Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome

Devorah Segal, Robert Heary, Sanjeev Sabharwal, Maureen T. Barry, Xue Ming

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1-3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement. Surgery was complicated by bleeding from intracranial vascular malformations. This report describes a very rapidly developing, massive holocord syringomyelia related to CSF obstruction due to an unusual congenital brain malformation and associated vascular overgrowth at the site. Serial, premorbid MRI studies demonstrated the very rapid progression from no Chiari malformation, to progressively greater cerebellar tonsillar herniation, to holocord syrinx. This complication has never been reported in MCAP syndrome and should be considered in any affected MCAP patient with a progressive neurological decline, even if previous spine imaging findings were normal. Surgical complications due to hemorrhage also need to be considered in this vascular brain malformation.

Original languageEnglish (US)
Pages (from-to)79-82
Number of pages4
JournalJournal of Neurosurgery: Pediatrics
Volume18
Issue number1
DOIs
StatePublished - Jul 1 2016

Fingerprint

Megalencephaly
Syringes
Syringomyelia
Central Nervous System Vascular Malformations
Encephalocele
Hemorrhage
Vascular Malformations
Laminectomy
Scoliosis
Leg
Spinal Cord
Seizures
Spine
Capillary Malformations, Congenital, 1
Brain
Polymicrogyria

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Segal, Devorah ; Heary, Robert ; Sabharwal, Sanjeev ; Barry, Maureen T. ; Ming, Xue. / Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome. In: Journal of Neurosurgery: Pediatrics. 2016 ; Vol. 18, No. 1. pp. 79-82.
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Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome. / Segal, Devorah; Heary, Robert; Sabharwal, Sanjeev; Barry, Maureen T.; Ming, Xue.

In: Journal of Neurosurgery: Pediatrics, Vol. 18, No. 1, 01.07.2016, p. 79-82.

Research output: Contribution to journalArticle

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