Lateral meningocele syndrome
Citation, DOI and case data
Patient with lumbar pain and abnormal x-ray
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MRI shows extensive asymmetric lateral lumbar meningoceles preferentially involving the left side of the lumbar neural foramina and paraspinal soft tissues from L2 to S1, with associated extensive remodeling of the neural arches, articular pillars, and neural foramina as well as a lesser degree of scalloping of the posterior surface of the involved Lumbar segments.
Also, intradural loculated compartments within the spinal canal produce asymmetric extrinsic distortion and displacement of the lower thoracic spinal cord within the field of view, which appears posteriorly displaced at T8-T10 and anteriorly displaced at T10-L1 levels.
Lateral meningocele syndrome is an extremely rare hereditary connective tissue disorder characterized by multiple lateral lumbar meningoceles, distinctive facial features, joint hypermobility, hypotonia, skeletal abnormalities, congenital cardiovascular malformations, urogenital anomalies and neurologic findings.
- 1. Erik C. Brown, Kunal Gupta, Christina Sayama. Neurosurgical management in lateral meningocele syndrome: case report. (2017) Journal of Neurosurgery: Pediatrics. 19 (2): 232. doi:10.3171/2016.9.PEDS16311 - Pubmed
- 2. Resham Ejaz, Wen Qin, Lijia Huang, et al. Lateral meningocele (Lehman) syndrome: A child with a novel NOTCH3 mutation. (2016) American Journal of Medical Genetics Part A. 170 (4): 1070. doi:10.1002/ajmg.a.37541 - Pubmed