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

Orthop Traumatol Surg Res

Service de chirurgie pédiatrique, hôpital Jeanne-de-Flandre, CHU de Lille, avenue Eugène-Avinée, 59037 Lille cedex, France; Université Nord-de-France Lille, 59000 Lille, France. Electronic address:

Published: October 2013

AI Article Synopsis

  • Dyggve-Melchior-Clausen syndrome is a rare condition that commonly results in hip degeneration and dislocation, with limited successful surgical management reported.
  • Two siblings were treated with different surgical techniques; however, both experienced progression of hip issues in follow-ups, suggesting that surgery should be avoided unless absolutely necessary due to pain.
  • Total hip arthroplasty is deemed the most reliable solution for adults, but previous surgeries in children complicate future procedures.

Article Abstract

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.

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http://dx.doi.org/10.1016/j.otsr.2013.04.006DOI Listing

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