Hecht Syndrome

At a glance

Rare, inherited disorder, characterized by trismus and pseudocamptodactyly. This medical condition called “Trismus-pseudocamptodactyly Syndrome” is a disorder characterized by short muscles and tendons resulting in limited range of motion of the hands, legs, and mouth. The most serious complication includes the limited opening of the mouth, a very serious considerations in anesthesia.

Synonyms

Trismus Pseudocamptodactyly Syndrome; Camp­todactyly Limited Jaw Excursion; Hecht-Beals-Wilson Syndrome; Dutch-Kentucky Syndrome; Distal Arthrogryposis Type 7.

History

A genetic disorder that was first described in 1969 by Frederik Hecht, an American pediatrician, and Rodney Kenneth Beals, an American surgeon.

Incidence

Rare syndrome; possible same familial origin for all cases has been evocated.

Genetic inheritance

Autosomal dominant; more common in females.

Diagnosis

Clinically evocated in patients presenting with an inability to open their mouth completely, combined with abnormally short muscle-tendon units in the fingers, causing the fingers to curve or bend with wrist dorsiflexion.

Clinical aspects

Features involve mouth (trismus), muscle, and bones (short stature, symphalangy, muscle anomalies, restricted joint mobility, pseudocamptodactyly). Ptosis, prognathism, dislocated hip and foot anomalies (metatarsus adductus, down-turning or hammer toes, talipes equinovarus), and heart malformations (mitral valve prolapse, aortic root dilatation) may occur. Feeding difficulties are frequent.

Precautions before anesthesia

An anesthetic consultation is highly recommended. Evaluate the airway for potential difficulty during direct laryngoscopy and tracheal intubation (clinical, radiographs), and orthopedic function (clinical).

Anesthetic considerations

Airway management is complicated by the presence of trismus, which does not improve after the induction of anesthesia. Mask anesthesia with sustained spontaneous ventilation is mandatory until the airway is secured. The use of a blind nasotracheal intubation may be an alternative in older patients but not recommended in children. Bleeding may be induced during attempts to secure the airway throughout the nose due to the possible presence of adenoid tissues that contributes to make this approach contraindicated in these patients. Laryngoscopy and orotracheal intubation can be achieved using either direct laryngoscopy (in milder cases), fiberoptic laryngoscopy, or Bullard laryngoscope. In the presence of severe trismus and limitation of mouth opening, it is recommended to ensure the presence of an otorhinolaryngologist in case of acute need of a tracheostomy to secure the airway. Careful intraoperative positioning to prevent pressure points is essential in the presence of skeletal malformations.

Pharmacological implications

Avoid muscle relaxant until airway is secured and lung ventilation confirmed. Trismus is not ...