Literature DB >> 24035654

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

E Nectoux1, B Hocquet, D Fron, A Mezel, A Paris, B Herbaux.   

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.
Copyright © 2013. Published by Elsevier Masson SAS.

Entities:  

Keywords:  Dislocation; Dyggve-Melchior-Clausen; Hip

Mesh:

Year:  2013        PMID: 24035654     DOI: 10.1016/j.otsr.2013.04.006

Source DB:  PubMed          Journal:  Orthop Traumatol Surg Res        ISSN: 1877-0568            Impact factor:   2.256


  1 in total

1.  A Case of Growth Hormone Use in Dyggve-Melchior-Clausen Syndrome.

Authors:  Ravi Upadhyay; Claire Ruane; Rachel Umans; Beth A Pletcher; Aditi Khokhar; Kristin Wong
Journal:  Case Rep Endocrinol       Date:  2022-03-15
  1 in total

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