Foot Deformities in Hajdu-Cheney Syndrome: A Rare Case Report and Review of the Literature.

Academic Article

Abstract

  • Introduction: Hajdu-Cheney syndrome (HCS) is a rare autosomal dominant disease characterized by acroosteolysis, wormian skull bones with persistent skull sutures, premature loss of teeth, micrognathia, short stature, hypermobility of the joints, neurologic manifestations such as basilar invagination with subsequent paresthesia, hearing loss, and speech alterations, and osteoporosis with tendency to pathologic fractures of long bones and vertebrae as well as painful hands and feet. Very few cases have been earlier reported in the literature. Case Report: We report a case of a 50-year-old female with bilateral foot deformities as a manifestation of the rare genetic disorder HCS. Surgical management of the left foot consisted of Morton's neuroma excision and Weil osteotomy with proximal interphalangeal joint resection and Kirschner wire fixation of the second and third metatarsophalangeal (MTP) joints. Recurrent subluxation of the left second MTP joint was observed at 5-week follow-up. The right foot was treated similarly 7weeks after the initial operation. The post-operative course of the right foot was complicated by bone resorption and nonunion of the second and third metatarsal Weil osteotomies. Conclusion: Management of complex foot deformities associated with HCS can be challenging and have not previously been described in the literature. Underlying bone and connective tissue abnormalities intrinsic to the syndrome may increase the risk of recurrence after surgical correction. Consideration should be given to such post-operative complications when treating foot deformities in a patient with HCS.
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    Keywords

  • Acroosteolysis, Hajdu-Cheney syndrome, basilar invagination, osteoporosis, wormian skull bones
  • Digital Object Identifier (doi)

    Author List

  • Shah AB; Tisano BK; Elattar O; Staggers JR; Naranje S
  • Start Page

  • 11
  • End Page

  • 15
  • Volume

  • 7
  • Issue

  • 5