Noncraniofacial manifestations of Crouzon's disease

J Craniofac Surg. 1994 Sep;5(4):218-22. doi: 10.1097/00001665-199409000-00004.

Abstract

Although most interest centers on the craniofacial region in Crouzon's disease, noncraniofacial manifestations are important because they may complicate both diagnosis and management. A population of 59 patients with Crouzon's disease was reviewed to determine the frequency of these deformities. Stylohyoid ligament calcification (50%) and cervical spine (40%) and elbow (18%) abnormalities were the most common. Minor hand deformities (10%), other musculoskeletal deformities (7%), and visceral anomalies (7%) were also seen. Acanthosis nigricans was not present in this population. Recommendations are made for the assessment and management of Crouzon's disease with reference to these areas.

Publication types

  • Review

MeSH terms

  • Acanthosis Nigricans / pathology
  • Craniofacial Dysostosis / pathology*
  • Elbow / abnormalities
  • Female
  • Hand Deformities, Congenital / pathology
  • Humans
  • Hyoid Bone
  • Ligaments / abnormalities
  • Limb Deformities, Congenital
  • Male
  • Retrospective Studies
  • Spine / abnormalities
  • Temporal Bone