Hecht syndrome
Overview
A rare genetic disorder characterized by the inability to open the mouth due to short muscles as well as hand movement abnormalities also due to shortened muscles.
Symptoms
* Limited opening of mouth * Short flexor tendons * Down-turned toes * Clubfoot * Metatarsus adductus * Short gastrocnemius * Tightly fisted infant hands * Infants crawl on knuckles
Causes
Simple fractures or dislocations are usually caused by a manual blow along the jawline; more serious compound fractures commonly result from automobile accidents. Other causes include industrial accidents, recreational or sports injuries, assaults, or other trauma. Recurrence of a dislocated jaw is common.
Diagnosis
Ask the patient if he experienced a recent injury (even a slight wound), infection, or animal bite. Does he have a history of epilepsy, neuromuscular disease, or endocrine or metabolic disorders? Obtain a complete drug history, including self-injected drugs because the use of a contaminated needle may produce tetanus. Also, ask about paresthesia or pain in the throat, jaw, neck, or shoulders. Examination of the oral cavity may be difficult or impossible to perform. If possible, examine the pharynx, tonsils, oral mucosa, gingivae, and teeth. Perform a neurologic assessment, evaluating cranial nerve, motor, and sensory function and deep tendon reflexes. Also, check the jaw jerk reflex. An extremely hyperactive response and a careful patient history usually establish the diagnosis. (See Performing the jaw jerk test, page 772.)
Treatment
As in all traumatic injuries, check first for a patent airway, adequate ventilation, and pulses; then control hemorrhage and check for other injuries. As necessary, maintain a patent airway with an oropharyngeal airway, nasotracheal intubation, or a cricothyrotomy. Relieve pain with analgesics as needed. After the patient stabilizes, surgical reduction and fixation by wiring restores mandibular and maxillary alignment. Maxillary fractures may also require reconstruction and repair of soft-tissue injuries. Teeth and bones are never removed during surgery unless unavoidable. If the patient has lost teeth from trauma, the surgeon will decide whether they can be reimplanted. If they can, he’ll reimplant them within 6 hours, while they’re still viable. Viability is increased if the tooth is placed in milk, saliva, or normal saline solution. Dislocations are usually reduced manually under anesthesia.