title: Neurosurgical management in lateral meningocele syndrome: case report.
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.
authors.affiliation: Department of Neurological Surgery and Doernbecher Children's Hospital, Oregon Health & Science University, Portland, Oregon.
authors.forename: Erik C
authors.initials: EC
authors.lastname: Brown
authors.affiliation: Department of Neurological Surgery and Doernbecher Children's Hospital, Oregon Health & Science University, Portland, Oregon.
authors.forename: Kunal
authors.initials: K
authors.lastname: Gupta
authors.affiliation: Department of Neurological Surgery and Doernbecher Children's Hospital, Oregon Health & Science University, Portland, Oregon.
authors.forename: Christina
authors.initials: C
authors.lastname: Sayama
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D001921:Brain / Q000000981:diagnostic imaging / Q000601:surgery; D002972:Cleft Palate / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D002658:Developmental Disabilities / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D008588:Meningocele / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods*
keywords: CM-I = Chiari malformation Type I; CPAP = continuous positive airway pressure; Chiari malformation; ETV = endoscopic third ventriculostomy; LMS = lateral meningocele syndrome; Lehman syndrome; NF1 = neurofibromatosis Type 1; VP = ventriculoperitoneal; congenital; endoscopic third ventriculostomy; lateral meningocele; lateral meningocele syndrome; shunt