Surgical treatment of intraventricular tumors associated with tuberous sclerosis

Abstract

Six children with intraventricular tumors associated with tuberous sclerosis (TS) were treated at the Children's Health Center, Department of Pediatric Neurosurgery, in the period 1987-1992. The age of the patients ranged from 7 to 15 years. TS was diagnosed according to Gomez diagnostic criteria. Computer tomography (CT) and magnetic resonance imaging showed intraventricular tumors associated with ventricular enlargement and multiple subependymal nodules commonly observed in cases of TS. All tumors were removed totally through frontal transcortical approach, with uneventful postoperative recovery. One patient, with two parallel tumors in the two frontal horns, underwent one-stage surgery with successful total removal. Histopathological examination in all cases showed subependymal giant cell astrocytoma (SGCA). The growth pattern of SGCA associated with TS, documented by sequential CT scans over several years, is described. The diagnosis and surgical treatment of the tumor are discussed, and periodic CT scanning, at least every 2 years, is recommended for patients with TS.

Transcortical surgery for lateral ventricular tumors

“…These symptoms can include headache, imbalance, visual field deficits, memory difficulty, personality changes, cognitive impairment, weakness, and seizures. 9,16,23,25,30,35,40 The signs and symptoms prior to diagnosis in this series were typical of patients with largesized tumors located in the lateral ventricle. The majority of the patients were children who presented with signs of increased ICP.…”

“…48 Transcortical approaches to the lateral ventricle often broach the tracts of the visual pathways, resulting in varying degrees of temporary and permanent deficits (Tables 2-4). 16,30,35,40 The optic tract can be found in the superior medial region of the temporal horn as it courses toward the lateral geniculate body. The radiations then spread toward the calcarine cortex along the Meyer-Archambault loop in the superior and lateral aspect of the temporal horn and in the tapetum over the roof and lateral aspect of the atrium and occipital horn.…”

“…In this location, they often reach a large size before being diagnosed. 24,31,40 Astrocytoma can be found in all parts of the ventricle and frequently will arise from the thalamus, where, based on MR documentation, they can appear to infiltrate. Ependymoma, when supratentorial, can be intraventricular as well as intraparenchymal.…”

“…The deaths in the microsurgical era are usually secondary to catastrophic postoperative hemorrhage or pulmonary emboli. 1,10,16,19,30,31,34,37,40 Nonetheless, even with our current technology a flexible surgical approach is important. A staged removal of a very large, vascular lesion via combined approaches is preferable to a single, long, difficult procedure.…”

Giant Cells: Contradiction to Two-Hit Model of Tuber Formation?

“…From our previous observations, we hypothesize that subependymal nodules may grow and differentiate into SEGAs (Roszkowski et al, 1995). Nevertheless, even benign brain lesions in TSC lead to seizures, mental retardation and autism (Jozwiak et al, 1998).…”