Objective: To evaluate the course and prognosis of airway obstruction and feeding difficulty in the Pierre Robin sequence (PRS).
Methods: Retrospective review of 60 patients with PRS between 1993 and 2002 at the University of California, Davis Medical Center. Patients were placed into diagnostic subgroups: (1) Isolated PRS; (2) Syndromic PRS (known syndrome with PRS); (3) Unique PRS (unique anomalies with PRS). Data regarding severity, duration, and management of airway obstruction and feeding difficulty were collected.
Results: Airway obstruction requiring intervention beyond positional therapy was seen in 28% isolated, 42% syndromic, and 58% unique PRS. One-third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two-thirds of patients, who failed positional therapy required a surgical airway procedure. Four patients underwent mandibular distraction osteogenesis, resulting in successful decannulation or avoidance of tracheostomy. Thirteen patients underwent tracheostomy; mean duration of tracheostomy-dependence was 17.0 months in Isolated PRS and 31.7 months in Unique PRS (p < 0.01). Successful decannulation by age of 3 years was confirmed in 85% of patients who underwent tracheostomy. Tube feeding was required in 53% Isolated, 67% Syndromic, and 83% Unique PRS. Forty-two percent of PRS patients with a successful positional airway still demonstrated feeding difficulty. Short-term (0-3 months) and intermediate (4-18 months) tube feeding was more commonly required in Isolated and Syndromic PRS, while long-term (beyond 18 months) gastrostomy tube feeding was more commonly required in Unique PRS (p < 0.01). By 3 years of age, a successful oral diet was seen in 91% Isolated, 92% Syndromic, and 78% Unique PRS.
Conclusions: Diagnostic subgroups based on the presence of additional anomalies help families and physicians in understanding the severity and duration of feeding and airway difficulty in PRS. Two-thirds of PRS patients who fail positional therapy may ultimately require a surgical airway procedure. Feeding difficulty can be present in the absence of clinically significant airway obstruction. Families and physicians should be encouraged that by 3 years of age, most patients were successfully taking an oral diet without airway obstruction.