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

Pierre Laumonerie1, Meagan E Tibbo2, Aissa Ibnoulkhatib1

  • 1Pediatric Orthopaedics Unit, Children's Hospital.

Journal of Pediatric Orthopedics
|September 4, 2019
PubMed

Insights

Hip dysplasia in Prader-Willi syndrome (PWS) patients shows favorable radiographic changes with early growth hormone treatment. Orthopedists may consider observation alone for hip dysplasia management in PWS.

Area of Science:

  • Orthopedics
  • Genetics
  • Pediatrics

Background:

  • Prader-Willi syndrome (PWS) is a rare genetic disorder associated with musculoskeletal issues, including hip dysplasia (HD).
  • Characterizing the evolution of HD in pediatric PWS patients is crucial for effective management.

Purpose of the Study:

  • To analyze the clinical and radiographic progression of hip dysplasia in children with Prader-Willi syndrome.
  • To assess the impact of demographic factors on hip dysplasia in this population.

Main Methods:

  • Retrospective cohort study of 72 pediatric patients with PWS, analyzing 147 pelvic radiographs.
  • Radiographic measurements included Wiberg center-edge angle (CEA), acetabular index (AI), and neck-shaft angle (NSA).
  • Statistical analysis assessed relationships between radiographic parameters and age, sex, and BMI z-score (BMIzs).

Main Results:

  • Hip dysplasia was identified in 29% of radiographs and varied significantly with age.
  • Age demonstrated a statistically significant association with CEA, AI, and NSA.
  • Sex and BMIzs were not independent predictors of radiographic hip angles.

Conclusions:

  • Favorable radiographic evolution of hip parameters was observed in PWS patients treated with growth hormone early in life.
  • These findings suggest that observation alone may be a viable management strategy for hip dysplasia in PWS patients.
Abstract

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