title: Unpredictability of hip behavior in Dyggve-Melchior-Clausen syndrome: a mid-term assessment of siblings.
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.
authors.affiliation: 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: eric.nectoux@chru-lille.fr.
authors.forename: E
authors.initials: E
authors.lastname: Nectoux
authors.forename: B
authors.initials: B
authors.lastname: Hocquet
authors.forename: D
authors.initials: D
authors.lastname: Fron
authors.forename: A
authors.initials: A
authors.lastname: Mezel
authors.forename: A
authors.initials: A
authors.lastname: Paris
authors.forename: B
authors.initials: B
authors.lastname: Herbaux
mesh_terms: D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004392:Dwarfism / Q000175:diagnosis / Q000601:surgery*; D005593:Fracture Fixation, Internal / Q000379:methods; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000601:surgery*; D006617:Hip Dislocation / Q000000981:diagnostic imaging / Q000601:surgery*; D006620:Hip Fractures / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000601:surgery*; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D010009:Osteochondrodysplasias / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D011379:Prognosis; D011859:Radiography; D018570:Risk Assessment; D035781:Siblings; D016896:Treatment Outcome
keywords: Dislocation; Dyggve-Melchior-Clausen; Hip