Adult bilateral idiopathic occlusion of foramina of Monro: is foraminoplasty really safe and effective?

Idiopathic bilateral occlusion of foramina of Monro is an extremely rare condition in adults and only few cases are reported. Currently, foraminoplasty is indicated as first-line treatment. We reported the case of a 52-year-old woman who presented with headache, instability and urinary incontinence. The imaging demonstrated bilateral occlusion of foramina of Monro and consequent biventricular hydrocephalus. A ventriculo-peritoneal shunt allowed to treat the hydrocephalus, but it was necessary to remove it for the appearance of peritonitis from salpingitis. Since the initial symptomatology reappeared, according to the literature, we performed an endoscopic foraminoplasty with septostomy. The patient immediately developed short-term memory impairment and subsequently we observed the hydrocephalus recurrence. The positioning of a ventriculo-atrial shunt allowed to improve the symptoms, but the memory remained impaired. In case of bilateral true stenosis of foramina of Monro, foraminoplasty may lead to severe memory impairment. Therefore, cerebrospinal fluid shunting should be considered as an effective and safer treatment.

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Prenatal diagnosis and management of fetal discordant alpha-thalassaemia in dichorionic diamniotic (DCDA) twins

A 29-year-old nulliparous woman with a dichorionic diamniotic (DCDA) twin pregnancy was referred to our hospital at 16 weeks' gestation for prenatal diagnosis. She was diagnosed of Haemoglobin H Constant Spring (Hb H CS; --^SEA/α^CSα) and her husband of alpha thalassemia-1 trait (--^SEA/αα). Detailed ultrasound showed that left twin had fetal anaemia and early signs of hydrops while the right one was normal. Both twins were female. Amniocentesis in each sac was performed for prenatal diagnosis of thalassemia after a proper counselling with the couple. DNA analysis confirmed that the left fetus was affected with haemoglobin Bart's hydrops fetalis (--^SEA/--^SEA) while the right one was alpha thalassemia-1 trait (--^SEA/αα). Selective feticide with intracardiac injection of KCl was successfully performed on the hydropic fetus. Identification of the affected fetus is crucial for selective termination. Family counselling about the procedure and complications is also necessary.

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Histologically confirmed case of complete atrioventricular block due to hepatocellular carcinoma

A 72-year-old man, who had been diagnosed as having hepatocellular carcinoma (HCC) with multiple extrahepatic metastasis, complained a general fatigue which appeared 2 weeks before admission. Because bradycardia was detected on physical examination, ECG was performed which revealed the complete atrioventricular (AV) block. We stopped Ca-blocker and β-blocker, but the bradycardia persisted. He was admitted to our hospital for an emergent pacemaker implantation. On admission, he complained dyspnoea. After the surgery, he died due to deterioration of heart failure. The autopsy revealed cardiac metastasis of HCC on AV node, so it was suspected that cardiac metastasis caused the AV block. We thought that the cause of his death was the exacerbation of heart failure associated with bradycardia. It was likely that complete AV block as a very rare complication caused by cardiac metastasis of HCC influenced the prognosis of this patient.

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Umbilical cord anomalies: antenatal ultrasound findings and postnatal correlation

Umbilical cord anomalies are rare. The differential diagnosis for a cystic structure around the umbilical cord and its insertion include pseudocyst, omphalomesenteric duct cyst, haemangioma, omphalocele or anterior abdominal wall defects. Although cord anomalies can be detected through antenatal ultrasound scans (US), very often a definitive diagnosis cannot be made. This may affect the management of the infant at birth. In cases where antenatal US was not diagnostic, current evidence supports the use of MRI to help in making an accurate diagnosis. We report two cases of umbilical cord anomalies. The first case was diagnosed in antenatal US as an omphalocele, but was found to be an allantoic cyst with hamartoma on postnatal diagnosis. The second case was not detected on antenatal US, and was diagnosed postnatally as a small omphalocele with vitellointestinal duct remnants.

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Coronary artery vasospasm in a patient withChurg-Strauss syndrome

Patients with Churg-Strauss syndrome often suffer from unusual cardiac manifestations and sudden cardiac death. This differs from patients with other autoimmune disorders, who typically present with premature ischaemic heart disease. We report the case of a 56-year-old man with recurrent coronary vasospasm, including an inferoposterior ST-elevation myocardial infarction, complicated by bradycardic arrest. There was only minor coronary artery disease on coronary angiography. An elevated eosinophil count was noted. His medical history included allergic rhinitis with polyposis, adult-onset asthma and biopsy-proven eosinophilic oesophagitis. Review of his sinus biopsies demonstrated blood vessels with marked accumulation of eosinophils in extravascular areas. The patient, therefore, met the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient was commenced on immunosuppression, with the return of the eosinophil count to within normal limits, and remains free of cardiovascular events over 24 months.

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Not just another diplopia: neurocysticercosis in a postpartum woman

Description 

A 23-year-old woman with a history of child birth 2 weeks back presented to the outpatient department with binocular diplopia and slight blurring of vision in both eyes (BE) for last 1 week. She also had four bouts of vomiting with associated nausea and hemicranial dull headache for last 3 days. The antenatal and immediate perinatal period was uneventful. Previous history as well as systemic history was not significant. The patient was afebrile. On systemic examination, neck stiffness could be elucidated. The blood pressure was recorded to be 124/74 mm Hg. Ocular examination revealed the visual acuity to be 20/40 in BE. There was restricted abduction of the right eye (figure 1) and diplopia charting revealed isolated sixth cranial nerve palsy. Pupillary reaction was brisk in BE with no evidence of relative afferent pupillary defect. Slit lamp evaluation of the anterior segment showed no abnormality. On fundus evaluation, bilateral disc oedema with ill-defined...

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Giant vesicle calculi leading to spontaneous bladder rupture and acute renal failure: an unusual presentation

Spontaneous rupture of the urinary bladder is extremely rare. We report a case of a 70-year-old man with spontaneous bladder rupture secondary to neglected giant vesicle calculi who presented as acute renal failure. The patient was stabilised with per-urethral catheterisation and extravesical drain placement. About 700 mL pus mixed with urine was drained through the per-urethral catheter and approximately 2000 mL of pus was drained through the extravesical drain. Cystolithotomy showed two large calculi which were removed.

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Acute aortic dissection: a missed diagnosis

A 60-year-old man with a history of indigestion and untreated hypertension presented with sudden-onset central chest pain which radiated to his back. Acute coronary syndrome was initially suspected but excluded in the emergency department before the patient was discharged. The pain subsequently abated to mild intermittent episodes and was misdiagnosed as indigestion. A week later the patient developed new shortness of breath and 'flu-like' symptoms with a positive d-dimer test. CT angiography revealed a Stanford type B aortic dissection which was causing hypoperfusion of the right kidney, resulting in an acute kidney injury. Due to uncontrolled hypertension despite rigorous antihypertensive medication and his failing renal function, the patient underwent endovascular repair and made a good recovery postoperatively.

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Proximal humerus deformity, in a four-limb amputee following meningococcal septicaemia

We present a challenging case of proximal humerus varus deformity in a four-limb amputee, caused by growth arrest from meningococcal septicaemia. The deformity resulted in a loss of function for our patient with inhibition of activities of daily living, requiring corrective osteotomy to help improve the range of motion of the shoulder. We describe in detail the management of our patient, highlight the importance of the orthopaedic manifestations of meningococcal septicaemia, and demonstrate the importance of monitoring potential deformities from growth arrest in these patients.

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Tuberous sclerosis complex: a clinical case with multiple ophthalmological manifestations

The tuberous sclerosis complex is a rare disease, with autosomal dominant transmission, with multisystemic involvement including ophthalmologic. Retinal hamartomas and retinal achromic patch are the most frequent ocular findings. Other ophthalmic signs and symptoms are relatively rare in this disease.

We describe the case of a young woman with tuberous sclerosis who presented with horizontal binocular diplopia and decreased visual acuity without complaints of nausea, vomiting or headache. She had right abducens nerve palsy, pale oedema of both optic discs and retinal hamartomas. An obstructive hydrocephalus caused by an intraventricular expansive lesion was identified in brain CT.

Observation by the ophthalmologist is indicated in all confirmed or suspected cases of tuberous sclerosis to aid in clinical diagnosis, monitoring of retinal hamartomas or identification of poorly symptomatic papilloedema.

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Chest pain following permanent pacemaker insertion... a case of pneumopericardium due to atrial lead perforation

Permanent pacemaker (PPM) implantation is an increasingly common procedure with complication rate estimated between 3% and 6%. Cardiac perforation by pacemaker lead(s) is rare, but a previous study has shown that it is probably an underdiagnosed complication. We are presenting a case of a patient who presented 5 days after PPM insertion with new-onset pleuritic chest pain. She had a normal chest X-ray (CXR), and acceptable pacing checks. However, a CT scan of the chest showed pneumopericardium and pneumothorax secondary to atrial lead perforation. The pain only settled by replacing the atrial lead. A repeat chest CT scan a few months later showed complete resolution of the pneumopericardium and pneumothorax. We believe that cardiac perforation can be easily missed if associated with normal CXR and acceptable pacing parameters. Unexplained chest pain following PPM insertion might be the only clue for such complication, although it might not always be present.

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Solitary fibrous tumour of caecum wall: an unlikely cause of low gastrointestinal haemorrhage

Solitary fibrous tumour (SFT), previously denominated as haemangiopericytoma, is a rare, spindle cell neoplasm that was first described in the thoracic pleura. It is now known that this tumour may develop from almost any anatomic location. We report a case of SFT, in a 65-year-old man, which was located in the muscularis propria layer of the caecum with involvement of the serosa and the ileocecal appendix, location never described in the literature, and with an uncommon clinical presentation of hematochezia. A radical right hemicolectomy was performed, and the patient was asymptomatic without evidence of metastasis or relapse after 6 months of follow-up.

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Neck carbuncle associated with methicillin-susceptible Staphylococcus aureus bacteraemia

Description 

A 66-year-old man presented with a 14-day history of painful posterior neck swelling and fever for a week prior to presentation. A restricted neck movements was noted over the last 3 days. Generally, he had a fever of 37.5°C and tachycardia at 121 bpm, whereas the other vital signs were normal. Local physical examination showed large posterior neck swelling with erythema and multiple sinuses discharging pus.

A neck carbuncle was diagnosed (figure 1).1 2 Skin eruptions were noted also on the lower extremities and gluteal region.

Figure 1

Physical examination. (a) A pustule in posterior neck which is surrounded by an extensive erythematous swelling (white dotted circle). (b) The pustules that have the same features were seen at lower extremity and gluteal region.

Laboratory evaluation showed a haemoglobin level of 14.3 g/dL, leucocyte count of 38 800x109/L, sodium of 132 mmol/L, creatinine of...

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Iris Morphological Features in Patients with 360° Angle-Closure Neovascular Glaucoma: An Anterior Segment Optical Coherence Tomography Study

Purpose: To investigate iris morphological features in 360° angle-closure neovascular glaucoma (NVG) by swept-source anterior segment optical coherence tomography (ASOCT). Patients and Methods: In this retrospective, clinic-based, comparative study, 14 patients with 360° angle-closure NVG and 14 healthy age-matched control subjects were enrolled. All patients enrolled had no prior glaucoma surgery but underwent cataract surgery with intraocular lens implantation. Horizontal scanning images of swept-source ASOCT were analyzed using software calipers in temporal and nasal angle areas. The iris thickness at 1 and 2 mm from the pupil edge, iris length, trabecular meshwork length, peripheral anterior synechia (PAS) length, PAS height ratio (PAS length/trabecular meshwork length), and pupil diameter were measured. Results: Between the groups, there were no statistically significant differences in iris length, trabecular meshwork length, and pupil diameter (p #x3e; 0.05). However, the iris thickness was significantly reduced in the NVG group compared with the control group in the temporal and nasal areas (0.306 vs. 0.563 mm/0.326 vs. 0.645 mm at 1 mm, 0.278 vs. 0.523 mm/0.282 vs. 0.546 mm at 2 mm, respectively) (mean, all p #x3c; 0.001). In the NVG group, PAS height ratios were 1.55 ± 0.45 (mean ± standard deviation) (range, 0.58–2.30) and 1.55 ± 0.78 (range, 0.68–3.68) at the temporal and nasal angles, respectively. Conclusions: In patients with 360° angle-closure NVG, the iris thickness decreased to about 50% of that in healthy subjects, and the PAS length exceeded the trabecular meshwork length by about 1.5 times.
Case Rep Ophthalmol 2018;9:449–456

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Hitherto unreported involvement pattern of Carvajal phenotype of cardiocutaneous syndrome: evaluation on cardiac MRI

Description 

An 11-year-old boy, born out of a consanguineous marriage, was admitted with gradually progressive dyspnoea, and facial and abdominal swelling for the past 1 year. On physical examination, he had woolly hair and patchy keratosis over the palms and soles (figure 1A,B). Chest radiograph showed gross cardiomegaly (figure 1C). ECG revealed left axis deviation. Transthoracic echocardiogram demonstrated biventricular dilatation with severe left ventricular dysfunction, trabecular configuration of both ventricles along with mild tricuspid and mitral valve regurgitation. Cardiac MRI revealed biventricular dilatation with severe global hypokinesia (left ventricular ejection fraction: 10%) and increased trabeculations in both the ventricles (figure 2A–D). Late gadolinium enhanced images showed extensive transmural enhancement predominantly of septum and lateral wall of left ventricle (figure 2E–F). In addition, enhancement was also seen in the right ventricular wall as well as involving the trabeculations. No genetic study was performed in...

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Superior mesenteric artery syndrome: a vicious cycle

Description 

A middle-aged cachectic woman with significant medical history of quadriplegia, chronic osteomyelitis, chronic obstructive pulmonary disease, kyphoscoliosis and malnutrition presented with 3 days of persistent nausea and vomiting, absolute constipation and increasing abdominal distention. She has had similar episodes in the recent past, which were milder in nature, and were diagnosed as gastroenteritis. She was hypotensive (blood pressure 86/60 mm Hg) and tachycardic (130 bpm) on arrival, with a grossly distended abdomen and active vomiting. Initial blood tests detected hypokalaemia (K+ of 3.0 mmol/L), a raised white cell count of 23.4x10⁹/L and features of acute kidney injury. A nasogastric tube was inserted and over a litre of bilious gastric contents was immediately aspirated.

A non-contrast CT scan of the abdomen and pelvis was performed (as the patient is allergic to iodine contrast), which demonstrated marked distension of the stomach and duodenum terminating abruptly where the third part of the duodenum crosses over the...

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Non-uraemic calciphylaxis (NUC) postliver transplantation

Calciphylaxis is a rare and life-threatening disease characterized by cutaneous arteriolar stenosis and vascular thrombosis leading to skin ischaemia and necrosis. While calciphylaxis occurs mostly in patients with end-stage renal disease, the disorder has been described in patients with normal renal function, namely non-uraemic calciphylaxis (NUC). A 41-year-old African-American woman presented with a painful ulcerative rash on her thighs and right buttock 2 months after undergoing an orthotopic liver transplantation. She underwent debridement of the lesions and an excisional biopsy of one of the lesions, which revealed calciphylaxis. She was treated with sodium thiosulfate, cinacalcet and hyperbaric oxygen with complete resolution of the lesions 4–5 months after presentation. While she was treated with a course of high-dose glucocorticoids after the transplant, she did not have other risk factors for calciphylaxis. NUC should be considered in the differential diagnosis of necrotic skin lesions in postliver transplant patients.

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Anterior spinal cord syndrome as a rare complication of acute bacterial meningitis in an adult

Acute bacterial meningitis is not an uncommon central nervous system infection. In severe cases, it can be associated with various neurological or systemic complications. However, acute spinal cord dysfunction rarely occurs. We report a case of bacterial meningitis complicated with spinal cord infarction despite adequate treatment with antibiotics and corticosteroid therapy. He had residual paraplegia and was fully dependent in the activity of daily living.

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Rare recurrence of apical ballooning (takotsubo) syndrome in an elderly man

Apical ballooning syndrome (ABS) is an under recognised clinical entity characterised by acute reversible left ventricular systolic dysfunction that mimics acute myocardial infarction in the absence of obstructive coronary artery disease; typically occurring in the setting of profound stress.¹ ABS disproportionately affects older women and recurrences are infrequent. We, hereby, describe a rare phenomenon of recurrent ABS in an elderly male patient, 10 years apart, presenting with the same left ventricular morphological appearance following non-cardiac surgeries. The case illustrates the importance of considering ABS in the differential diagnosis of perioperative acute myocardial infarction in older men undergoing major surgery.

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Retinoblastoma presenting as haemorrhagic choroidal detachment in a 10-year-old girl

To report a case of retinoblastoma presenting as haemorrhagic choroidal detachment in a 10-year-old girl. The patient, apparently well, presented with sudden blurring of vision in the setting of blunt trauma. On examination and ocular ultrasound, haemorrhagic choroidal detachment was suspected. She underwent external drainage via sclerostomies. The haemorrhage resolved ultrasonographically, but the vision did not improve. Five months postoperatively, she presented with sudden eye pain, lid swelling and proptosis. The ancillary workups were consistent with panophthalmitis and was treated as such. Six months postoperatively, the pain and lid swelling persisted; hence, the painful near absolute eye was enucleated. The histopathology revealed small round blue cells with necrosis consistent with retinoblastoma. Immunohistochemistry was positive for synaptophysin and negative for S100 confirming retinoblastoma. Retinoblastoma is the most common intraocular malignancy in children. The importance of increased awareness of its various presentations is of utmost importance to prevent life-threatening and vision-impairing complications.

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Triphasic response of pituitary stalk injury following TBI: a relevant yet uncommonly recognised endocrine phenomenon

The triphasic response of pituitary stalk injury has previously been described in a minority of patients following intracranial surgery, however, this phenomenon can also occur after traumatic brain injury. We present the case of a 20-year-old male who experienced the triphasic response of pituitary stalk injury (central diabetes insipidus, syndrome of inappropriate antidiuretic hormone and central diabetes insipidus again) after striking his head on a concrete curb. His history and presentation highlight the importance of recognising the distinctive symptoms of each individual stage of pituitary stalk injury, and using the appropriate diagnostic tools and therapies to guide further management.

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Trans-Frontal Five-Fluorouracil (TraFFF): a novel technique for the application of adjuvant topical chemotherapeutic agents in sinonasal adenocarcinoma

Sinonasal adenocarcinoma has traditionally been treated with surgery (craniofacial or endoscopic) and adjuvant radiotherapy. Two large series have demonstrated favourable 5-year survival using surgery with adjuvant topical chemotherapy applied repeatedly over several weeks. The authors describe a novel, transnasal application of topical 5-fluorouracil. However, complete coverage of the frontal region of the anterior skull base can be difficult to achieve with a purely transnasal approach in an outpatient setting. We present a novel adjunct method of delivering chemotherapeutic agents into this key area used in a 37-year-old man with T2N0M0 ethmoid adenocarcinoma. The procedure was well tolerated in an outpatient setting and remains disease free at 3 years postoperatively. We suggest that frontal trephination is a useful adjunct to aid accurate placement of adjuvant topical chemotherapeutic agents in the treatment of sinonasal adenocarcinoma.

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