Trismus-pseudocamptodactyly syndrome: a 20 year follow-up.
نویسندگان
چکیده
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 orthognathic 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.
منابع مشابه
Trismus-pseudocamptodactyly syndrome: case report ten years after.
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 p...
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ورودعنوان ژورنال:
- European journal of paediatric dentistry : official journal of European Academy of Paediatric Dentistry
دوره 15 2 Suppl شماره
صفحات -
تاریخ انتشار 2014