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.

Total Hip Arthroplasty in Dyggve-Melchior-Clausen Syndrome: Literature Review and Case Report

“…Another case report is of a 9-year-old patient with DMC syndrome with painful wrists and bilateral hip subluxation [ 9 ]. The subluxation of the right hip became painful at age 17, and varisation of the upper femur with triple pelvic osteotomy of the right hip was performed.…”

“…The patient received bilateral femoral varisation with Pemberton osteotomies at age 11. Over 3 years, the patient rapidly developed progressive articular degeneration with subsequent subluxation of the right hip [ 9 ].…”

“…Spondyloepimetaphyseal dysplasia leads to progressively abnormal development of the hip epiphysis and growth of the hip joint [ 8 ]. The abnormal femoral head can later develop osteonecrosis and major arthrosis as a sequela of DMC [ 9 ]. Suggested treatment options include realignment of the proximal femur and/or acetabulum, realignment osteotomy, cheilectomy of the hip, and/or total hip replacement [ 5 , 6 , 9 ].…”

A Case of Growth Hormone Use in Dyggve–Melchior–Clausen Syndrome

“…When present, progressive hip deformities in patients with DMC syndrome invariably lead to bilateral hip subluxation and eventual bilateral dislocation of the hip joint. The pain associated with changes at the hip is ultimately managed surgically although this can be delayed until late adolescence in some cases [ 7 ]. At the time of our initial evaluation, our patient already had limited range of motion at several joints including the hips.…”