Background: Pierre Robin sequence is characterized by mandibular retrognathia and glossoptosis resulting in airway obstruction and feeding difficulties. When conservative management fails, mandibular distraction osteogenesis or tongue-lip adhesion may be required to avoid tracheostomy. The authors’ goal was to prospectively evaluate the airway and feeding outcomes of their comprehensive approach to Pierre Robin sequence, which includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion.
Methods: A longitudinal study of newborns with Pierre Robin sequence treated at a pediatric academic medical center between 2010 and 2015 was performed. Baseline feeding and respiratory data were collected. Patients underwent conservative management if they demonstrated sustainable weight gain without tube feeds, and if their airway was stable with positioning alone. Patients who required surgery underwent tongue-lip adhesion or mandibular distraction osteogenesis based on family and surgeon preference. Postoperative airway and feeding data were collected.
Results: Twenty-eight patients with Pierre Robin sequence were followed prospectively. Thirty-two percent had a syndrome. Ten underwent mandibular distraction osteogenesis, eight underwent tongue-lip adhesion, and 10 were treated conservatively. There were no differences in days to extubation or discharge, change in weight percentile, requirement for gastrostomy tube, or residual obstructive sleep apnea between the three groups. No patients required tracheostomy. The greatest reduction in apnea-hypopnea index occurred with mandibular distraction osteogenesis, followed by tongue-lip adhesion and conservative management.
Conclusions: Careful selection of which patients with Pierre Robin sequence need surgery, and of the most appropriate surgical procedure for each patient, can minimize the need for postprocedure tracheostomy. A comprehensive approach to Pierre Robin sequence that includes conservative management, mandibular distraction osteogenesis, and tongue-lip adhesion can result in excellent airway and feeding outcomes.
CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.
From the Department of Plastic Surgery, The Ohio State University College of Medicine; and the Department of Plastic and Reconstructive Surgery, the Department of Speech Pathology, and the Sleep Disorder Center, Division of Pulmonary Medicine, Nationwide Children’s Hospital.
Received for publication April 19, 2016; accepted September 12, 2016.
Disclosure: None of the authors has any financial interest in any products or techniques mentioned in this article.
Gregory D. Pearson, M.D., Department of Plastic Surgery, The Ohio State University Wexner Medical Center, 915 Olentangy River Road, Suite 2100, Columbus, Ohio 43212, firstname.lastname@example.org