Orthopedic problems in Lesch-Nyhan syndrome

Paul D. Sponseller, Nicholas U. Ahn, James C. Choi, Uri M. Ahn

Research output: Contribution to journalArticlepeer-review

Abstract

Lesch-Nyhan syndrome is an X-linked disorder of purine metabolism. The orthopedic problems and results of treatment of nine Lesch-Nyhan patients are reviewed. Associated orthopedic problems included hip subluxation or dislocation (nine of 18 hips), fractures (three), autoamputation, infections (three), minor scoliosis, and contractures. Lesch-Nyhan patients can safely undergo orthopedic procedures and the results of surgery are satisfactory and similar to those of patients with spastic cerebral palsy. All of the seven operated-on hips maintained good reduction at 6-year mean follow-up. With adequate cast technique, fractures and hip subluxation/dislocation may be treated successfully. The treating orthopedist should be aware of the increased incidence of heterotopic ossification in this population, as well as the potential for serious complications such as hardware failure or femur fracture, if appropriate immobilization is not used.

Original languageEnglish (US)
Pages (from-to)596-602
Number of pages7
JournalJournal of Pediatric Orthopaedics
Volume19
Issue number5
DOIs
StatePublished - Jan 1 1999

Keywords

  • Hip dislocation
  • Hip subluxation
  • Hypoxanthine-guanine phosphoribosyltransferase
  • Lesch-Nyhan syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

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