Chapter 71: TREACHER COLLINS SYNDROME

YOUR PATIENT

A 17-year-old male with Treacher Collins syndrome presents for mandibular and maxillary osteotomies.

Airway examination shows a Mallampati class 4 airway, large tongue, and free range of neck motion.

PREOPERATIVE CONSIDERATIONS

Treacher Collins syndrome is characterized by poorly developed supraorbital ridges, ophthalmic abnormalities, aplastic or hypoplastic zygomas, ear deformities, hearing loss, cleft lip or palate, macrostomia, malocclusion of the teeth, maxillary hypoplasia (narrowing of nasal passages, resulting in choanal stenosis or atresia), mandibular hypoplasia (tongue base is retropositioned, thereby obstructing oropharyngeal and hypopharyngeal spaces), and midface hypoplasia.

From birth, the adequacy of the airway is of primary concern. Repeated imaging may be needed prior to reconstructive procedures.

The degree of airway obstruction is related to the degree of maxillary and mandibular hypoplasia, choanal atresia, and glossoptosis. A narrow airway due to pharyngeal hypoplasia may cause respiratory distress and necessitate a tracheostomy. Tracheostomy may be required during infancy for those at highest risk for obstructive sleep apnea (OSA). Mandibular distraction procedures can be used to relieve airway obstruction and facilitate tracheal decannulation.

Limited oropharyngeal and hypopharyngeal space may lead to OSA, pulmonary hypertension, and in severe cases, cor pulmonale.

Aside from cleft lip and palate repair, the timing of the major reconstruction typically occurs during childhood or adolescence when the cranioorbital zygomatic bony development is nearly complete.

ANESTHETIC MANAGEMENT

• Give an antisialagogue for airway preparation.

• Mask ventilation is difficult.

• Direct laryngoscopy and tracheal intubation may be extremely difficult or impossible secondary to severe mandibular hypoplasia, a small mouth, and a narrow airway.

• Adequacy of mask ventilation should be determined. At this point, the airway can be visualized or managed with a variety of airway tools, with laryngeal mask anesthesia often being the only way to provide positive pressure ventilation prior to intubation.

• Use awake or sedated fiberoptic intubation if possible.

• Fiberoptic nasal intubation may be impossible due to choanal stenosis or atresia.

• If oral intubation is not desirable because of the nature of the surgery, tracheostomy may be required. Tracheostomy may be difficult if the patient has had a previous tracheostomy.

• Blood loss may be important during craniofacial reconstructive surgery and requires invasive monitoring of hemodynamic parameters (arterial line with or without a central venous pressure catheter), estimation of blood loss, arterial blood gas, and electrolyte analysis.

• Consider giving steroids to reduce airway swelling.

• Avoid excessive opioids to minimize risk of postoperative respiratory depression.

POSTOPERATIVE CONSIDERATIONS

Postoperative care often requires transfer of an intubated patient to the intensive care unit. In preparation for extubation, airway devices and staff support should be available in case the patient requires reintubation.