Gamma knife radiosurgery for glomus jugulare tumors: Therapeutic advantages of minimalism in the skull base.

Context: Glomus jugulare (GJ) tumors are paragangliomas found in the region of the jugular foramen. Surgery with/without embolization and conventional radiotherapy has been the traditional management option. Aim: To analyze the efficacy of gamma knife radiosurgery (GKS) as a primary or an adjunctive form of therapy. Settings and Design: A retrospective analysis of patients who received GKS at a tertiary neurosurgical center was performed. Materials and Methods: Of the 1601 patients who underwent GKS from 1997 to 2006, 24 patients with GJ underwent 25 procedures. Results: The average age of the cohort was 46.6 years (range, 22-76 years) and the male to female ratio was 1:2. The most common neurological deficit was IX, X, XI cranial nerve paresis (15/24). Fifteen patients received primary GKS. Mean tumor size was 8.7 cc (range 1.1-17.2 cc). The coverage achieved was 93.1% (range 90-97%) using a mean tumor margin dose of 16.4 Gy (range 12-25 Gy) at a mean isodose of 49.5% (range 45-50%). Thirteen patients (six primary and seven secondary) were available for follow-up at a median interval of 24 months (range seven to 48 months). The average tumor size was 7.9 cc (range 1.1-17.2 cc). Using a mean tumor margin dose of 16.3 Gy (range 12-20 Gy) 93.6% coverage (range 91-97%) was achieved. Six patients improved clinically. A single patient developed transient trigeminal neuralgia. Magnetic resonance imaging follow-up was available for 10 patients; seven recorded a decrease in size. There was no tumor progression. Conclusions: Gamma knife radiosurgery is a safe and effective primary and secondary modality of treatment for GJ.

Idiopathic chronic subdural hematoma, MCA infarct and cortical atrophy with status epilepticus in infants.

Spontaneous chronic subdural hematomas in infants are extremely rare. A very limited number of cases are known and reported in literature. The clinical presentation can be myriad varying from asymptomatic cases to gross neurological deficits. We report the cases of 2 infants who presented to us with repeated episodes of generalized tonic clonic seizures since birth. No etiology could be established for either of them. Subsequent imaging revealed chronic subdural hematoma, MCA infarct and cortical atrophy in both the cases. Both the cases improved following evacuation of the chronic subdural hematoma. One of the infants had secondary craniosynostosis for which additional coronal suturectomy had to be done. The management of such cases and a brief review of literature are discussed.

Neuronavigation in a developing country: A pilot study of efficacy and limitations in intracranial surgery.

CONTEXT: Neuronavigation provides a patient-specific, three-dimensional (3-D) anatomy for preoperative planning and intraoperative navigation. However, the initial and maintenance costs are quite prohibitive, especially in the Indian scenario. AIMS: To study the efficacy and limitations of neuronavigation, especially in the Indian scenario. SETTINGS AND DESIGN: A prospective nonrandomized study. MATERIALS AND METHODS: A total of 121 patients underwent intracranial surgery from 2002-2006, in which neuronavigation was used. In this, the initial part, we studied the efficacy and limitations of neuronavigation in the initial 37 patients. The efficacy of the image guidance was graded according to a point's scale in which points were awarded ranging from 0 to 3. Cranial image guided score (IGS) was calculated by the summation of grading during designing the flap/burr hole, delineation of the intraoperative anatomy, navigation and access to the lesion and resection / biopsy of the lesion or completion of the procedure. The scoring ranged from 0-12 and the utility of IGS in cranial neurosurgical procedures was calculated based on the total points for each surgery. RESULTS AND CONCLUSION: Cranial image guidance was useful in a variety of operative steps. Intraoperative approach and navigation was relatively easier with an increase in perception of safety. Limitations of IGS include learning curve, cost and the phenomenon of brain shift. Drawbacks of the study included that this was a subjective rather than a truly objective study and the relatively lesser number of patients. We hope to conduct a larger study with randomization but the question of ethical approval would be a primary concern.

Comparative analysis of diagnostic accuracy of different brain biopsy procedures.

BACKGROUND: Image-guided procedures such as computed tomography (CT) guided, neuronavigator-guided and ultrasound-guided methods can assist neurosurgeons in localizing the intraparenchymal lesion of the brain. However, despite improvements in the imaging techniques, an accurate diagnosis of intrinsic lesion requires tissue sampling and histological verification. AIMS: The present study was carried out to examine the reliability of the diagnoses made on tumor sample obtained via different stereotactic and ultrasound-guided brain biopsy procedures. MATERIALS AND METHODS: A retrospective analysis was conducted of all brain biopsies (frame-based and frameless stereotactic and ultrasound-guided) performed in a single tertiary care neurosciences center between 1995 and 2005. The overall diagnostic accuracy achieved on histopathology and correlation with type of biopsy technique was evaluated. RESULTS: A total of 130 cases were included, which consisted of 82 males and 48 females. Age ranged from 4 to 75 years (mean age 39.5 years). Twenty per cent (27 patients) were in the pediatric age group, while 12% (16 patients) were >or= 60-years of age. A definitive histological diagnosis was established in 109 cases (diagnostic yield 80.2%), which encompassed 101 neoplastic and eight nonneoplastic lesions. Frame-based, frameless stereotactic and ultrasound-guided biopsies were done in 95, 15 and 20 patients respectively. Although the numbers of cases were small there was trend for better yield with frameless image-guided stereotactic biopsy and maximum diagnostic yield was obtained i.e, 87% (13/15) in comparison to conventional frame-based CT-guided stereotactic biopsy and ultrasound-guided biopsy. CONCLUSIONS: Overall, a trend of higher diagnostic yield was seen in cases with frameless image-guided stereotactic biopsy. Thus, this small series confirms that frameless neuronavigator-guided stereotactic procedures represent the lesion sufficiently in order to make histopathologic diagnosis.

Craniofacial surgery for craniometaphyseal dysplasia.

Craniometaphyseal dysplasia (CMD) is a rare congenital bone dysplasia with abnormal bony overgrowth leading to characteristic facial features and cranial nerve compression. We present a 10-year-old child with bony swelling at the nasal root since birth along with decreased hearing in both ears. She had normal developmental milestones and intelligence. On examination, she had bossing of forehead with very broad nasal root, short septum, hypertelorism and epicanthic folds. CT scan with 3D reconstruction revealed grossly thickened calvarium and hyperostosis and sclerosis of the cranial base. As the major concern of the parents was cosmetic, craniofacial reconstruction was performed with good cosmetic outcome.

Spontaneous subdural empyema in falciparum malaria: a case study.

CLINICAL HISTORY: Malaria is one of the most common diseases in the tropical countries. Cerebral malaria is usually a diffuse symmetric encephalopathy with focal signs being unusual. METHODS : We present a three-year old girl lapsing into unconsciousness following a seizure while undergoing treatment for malaria. Imaging revealed a large heterogenous density, left hemispheric acute subdural haematoma with brain herniation. Investigations revealed anaemia, thrombocytopenia and positive peripheral blood smear for falciparum malaria. RESULTS: Treatment involved surgical evacuation of the clot and the associated subdural empyema, intravenous quinine and antibiotics. CONCLUSION: This is the second case report of spontaneous subdural empyema in complicated falciparum malaria and highlights a rare but surgically manageable complication.

Brain abscess--an unusual complication of ventriculo-peritoneal shunt.

The authors report a case of a 10-year-old boy, who was operated for a craniopharyngioma with hydrocephalus. He succumbed to delayed development of brain abscess and its consequence, secondary to incompletely treated shunt tract infection. This unfortunate complication developed, despite the fact that conventional steps (removal of VP shunt and intravenous antibiotics) recommended for the treatment of VP shunt infection were adopted. The pathogenesis, management and review of literature of this extremely rare clinical entity is presented.

Choroid plexus papilloma of the posterior third ventricle during infancy & childhood: report of two cases with management morbidities.

We report two cases of posterior third ventricular choroid plexus papilloma, one in an 8-month-old infant and another in a two-year-old child. These cases presented with features of obstructive hydrocephalus. Both these patients underwent a ventriculo-peritoneal (VP) shunt surgery prior to the tumor excision. Following the VP shunt surgery both patients developed ascitis requiring exteriorization of the abdominal end of the shunt. There was a clear proof of CSF overproduction: 1400-1500 ml/day in the eight-month-old infant and 900-1200 ml/day in the two-year-old child. In the former it was transient and could be treated with revision of the VP shunt whereas in the second case a ventriculo-arterial shunt had to be done. In the second case a staged reduction cranioplasty was also performed for an enormously enlarged head (head circumference--74 cm). Interesting clinical and radiological findings and useful management strategies are described.

Spontaneous subdural hematoma in a young adult with hemophilia.

We report a case of spontaneous acute subdural hematoma in a 30-year-old man, who was diagnosed with hemophilia during his hospital stay. He developed an extradural hematoma following evacuation of the acute SDH, which was also evacuated. He had a good outcome. Management of such a patient is discussed.

Craniofacial surgery and optic canal decompression in adult fibrous dysplasia.

A 29-year-old female had a 3-year history of bony swelling over the right frontal area. For 3 months she noticed proptosis of her right eye. Investigations revealed fibrous dysplasia involving the right half of the frontal bone and the right greater and lesser wings of the sphenoid bone. Visual evoked potentials (VEP) showed delayed latencies on the involved side. A craniofacial surgery with optic canal decompression was performed. Follow-up after 2 years revealed normalization of VEP.

Sudden bilateral foot drop: an unusual presentation of lumbar disc prolapse.

Bilateral acute foot drop is reported in a 30-year-old healthy male. He presented with a 7-day history of sudden severe backache, radiating to both the lower limbs and 1-day history of sudden bilateral ankle weakness that progressed to bilateral foot drop within 6 hours. He also developed retention of urine. Investigations revealed a large central disc prolapse at L3-4 with significant canal stenosis at that level. Following surgery the patient had progressive improvement.

Posterior fossa extradural hematoma.

OBJECTIVE: To review the clinical profile, radiology and treatment of Pediatric posterior fossa extradural hematoma (PPFEDH) and evaluate the outcome. METHODS: Posterior fossa extradural hematoma (PFEDH) is an uncommon trauma sequel and in pediatric age group is still rare. This article discusses 18 cases of PPFEDH seen over a period of 8 years and review the pertinent medical literature. Only a few studies discuss PPFEDH and in most of them outcome has been good. Trauma has been subtle and fracture of occipital bone is seen in majority of cases. Treatment has been operative in majority of the cases. RESULTS: Most of the patients were diagnosed only on high index of suspicion with a history of trauma on the occipit, fracture of the occipital bone and persistent symptoms suggestive of increasing intracranial pressure and posterior fossa space occupying lesion. CT scanning was done liberally to confirm the suspicion and the patients with hematoma were treated aggressively by surgical evacuation with good recovery in most of them. CONCLUSION: Authors suggest an aggressive approach in the event of PPFEDH. However some patients can be managed conservatively but they should be closely observed for clinical manifestation and CT scan routinely to evaluate the progress of hematoma radiologically. A strict criterion should be followed in choosing such patients for conservative treatment as highlighted in the study.

An unusual presentation of an intra-parenchymatous frontal yolk sac tumour : case report.

Yolk sac tumours are rare conditions among the germ cell tumours. Intracerebral germ cell tumours are exceedingly rare. A 15 year old girl presenting with a one week history of raised intracranial pressure is described. She had bilateral papilloedema and a right 6th nerve palsy. CT scan showed an intra-parenchymatous right frontal ring enhancing lesion of 2 cms diameter. The patient underwent microsurgical total excision of the tumour, followed by chemotherapy. She was asymptomatic at three years following surgery.

Hypersensitivity to glucocorticoids in patients with raised ICP : report of two cases.

Corticosteroids are widely used in patients with raised intracranial pressure associated with cerebral neoplasms, cerebral vascular malformations, cerebral ischaemia and benign intracranial hypertension. In general clinical practice, anti-allergic, anti-inflammatory and immuno-suppressive properties of corticosteroids are commonly utilised in the management of allergic and immunological diseases. However in exceptionally rare circumstances, steroids may be the cause of hypersensitive reactions. Authors report two patients with raised intracranial pressure who developed steroid hypersensitivity. A review of the relevant literature is discussed.

Spinal congenital dermal sinus: an experience of 23 cases over 7 years.

Spinal congenital dermal sinus is a rare entity, which supposedly results from the failure of neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The present study was undertaken to know the clinical profile of these patients, to study associated anomalies and to assess the results of surgical intervention. We had 23 patients with male : female ratio of 9:16. Only 2 patients were below 2 years of age and most cases (16) were between 2-16 years (mean age =10.2 years). Lumbar region (17 cases) was most frequently involved, followed by lumbosacral and thoracic region in 3 patients each. Only three patients were asymptomatic at the time of presentation. Most of the cases presented with evidence of neural compression or tethered cord syndrome. Only one case presented with spinal abscess. The motor, sensory and autonomic deficits were seen in 20, 11 and 12 patients respectively. Scoliosis and CTEV (congenital talipus equino varus) were the common associated anomalies. MRI revealed associated dysraphic state of spinal cord in 21(>90%) cases. All patients underwent surgical exploration and repair of dysraphic state and excision of the sinus. None of the asymptomatic patients deteriorated. Overall 8 patients improved, 14 got their neurological status stabilized, including 3 asymptomatic cases. Only one patient deteriorated. Postoperative wound infection was seen in 2 cases. As age advances, the chance of developing neurological deficit increases. Associated dysraphic state should be looked for and treated simultaneously, using microsurgical technique, whenever possible. It is better to treat all these cases with aggressive surgical intervention before the neurological deficits appear.

Sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh.

Malignant peripheral nerve sheath tumour (MPNST) is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot.

Nosocomial infections due to Acinetobacter baumannii in a neurosurgery ICU.

Invasive infections caused by Acinetobacter baumannii in a post-operative neurosurgery ICU were studied. Sixty one patients admitted during a span of 11 months were culture positive for acinetobacter species from blood and/or CSF samples. They were followed up prospectively for evidence of infection and clinical outcome. 40 cases had clinical evidence of infection due to acinetobacter species while in 21 patients, the isolation of the organism was considered a contaminant. Acinetobacter baumannii was the most common organism associated with invasive infections. Respiratory tract was found to be the most common primary source of infection in patients with bacteraemia or meningitis. The age, sex and pre-operative hospital stay were not significantly different in the two groups (p>0.05), while post-operative hospital stay and mortality was significantly higher in patients with invasive infection (p<0.05). Acinetobacter baumannii was isolated from multiple sites (p<0.05) and repeatedly from the same site (p<0.001) in a significantly higher number of patients with invasive infections. Mortality was high in the patients infected with Acinetobacter baumannii. Even amongst the infected group, the patient shaving meningitis showed a higher mortality as compared to the patients having bacteraemia.

Giant ocular nerve neurofibroma of the cavernous sinus: a series of 5 cases.

Five cases of giant cavernous sinus neurofibroma arising from the ocular cranial nerves are reported. These patients collected over a period of 5 years consisted of 3 males and 2 females with an age range of 9 to 40 years and a mean of 20.6 years. Clinically, all patients presented with ocular palsies over a long period (mean 3.4 years). All of them underwent a frontotemporal craniotomy along with an orbito-zygomatic osteotomy and excision of the tumour. In patients with extension of the tumour into the orbit, the superior orbital fissure was drilled, the tenon's capsule was cut and the intraorbital portion was excised. The management of these tumours is discussed and the literature reviewed.