Trismus-pseudocamptodactyly syndrome (Hecht-Beals' syndrome): case report and literature review.
The trismus-pseudocamptodactyly syndrome (TPS) is a rare condition inherited as an autosomal dominant trait with variable expressivity. Clinically the syndrome is characterized by decreased ability to open the mouth and curvature of the fingers at the level of interphalangic joints while attempting dorsiflexion of the wrist (pseudocamptodactyly). A 6-year-old male patient from Guatemala with this syndrome is presented. The surgical treatment consisted of bilateral coronoid amputation to resolve the pseudotrismus. The procedure was successful and the patient achieved normal function.
Carlos
R
R
Centro de Medicina Oral de Guatemala, Guatemala City, Guatemala.
Contreras
E
E
Cabrera
J
J
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery; D001176:Arthrogryposis / Q000175:diagnosis; D002648:Child; D006228:Hand Deformities, Congenital / Q000175:diagnosis; D006801:Humans; D008297:Male; D008334:Mandible / Q000002:abnormalities / Q000601:surgery; D009056:Mouth Abnormalities / Q000175:diagnosis / Q000601:surgery; D011859:Radiography; D013577:Syndrome; D014313:Trismus / Q000209:etiology / Q000601:surgery