Fetal radiation exposure and moyamoya disease.

Moyamoya disease is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis. We present the first reported patient with moyamoya disease possibly due to radiation exposure during fetal development. A 10-year-old male, whose mother had undergone radiotherapy when pregnant, presented with moyamoya disease. The relevant literature is discussed.

Suprasellar haemangiopericytoma--challenges in diagnosis and treatment.

Haemangiopericytomas of central nervous system (CNS) were first defined as a separate entity in 1942. Previously they were either considered to be a histological variant of an angioblastic meningioma or a distinctive mesenchymal neoplasm. Most commonly they are located in parasagittal and falcine region. Tumours in the sellar/parasellar location are very rare and commonly escape diagnosis before operation. They are characterised by high vascularity, a high rate of local recurrence and extraneuronal metastasis. We report a 35-year-old man with a suprasellar hemangiopericytoma who presented with bilateral diminution of vision in both eyes and frontal headache. Six months after the first operation, he developed a large local recurrence. He again underwent tumour decompression followed by postoperative conformal radiotherapy and is currently asymptomatic and stable clinically and radiologically. The various differential diagnoses, the importance of a preoperative suspicion of this diagnosis and management are issues discussed in this illustrated review.

Aspergilloma of the brain: an overview.

Fungal infections of the central nervous system (CNS) are almost always a clinical surprise. Their presentation is subtle, often without any diagnostic characteristics, and they are frequently mistaken for tuberculous meningitis, pyogenic abscess, or brain tumor. Granulocytopenia, cellular and humoral mediated immune dysfunction are predisposing factors to the development of CNS infections in immunosuppressed patients. Aspergillus fumigatus is the most common human pathogen in the genus Aspergillus. Maxillary sinusitis of dental origin or the lungs are the most common sites of primary Aspergillus infection. Infection reaches the brain directly from the nasal sinuses via vascular channels or is blood borne from the lungs and gastrointestinal tract. Single or multiple abscess formation with blood vessel invasion leading to thrombosis is a characteristic feature of Aspergillosis on neuropathologic examination. Aspergillosis should be considered in cases manifesting with acute onset of focal neurologic deficits resulting from a suspected vascular or space-occupying lesion especially in immunocompromised hosts. Aspergillosis is diagnosed on direct examinations and culture, however the diagnosis of aspergillosis of the CNS is difficult. Diagnosis of an intracranial mass lesion is best confirmed with a computed tomography or magnetic resonance imaging of the head with or without intravenous contrast. Aggressive neurosurgical intervention for surgical removal of Aspergillus abscesses, granulomas, and focally infracted brain; correction of underlying risk factors; Amphotericin B combined with flucytosine and treatment of the source of infection should form the mainstay of the management. Off late Liposomal Amphotericin B was found to be more effective and safe than conventional Amphotericin B in the management of Apergillus infections Only with a high index of suspicion, an aggressive approach to diagnosis, and rapid vigorous therapy may we hope to alter the clinical course in this group of patients.

Intradiploic meningioma of the orbit: a case report.

We report a case of a 12-year-old male child who presented with a gradual onset exopthalmos involving the left eye. The plain radiographs of the skull showed hyperostosis of the left orbital roof. The computerized tomography (CT) scan revealed an intradiploic orbital roof tumor with expansion of both the tables of the orbital roof. The tumor was completely excised by an extradural route using a basal frontal craniotomy. The histopathological diagnosis of the tumor was a psammomatous meningioma. A split calvarial frontal bone graft was used to reconstruct the orbital roof. The pathogenesis, radiological features and surgical technique involved in the management of intradiploic orbital roof meningioma are discussed and the relevant literature is reviewed.

Suprasellar arachnoid cyst presenting with bobble-head doll movements: a report of 3 cases.

We report a series of three patients with suprasellar arachnoid cysts who presented with a rare 'bobble-head doll' syndrome. The abnormal head movements improved after surgical evacuation of the cysts in all the three cases. Various pathophysiological mechanisms involved in the bobble-head doll syndrome are discussed. The literature on suprasellar arachnoid cysts is briefly reviewed.

Ischaemic complications after surgical resection of intracranial aspergilloma.

A 32-year-old male presented with a paranasal sinus Aspergillus fungal infection. The nasal infection was cleared by endoscopic sinus surgery and the patient was treated with antifungal agents. The patient was otherwise healthy with no evidence of immunosuppresion. Five months later, the patient had to undergo excision of the left frontal intracranial infection for symptoms of raised intracranial pressure and seizures. Within 48hours of surgery, the patient developed basilar artery thrombosis with infarction of the cerebellum and midbrain. The patient succumbed to this vascular catastrophe, which developed at a site distal to that from surgery. The pathophysiology of ischaemic complications after surgical resection of intracranial aspergilloma is discussed.

En-plaque ventricular ependymoma.

An extremely unusual case demonstrating rare behaviour of an ependymoma is presented. The tumour spread in an en-plaque fashion and involved the ependymal lining of the entire ventricular system. The patient, a 56-year-old male, presented with symptoms of raised intracranial pressure and truncal ataxia. The management issues are analyzed on the basis of a brief literature survey.

Cavernous sinus thrombosis and air embolism following surgery for acoustic neurinoma: a case report.

A 55 year old male patient was operated on for a massive and vascular acoustic neurinoma in a sitting position. The tumor was completely excised. Post-operatively, the patient developed irritability and clinical features suggestive of contralateral cavernous sinus thrombosis. CT scan showed air within the dural walls of the cavernous sinus on the side of surgery. However, there was no radiological evidence of cavernous sinus thrombosis on the contralateral side. Cavernous sinus thrombosis as a post-surgery complication has not been reported. Air within the dural confines of the cavernous sinus has also not been observed or radiologically recorded in the literature.

An unusual post-traumatic occipitocervical pseudomeningocele: case report.

BACKGROUND: A rare case of massive post-traumatic subcutaneous pseudomeningocele probably communicating with the cisterna magna, is reported. CASE DESCRIPTION: An 8-year-old boy sustained a severe injury to the nape of the neck, after which he developed a huge local subcutaneous swelling containing cerebrospinal fluid (CSF). Communication of the subcutaneous CSF collection with the cisterna magna or any other site of dural fistula could not be identified. After a lumbo-peritoneal CSF shunt, the swelling resolved completely. CONCLUSION: The clinical features of a rare case of symptomatic post-traumatic cerebrospinal fluid pseudomeningocele are elaborated.

Rupture of a giant posterior inferior cerebellar artery aneurysm in an infant following a ventriculoperitoneal shunt--Case report.

A 4-month-old female child presented with a huge posterior fossa mass lesion and severe hydrocephalus. Six hours after a ventriculoperitoneal shunt procedure, her condition worsened and she died. Autopsy showed extensive intracranial hemorrhage due to rupture of a posterior inferior cerebellar artery aneurysm. The probable causes of the rupture of the aneurysm were mechanical effects or changes in cerebral blood flow.

Midline posterior fossa teratoma--case report.

A 20-day-old female neonate presented with an immature teratoma in the midline posterior fossa. The tumor was totally removed but the patient died of pneumonia. Teratoma is a rare tumor, but very difficult to treat as the patients tend to be young, and the outcome is very poor.

Prognostic factors for cerebellar astrocytomas in children: a study of 102 cases.

OBJECTIVE: To evaluate the prognostic factors influencing the length of survival of pediatric patients with cerebellar astrocytomas. METHODS: The clinical data of 102 patients under the age of 12 years with cerebellar astrocytomas were retrospectively analyzed. The radiological features, surgical findings, histology and adjuvant radiotherapy were reviewed. Patients were followed up on an outpatient basis. Computerized tomography (CT) scans of the brain were performed to detect recurrence. RESULTS: The age of the patients at presentation varied from 10 months to 12 years. The mean age at presentation was 7 years and 11 months. The average duration of symptoms was 5.8 months. The clinical features were predominantly related to intracranial hypertension and the location of the tumor. Twenty-six tumors were located in the vermis and 76 in the cerebellar hemisphere. The brain stem was involved in 20 patients. All 102 patients had a preoperative contrast-enhanced CT scan. Midline vermian tumors were predominantly solid and enhancing, whilst the hemispheric tumors were cystic and nonenhancing. The tumors were graded into three subgroups based on histological characteristics: pilocytic astrocytoma, low-grade fibrillary astrocytoma and high-grade fibrillary astrocytoma. Total, radical and subtotal excision, as determined by the surgical impression, were performed in 82, 16 and 4 patients, respectively. Postoperative radiotherapy was administered to 12 patients. CSF diversion was carried out in 11 of 79 cases who had moderate to severe hydrocephalus. CONCLUSIONS: Our study has shown that the location of the tumor (p < 0.05), histological grade (p < 0.001) and the extent of tumor resection (p < 0.001) have a significant and definitive relationship to the length of survival of pediatric patients with cerebellar astrocytomas. The patient's age (p > 0.05) does not influence the outcome. We could not evaluate the effect of brain stem involvement and adjuvant radiotherapy in our study because of the small sample size.

Massive aneurysmal bone cyst of the anterior cranial fossa floor--case report.

A 19-year-old male patient presented with a midline facial, nose, and forehead hard and bony swelling associated with hypertelorism. Neuroimaging revealed a massive tumor involving the anterior cranial fossa floor, which had occupied and enlarged all paranasal air sinuses, and displaced the orbits outwards and the frontal lobes of the brain superiorly. A basal transcranial route was used for radical resection of the massive and vascular tumor. Histological examination confirmed an aneurysmal bone cyst. Such tumors only rarely involve the cranial bones or paranasal air sinuses.

Pathological laughter as a presenting symptom of massive trigeminal neuromas: report of four cases.

OBJECTIVE AND IMPORTANCE: This is a report of four cases of huge trigeminal neuromas that presented with the principle symptom of pathological laughter (PL). CLINICAL PRESENTATION: All four patients were male and were in either the third or fourth decade of life. In addition to PL, there were neurological deficits related to trigeminal nerve, brainstem, and cerebellar dysfunctions. INTERVENTION: All tumors were radically excised via a lateral basal temporal approach. The PL was cured immediately after surgery. CONCLUSION: PL sometimes precedes other neurological manifestations and may be a useful localizing sign. The clinical and radiological features in our cases suggest that PL is a result of extra-axial compression of the pons and adjoining neural structures.

Primary petrous bone Ewing's sarcoma.

A case of primary Ewing's sarcoma involving a large portion of the petrous temporal bone in an eighteen month old child is reported. The lesion was treated by radical surgery and adjuvant chemotherapy and radiotherapy. An extremely rare age of presentation, an unusual location and an excellent response to treatment are the highlights of this case.

Cavernous malformation of the pineal region: short report and review of the literature.

A case of a cavernous malformation of the pineal region is reported. Unique magnetic resonance images of a histologically-verified cavernous malformation of the pineal region are presented and the relevant literature on the subject is reviewed.