Trismus-pseudocamptodactyly syndrome (Hecht-Beals' syndrome): case report and literature review

Oral Dis. 2005 May;11(3):186-9. doi: 10.1111/j.1601-0825.2005.01005.x.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abnormalities, Multiple* / diagnostic imaging
  • Abnormalities, Multiple* / surgery
  • Arthrogryposis / diagnosis
  • Child
  • Hand Deformities, Congenital* / diagnosis
  • Humans
  • Male
  • Mandible* / abnormalities
  • Mandible* / surgery
  • Mouth Abnormalities* / diagnosis
  • Mouth Abnormalities* / surgery
  • Radiography
  • Syndrome
  • Trismus* / etiology
  • Trismus* / surgery