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.
MeSH terms
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Eye Diseases / complications
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Eye Diseases / pathology*
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Eye Diseases / surgery*
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Humans
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Hydrocephalus / etiology
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Hydrocephalus / pathology
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Hydrocephalus / surgery
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Infant
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Lipomatosis / complications
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Lipomatosis / pathology*
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Lipomatosis / surgery*
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Male
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Neural Tube Defects / etiology
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Neural Tube Defects / pathology
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Neural Tube Defects / surgery
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Neurocutaneous Syndromes / complications
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Neurocutaneous Syndromes / pathology*
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Neurocutaneous Syndromes / surgery*
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Neurosurgical Procedures*
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Scoliosis / etiology
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Scoliosis / pathology
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Scoliosis / surgery
Supplementary concepts
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Encephalocraniocutaneous lipomatosis