Surgical correction of trismus in a child with Hecht syndrome.

Abstract

Hecht syndrome is a rare condition that is also known as trismus pseudocamptodactyly syndrome. Short muscle and tendon units limiting the range of motion of upper and lower extremities and mouth characterize this disorder of muscle development. There is no consensus on the optimal treatment of temporomandibular joint (TMJ) ankylosis in this patient population. Endoscopic release in one patient resulted in early postoperative recurrence. The authors present a 28-month old boy who had a limited mouth opening of 6 mm. CT scan showed no bony ankylosis. The range of mouth opening did not to improve with physical therapy. The patient underwent extensive subperiosteal dissection of the mandible, bilateral coronoidectomy, and TMJ exploration. An intraoperative opening of 18 mm was achieved. The patient remained intubated until postoperative swelling resolved. He was extubated in the operating room 6 days later. The patient continued to improve with physical therapy. He had a 25-mm mouth opening at 12 months of follow-up.