Open wide: Anesthetic management of a child with Hecht–Beals syndrome

Abstract

Hecht–Beals syndrome (HBS) is a rare disorder characterized by trismus and deformity of the extremities. The etiology of trismus is unknown; theories suggest invasion of enlarged coronoid processes into the zygomatic bone. Of primary concern is the limited mouth opening and possible difficult airway. Since the syndrome was first described in 1969, there have been several articles in the pediatrics and dental literature but only 6 case reports describing the anesthetic management of these patients. Successful airway approaches have utilized various techniques including blind nasal intubation, fiberoptic intubation, and tracheal tube introducer guidance. In this case report, we discuss a multidisciplinary approach to the anesthetic management of a child with HBS undergoing MRI and outpatient surgery.

Keywords

• congenital trismus; hecht-beals syndrome; pseudocamptodactyly