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KMID : 1155520110060010085
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Anesthesia and Pain Medicine
2011 Volume.6 No. 1 p.85 ~ p.88
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An anesthetic experience in a patient with Stickler sydrome: A case report
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Choi Youn-Hee
Shin Yong-Sup
Lee Sun-Yeul
Park Hae-Jin
Choi Guen-Seok
Hong Boo-Hwi
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Abstract
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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.
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KEYWORD
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General anesthesia, Micrognathia, Stickler syndrome
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