Παρασκευή 12 Οκτωβρίου 2018

Sleeping beauty syndrome presenting with insomnia

A young man previously diagnosed with Kleine-Levin syndrome (KLS) presented with abnormal behaviour over the last 8 days. This included decreased sleeping hours and appetite, hypersexuality, aggressiveness and visual hallucinations. All blood tests and investigations in the emergency department yielded normal results. A preliminary diagnosis of a KLS episode with psychosis was made and the patient was started on a regimen of aripiprazole 10 mg once daily along with lorazepam 2 mg intravenously in two divided doses in the event of agitation or insomnia. On discharge 5 days later, the patient had returned to his premorbid level of functioning and was willing to follow up in the neurology clinic. He was discharged on aripiprazole 10 mg once daily and lorazepam 2 mg two times daily as needed for 2 weeks to help with his agitation and insomnia, as well as lithium carbonate 400 mg at night.



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Takotsubo syndrome with severe bradycardia initiated by seizure: Is the implantation of a permanent pacemaker necessary?

Although arrhythmias are frequent in patients with Takotsubo syndrome (TTS), data on sick sinus syndrome remain elusive. Here, we report a case of TTS initiated by a seizure as a physical trigger that led to sinus arrest. The patient presented with cardiogenic shock and bradycardia which required intensive cardiovascular care. However, in the subacute phase of TTS, the sinus function recovered significantly, and pacemaker implantation was deferred.



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Living donor renal transplant in a patient with end-stage renal disease due to Hermansky-Pudlak syndrome

Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder characterised by oculocutaneous albinism, bleeding diathesis and end-stage renal disease (ESRD), due to interstitial deposition of ceroid lipofuscin. Renal transplantation is potentially a definitive treatment option for patients with ESRD due to HPS. Herein, we describe the case of a 55-year-old male patient with HPS that successfully underwent a living donor kidney transplant. We also emphasise the importance of multidisciplinary input during the preoperative, perioperative and postoperative phases in this high-risk clinical scenario.



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Living donor liver transplantation and situs inversus totalis: cutting the Gordian knot

Liver transplantation for biliary atresia splenic malformation syndrome associated with situs inversus totalis is a challenging task due to the complexity of associated malformations and the technical proficiency required to overcome them. We present the case of a 6-month-old infant who underwent liver transplantation for biliary atresia. A reduced left lateral segment liver graft from a live donor (his mother) was implanted. The postoperative period was uneventful, and the child remained well on follow-up. Thus, such rare congenital anomalies no longer prove to be a deterrent for successful liver transplantation.



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Unusual case of unilateral conductive hearing loss: chronic lymphocytic leukaemia

This presentation reports a novel case of chronic lymphocytic leukaemia (CLL), presenting with an early cutaneous lesion within the external auditory canal, in a patient being assessed for conductive hearing loss. It has previously been reported that infiltrative CLL can involve the head and neck; however, isolated external ear canal involvement is rare. Given that the incidence of CLL in Australia is rising, this case highlights the importance of considering CLL as a differential diagnosis for presentations of unilateral conductive hearing loss.



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Corneal ulcer as the presenting feature of type 2 diabetes mellitus

A 60-year-old woman, with no significant medical or ophthalmic history, presented with a unilateral large corneal ulcer and hypopyon. Despite a severely injected conjunctiva and large corneal epithelial defect, the patient denied any discomfort in the eye. The ulcer was extremely slow to heal requiring prolonged treatment with topical and systemic broad-spectrum antibiotics. Due to the corneal opacification, fundal examination was not possible. Subsequent examination of the fellow eye revealed an appearance consistent with proliferative diabetic retinopathy. She was given treatment with panretinal laser photocoagulation. There was no history of diabetes and the patient denied experiencing any of the classical symptoms of hyperglycaemia. With blood tests revealing a raised plasma glucose and Haemoglobin A1c (HbA1c), a new diagnosis of diabetes mellitus was made. With the commencement of treatment for diabetes, there were signs of improvement in the corneal appearance.



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Mesenteric panniculitis: diagnostic precision

A previously healthy 74-year-old woman was admitted with vespertine fever, tremors, shivers and loss of appetite within the previous month. Blood tests revealed an elevated C reactive protein serum level. Serologies for infection were negative. Blood cultures grew no organisms. Colonoscopy revealed normal findings. CT showed typical findings of mesenteric panniculitis with infiltration of mesenteric fat that was circumscribed by hyperattenuating capsule and contained enlarged homogenous lymph nodes. The histopathological analysis from mesenterium revealed non-specific signs of chronic inflammation. On institution of prednisolone, the clinical symptoms subsided, and we replaced it with azathioprine after 1 month. After 12 months of therapy, the patient remained asymptomatic, normalised the serological inflammatory markers and repeat CT revealed normal mesenteric fat.



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Bilateral congenital eventration of diaphragm: keep in mind, the other side

Eventration of diaphragm is an uncommon disorder in which diaphragmatic muscle is replaced by fibroelastic tissue, either partially or completely. Bilateral eventration is even rarer. We present a case of bilateral eventration of diaphragm in newborn with a fibroelastic sac on left side and diaphragmatic eventration with good muscular lips on right side. The right-sided diaphragmatic eventration was not evident initially, but manifested after surgical repair of the left-sided eventration.



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Cerebrospinal fluid hydrocele caused by scrotal migration of a ventriculoperitoneal shunt

A 6-month-old boy presented with painless right hemiscrotal swelling. The scrotal ultrasound revealed a cerebrospinal fluid hydrocele caused by the migration of a ventriculoperitoneal shunt (VPS) catheter tip into the right hemiscrotum and associated undescended left testis. Earlier, he underwent a VPS placement for hydrocephalus secondary to neonatal bacterial meningitis and ventriculitis. The patient was treated with bilateral herniotomy, left-sided orchidopexy and repositioning of VPS into the peritoneal cavity.



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Methicillin-sensible Staphylococcus aureus causing endocarditis, with cerebral and orthopaedic complications, in a 17-month-old child with no risk factor

We report a case of Staphylococcus aureus endocarditis, with large vegetation, in a 17-month-old male infant, complicated with meningitis, ischaemic strokes and osteoarthritis leading to haemorrhagic stroke by aneurysm rupture. He did not present any risk factor for endocarditis. The final course was favourable through, after valve replacement. The strain was sensible to methicillin and belongs to complex clonal 398, with accessory gene regulator I. We did not found immunodeficiency.



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Unilateral localized extraocular muscle metastasis by lobular breast carcinoma

Here is a case of an isolated orbital metastasis in a previously diagnosed metastatic breast cancer of a 46-year-old woman presenting diplopia and pain in her left eye. The magnetic resonance showed a suspected metastatic localisation in the upper left oblique muscle, which was confirmed by the fine-needle aspiration biopsy. The systemic therapy with liposomal doxorubicin as well as local stereotactic radiotherapy showed a gradual improvement of the local symptoms and signs.



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A snotty nose: more than just a cold

Description 

A healthy 3-year-old girl presented with a 5-day history of right-sided cheek and eye swelling and pain, associated with right-sided green nasal discharge. Examination revealed diffuse erythematous swelling around the right cheek, jawline and eye. Oral antibiotics for suspected periorbital cellulitis were ineffective. Blood inflammatory markers and ultrasound of her neck and parotid glands were normal. She was discharged as having viral parotitis. Antibiotics and nasal drops were later restarted for clinical sinusitis; however, she showed no improvement, now having 3 weeks of right-sided facial swelling with thick green and intermittently bloody nasal discharge. Further history revealed possible squint, mastication pain and breathing difficulty through the right nostril.

Cranial imaging (figure 1) demonstrated a large central but predominantly right-sided nasopharyngeal tumour, extending into the ethmoid sinuses and orbits (with medial recti distortion), infratemporal fossa and pterygoid muscles and anterior and middle cranial fossae. There was...



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Disseminated nocardiosis with infective endocarditis of a transplanted heart

Nocardiosis is caused by various species of Nocardia and typically occurs as an opportunistic infection. It frequently disseminates, most often involving the lungs, subcutaneous tissues and central nervous system. It has rarely been reported to affect native heart valves. We report the case of a 64-year-old man with disseminated nocardiosis involving the brain, lungs, muscle and tricuspid valve of a transplanted heart. Following antimicrobial therapy, the patient improved clinically and there was no evidence of residual infection on follow-up imaging. This case highlights the presentation of nocardiosis, current therapeutic guidelines and the question of prophylaxis against Nocardia in immunocompromised patients.



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Toxic epidermal necrolysis: the red eye and red herrings in casualty

A 38-year-old woman presented to casualty with bilateral red eyes associated with a recent upper respiratory tract infection. This was initially diagnosed as conjunctivitis, however systemic review revealed an erythematous facial and skin rash, mildly swollen lips and mild swallowing difficulties. The patient was referred for an urgent medical assessment, by which time she was found to have erythema affecting 54% of her body surface area and diagnosed with suspected toxic epidermal necrolysis (TEN). She rapidly deteriorated over 24 hours with a spreading blistering skin rash and airway compromise requiring urgent intubation and admission to the intensive treatment unit (ITU). Subsequent skin biopsies confirmed the diagnosis of TEN, attributed to recent use of ibuprofen. Treatment included broad-spectrum antibiotics and high-dose corticosteroids. The patient had a prolonged hospital stay and developed severe scarring of the ocular surface. She was discharged home and remains under continuing outpatient follow-up with ophthalmology and dermatology teams.



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Neonate with urinary ascites but no hydronephrosis: unusual presentation of posterior urethral valves

Posterior urethral valves (PUV) are an important cause of paediatric obstructive uropathy. PUV are usually diagnosed by prenatal ultrasonography (US) revealing hydronephrosis and bladder distention. We describe a 17-day-old male infant with abdominal distention who had no hydronephrosis on prenatal US. Laboratory investigations showed serum creatinine of 12 mg/dL, hyperkalaemia and metabolic acidosis. Abdominal US showed large amount of ascites, normal-sized kidneys without hydronephrosis and incompletely distended bladder. Paracentesis revealed clear, yellow ascitic fluid with creatinine level of 27 mg/dL compatible with urinary ascites. Voiding cystourethrogram (VCUG) demonstrated PUV with a dilated posterior urethra, grade 5 right vesicoureteral reflux and a ruptured kidney fornix with peritoneal extravasation of contrast. Foley decompression resulted in normalisation of creatinine within 72 hours. Transurethral resection of PUV was performed, and a repeat VCUG showed recovery of forniceal rupture. This case illustrates an unusual presentation of a potentially life-threatening but treatable cause of urinary tract obstruction.



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Acute pancreatitis-induced takotsubo cardiomyopathy and cardiogenic shock treated with a percutaneous left ventricular assist device

A 63-year-old man was admitted for severe acute pancreatitis. On day 3 of hospitalisation, he developed shortness of breath and acute pulmonary oedema. Echocardiogram revealed global hypokinesis with a left ventricular ejection fraction of 20%, and he was diagnosed with takotsubo cardiomyopathy. He developed cardiogenic shock which was treated successfully with a percutaneous left ventricular assist device. His left ventricular ejection fraction improved by hospital follow-up 3 weeks later.



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Secondary bacterial peritonitis and pelvic abscess due to Clostridium difficile

A 70-year-old man with a history of hepatic cirrhosis presented with abdominal discomfort and distention. Physical examination revealed abdominal distention, positive fluid wave and abdominal tenderness. Due to concerns for spontaneous bacterial peritonitis (SBP), paracentesis was performed. Fluid analysis revealed 5371 total nucleated cells with 48% neutrophils. Ceftriaxone was then initiated for the treatment of SBP. Bacterial cultures of the fluid, however, grew Clostridium difficile. Therefore, metronidazole was added. An abdominal ultrasound revealed a pelvic fluid collection that was suspicious for an abscess on an abdominal CT scan. The patient underwent CT-guided drain placement into the pelvic fluid collection. The fluid aspirate was consistent with an abscess. However, cultures were negative in the setting of ongoing antibiotic therapy. The patient was treated with a 10-day course of ceftriaxone and metronidazole and was discharged home with outpatient follow-up.



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Stage III Kienböcks disease treated with hyperbaric oxygen: the role of an unusual approach to a rare condition

Kienböck's disease is a rare condition characterised by avascular necrosis of the lunate bone. Its natural history and aetiopathogenesis have not yet been clarified, nor are its triggering factors identified. We present a case of a 17-year-old male gymnast, without relevant medical/family history, with stage IIIA Kienböck's disease diagnosed in 2016. Initially, submitted to conservative treatment that proved to be insufficient. Consequently, surgical treatment was proposed, but refused. The patient instead underwent experimental treatment with hyperbaric oxygen (120 sessions, 100% oxygen at 2.5 atm, for 70 min periods, once daily, five times per week). In April 2018, a favourable clinical and radiological evolution was observed, with an improvement in the patterns of pain, motion and strength and an almost complete involution of the process of aseptic necrosis of the semilunar. To the best of our knowledge, this is the first report of Kienböck's disease treated with hyperbaric oxygen.



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Case of newly diagnosed bilateral anorchia in a 42-year-old male patient

A 42-year-old African man presented with hypogonadic phenotypical features, including gynoid body distribution, gynaecomastia, absent facial and truncal hair and micropenis. He denied ever experiencing development of male secondary sex characteristics. Endocrine testing revealed hypergonadotropic hypogonadism and undetectable AMH. Human chorionic gonadotropin (hCG) stimulation test failed to increase testosterone levels. Peripheral blood karyotype was 46, XY. Clinical examination and abdominal/pelvic/scrotal ultrasound and MRI failed to identify any testicular structures/remnants. Given the clinical course and the biochemical–radiological presentation, the diagnosis of bilateral anorchia was made (after more than four decades of its probable onset), and surgical exploration was decided against. The patient was subsequently started on monthly intramuscular testosterone experiencing progressive normal virilisation.



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Early initiation of venovenous extracorporeal membrane oxygenation in a mechanically ventilated patient with severe acute respiratory distress syndrome

A 49-year-old man presented to the emergency department with acute-onset dyspnoea and hypoxaemia 1 day following nasal surgery for obstructive sleep apnoea. A chest X-ray showed diffuse bilateral pulmonary infiltrates. Supplemental 100% fractional inspired oxygen (FiO2) via non-rebreather mask was delivered with resulting arterial oxygen tension:FiO2 ratio of 67. Transthoracic echocardiogram demonstrated normal heart function. A clinical diagnosis of severe acute respiratory distress syndrome (ARDS) was promptly made. Based on patient preference to avoid intubation and following a multidisciplinary approach, we decided to initiate venovenous extracorporeal membrane oxygenation (VV-ECMO) as an alternative strategy to mechanical ventilation. Though he ultimately required brief mechanical ventilation during ECMO cannulation, the patient spent a total of 5 days on VV-ECMO and a total of 8 days in the intensive care unit. Six days after discharge, his pulmonary function test demonstrated no significant abnormalities. We present a rare case of early initiation of VV-ECMO in a patient with severe ARDS that served as a bridge to recovery.



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Case 31-2018: A 37-Year-Old Man with a Self-Inflicted Gunshot Wound

Presentation of Case. Dr. Jordan P. Bloom (Surgery): A 37-year-old man was admitted to the surgical intensive care unit (ICU) of this hospital because of a self-inflicted gunshot wound. The patient had been in his usual state of health until 2 days before admission. That evening, while he was at…

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A Tricky Diagnosis

Foreword. In this Journal feature, information about a real patient is presented in stages (boldface type) to an expert clinician, who responds to the information by sharing relevant background and reasoning with the reader (regular type). The authors' commentary follows. Stage. A 70-year-old man…

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Case 29-2018: A 31-Year-Old Woman with Infertility

Presentation of Case. Dr. Caitlin Sacha (Obstetrics and Gynecology): A 31-year-old woman with infertility was referred to the fertility center of this hospital by a surrogate parenting agency to discuss the possibility of family building with an oocyte donor and a gestational carrier. The patient…

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Preoperative Evaluation: Is It Time to View It as a Component of Perioperative Optimization?

Publication date: Available online 11 October 2018

Source: Anesthesiology Clinics

Author(s): Lee A. Fleisher



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