Trismus Pseudocamptodactyly Syndrome: A Sporadic Cause of Trismus

Trismus Pseudocamptodactyly Syndrome: A Sporadic Cause of Trismus

Trismus pseudocamptodactyly syndrome is a very rare autosomal dominant inherited disorder characterized by the inability to completely open the mouth (trismus) and the presence of abnormally short tendon units causing the fingers to curve (camptodactyly). Early diagnosis and management of this condition is important to prevent facial deformities in the patient. Reporting such a case is importan...

Trismus-pseudocamptodactyly syndrome: a case report.

BACKGROUND Hecht and Beals in 1969 described an autosomal dominant syndrome characterised by severe restriction of mouth opening, camptodactyly, shortness of leg muscles and, as a direct consequence, foot deformities. CASE REPORT A case of a 4-year-old girl affected by this unusual syndrome is described. The patient underwent bilateral resection of coronoid processes by intraoral approach. An...

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

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 s...

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...

The trismus-pseudocampylodactyly syndrome.