Anesth Pain Med.  2011 Jan;6(1):85-88.

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

Affiliations
  • 1Department 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.

Keyword

General anesthesia; Micrognathia; Stickler syndrome

MeSH Terms

Anesthesia, General
Child
Cleft Palate
Collagen
Connective Tissue
Cryotherapy
Humans
Incidence
Intubation, Intratracheal
Laryngoscopes
Masks
Neuromuscular Blockade
Procollagen
Scleral Buckling
Ventilation
Collagen
Procollagen
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