Surgery for subependymal giant cell astrocytomas in children with tuberous sclerosis complex

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Martine FohlenGeorg Dorfmüller

Abstract

Subependymal giant cell astrocytomas (SEGAs) are low-grade intraventricular glial tumors that develop in 10-15% of patients with tuberous sclerosis complex; they often cause hydrocephalus and are potentially accessible to a surgical treatment. Our aim is to evaluate morbidity and results after surgery in symptomatic and asymptomatic patients. We present a retrospective series of 18 pediatric patients operated on for SEGA between 2006 and 2016 at our institution. We reviewed surgical indications, preoperative clinical and radiologic data, surgical management, and clinical and radiological follow-up. Mean age at surgery was 10.7 years. The surgical decision was based on clinical signs of raised intracranial pressure due to hydrocephalus in 8 and on radiological findings without any clinical signs in the other 10 patients (increased in SEGA volume with or without ventricular enlargement). Surgical treatment consisted in a frontal trans-ventricular microsurgical approach in 17 patients and an endoscopic approach in 1. External ventricular drainage was placed in all the patients but 1. Ventriculoperitoneal shunting (VPS) became necessary in 6 patients, all of them presenting with a preoperative active hydrocephalus. Morbidity appear...Continue Reading

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Citations

Feb 28, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Martine FohlenRenaud Touraine
Sep 27, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Paolo FrassanitoGianpiero Tamburrini
Aug 17, 2021·Pediatric Neurology·Hope NorthrupUNKNOWN International Tuberous Sclerosis Complex Consensus Group

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