Encephalocraniocutaneous lipomatosis: a review of its clinical pathology and neurosurgical indications

Journal of Neurosurgery. Pediatrics
Robert E Ayer, Alexander Zouros


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.


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Mar 15, 2006·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·R Shane TubbsW Jerry Oakes
Nov 15, 2007·American Journal of Medical Genetics. Part a·Ute MoogVirginia E Kimonis
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Jul 16, 2009·Spine·Mario Cardoso, Robert F Keating

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Aug 27, 2013·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Chia-Chun ChiangTai-Tong Wong
Jan 5, 2014·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Hali Bauld, P Daniel McNeely
Mar 3, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Zehra Filiz Karaman, Şerife Ebru Özüdoğru

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