Pediatric Radiology

, Volume 45, Issue 10, pp 1567–1570 | Cite as

Craniovertebral junction stenosis in Lenz–Majewski syndrome

  • Koichi MizuguchiEmail author
  • Osamu Miyazaki
  • Gen Nishimura
  • Akira Ishigro
Case Report


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.


Lenz-Majewski syndrome Craniovertebral junction stenosis Hyperostosis Child Computed tomography Magnetic resonance imaging Radiography Skeletal dysplasia 



We thank Dr. Julian Tang of the Department of Education for Clinical Research, National Center for Child Health and Development, for editing this manuscript.

Conflicts of interest



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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Koichi Mizuguchi
    • 1
    Email author
  • Osamu Miyazaki
    • 2
  • Gen Nishimura
    • 3
  • Akira Ishigro
    • 1
  1. 1.Department of General Pediatrics and Interdisciplinary MedicineNational Center for Child Health and DevelopmentSetagaya-kuJapan
  2. 2.Department of RadiologyNational Center for Child Health and DevelopmentTokyoJapan
  3. 3.Department of Pediatric ImagingTokyo Metropolitan Children’s Medical CenterTokyoJapan

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