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Trismus-pseudocamptodactyly syndrome. Case report ten years after

Type:  Articles

Pubblication date:  /4/2008

Authors:   G. Gasparini, R. Boniello, A. Moro, G. Zampino*, S. Pelo

Language:  English

Institution:  Maxillofacial Surgery Unit, Catholic University Medical School, Rome, Italy *Department of Paediatrics, Catholic University Medical School, Rome, Italy

Publication:  European Journal of Paediatric Dentistry

Publisher:  Ariesdue Srl

Keywords:  Pseudocamptodactyly; Carney syndrome; Coronoidotomies; Temporal muscles trismus; Dutch–Kentucky syndrome

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Email:  [email protected]

Title:  Trismus-pseudocamptodactyly syndrome. Case report ten years after

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.

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