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[Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management].
An Esp Pediatr. 2002 Feb; 56(2):175-9.AE

Abstract

We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. The boy showed characteristic deformities of distal arthrogryposis in the hands and feet, as well as the typical features of "whistling face syndrome". In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome. We describe the preanesthetic management, anesthetic method and surgical technique performed when the child was aged 9 months. The delay was due to recurrent episodes of bronchopneumonia.

Authors+Show Affiliations

Hospital Infantil del Niño Jesús, Servicio de Cirugía Pediátrica, Madrid, Spain. criped@hnjs.insalud.esNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

spa

PubMed ID

11827658

Citation

Alonso Calderón, J L., and K Ali Taoube. "[Freeman-Sheldon Syndrome: Clinical Manifestations and Anesthetic and Surgical Management]." Anales Espanoles De Pediatria, vol. 56, no. 2, 2002, pp. 175-9.
Alonso Calderón JL, Ali Taoube K. [Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management]. An Esp Pediatr. 2002;56(2):175-9.
Alonso Calderón, J. L., & Ali Taoube, K. (2002). [Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management]. Anales Espanoles De Pediatria, 56(2), 175-9.
Alonso Calderón JL, Ali Taoube K. [Freeman-Sheldon Syndrome: Clinical Manifestations and Anesthetic and Surgical Management]. An Esp Pediatr. 2002;56(2):175-9. PubMed PMID: 11827658.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management]. AU - Alonso Calderón,J L, AU - Ali Taoube,K, PY - 2002/2/6/pubmed PY - 2002/6/12/medline PY - 2002/2/6/entrez SP - 175 EP - 9 JF - Anales espanoles de pediatria JO - An Esp Pediatr VL - 56 IS - 2 N2 - We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. The boy showed characteristic deformities of distal arthrogryposis in the hands and feet, as well as the typical features of "whistling face syndrome". In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome. We describe the preanesthetic management, anesthetic method and surgical technique performed when the child was aged 9 months. The delay was due to recurrent episodes of bronchopneumonia. SN - 0302-4342 UR - https://www.unboundmedicine.com/medline/citation/11827658/[Freeman_Sheldon_syndrome:_clinical_manifestations_and_anesthetic_and_surgical_management]_ L2 - http://www.diseaseinfosearch.org/result/2921 DB - PRIME DP - Unbound Medicine ER -