Hecht Syndrome

Rare, inherited disorder, characterized by trismus and pseudocamptodactyly. Patients cannot open completely their mouth.

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Trismus Pseudocamptodactyly Syndrome; Camptodactyly Limited Jaw Excursion; Hecht-Beals-Wilson Syndrome; Dutch-Kentucky Syndrome.

Genetic disorder first described in 1969 by Frederik Hecht, an American pediatrician, and Rodney Kenneth Beals, an American surgeon.

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

Autosomal dominant; more common in females.

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.

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.

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

Direct laryngoscopy and tracheal intubation can be difficult because of trismus and may require adapted anesthetic techniques such as fiberoptic intubation. Careful intraoperative positioning is needed because of skeletal malformations but can be difficult to realize.

Avoid muscle relaxant until airway is secured and lung ventilation confirmed. Trismus is not related to a structural muscle anomaly, and the administration of succinylcholine and volatile anesthetic agents does not have to be avoided. Prophylactic antibiotics in case of cardiopathy may be indicated.