Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis - A case report and review of the literature

Chia Chun Chiang, Shih Chieh Lin, Hsiu Mei Wu, Jia Chi Wang, Tsui Fen Yang, Hsin Hung Chen, Donald M. Ho, Tai-Tong Wong

Research output: Contribution to journalArticle

6 Citations (Scopus)


Introduction: Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida. Case report: Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant.

Original languageEnglish
Pages (from-to)13-17
Number of pages5
JournalChild's Nervous System
Issue number1
Publication statusPublished - Jan 1 2014
Externally publishedYes



  • Compressive cervicalmyeloradiculopathy
  • Encephalocraniocutaneous lipomatosis
  • Spine
  • Surgical decompression

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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