[Freeman-Sheldon's syndrome. Report of five cases]

Acta Ortop Mex. Nov-Dec 2007;21(6):338-43.
[Article in Spanish]

Abstract

Introduction: Freeman-Sheldon syndrome, described in 1938, is an infrequent form of arthrogryposis.

Material and methods: We reviewed the files of the patients with the diagnosis of Freeman-Sheldon syndrome from 1990 to 2003, and carried out an evaluation of the clinical data, family history and malformations of extremities.

Results: 5 patients were included, 4 male and 1 female. Upper extremities: All patients presented adducted thumbs, 3 patients were treated with a first web release and dorsal graft with improvement of grip on larger objects. Feet: Four patients had severe bilateral rigid clubfeet, they were operated on with a posteromedial release and bilateral talectomy, satisfactory results were obtained in 4 of 6 feet according to Niki criteria. Other non orthopedic anomalies: In three patients unilateral right undescended testis; 1 right inguinal hernia.

Conclusions: The patients have as common factor the whistler appearance, accompanied with mill crosses hand deformity, adducted thumbs, accompanied or not with inferior extremities deformities, intellectual capacity was normal.

Publication types

  • English Abstract

MeSH terms

  • Adult
  • Arthrogryposis* / diagnosis
  • Arthrogryposis* / surgery
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Phenotype