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Case Reports in Anesthesiology
Volume 2015 (2015), Article ID 509196, 3 pages
http://dx.doi.org/10.1155/2015/509196
Case Report

Anesthetic Approach for a Patient with Jeune Syndrome

1Department of Anesthesiology, Istanbul Medical Faculty, Istanbul University, Fatih, 34093 Istanbul, Turkey
2Department of Anesthesiology, Suleymaniye Obstetrics & Pediatrics Training and Research Hospital, Zeytinburnu, 34116 Istanbul, Turkey

Received 21 May 2015; Accepted 13 August 2015

Academic Editor: Mark Coburn

Copyright © 2015 Mehmet I. Buget et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a case of JS patient, who was scheduled for femoral extension under general anesthesia. The severity of respiratory problems in JS patients is thought to diminish with age. Our case supported this theory, and we managed the anesthetic process uneventfully.