Long Term Speech and Feeding Outcomes in Patients With Pierre Robin Sequence

Airway obstruction in newborns with Pierre Robin sequence (PRS) may be managed with tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), or tracheostomy to prevent airway compromise when conservative airway interventions fail or are contraindicated based on the type of obstruction present. Unfortunately, some of these procedures have the potential to affect a child's speech and feeding development.

The authors retrospectively reviewed the records of all children with PRS treated at our institution in the last 25 years. Our primary outcomes of interests were: (1) consonant production errors; (2) achievement of full oral feeds; (3) need for prolonged gastrostomy tube feeds; and (4) avoidance of tracheostomy.

Seven (7/73, 10%) children required intubation at birth for respiratory failure. Forty-two children were treated with TLA (42/73, 58%), 2 with MDO (2/73, 3%), and 1 (1/73, 1%) with tracheostomy. Twenty-one (21/73, 29%) were treated with conservative airway interventions. Of the 7 children requiring intubation, 1 required tracheostomy, and 6 required TLA. Five children who received TLA initially proceeded to tracheostomy. Of the 3 children who underwent MDO, 2 required tracheostomy. Of the 2 patients who underwent MDO, 2 failed, requiring tracheostomy. One child required tracheostomy as the first airway intervention. Among all children with PRS, /s,z/ speech errors were the most common. Children treated with conservative airway interventions had significantly fewer /sh/ errors at age 3 (X = 6.604, P < 0.05) relative to those treated with TLA, MDO, and/or tracheostomy. Consonant production errors extinguished over time, with significantly less /s,z/ errors produced at age 8 compared to at ages 3 (Z = -2.263, P < 0.01), 4 (Z = -2.449, P < 0.05), 5 (Z = -2.775, P < 0.01), and 6 (Z = -2.049, P < 0.05). Among all children, 70% (51/70) were able to achieve full oral feeds.

This study describes speech-production and feeding outcomes in children with PRS. Tongue-tip sound errors, including /s,z/, are prominent early in speech development but later extinguish, a pattern of speech maturation that follows that of typically-developing children.Most children were able to achieve full oral feeds, with few requiring prolonged g-tube placement. We hope these results serve as a useful tool in managing speech and feeding in children with TLA, and when counselling patients with PRS requiring definitive airway surgery.