Open AccessNeurosurgical Management of Lateral Meningocele Syndrome: A Clinical Update for the Pediatric Neurosurgeon
Author(s) -
Joshua A. Cuoco,
Brendan J. Klein,
Christopher Büsch,
Hailey L Gosnell,
Ayesha Kar,
Eric A. Marvin,
Lisa S. Apfel
Publication year - 2019
Publication title -
pediatric neurosurgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.385
H-Index - 72
eISSN - 1423-0305
pISSN - 1016-2291
DOI - 10.1159/000504060
Subject(s) - medicine , syringomyelia , hydrocephalus , neurosurgery , marfan syndrome , chiari malformation , connective tissue disorder , spinal cord , ehlers–danlos syndrome , pathology , surgery , psychiatry
Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos syndrome, and Loeys-Dietz syndrome. However, this syndrome is invariably associated with the presence of multiple lateral thoracolumbar spinal meningoceles: a distinct point of phenotypic divergence from other connective tissue disorders. The etiopathogenesis of this syndrome has recently been linked to truncating mutations within exon 33 of NOTCH3. Despite numerous reports, neurosurgical management of multiple spinal meningoceles remains poorly defined in the literature. We conducted a literature review to provide insight into the nosology, clinical significance, and neurosurgical management strategies of this distinct connective tissue disorder.