Neurosurgical management in lateral meningocele syndrome: case report

Erik C. Brown, Kunal Gupta, Christina Sayama

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder. It is associated with morphological changes similar to those of other connective tissue disorders, with the unique distinction of multiple, often bilateral and large, lateral meningoceles herniating through the spinal foramina. In some cases, these lateral meningoceles can cause pain and discomfort due to their presence within retroperitoneal tissues or cause direct compression of the spinal nerve root exiting the foramen; in some cases compression may also involve motor weakness. The presence of lateral meningoceles imposes unique challenges related to CSF flow dynamics, especially with concurrent Chiari malformation, which also occurs with increased frequency in individuals with LMS. The authors present the case of a 6-month-old female with LMS with multiple lateral meningoceles throughout the thoracic and lumbar spine. The infant experienced a focal neurological abnormality due to enlargement of her lateral meningoceles following decompression of a symptomatic Chiari malformation and endoscopic third ventriculostomy. The finding was reversed through implantation of a ventriculoperitoneal shunt, which reduced the burden of CSF upon the lateral meningoceles. Such a case compels consideration that CSF flow dynamics in addition to altered connective tissue play a role in the presence of lateral meningoceles in patients within this and similar patient populations.

Original languageEnglish (US)
Pages (from-to)232-238
Number of pages7
JournalJournal of neurosurgery. Pediatrics
Volume19
Issue number2
DOIs
StatePublished - Feb 1 2017

Fingerprint

Meningocele
Connective Tissue
Ventriculostomy
Ventriculoperitoneal Shunt
Spinal Nerve Roots
Decompression
Lateral meningocele syndrome
Spine
Thorax
Pain

Keywords

  • Chiari malformation
  • CM-I = Chiari malformation Type I
  • congenital
  • CPAP = continuous positive airway pressure
  • endoscopic third ventriculostomy
  • ETV = endoscopic third ventriculostomy
  • lateral meningocele
  • lateral meningocele syndrome
  • Lehman syndrome
  • LMS = lateral meningocele syndrome
  • NF1 = neurofibromatosis Type 1
  • shunt
  • VP = ventriculoperitoneal

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Neurosurgical management in lateral meningocele syndrome : case report. / Brown, Erik C.; Gupta, Kunal; Sayama, Christina.

In: Journal of neurosurgery. Pediatrics, Vol. 19, No. 2, 01.02.2017, p. 232-238.

Research output: Contribution to journalArticle

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