An anesthetic experience in a patient with Stickler sydrome: A case report.
- Author:
Youn Hee CHOI
1
;
Yong Sup SHIN
;
Sun Yeul LEE
;
Hae Jin PAK
;
Guen Seok CHOI
;
Boo Hwi HONG
Author Information
1. Department of Anesthesiology and Pain Medicine, Chungnam National University School of Medicine, Daejeon, Korea. ysshin@cnu.ac.kr
- Publication Type:Case Report
- Keywords:
General anesthesia;
Micrognathia;
Stickler syndrome
- MeSH:
Anesthesia, General;
Child;
Cleft Palate;
Collagen;
Connective Tissue;
Cryotherapy;
Humans;
Incidence;
Intubation, Intratracheal;
Laryngoscopes;
Masks;
Neuromuscular Blockade;
Procollagen;
Scleral Buckling;
Ventilation
- From:Anesthesia and Pain Medicine
2011;6(1):85-88
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Stickler syndrome is a connective tissue dysplasia disorder with characteristic midface hypoplasia, retromicrognathia, cleft palate, and a moon-shaped appearance. Incidence of the syndrome is estimated at around 1/10000 and the disorder is considered to be caused by mutations in the COL2A1, COL11A1, COL11A2, COL9A1 procollagen genes of type 2 and 11 collagen. Patients with a mandibular hypoplasia like Stickler syndrome present the anesthesiologist with considerable problems when mask ventilation or endotracheal intubation is attempted. We report a successful anesthetic experience, including blind endotracheal intubation with rigid laryngoscope without neuromuscular blockade, in a 9-year-old boy with Stickler syndrome for scleral buckling with cryotherapy.
