An anesthetic experience in a patient with Stickler sydrome: A case report

Choi, Youn Hee; Shin, Yong Sup; Lee, Sun Yeul; Pak, Hae Jin; Choi, Guen Seok; Hong, Boo Hwi

Case Report

Pediatric Anesthesia

Anesthesia and Pain Medicine 2011;6(1):85-88.
Published online January 31, 2011.

An anesthetic experience in a patient with Stickler sydrome: A case report

Youn Hee Choi, Yong Sup Shin, Sun Yeul Lee, Hae Jin Pak, Guen Seok Choi, Boo Hwi Hong

Department of Anesthesiology and Pain Medicine, Chungnam National University School of Medicine, Daejeon, Korea. ysshin@cnu.ac.kr

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.

Key Words: General anesthesia, Micrognathia, Stickler syndrome