaedic colleagues regarding the use of antifungal-impregnated cement beads as an adjunctive therapy. Voriconazole was recommended as the agent of choice to be incorporated into the drug-eluting cement beads fashioned intraoperatively.
Seven Palacos (nonbiodegradable) cement beads impregnated with 600 mg of voriconazole were inserted in December 2018 during proximal row carpectomy, washout, and debridement (). A total of nine sessions of joint irrigation and debridement were performed from November 2018 to January 2019. The Palacos beads were in situ for a total of three weeks before they were removed at one of the above sessions. Samples taken since the insertion of voriconazole beads were consistently negative for L. prolificans on microbiology, and the right wrist showed clinical improvement, eventually to complete resolution of pain with no ongoing signs of infection. External fixation was applied to the now flail right wrist in January 2019 to achieve skeletal stability, and the patient was discharged on oral voriconazole and Terbinafine (Figures and ).
The patient remained well in subsequent outpatient reviews and was eventually deemed fit for right wrist fusion with iliac crest bone graft and internal fixation, which was completed in July 2019. Voriconazole-impregnated Stimulan (biodegradable) was used throughout the deep dead space during the procedure. The plastics team performed a myocutaneous flap to close the dorsal wrist wound. At four months post right wrist fusion, the patient remained clinically well and pain-free and had regained some function in relation to the use of the right hand (). Follow-up imaging showed a stable right wrist post fusion with no evidence of collection or hardware failure. The patient is currently on voriconazole (200 mg BD) and Terbinafine (250 mg BD), with regular trough voriconazole levels monitored by the ID team; he is to complete at least 12 months of oral voriconazole in total, before cessation of treatment. It is not believed that the patient will need life-long suppressive treatment with either antibiotics or antifungal agents at this stage.


Patient A, a 28-year-old, otherwise-healthy, male recreational regular-footed (right foot forward) wakeboarder made a sit-down start from a starting ramp in a cable park. When he did so, the tip of his wakeboard sank in the water, and the board rotated, along with both of his attached lower limbs. This resulted in a violent twisting force to his left pelvis and thigh. He collapsed into the water, and simultaneously felt a sharp pain in his left thigh. He was rescued from the water immediately by the staff of the cable park and attended to by paramedics at the hospital around 20 minutes after the accident. Upon arrival in the hospital, a spiral femur shaft fracture (AO classification: 32-A1.2) was confirmed on radiographs (); no motor or sensory disabilities were identified. There was swelling and crepitation in the mid-third of the left thigh. After a short preoperative preparation, anterograde reamed locked nailing was performed using a rigid locked nail while the patient was under spinal anaesthesia (). He had no postoperative complications and recovered well through a physical therapy program. At 6 months follow-up, the patient had full, painless ROM, with a little muscle atrophy in the injured thigh. He returned to wakeboarding 155 days after injury.


Patient B, a 28-year-old, otherwise healthy, female, advanced, goofy-footed (left foot forward) wakeboarder tried to perform a wake jump. As she came back down upon the surface of the water, she fell on her right leg. No solid object, such as a tree branch or rock, was reported to have been in the water. Similar to patient A, we assume that during the landing, the tip of the wakeboard submerged into the water and was immediately followed by a rotation of the board, causing a twisting force on her right pelvis and thigh. Moreover, as the athlete was prepared for high forces, she contracted all muscles, particularly, of the knee and ankle, for a maximum of stability. When she arrived in the hospital, she presented with swelling and crepitation in the second third of the right femur. A wedge, bending, femoral shaft fracture (AO classification: 32-B2.2) was diagnosed after X-ray examination. Similar to patient A, after a short preoperative preparation, an anterograde reamed locked nailing was performed under a rigid lock nail in spinal aesthesia (). No postoperative problems could be observed, and she recovered well and quick through a physical therapy program.
It is likely that in both cases, similar dynamics caused this type of injury. Typically, the forward foot is slightly more bent in order to lift the tip of the board out of the water. Once the tip sinks in the water, the board submerges, and the maximum forces applied to the back foot (standing foot) which is nearly fully extended. Hereby, the highest stress is forwarded onto the femoral shaft, leading to an isolated fracture ().


A 16-year-old male Chinese patient was admitted to our hospital due to a 4-year history of recurrent upper and lower extremity pain with low-grade fever up to 37.5°C, as well as a 2-year history of chest distress and dyspnea after activity. He complained of an aggravation of chest distress and dyspnea 3 months before admission. No other medical history was reported. Physical examination revealed sporadic angiokeratomas on his waist and back (Fig. C). His blood pressure was normal when he had no extremity pain and could rise to approximately 160/110 mm Hg, with a heart rate of 110 bpm, when the pain attacked. Twelve-lead electrocardiogram showed QT prolongation (QTc = 501 ms) and intraventricular conduction block (QRS = 152 ms) (Fig. A and B). Transthoracic echocardiography (TTE) revealed dilated left and right ventricles, mild mitral and tricuspid valve insufficiency, and an ejection fraction of 61%, with no evidence of myocardial hypertrophy (Table , Fig. D). Cardiovascular magnetic resonance (CMR) confirmed that the ventricles were dilated (Fig. F and G, Table ). No significant abnormalities were found in first-pass perfusion or late gadolinium enhancement (LGE) CMR. Native T1 mapping of the left ventricle is shown in Fig. E. The mean T1 was 1262 ms. Routine blood, urine, and stool tests were all unremarkable, as were hepatic and renal function, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), joint X-ray and magnetic resonance imaging (MRI), 24-hour urine catecholamine assay, 99mTc-octreotide scan, and ophthalmologic examination. The patient was negative for human leukocyte antigen-B27 (HLA-B27). Leukocyte α-galactosidase A activity was 0.1 nmol/hour/mg protein, which is below the normal range of 29.0 to 64.4 nmol/hour/mg protein. Polymerase chain reaction amplification and Sanger sequencing of the GLA exons from genomic DNA of the patient revealed an insertion of 1 nucleotide between positions 76 and 77 in the cDNA (c.76_77insT; Fig. H). The present report is the first to describe this novel mutation, which can generate a premature termination codon at amino acid position 30. The patients mother was heterozygous for the mutation, as shown in Figure I.
The patient was treated using a non-steroidal anti-inflammatory drug (loxoprofen sodium 60 mg q8 hour) after admission, but his extremity pain did not improve. After the definitive diagnosis of FD, his medication was changed to tramadol (100 mg prn), metoprolol (23.75 mg qd), and angiotensin-converting enzyme inhibitor (fosinopril sodium 5 mg qd). Metoprolol and fosinopril sodium were used to delay ventricular remodeling and the possible future decline of renal function. The patient refused enzyme replacement therapy for financial reasons. The tramadol treatment only partially relieved the patients extremity pain, and was discontinued 2 weeks later. The patient was followed up for 29 months. Twenty months after his discharge from the hospital, echocardiography, electrocardiography, renal function, and routine blood and urine tests showed no significant changes. Starting approximately 24 months after his discharge, the patient reported a slow and gradual decrease in the frequency and degree of extremity pain and chest distress, which corroborated previous findings that pain tends to diminish with age.[