Neglected Chronically Dislocated Hip in a Prader-Willi Child: A Case Report and Literature Review.

Abstract

Introduction: Prader-Willi syndrome (PWS) is an uncommon genetic disorder resulting from the loss of function of genes in the paternal copy of chromosome 15q11.2-q13. Although the clinical features and diagnosis of PWS are well described, the management protocol for hip dysplasia (HD) is still controversial.

Case report: We present a case of a 4-year-old female child who was diagnosed with PWS and had developmental dysplasia of the hip (DDH) as a skeletal manifestation. She underwent an open reduction of the hip, femoral shortening with fixation using a plate, Dega osteotomy, capsulorrhaphy, and temporary stabilization of the hip joint with a K-wire, similar to any other case of DDH. Postoperative recovery was uneventful. However, there were challenges associated with her management from both a surgical and an anesthetic point of view.It is imperative that once diagnosed, the condition be addressed comprehensively. A detailed preoperative evaluation by the anesthesia team is required to ensure preparedness for potential surgical challenges. Similarly, the surgical team must be equipped with all necessary instruments and a backup plan.

Conclusion: Hip dysplasia (HD) should be diagnosed as early as possible. Nonoperative treatment is usually sufficient before the age of 6 months. However, in PWS, it may present late and be neglected, requiring surgical management similar to a case of DDH, with the associated challenges.