Hydrocephalus in neurofibromatosis. Contribution of magnetic resonance imaging to its diagnosis, control and treatment.
Cerebral ventricular dilation and/or frank hydrocephalus unrelated to brain tumors is not a rare feature of von Recklinghausen neurofibromatosis (NF-1). In our experience, it was observed in 23% of patients consulting for neurological problems. From 1984 to 1989, 30 patients with documented NF-1 were referred to for cranial or spinal magnetic resonance imaging (MRI). In 9 of these patients, clinical features or previous computed tomographic scanning revealed hydrocephalus or ventriculomegaly. One patient had biventricular hydrocephalus (enlarged lateral ventricles), 7 had triventricular hydrocephalus (aqueductal stenosis) and 1 had tetraventricular hydrocephalus (obstruction of the foramina of Magendie and Luschka). MRI showed anomalies in all 9 patients. The importance of MRI for the diagnosis, control and treatment of the hydrocephalus as well as for understanding the embryologic pathogenesis of these conditions is emphasized.
Pou-Serradell
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Department of Neurology, Hospital Nuestra Señora del Mar, Autonomous University of Barcelona, Spain.
Ugarte-Elola
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D000328:Adult; D002557:Cerebrospinal Fluid Shunts; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000601:surgery