Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state.

INTRODUCTION: The authors describe the case of a 29-month-old boy who presented with acute non-communicating hydrocephalus caused by a small tumor in the fourth ventricle. He became brain-dead immediately and remained stable in that condition. MATERIALS AND METHODS: Six months later, despite being in a brain-dead state, a rapid direct tumor extension from the intracranial to extracranial region was observed, and chemoradiotherapy was performed following tumor biopsy. The histopathological diagnosis was large cell medulloblastoma. Although treatment was initially effective, the tumor again aggressively invaded the cervical muscles via the spinal canal. Comparative genomic hybridization (metaphase) analysis revealed a pattern of aberrations predictive of a poor prognosis (+1q, ?17p, +17q, and probable amplification of c-myc gene), and he eventually died 11 months after onset. RESULTS: Direct invasion of medulloblastoma from the intracranial to extracranial region is extremely rare, and, to our knowledge, this is the first report of medulloblastoma exhibiting rapid extension to the extracranial region in brain-dead state. CONCLUSIONS: For patients with medulloblastomas, careful observation is needed even in brain-dead state. The etiology of this rare condition as well as the genetic characteristics responsible for aggressive tumor behavior are discussed.

[Clinical prognosis and therapeutic aspects in management of pediatric moyamoya disease].

We reviewed the clinical prognosis and therapeutic aspects in the management of pediatric moyamoya disease from our data base for the last 8 years since establishment of our institution. We found 99 cerebrovascular disease cases among the total of 1,159 cases, and 20 out of them were moyamoya disease. Only 13 cases were analyzed as the rest were not available for follow up. Nine girls and 4 boys were admitted with an average age of 5 years-old 4 months. Initial symptom were TIA in 5, cerebral infarction in 5, migraine in 2, and epilepsy in one. The type of moyamoya disease was bilateral type in 10 cases, and unilateral type in 3 cases. Clinical condition and investigations, as well as the effect of conservative management, required surgery in 9 cases (8 indirect revascularization and one direct-bypass surgery), and the rest 4 cases were managed conservatively. Only 10 cases were followed-up, 9 operated cases and one conservatively managed case. In 6 of 9 of the operated cases, there was recurrence: cerebral infarction in 3, TIA recurrence in 2 and epileptic seizure in one. On MRA a steno-occlusive lesion of PCA was found in 4 of the 6 recurrence patients, and improvement was achieved by additional surgery in one of them. All of the cases developed good postoperative collateral circulation. The conservatively treated patient did not show neither steno-occlusive lesion of PCA, nor a clinical recurrence for 7 years observation. Therefore, posterior circulation system in moyamoya disease is important as a collateral circulation in addition to the revascularization procedures, and we believe that the progression of the steno-occlusive lesion of PCA is an important indicator, predicting recurrence.

Ventricular anatomy of hydrocephalus associated with myeloschisis and endoscopic third ventriculostomy.

OBJECTS: To characterize the anatomical features of the ventricular regions in patients with myeloschisis and related to successful performance of endoscopic third ventriculostomy (ETV). METHODS: Radiological and endoscopic findings of 21 myeloschisis patients were retrospectively reviewed. Radiological features that could interfere with endoscopic procedures were (1) a huge massa intermedia (12/19), (2) sloping of the third ventricular floor (3/10), (3) narrow anteroposterior length of the third ventricular floor (2/10), and (4) narrow prepontine cistern (8/21). Endoscopic findings were (a) a narrow foramen of Monro (0/3), (b) hypertrophy of the anterior commissure (1/3), (c) sloping of the third ventricle floor (1/3), (d) a huge massa intermedia (3/3), and (e) opaque third ventricular floor (3/3). These endoscopic findings did not interfere with endoscopic procedures by using the Oi-HandyPro neuroendoscope without the above-mentioned radiological features 3 or 4. CONCLUSION: Narrow anteroposterior length of the third ventricular floor and narrow prepontine cistern are not infrequently observed. Preoperative evaluation and intraoperative inspection of these findings are very important in successful performance of ETV.