Craniovertebral junction stenosis in Lenz-Majewski syndrome.

Abstract:

:We report a girl with Lenz-Majewski syndrome associated with craniovertebral junction stenosis that led to communicating hydrocephalus and cervical myelopathy. The life-threatening complication was related to progressive craniovertebral hyperostosis that rapidly exacerbated during early childhood. Despite initial success of surgical intervention at 2 years of age, she developed apneic spells and died suddenly at age 5 years. Close monitoring for craniovertebral junction stenosis is essential to reduce morbidity and mortality in children with Lenz-Majewski syndrome.

journal_name

Pediatr Radiol

journal_title

Pediatric radiology

authors

Mizuguchi K,Miyazaki O,Nishimura G,Ishigro A

doi

10.1007/s00247-015-3329-5

subject

Has Abstract

pub_date

2015-09-01 00:00:00

pages

1567-70

issue

10

eissn

0301-0449

issn

1432-1998

journal_volume

45

pub_type

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