[Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management].
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.
Alonso Calderón
J L
JL
Hospital Infantil del Niño Jesús, Servicio de Cirugía Pediátrica, Madrid, Spain. criped@hnjs.insalud.es
Ali Taoube
K
K
D000015:Abnormalities, Multiple / Q000601:surgery*; D000758:Anesthesia; D001176:Arthrogryposis / Q000601:surgery; D019465:Craniofacial Abnormalities / Q000601:surgery; D005532:Foot Deformities, Congenital / Q000601:surgery; D006228:Hand Deformities, Congenital / Q000601:surgery; D006552:Hernia, Inguinal / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D013577:Syndrome