Trismus-pseudocamptodactyly syndrome: a case report

Eur J Paediatr Dent. 2003 Mar;4(1):33-6.

Abstract

Background: Hecht and Beals in 1969 described an autosomal dominant syndrome characterised by severe restriction of mouth opening, camptodactyly, shortness of leg muscles and, as a direct consequence, foot deformities.

Case report: A case of a 4-year-old girl affected by this unusual syndrome is described. The patient underwent bilateral resection of coronoid processes by intraoral approach. An intraoral device was used in the immediate postoperative period in order to maintain mouth opening. Once at home, the patient has had, for six months, phisiokinesic therapy by means of a modified Darcissac device.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / surgery
  • Abnormalities, Multiple / therapy*
  • Arthrogryposis
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Foot Deformities, Congenital
  • Hand Deformities, Congenital
  • Humans
  • Ligaments / abnormalities*
  • Ligaments / surgery
  • Mandible / abnormalities*
  • Mandible / surgery
  • Mouth / physiopathology*
  • Syndrome
  • Treatment Outcome
  • Trismus / therapy*