Trismus-pseudocamptodactyly syndrome: case report ten years after

Eur J Paediatr Dent. 2008 Dec;9(4):199-203.


Background: In 1969, Hecht and Beals described for the first time a rare dominant autosomal syndrome characterised by reduced mouth opening, pseudocamptodactyly, short stature, and foot deformities. Recent studies have confirmed that TPS is caused by a mutation of MYH8 that is common to another disease called Carney syndrome.

Case report: The authors describe the long term follow-up of a case presented in 2003, ten years after the first surgical procedure: a 14-year-old girl, affected by this rare syndrome, had underwent an early (at 4 years) surgical treatment of bilateral coronoidotomies to ensure safe airway management to allow subsequent surgical treatment to correct foot deformities. After six years, a complete relapse of the trismus occurred. Three years later, the patient underwent a second surgery of bilateral coronoidotomies to definitely solve trismus. At the 18 months follow-up, the mouth opening was stable.

Publication types

  • Case Reports

MeSH terms

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