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Trismus-pseudocamptodactyly syndrome: a 20 year follow-up
 

Type:  Articles

Pubblication date:  07/2014

Authors:  T.M. Marianetti*, L. Dall’Asta**, A. Torroni*, G. Gasparini*, S. Pelo*

Language:  English

Institution:  *Maxillofacial Surgery Department, Catholic University Medical School, Complesso Integrato Columbus, Roma, Italy **Private practitioner, Roma, Italy

Publication:  European Journal of Paediatic Dentistry

Publisher:  Ariesdue Srl

Keywords:  Coronoidectomy; Coronoidotomy; Trismus-Pseudocamptodactyly Syndrome.

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Title:  Trismus-pseudocamptodactyly syndrome: a 20 year follow-up

Abstract:  Background Trismus-Pseudocamptodactyly Syndrome (TPS) is a rare autosomal syndrome characterised by the inability to open the mouth fully, pseudocamptodactyly, short stature and foot deformities. The maxillofacial feature entails hyperplasia of the coronoid processes which mechanically interfere with the zygomatic processes during mouth opening. Case Report A 22-year-old girl affected by a severe form of TPS was followed from the age of three years. Bone reossification was observed after two coronoidotomies of both hyperplasic coronoid processes. After the decision to perform a coronoidectomy, the four-year follow-up showed a favourable outcome. Meanwhile the patient developed an anterior open bite which was treated with a fourth orthognatic surgery. The follow-up underscores how the correction of malformation leads to the generation of EMG activity of the masticatory muscles after many years of passiveness.

 
 
 
 
 
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