Lateral meningocoele syndrome

Lateral meningocoele syndrome is an extremely  rare hereditary connective tissue disorder characterized by multiple lateral lumbar meningocoeles, distinctive facial features, joint hypermobility, hypotonia, skeletal abnormalities, congenital cardiovascular malformations, urogenital anomalies and neurologic findings. 1

Lateral meningocoele syndrome is inherited in an autosomal dominant manner as a result of a de-novo NOTCH3 pathogenic variant.1-2

Lateral meningocoeles are comprised by protrusion of the arachnoid and dura through the foramina and represent the severe and extreme of the dural ectasia spectrum.5

Associations

It can be associated with Chiari I malformationsyringomyelia or idiopathic "empty sella".

CT and MRI

Lateral meningocoele syndrome typically manifests as extensive but asymmetric lateral lumbar meningocoeles involving the neural foramina and paraspinal soft tissues with associated extensive remodeling of the neural arches, articular pillar and neural foramina as well as some degree of scalloping of the posterior surface of the involved segments. Intradural compartments in the interior   of the spinal canal produce asymmetric extrinsic distortion, displacement or compression to the spinal cord.

Symptomatic treatment for lateral meningocoeles, may require surgical intervention in large lesions.

Lateral meningocoele syndrome was first described in 1977 by Lehman et al 4.

The main differential diagnosis is that of other connective tissue disorders which have meningocoeles. These include: 

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Article information

rID: 48201
Section: Syndromes
Synonyms or Alternate Spellings:
  • Lateral lumbar meningocoele
  • Lateral meningocoele syndrome (LMS)
  • Lehman Syndrome

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