Evolution of Hip Dysplasia in Pediatric Patients With Prader-Willi Syndrome Treated With Growth Hormone Early in Development

Background: Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by obesity, hypotonia, feeding difficulties, obesity, musculoskeletal manifestations including scoliosis, and hip dysplasia (HD). The aim of this study was to characterize the clinical and radiographic evolution of HD in the pediatric PWS population. Methods: The authors performed a retrospective cohort study of 72 patients (147 anteroposterior pelvic radiographs) between January 2004 and December 2016. Center-edge angle (CEA) of Wiberg, acetabular index (AI), and neck-shaft angle (NSA) were measures in all hips. The relationship between radiographic and demographic parameters of age, sex, and body mass index z-score (BMIzs) were assessed. Results: A total of 274 radiographic measurements were performed and analyzed in 72 patients. The mean CEA, AI, and NSA were 21.8±7.1 degrees (range, 5 to 35 degrees), 16.7±7 degrees (range, 5 to 45 degrees), and 142±8.5 degrees (range, 128 to 165 degrees), respectively. HD was diagnosed in 79 (29%) hip radiographs and varied significantly between the age groups (P
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Syndromes Source Type: research