A 4-month-old male with Pierre Robin sequence presents for a tongue-lip adhesion procedure.
Pierre Robin sequence is characterized by retrognathia, glossoptosis (tongue falling to the back of the throat), cleft soft palate, and airway obstruction. With early mandibular hypoplasia in utero, the tongue is placed posteriorly, keeping the palatal shelves (which normally must grow over the tongue) from closing in the midline and causing a cleft. The rounded contour of the cleft differs from the usual inverted V shape of most palatal clefts. Pierre Robin sequence may occur as a component of many syndromes, such as Stickler syndrome, velocardiofacial syndrome, fetal alcohol syndrome, and bilateral hemifacial microsomia.
Neonates and infants with Pierre Robin sequence will have some degree of airway obstruction. Mild airway obstruction may require only lateral or prone positioning to relieve the obstruction. However, 25% of infants with Pierre Robin sequence have more severe obstruction, necessitating surgical intervention. Cor pulmonale can develop with severe chronic airway obstruction. Patients may also have vagal hyperactivity and brainstem dysfunction with periods of central apnea.
These patients often present with significant reflux and feeding difficulties secondary to anatomic abnormalities and with swallowing problems due to brainstem dysfunction.
The tongue-lip adhesion involves suturing the inferior portion of the tongue to the lower lip to prevent the tongue from falling to the back of the pharynx and causing obstruction. Once the mucosal flaps from the tongue and the lower lip are approximated, a temporary retention suture with a button is placed through the floor of the mouth and around the mandible to secure the repair. The goal of the tongue-lip adhesion is to relieve the airway obstruction and improve feeding until the mandible has had a chance to grow. The adhesion is left intact until the patient is approximately 1 year old, by which time the mandible has had time to grow and the adhesion can be taken down.
In infants with severe obstruction, mandibular distraction can be performed in infants less than 6 months old to rapidly distract the jaw.
Tracheostomy is a final option for the patients who fail conservative treatment, tongue-lip adhesion, and mandibular distraction. After the mandibles are distracted, the patient can be decannulated; it takes an average of 3 years for decannulation to occur.
Mask ventilation is difficult.
Direct laryngoscopy and tracheal intubation may be extremely difficult or impossible secondary to severe mandibular hypoplasia.
Consider awake, sedated, or anesthetized fiberoptic intubation, while maintaining spontaneous ventilation. Dexmedetomidine and/or ketamine may be titrated to produce the desired effect.
When infants have significant difficulty with ventilation or intubation, aside from oral pharyngeal and nasal airways, a suture (0 silk) can be placed at the base of the tongue to displace the tongue anteriorly to assist with ventilation or intubation.