Skin eruption and long-lasting fever in a young man

Description

A 20-year-old man presented to emergency room with a 3-week history of general weakness, fever, diffuse arthralgia and skin eruption. His medical history was unremarkable; he did not travel recently, had no contact with an ill person, nor had risky sexual behaviour and took no medication. Physical examination only showed a diffuse, non-painful, infracentimetric and non-confluent macular eruption over the trunks and limbs (figures 1 and 2). Blood test showed elevated C reactive protein and neutrophilic leucocytosis. Serologies for hepatitis C and B viruses, rubella, Epstein-Barr virus, cytomegalovirus, toxoplasmosis and HIV were negative. Urinalysis and chest X-ray were normal. Blood cultures became positive for Neisseria meningitidis, whereas skin biopsy only showed a dermic inflammatory polymorphic (lymphocytic and neutrophilic) infiltrate without specificity with negative aerobic and anaerobic cultures.

Figure 1

Macular lesions on the trunk.

Figure 2

Macular lesions...

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Diencephalic syndrome: a rare cause of failure to thrive

Timely diagnosis of diencephalic syndrome is not often the case for patients presenting with failure to thrive (FTT) because of its rarity and lack of specific symptoms. Herein, we report two cases of diencephalic syndrome (2-year-old girl and 10-month-old boy) presenting with severe emaciation. Both patients had histories of poor weight gain for months despite having good appetites prior to diagnosis. Initial work-up did not reveal the diagnosis. Horizontal nystagmus was noted in both patients: by a neurologist in the first patient and by a family member in the second patient. MRI of the brain showed large suprasellar mass and pilocytic astrocytoma was confirmed by pathology in each case. The patients were started on appropriate chemotherapy with interval improvements in weight gain. These cases illustrate the importance of cranial imaging and consideration of diencephalic syndrome for children presenting with FTT despite normal or increased caloric intake.

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Substantial harm associated with failure of chronic paediatric central venous access devices

Central venous access devices (CVADs) form an important component of modern paediatric healthcare, especially for children with chronic health conditions such as cancer or gastrointestinal disorders. However device failure and complications rates are high.

Over 21/2 years, a child requiring parenteral nutrition and associated vascular access dependency due to 'short gut syndrome' (intestinal failure secondary to gastroschisis and resultant significant bowel resection) had ten CVADs inserted, with ninesubsequently failing. This resulted in multiple anaesthetics, invasive procedures, injuries, vascular depletion, interrupted nutrition, delayed treatment and substantial healthcare costs. A conservative estimate of the institutional costs for each insertion, or rewiring, of her tunnelled CVAD was $A10 253 (2016 Australian dollars).

These complications and device failures had significant negative impact on the child and her family. Considering the commonality of conditions requiring prolonged vascular access, these failures also have a significant impact on international health service costs.

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Renal keratinising desquamative squamous metaplasia: all that hurts is not stone

Description

A 66-year-old Caucasian man with history of hypertension, hyperlipidaemia and 16 pack-year smoking was referred to us for evaluation of recurrent right-sided flank pain and suspected nephrolithiasis. His first episode of pain was 2 years prior to presentation, which was recurrent. There was no associated haematuria, dysuria, fever, chills, urinary hesitancy or incontinence. There was no family history of stones. He underwent multiple ureteroscopies, which have shown glistening, soft, acellular debris in the upper ureter. Last ureteroscopy and pyeloscopy showed normal underlying mucosa, renal pelvis and calyces with no evidence of malignancy. Subsequent MRI also did not show any malignancy. The last available pathology showed minute fragments of acellular keratin debris. Interestingly, he never had imaging evidence of renal stone, although had mild hydronephrosis one time. CT urogram demonstrated an ill-defined filling defect in the anterior right renal pelvis measuring approximately 13x3 mm in axial dimensions (figure 1). During...

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Transient neonatal hypercalcaemia secondary to excess maternal vitamin D intake: too much of a good thing

We report a case of transient neonatal hypercalcaemia secondary to excess maternal vitamin D intake in pregnancy. Vitamin D insufficiency and deficiency in pregnancy are associated with adverse pregnancy outcomes, but there is no definite benefit to supplementation. The Royal College of Obstetrics and Gynaecology recommends routine supplementation with vitamin D₃ 400 IU/day, but higher dose preparations usually recommended for the treatment of vitamin D deficiency are readily available over the counter. This case highlights the risks of excess supplementation, especially at higher doses and in women without evidence of vitamin D deficiency. The amount used in this case was at the upper end of the generally accepted safe dose range, but still less than that commonly recognised to cause problems. Neonatal hypercalcaemia is a potentially serious condition. The current local or national recommendations for vitamin D supplementation and the possible adverse effects of excess vitamin D consumption should be clearly communicated to pregnant women.

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Intraparenchymal pericatheter cyst in ventriculoperitoneal shunt failure

Description

A 60-year-old man status post right parieto-occipital ventriculoperitoneal shunt with programmable valve for indication of delayed hydrocephalus after aneurysmal subarachnoid haemorrhage returns to clinic 7 weeks after placement for insidious onset of headaches, confusion and gait imbalance. CT scan of the head without contrast revealed an intraparenchymal pericatheter cystic collection with severe oedema (figure 1). The patient denied systemic signs of illness, and laboratory markers for infection were low. A tap of the shunt reservoir revealed no spontaneous flow and difficulty in aspirating cerebrospinal fluid (CSF). Laboratory analysis of the fluid showed negative Gram stain, normal chemistry profile and cell counts. MRI of the brain showed a simple cyst with significant oedema, but no restricted diffusion or contrast enhancement of the cyst wall (figures 2 and 3). CT of the abdomen was benign, without evidence of pseudocyst or other cause of distal obstruction.

...

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Sternoclavicular joint osteophytosis: a difficult diagnosis to swallow

Unexplained dysphagia requires prompt investigation to rule out a possible underlying malignancy. We describe the case of a 60-year-old man who presented to his family practitioner with a 1-year history of increasing dysphagia with associated pain over the front of his chest. He was referred on to an ear, nose and throat specialist where no obvious laryngeal pathology was found at direct laryngoscopy, but an 'indentation' of the right anterior larynx, which increased with external pressure on the sternoclavicular joint (SCJ), was noted. A subsequent CT scan of his neck demonstrated osteoarthritis of the right SCJ with an abnormally large posterior osteophyte. The patient was subsequently referred on to an orthopaedic surgeon specialising in SCJ surgery and underwent an arthroscopic excision of his right SCJ. Soon after surgery, the patient's dysphagia had settled and his symptoms remain resolved 1 year post surgery.

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Cupping at the ends of ribs is not always rickets

Description

A 7-week-old baby boy presented with a history of cough, loose stools and respiratory distress since last 7 days. At admission he had a respiratory rate of 64/min, a heart rate of 144/min and an oxygen saturation of 56%. Chest examination revealed crepitations in both lung fields. Rest of the examination was unremarkable. Investigations revealed haemoglobin 82 g/L; white cell count 11.2x10⁹/L (differential counts: polymorphs 68%, lymphocytes 1%, monocytes 26% and eosinophils 5%); absolute lymphocyte count 0.11x10⁹/L; platelet count 102x10⁹/L and C reactive protein 239 g/L. Chest X-ray showed non-homogenous opacities in bilateral lung fields (more on right side) with an absent thymic shadow, cupping at the anterior end of ribs (black arrow, figure 1), flattening of lower end of the right scapula (white arrow, figure 1) and a spur at the inferior-lateral angle of the left scapula (white arrow head, figure 1). These characteristic radiological changes (ie, cupping at...

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Isolated insulin-derived amyloidoma of the breast

Isolated amyloidomas derived from insulin are extremely rare, and there is only one reported case to date of insulin-derived amyloidoma in the breast.

We present the case of a 36-year-old woman reporting a lump in the right breast. It was clinically assessed as a probable fibroadenoma but was removed surgically given the size of the lesion. On histological analysis, the lesion had features consistent with amyloid. Further investigations showed the amyloid to be derived from insulin. The lump was removed in its entirety, and the patient made a full recovery.

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Bilateral interstitial keratitis with anterior stromal infiltrates associated with reactive arthritis

A previously healthy 48-year-old man presented with a 1-week history of migrating polyarthropathy preceded by a viral illness, dysuria and bilateral red eyes. Ocular examination revealed anterior and interstitial stromal keratitis. He was systemically well but had raised erythrocyte sedimentation rate and C reactive protein and was positive for human leucocyte antigen B27 on extensive infective, rheumatological and autoimmune investigations. Although the exact triggering pathogen was not identified, clinical findings were consistent with reactive arthritis. Bilateral interstitial keratitis is a rare manifestation of reactive arthritis which, along with the anterior stromal keratitis, responded well to topical prednisolone sodium phosphate 0.5%. Systemic joint symptoms improved on oral sulfasalazine, non-steroid anti-inflammatory agent and low-dose prednisolone.

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Use of low-dose thrombolytics for treatment of intracardiac thrombus and massive pulmonary embolus after aborted liver transplant leads to recovery of right ventricular function and redo liver transplantation

This is a 61-year-old man with end-stage liver disease who experienced cardiac arrest secondary to a massive pulmonary embolus and intracardiac thrombus during cannulation for veno-venous extracorporeal membrane oxygenation (ECMO) in preparation for orthotopic liver transplantation (OLT). Surgery was aborted and the patient was taken back to the intensive care unit in cardiogenic shock on multiple vasopressors. The patient was unresponsive to heparin bolus and too high risk for systemic thrombolytics or embolectomy. He was ultimately treated with 12 mg total of alteplase through his pulmonary artery catheter over 3 hours. He had subsequent resolution of his cardiogenic shock and proceeded with successful liver transplantation 5 days after his initial event without any bleeding complications. Low-dose thrombolytic therapy in the setting of absolute contraindications to thrombolysis allowed for recovery of cardiac function and, ultimately redo OLT in a patient with otherwise little hope of survival.

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Adrenal crisis in metastatic breast cancer

A female patient with oestrogen receptor-positive and human epidermal growth factor receptor 2 (HER2)-positive invasive lobular breast cancer presented with progressive disease on CT scan. Some days after initiation of antineoplastic chemotherapy and anti-HER2 targeted antibody therapy, the patient presented with profuse diarrhoea, neutropaenia, nausea and weakness. Although Clostridium difficile was rapidly tackled as a causative agent of gastrointestinal complaints, clinical situation did not markedly improve despite proper antimicrobial treatment. The patient reported profound lack of energy, while nausea, vomiting and loose stools still persisted. Additionally slightly exaggerated pigmentation of nonsunexposed skin and mucosal areas led us to the assumption of proopiomelanocortin-derived peptide hypersecretion. The combination of highly elevated adrenocorticotropic hormone and low basal cortisol levels taken from a morning blood sample established the diagnosis of adrenal insufficiency due to metastatic burden, leading to a near Addison crisis by gastrointestinal complications of chemo-immune therapy. Administration of hydrocortisone immediately relieved general symptoms .

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Contribution of arterial spin-labelling MRI in a case with immune reconstitution inflammatory syndrome

Central nervous system immune reconstitution inflammatory syndrome (CNS-IRIS), which occurs most often in HIV-infected patients, is an exacerbation of inflammatory reactions related to opportunistic infections as well as primary CNS malignancies both of which mostly occur in HIV-infected patients. However, differential diagnoses are challenging both clinically and radiologically. We describe a patient with CNS-IRIS due to toxoplasmosis whose ¹¹C-methionine uptake suggested lymphoma but whose arterial spin-labelling MRI led to the correct diagnosis.

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Hiding in plain sight: a case of chronic disseminated histoplasmosis with central nervous system involvement

A 64-year-old man presented with gradual onset of confusion, ataxia and 25-pound weight loss over 3 months. MRI of the brain revealed two enhancing cerebellar lesions suspicious for metastases. Positron emission tomography-CT showed enhancement of cervical and axillary lymph nodes. Left axillary lymph node biopsy showed no evidence of malignancy but instead showed fungal organisms morphologically consistent with Histoplasma spp. Disseminated histoplasmosis with central nervous system involvement was suspected. Further history revealed that the patient had been having subjective fever for the past several months. He has had mild pancytopenia for about 2 years, which had not been further evaluated. Additionally, he had an oesophagogastroduodenoscopy 3 months prior to admission, which had shown granulomatous gastritis. Subsequently, the diagnosis of disseminated histoplasmosis was confirmed by serological testing and bone marrow biopsy. The patient was started on liposomal amphotericin B. Unfortunately, the patient had a catastrophic stroke and was transitioned to comfort care measures.

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Persistent sacral chloroma in refractory acute myelogenous leukaemia

Acute myelogenous leukaemia (AML) is a clonal process involving the myeloid subgroup of white blood cells. Chloromas, or myeloid sarcomas, are masses of myeloid leukaemic cells and are a unique aspect of AML. This case involves a 14-year-old boy with AML who presented with multiple chloromas at diagnosis. The patient's extra-calvarial masses and bone marrow involvement responded to chemotherapy; however, his sacral epidural chloromas persisted despite four courses of chemotherapy. The central nervous system, bone marrow and testes have been known to be sanctuary sites for AML. This case illustrates that the sacral spinal canal may potentially be a sanctuary site for the disease process also.

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'Scissor deformity of the toes

Description

A 63-year-old woman presented with an unusual foot deformity involving the left first and second toe causing persistent pain (subjective functional grade 1),1 difficulty in walking and inability to accommodate the foot in regular footwear. She has type 2 diabetes mellitus for the past 8 years and sensorimotor peripheral neuropathy as assessed by vibration perception threshold >25 mV, absence of Semmes-Weinstein monofilament perception and absent ankle reflex. She was a home maker and wore footwear with thumb-hold most of the time. On examination, she had severe hallux valgus (HV) (Manchester Scale: severe score=3)², anatomical grade 11 and over-riding of second toe over the great toe causing 'scissor deformity' (figure 1). In addition, she had hammer and claw toe deformity involving other digits. She was provided with modified footwear to accommodate great toes with a bunion aid.

Figure 1

Both feet showing...

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Cryptococcal meningitis causing obstructive hydrocephalus in a patient on fingolimod

Cryptococcosis is a recognised opportunistic infection in immunocompromised patients. The long-term adverse effect profile of fingolimod, an immunomodulating agent approved for use in multiple sclerosis in 2010, is only just emerging. We report the first case to our knowledge of a patient presenting with obstructive hydrocephalus secondary to cryptococcal meningitis in the setting of fingolimod therapy. Extensive posterior fossa leptomeningeal inflammation with associated cerebellar oedema resulted in effacement of the fourth ventricle and obstructive hydrocephalus requiring urgent ventriculostomy. Induction, consolidative and maintenance antifungal therapy was prescribed and subsequent conversion to a ventriculoperitoneal shunt was successful in relieving the patient's ventriculomegaly. Awareness of these rare, novel and life-threatening complications of fingolimod-associated immunocompromise is critical as the use of such drugs is expected to rise.

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A rare case of malposition of central venous catheter detected by ultrasonography-guided saline flush test

Central venous catheter (CVC) insertion is associated with many potential complications; malposition of the catheter is one of them. A chest X-ray is routinely done to detect the malposition of catheter, but sometimes it has been seen that X-ray is time-consuming and its accuracy is also low for determining the exact position of the catheter tip. In our case, an ultrasonography (USG)-guided CVC was placed into the right internal jugular vein of the patient. As there was no ECG change obtained during insertion of guidewire and catheter, malposition was suspected, which was easily detected by a novel USG-guided saline flush test. We present a case report where USG was used for detection of a misplaced CVC (from right internal jugular vein to right subclavian vein). With ultrasound, the location of the catheter tip can be confirmed in very less time compared with chest X-ray.

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Vascularisation of the anterior lens capsule in an eye with excellent visual acuity

A 53-year-old phakic female with a history of nanophthalmos and hyperopia was incidentally found to have unilateral neovascularisation on the left inferoanterior lens surface on routine review for ocular hypertension.

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A rare case of Epstein-Barr virus mucocutaneous ulcer of the colon

Epstein-Barr virus mucocutaneous ulcer (EBVMCU) is a rare form of EBV lymphoproliferative disorder. The disease was recently described in 2010 for the first time in a case series and it was recently identified by the WHO classification of haematological malignancies as a separate category among the EBV lymphoproliferative disorders. We present a case of EBVMCU of the colon presenting as an ulcerating inflammatory mass in a female in her mid-60s who presented initially with abdominal pain and diarrhoea. The patient had extensive workup for her disease and due to progression of her symptoms, she was taken for an exploratory laparotomy. During the procedure, there was an inflammatory mass at the caecum and severe inflammation of the caecum and the terminal ileum and right hemicolectomy was performed. Diagnosis was confirmed by histopathology as EBV-positive lymphoproliferative disorder best classified as EBV-positive mucocutaneous ulcer.

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Seronegative Autoimmune Hepatitis A Clinically Challenging Difficult Diagnosis

Autoimmune hepatitis (AIH) is a complex liver disease of unknown cause which results in immune-mediated liver injury with varied clinical presentations. Seronegative AIH follows a similar course to autoantibody-positive disease and diagnosis may be challenging. There are no single serologic tests of sufficient diagnostic specificity, and delay in appropriate treatment may lead to progression of the liver disease and liver failure. The revised conventional diagnostic criteria (RDC) scoring for AIH is complex and not routinely used in the clinical practice. The more recent simplified diagnostic criteria (SDC) scoring proposed by International Autoimmune Hepatitis Group in 2008 has wider application in routine practice facilitating the diagnosis of AIH with a specificity and sensitivity of ~90%. In this report, we describe a case of seronegative autoimmune hepatitis diagnosed using RDC. SDC score calculated in our case was 4 and was not diagnostic for AIH. We subsequently used the complex revised diagnostic criteria for definitive diagnosis. Some of the patients previously diagnosed as cryptogenic active hepatitis of unknown etiology probably had an unrecognized diagnosis of seronegative autoimmune hepatitis. SDC scoring may not be applicable in patients with seronegative autoimmune hepatitis. These patients should be reassessed by using RDC.

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Partial response of pulmonary adenocarcinoma with symptomatic brain metastasis to nivolumab plus high-dose oral corticosteroid: a case report

Nivolumab, a monoclonal antibody targeting the programmed death-1 receptor, is indicated in locally advanced or metastatic non-small cell lung cancer, with progression after platinum-based chemotherapy. Up-to-...

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Idiopathic abdominal cocoon: a rare presentation of small bowel obstruction in a virgin abdomen. How much do we know?

Abdominal cocoon is an extremely rare condition that has been mainly associated with young adolescent women. It was first described in 1978 by Foo et al. We present here a case that describes an otherwise healthy adult man who presented with intestinal obstruction and was found to have an abdominal cocoon, also known as a peritoneal sac. The patient was taken for a laparotomy and the sac was released through blunt dissection along the avascular planes. He was discharged in good condition 3 days postoperatively. We discuss some of the current literature and previously reported cases on this condition.

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Two otherwise healthy young brothers present with intermittent claudication, just a coincidence?

Popliteal artery entrapment syndrome (PAES) is a recognised cause of lower limb peripheral arterial disease in young adults. We describe the cases of two otherwise healthy brothers who presented with the condition 5 years apart. The first brother, who is also the first author of this case report, presented aged 19 with worsening, right-sided, exercise-induced lower leg pain and transient foot pallor. Imaging confirmed PAES and irreversible localised arterial damage. Surgery was performed to release the entrapment and resect the section of diseased artery. The limb was revascularised using an autologous interposition saphenous vein graft. The second brother began experiencing left-sided, exercise-induced lower leg pain aged 24. Again, imaging revealed PAES and irreversible arterial damage. A similar revascularisation procedure was performed. Both siblings fully recovered and are symptom free. Arterial duplex scans have confirmed patent grafts. A correlation in siblings has only been reported in the literature five times previously.

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Black swans: challenging the relationship of anaesthetic-induced unconsciousness and electroencephalographic oscillations in the frontal cortex

The study of general anaesthesia and electroencephalographic oscillations in the frontal cortex spans at least four decades, with several notable findings:

• In 1977, Tinker and colleagues¹ proposed, based on studies of the nonhuman primate, that anteriorization—the shift of electroencephalographic power from posterior cortex to frontal cortex—correlated with unresponsiveness during general anaesthesia.
• In the early 1990s, Steriade contributed to our understanding of the neurophysiology of sleep and general anaesthesia in animals, including descriptions of three distinct oscillations involving corticothalamic networks: a slow rhythm at < 1 Hz, a delta rhythm at 1-4 Hz, and a faster theta/alpha rhythm at 7-14 Hz.²³
• In the mid-1990s, the shift of alpha oscillations to more anterior structures was identified during both propofol sedation⁴ and isoflurane/nitrous oxide anaesthesia.⁵
• In 2001, anteriorized alpha and slow-wave activity was posited by John and colleagues⁶ to be an agent-invariant marker of anaesthetic-induced unconsciousness, based on a study of 176 surgical patients.
• In 2004, the disappearance of occipital alpha oscillations and shift to high-power frontal alpha oscillations was found to be associated with propofol-induced unconsciousness in healthy human participants.⁷
• In 2013, Purdon and colleagues⁸ found, using high-density electroencephalography in human volunteers, that anteriorization of alpha and phase-amplitude coupling patterns correlated with propofol-induced unconsciousness.
• In 2014, both propofol and sevoflurane anaesthesia were found to be associated with anterior alpha and slow oscillations in surgical patients.⁹

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Beating, Fast and Slow

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, sharing his or her reasoning with the reader (regular type). The authors' commentary follows. Stage. A 52-year-old man presented to his…

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