title: Encephalocraniocutaneous lipomatosis: a review of its clinical pathology and neurosurgical indications.
abstract: Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome whose hallmark lesions are benign lipomas of the brain and spinal cord. The authors present a case of a male infant with ECCL who had extensive brainstem and spinal cord lipomas. The management of this patient's hydrocephalus, cervicomedullary compression, tethered cord, and scoliosis over the course of his first 2 years of life is described. This case report and review of the literature is presented to provide a synopsis of the problems likely to be encountered by neurosurgeons who treat patients with this syndrome.
authors: Department of Neurosurgery, Loma Linda University Medical Center, Loma Linda, California 92354, USA. rayer77@yahoo.com
authors: Robert E
authors: RE
authors: Ayer
authors: Alexander
authors: A
authors: Zouros
mesh_terms: D005128:Eye Diseases / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000473:pathology / Q000601:surgery; D007223:Infant; D008068:Lipomatosis / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D008297:Male; D009436:Neural Tube Defects / Q000209:etiology / Q000473:pathology / Q000601:surgery; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D019635:Neurosurgical Procedures; D012600:Scoliosis / Q000209:etiology / Q000473:pathology / Q000601:surgery