Airway And Feeding Outcomes Of Pierre Robin Sequence: A Retrospective Review Of Surgical And Conservative Management
Edgar Soto, Carter Boyd, BS, Shivani Ananthasekar, BS, Timothy W. King, MD, PhD, Rene P. Myers, MD.
University of Alabama at Birmingham School of Medicine, Birmingham, AL, USA.
PURPOSE: Pierre Robins sequence (PRS) is a triad of congenital facial abnormalities that includes micrognathia, glossoptosis and upper airway obstruction. Current evidence suggests that the incidence of PRS is 1 in 8500 live births with a mortality rate of between 10-30%. PRS potentially leads to life-threatening obstructive apnea and feeding difficulties during the neonatal period. Understanding potential airway lesions in concordance with potential feeding problems is important for determining appropriate treatment: observation, tounge-lip adhesion, mandibular distraction osteogenesis (MDO), or tracheostomy. MDO has shown superior outcomes in many studies, but there is currently not enough studies evaluating the effects of MDO on the treatment of feeding and airway obstructions in PRS patients.
METHODS: A retrospective review of patients presented with PRS at a tertiary referral hospital - Children's of Alabama between January 2015 and December 2018 was performed. The patients were stratified into two groups based on the genetic information either isolated (iPRS) or syndromic (sPRS) PRS. Patients with associated clinical features or genetic anomalies were classified as sPRS. The primary outcome measure was improvement in airway and feeding outcomes as well as the presence of complication following treatment. The airway outcome measure included avoidance of tracheostomy (primary airway), decannulation, relief of OSA, improvement in oxygen saturation and the repeat of procedure. The primary feeding measure included achievement of full oral feeds at latest follow up (primary feeding), growth/weight gain, and improvement in gastroesophageal reflux. Complications included external scarring, infection, hardware exposure, device dislodgement, facial nerve problems, and tooth-bud damage. Multivariate analyses were then conducted through logistic regression models involving respiratory disorders for one model, feeding disorders in a second model as binary dependent variables using IBM SPSS Statistics.
RESULTS: Over the study period, we identified 26 infants with PRS who were treated with MDO and managed by a single surgeon. PRS was isolated in 52% of patients and syndromic in 48%. Associated syndromes in sPRS patients was noted. The timing of the operative intervention, the avoidance of tracheostomy, the size of the mandible pre and post distraction, and the achievement of full oral feeds was reported. Greater than 50% of subjects treated with MDO achieved a positive feeding outcome and at least 60% achieved a positive airway outcome. We will also discuss in detail the type of MDO performed, the mandible measurement variability in PRS patients, and the use of latency and activation phase during distraction.
CONCLUSION: he avoidance of an additional surgical intervention and decreasing time needed to care for a neonate are important potential benefits for patient quality of life and caretaker burden in PRS patients. This study presents the use of MDO as an effective surgical technique in improving outcomes of speech and mandibular defects in PRS patients at a tertiary referral center.
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