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

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.

• Authors: Henry Juver Vergara Fernández, Leonori Román Capdevila
• Journal: Acta ortopedica mexicana (Acta Ortop Mex) Vol. 21 Issue 6 Pg. 338-43 ( 2007) ISSN: 2306-4102 [Print] Mexico
• Vernacular Title: Síndrome de Freeman-Sheldon. Informe de cinco casos.
• PMID: 18386533 (Publication Type: English Abstract, Journal Article)

Topics

• Adult
• Arthrogryposis (diagnosis, surgery)
• Child, Preschool
• Female
• Humans
• Male
• Phenotype