Clinical and surgical management of a congenital Type II split cord malformation presenting with progressive cranial neuropathies: case report

Patrick R. Maloney, Meghan E. Murphy, Molly J. Sullan, Kathryn M. Van Abel, Shelagh A. Cofer, John C. Cheville, Nicholas M. Wetjen

Research output: Contribution to journalArticle

Abstract

Split cord malformation (SCM) is a rare abnormality of notochord development. The majority of cases occur in the thoracolumbar region, with more than 30 cases of cervical SCM reported. The clinical impact of SCMs involving the cervical cord is therefore largely unknown. In addition, the concomitant finding of brainstem involvement is presumably incompatible with life in the majority of patients, resulting in a paucity of data regarding this clinical scenario. In this paper the authors present the first case, to their knowledge, of an incomplete cervical SCM involving the brainstem and discuss its clinical impact, diagnosis, and management.

Original languageEnglish (US)
Pages (from-to)349-353
Number of pages5
JournalJournal of neurosurgery. Pediatrics
Volume19
Issue number3
DOIs
StatePublished - Mar 1 2017

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Keywords

  • AHI = apnea-hypopnea index
  • BAER = brainstem auditory evoked response
  • brainstem hypoplasia
  • cervical cord malformation
  • diplomyelia
  • duplicated spinal cord
  • FEES = functional endoscopic examination of swallowing
  • SCM = split cord malformation
  • spine
  • split cord malformation
  • TCS = tethered cord syndrome
  • unilateral vocal cord palsy

ASJC Scopus subject areas

  • Medicine(all)

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