Anesthesia and Pain Medicine  2011;6(1):85-88

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

Youn Hee CHOI 1 ; Yong Sup SHIN ; Sun Yeul LEE ; Hae Jin PAK ; Guen Seok CHOI ; Boo Hwi HONG

Affiliations

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Keywords

General anesthesia; Micrognathia; Stickler syndrome

Country

Republic 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.