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Case Reports
. 2015 Sep;45(10):1567-70.
doi: 10.1007/s00247-015-3329-5. Epub 2015 Mar 17.

Craniovertebral junction stenosis in Lenz-Majewski syndrome

Koichi Mizuguchi  1 , Osamu Miyazaki, Gen Nishimura, Akira Ishigro
Affiliations
Case Reports

Craniovertebral junction stenosis in Lenz-Majewski syndrome

Koichi Mizuguchi et al. Pediatr Radiol. 2015 Sep.

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.

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