Klippel-Trenaunay syndrome and cerebral haemangiopericytoma: a potential association.

Klippel-Trenaunay syndrome (KTS) a rare mesodermal phakomatosis consisting of capillary malformations, varicose veins, and limb hypertrophy, often associated with vascular malformations and benign tumours. A 33-year-old male presented with headaches secondary to a previously diagnosed intracranial tumour. He had a large blanching port-wine stain and hypertrophy of the left side of his body and limbs partial syndactyly of the 2(nd) and 3(rd) digits in all four extremities. The lesion was surgically resected and histology showed a Haemangiopericytoma. Thus KTS may be associated with intracranial Haemangiopericytomas, a malignant vascular tumour.

Streptococcus pneumoniae from ophthalmic infections: serotype distribution and penicillin susceptibility.

Streptococcus pneumoniae is one of the pathogens causing infection of the conjunctiva and the uveal tract. The present study began with the observation of two ophthalmic S. pneumoniae isolates showing intermediate resistance to penicillin. Among the 25 isolates of S. pneumoniae from 617 ophthalmic specimens, during the period of 14 months, four were found to exhibit an intermediate resistance to penicillin. Minimum Inhibitory Concentration values ranging from 0.125 microg/mL to 0.25 microg/mL was observed. No multidrug resistant strains were isolated. Serogrouping/typing of the S. pneumoniae revealed the following serogroups/types; 6A (n = 3), 6B (n = 2), 22 (n = 3), 14 (n = 3), 23A (n = 2), and 1 each of 23B, 19A, 7B, 32, 9, 42, 21, 39, 10, 3, and 34. One strain showed cross reaction in pool 29, 35, and 47. These findings represent the first such observation of ophthalmic isolates from India.

Emerging importance of Pneumocystis carinii among indian immunosuppressed patients.

Pneumocystis carinii is known to cause both significant morbidity and mortality in immunosuppressed individuals. In a six and a half year period starting with 1992, a total of 204 samples including bronchial aspirates, induced sputum and suction catheter tips were examined for P. carinii by direct microscopic examination of Giemsa's and toluidine 'O' stained smears. At a later stage of the investigation, immunofluorescent staining using monoclonal reagent (Meriflour, USA) was also used for examination of the specimens. In all 24 (11.8%) of 204 samples were positive for P. carinii. In addition, Pneumocystis carinii pneumonia (PCP) was diagnosed in five (33%) of 15 patients of acquired immunodeficiency syndrome (AIDS) with opportunistic infections. All these 15 patients had CD4 T cell counts of less than 500 T lymphocyte equivalent (TLE) /ml as measured by a Trax ELISA assay. Laboratories in India have to be better equipped for an early and correct diagnosis of PCP that is bound to rise with the increase in the number and variety of immunosuppressed patients.

Successful renal transplantation following treatment of Aspergillus terreus peritonitis in a continuous ambulatory peritoneal dialysis patient.

Peritoneal dialysis (PD) related peritonitis caused by fungi is a potentially life-threatening complication. It diminishes prospects of continuing PD. We report a patient with Aspergillus terreus peritonitis treated successfully with catheter removal and antifungal therapy and subsequently had a live-related renal transplantation. There was no recurrence of the infection in 3 years of follow-up.

Unusual presentation of entomophthoromycosis.

Rhinoentomophthoromycosis caused by Conidiobolus sp commonly presents as a chronic granulomatous lesion that affects the rhinofacial subcutaneous tissue. We present an 18-year-old girl who presented with progressive bilateral proptosis and loss of vision since 2 weeks. Biopsy and fungal cultures confirmed diagnosis of Conidiobolus sp infection of the paranasal sinuses bilaterally with orbital extension and blindness. The clinical picture was complicated by the presence of sputum-positive cavitatory pulmonary tuberculosis, which was diagnosed at the same time. To our knowledge, this is the first such case to be reported from India. We also discuss the management of entomophthoromycosis. Despite many reports of success, there remains no consensus on the treatment of Conidiobolus infections of the nose and the paranasal sinuses with antifungal agents.

Brown-Séquard syndrome secondary to spontaneous bleed from postradiation cavernous angiomas.

SUMMARY: The spontaneous occurrence of acute Brown-Séquard syndrome is an extremely rare event, with most reported cases being secondary to spontaneous epidural hematomas and spinal cord ischemia. We report a rare case of Brown-Séquard syndrome from spontaneous intraspinal hemorrhage in a patient with multiple cavernous angiomas in the spinal cord secondary to craniospinal radiation in childhood. Postulated mechanisms leading to the condition include postradiation molecular changes and venous occlusion.

Apophysomyces elegans - renal mucormycosis in a healthy host: a case report from south India.

Zygomycosis is an opportunistic fungal infection that seldom occurs in individuals with a competent immune system. Isolated involvement of any organ is rare and only a few cases of renal zygomycosis have been reported. We present an unusual case of renal zygomycosis caused by Apophysomyces elegans in a patient with no known predisposing factor. He presented with flakes in the urine and was found to have a poorly functioning right kidney. Ureterorenoscopy was performed, fungal elements removed and pathological confirmation obtained. The patient subsequently underwent nephrectomy after treatment with amphotericin B. He made an uneventful recovery.

Prevalence of inducible clindamycin resistance in gram positive organisms in a tertiary care centre.

Gram positive organisms are one of the leading pathogens causing skin and soft tissue infections. For these infections, clindamycin is a useful alternate drug in penicillin-allergic patients. This study was conducted to investigate the prevalence of erythromycin-induced clindamycin resistance in gram positive organisms in the southern part of the country. A total of 522 consecutive clinical isolates from blood, CSF, sputum, throat, pus, and urine were collected between November 2006 and April 2007 and tested for erythromycin resistance and inducible clindamycin resistance. There was a relatively higher incidence of inducible clindamycin resistance among the MRSA isolates. We conclude, therefore, that clindamycin is not a suitable alternative antibiotic for use in staphylococcal skin and soft tissue infections.

Acute abdomen: an unusual presentation of disseminated Penicillium marneffei infection.

Varied clinical presentations of Penicillium marneffei, an opportunistic pathogen in HIV disease has been rarely described in literature. We report a patient with advanced AIDS who presented to us with prolonged fever and had features of an acute abdomen. On radiologic imaging he had features of intestinal obstruction and mesenteric lymphadenitis. A diagnosis was made possible by endoscopic biopsies of the small bowel and bone marrow culture which grew P. Marneffei. He was treated with intravenous amphotericin for 2 weeks followed by oral itraconazole. This case is reported for its rarity and unusual presentation and to sensitise clinicians and microbiologists to consider this as an aetiology in patients with advanced HIV/AIDS who present with acute abdomen, more so in patients from a distinct geographic region--South-East Asia.

CT Guided Aspiration of a Cervical Synovial Cyst. Case Report and Technical note.

SUMMARY: Synovial cysts are cystic dilations of synovial sheaths with or without anatomical continuity within a joint space that occasionally involve the lumbar spine. Image-guided aspiration has been used successfully in the management of lumbar synovial cysts. Synovial cysts of the cervical spine are rare, tend to present with myelopathic symptoms, and are classically treated using operative intervention.We report a novel approach for cervical synovial cyst aspiration under CT guidance. A 66-year-old woman presented to the clinic complaining of severe pain, tingling, and dysesthesia in her right upper extremity in the C5, C6 and C7 nerve root distribution. MR imaging of her cervical spine showed a synovial cyst within the right C6-C7 neural foramen with significant compression and obliteration of the C7 nerve root. The patient underwent CT-guided aspiration of the cyst using a double needle technique, where one needle was used to open up the epidural space while another aspirated the cyst. This was followed by epidural injection of local anesthetic and steroids. Immediately after injection, the patient experienced complete relief of her right forearm pain, tingling, and dysesthesias. The patient tolerated the procedure well and there were no postprocedure complications. At four year follow-up the patient is free of any symptoms. Image-guided aspiration of cervical synovial cysts is feasible using CT fluoroscopy. Aspiration of these cysts combined with epidural steroid injection can provide long-lasting symptomatic relief in some patients.

Minimally invasive approach for drainage of a sacral epidural abscess. A case report and technical note.

SUMMARY: Sacral epidural abscesses are rare infections, often managed with open surgery, especially in the presence of acute neurological symptoms. We report a novel approach for minimally invasive drainage of sacral epidural abscesses. A 51-year-old man presented to the emergency department complaining of low back pain, generalized muscle pain, pain across several large joints, low-grade fever, and weakness of both legs for ten days. MRI of the patient's lumbosacral spine showed osteomyelitis involving his L5, S1 vertebrae, L5-S1 discitis, as well as anterior and posterior epidural abscesses extending from L5-S1 disc space to the S2 vertebral level. Under CT fluoroscopic guidance a 20-gauge spinal needle was inserted into the sacral hiatus, parallel to the pelvic surface of the sacral canal, and directed cranially. A 0.18- gauge microwire was then advanced through the 20-gauge needle. The 20-gauge needle was exchanged over the guidewire for an 18-gauge blunt tipped needle which was curved to approximate the contours of the sacral canal. The curved needle was inserted through the sacral hiatus with its concavity initially facing upwards, and then rotated 180 degrees to gain access to epidural abscess. Once anatomic access was established 5cc of thick purulent material was evacuated. The patient tolerated the procedure well, and no focal nerve root symptoms were noted following the procedure. Image guided aspiration of sacral epidural abscesses can be carried out in a safe and effective manner using CT fluoroscopy. Aspiration of these abscesses combined with intravenous antibiotics may be an alternative to open surgery in select patients.

Spontaneous subdural hematoma in the setting of immune thrombocytopenia complicated by ischemic infarcts.

The occurrence of spontaneous subdural hematoma (SDH) in immune thrombocytopenia (ITP) is rare. We report a spontaneous subacute subdural hematoma in a patient with chronic ITP. The patient presented with headache and a noncontrast head CT scan showed an 11 mm subacute right frontoparietal SDH causing an 8 mm right to left midline shift. The patient underwent medical management with platelets, FFP and packed RBC transfusions, steroids, IVIG and mannitol, which failed to prevent deterioration of her clinical condition. The patient then underwent burr hole drainage of the SDH. Her postoperative course was complicated by ischemic infarcts in the right posterior cerebral artery territory leaving her with a residual left homonymous hemianopia. Our patient was unique in the concurrent development of ischemic infarcts postoperatively, following burr hole drainage of the SDH in the setting of ITP. This case highlights the diagnostic and therapeutic dilemmas involved in taking care of such patients.

Facial steal syndrome: identification and endovascular management.

We describe a case of intraprandial vertebro-basilar insufficiency secondary to posterior cerebral circulation ischemia. Secondary collateralization was seen from facial and external carotid artery branches to the posterior circulation causing facial arterial steal during mastication. This condition was treated endovascularly. Occluded common and external carotid arteries were revascularized via angioplasty and stenting. The patient was able to undergo coronary artery bypass and right hip replacement in the following two months without cerebrovascular complications.

Traumatic retropharyngeal pseudomeningocele with atlanto-occipital dislocation in a neurologically intact patient.

Traumatic retropharyngeal pseudomeningoceles occur rarely, are associated with severe trauma, and have been reported in patients with significant neurologic deficits at presentation. We report the rare occurrence of a pseudomeningocele following a high-speed motor vehicle accident. Neurological examination showed the patient to be briskly following commands, with intact cranial nerve, motor, and sensory function. CT/MR imaging showed subarachnoid hemorrhage involving the interpeduncular cistern, a clivus fracture, a right occipital condyle fracture, an atlanto-occipital subluxation, aortic arch transection (stable and contained on CT angiogram), multiple rib fractures on the right side with associated pneumothorax, hemothorax and pulmonary contusions. His cervical spine was stabilized in a halo. He was subsequently managed in the intensive care unit and remained neurologically intact. A repeat MRI showed the interval development of a 2×1.5 cm pseudomeningocele at the craniocervical junction medial to the left occipital condyle communicating with the left anterolateral aspect of the spinal canal. Traumatic pseudomeningoceles are associated with large deceleration forces at the time of injury and are usually associated with significant neurologic deficits at presentation. However, they can arise and give rise to symptoms in a delayed fashion in trauma patients who are neurologically intact at initial presentation.

Angiographic exclusion of intracranial arteriovenous malformations using onyx®.

Historically, endovascular treatment for cerebral arteriovenous malformations (AVMs) has been reserved as an adjunct to other modalities, namely radiosurgery and microsurgery, as the cure rate for cerebral AVMs treated solely with endovascular technique and older embolic agents is low. We report a series of five consecutive patients treated successfully with Onyx®, the newest available embolic agent, with resultant angiographic cure. Five patients aged three, nine, 33, 49, and 63 years were treated endovascularly for cerebral AVMs with a total of 19 embolizations with Onyx(®). All procedures were performed under general anesthesia with biplanar fluoroscopy. Complete angiographic obliteration of the AVM nidus was obtained in all cases with no residual filling. No immediate hemorrhagic or thromboembolic complications were observed. This early experience suggests that Onyx® has excellent embolization potential, and endovascular cure of AVMs may now be achieved with single modality therapy. Long terms data needs to be collected to further validate this observation.

Central neurocytoma: case report of an 81-year-old and histopathologic findings.

Central Neurocytomas are rare and usually benign tumors found primarily in the lateral ventricles of the brain. Central Neurocytomas are composed of uniform round cells exhibiting neuronal differentiation and are found almost exclusively in young adults between 15 and 60 years of age. The authors report the case of an 81-year-old man with a history of severe psychosis and depression, who presented with progressive confusion and ataxia likely unrelated to a central neurocytoma of his right lateral ventricle. The patient underwent a stereotactic biopsy of the lesion, followed by Gamma Knife radiosurgery. Histopathology showed immunohistological staining for synaptophysin, neuron specific enolase (NSE) and neuronal nuclear antigen (NeuN). A review of published literature on central neurocytomas revealed that this tumor occurs most frequently in young adults with a median age between 25 and 30 years. This pathology has never been reported in patients over the age of 80. Gamma Knife radiosurgery was successful in decreasing the tumor volume by 20% at the four month follow-up, but the fact that the patient died of unrelated occurrences in the elderly cannot be ruled out. Patients with central neurocytomas commonly present with obstructive hydrocephalus and immediate treatment may be necessary to tide over urgent situations.