Pierre Robin sequence: Secondary respiratory difficulties and intrinsic feeding abnormalities

Objective: There is considerable variation in opinion regarding the optimal management of patients with Pierre Robin sequence (PRS), No single method of airway intervention or feeding strategy is universally appropriate and effective, This study was performed to examine methods used for airway and feeding management and to identify specific problems encountered. Study Design: A retrospective study of 252 patient charts between 1989 and 1997 at Children's Hospital of Wisconsin. Methods: Patient information was collected regarding perinatal history, genetics evaluation, and airway and feeding evaluations and intervention, A group of 47 patients was determined as having PRS. Results: Secondary respiratory difficulties, defined as respiratory abnormalities in addition to the expected PRS obstruction, were identified in 23% of patients. Also, intrinsic feeding abnormalities not associated with airway obstruction were identified in 11% of patients. Analysis by Fisher's Exact Test revealed patients with a syndromic diagnosis to have a significantly higher rate for tracheotomies and gastrostomy tube placement (P = .041, and P = .0004, respectively), Syndromic patients were also found to have significantly lower Apgar scores and longer hospital stays. Positioning techniques, tongue-lip adhesion, and tracheotomy were also employed effectively with specific indications and specific difficulties that need to be considered. Conclusion: Patients with PRS require thorough airway and feeding evaluation. Those with additional syndromic diagnoses demonstrate higher rates of more invasive interventions. Patients with PRS must undergo individualized approaches with consideration of multiple factors for successful management.