Trismus-pseudocamptodactyly syndrome: case report ten years after

Giulio Gasparini, Alessandro Moro, Giuseppe Zampino, Roberto Boniello, Sandro Pelo

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7 Citazioni (Scopus)

Abstract

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.
Lingua originaleEnglish
pagine (da-a)199-203
Numero di pagine5
RivistaEUROPEAN JOURNAL OF PAEDIATRIC DENTISTRY
Volume9
Stato di pubblicazionePubblicato - 2008

Keywords

  • Abnormalities, Multiple
  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Foot Deformities, Congenital
  • Hand Deformities, Congenital
  • Humans
  • Ligaments
  • Mandible
  • Mouth
  • Neuromuscular Diseases
  • Syndrome
  • Treatment Outcome
  • Trismus

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