Unpredictability of hip behavior in Dyggve-Melchior-Clausen syndrome: a mid-term assessment of siblings

Orthop Traumatol Surg Res. 2013 Oct;99(6):745-8. doi: 10.1016/j.otsr.2013.04.006. Epub 2013 Sep 12.


Dyggve-Melchior-Clausen syndrome is a rare spondylo-epiphyseal disease, which almost constantly leads to both bilateral hip degeneration and dislocation. Few authors have reported to date the surgical management of this orthopaedic disorder. We present two new cases affecting siblings. One brother was treated by unilateral triple pelvic osteotomy combined with varus osteotomy of the proximal femur; the other was treated by bilateral Pemberton osteotomies with varus osteotomy of the proximal femur. At a respective 5-year and 3-year follow-up delay, both cases had evolved towards progressive subluxation recurrence along with severe hip degeneration. Based on both our experience and literature review, it seems that one should avoid operating these hips unless pain renders surgery mandatory. Total hip arthroplasty seems the only reliable surgical solution at the adult age and paediatric surgeons should keep in mind that previous femoral osteotomies will make it more challenging for adult orthopaedic surgeons to implant on a remodeled anatomy.

Keywords: Dislocation; Dyggve-Melchior-Clausen; Hip.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Disease Progression
  • Dwarfism / diagnosis
  • Dwarfism / surgery*
  • Fracture Fixation, Internal / methods
  • Genetic Diseases, X-Linked / diagnosis
  • Genetic Diseases, X-Linked / surgery*
  • Hip Dislocation / diagnostic imaging
  • Hip Dislocation / surgery*
  • Hip Fractures / diagnostic imaging
  • Hip Fractures / surgery*
  • Humans
  • Intellectual Disability / diagnosis
  • Intellectual Disability / surgery*
  • Male
  • Monitoring, Physiologic / methods
  • Osteochondrodysplasias / congenital*
  • Osteochondrodysplasias / diagnosis
  • Osteochondrodysplasias / surgery
  • Prognosis
  • Radiography
  • Risk Assessment
  • Siblings
  • Treatment Outcome

Supplementary concepts

  • Dyggve-Melchior-Clausen syndrome