Improved Outcome in Pierre Robin Sequence: Effect of Multidisciplinary Evaluation and Management

Abstract

Infants with complications of Pierre Robin sequence are at increased risk of airway obstruction and resultant hypoxia, cor pulmonale, failure to thrive, and cerebral impairment. In an effort to minimize such complications, patients with Pierre Robin sequence were examined prospectively by a multidisciplinary team using polysomnography and continuous oximetry. Obstructive apnea and desaturation occurred in 18 of the 21 patients studied. Four children required only home apnea monitoning, and six required only monitoring and supplemental oxygen. Seven children had lip-tongue adhesion procedures performed, and four required tracheostomy. No patients died. All patients with isolated Pierre Robin sequence had normal development at follow-up except for one child who had experienced a respiratory arrest before referral. With improvements in neonatal intensive care, testing for respiratory assessment, improved surgical and postoperative intervention and home monitoring, the morbidity and mortality for children with Pierre Robin sequence can be reduced markedly.

Neonates with Tongue‐Based Airway Obstruction

“…Six studies were evaluated that report on neurodevelopmental impairment (NDI). When cohorts of PRS patients have been evaluated, rates of NDI range from 11.5% to 23.8% 15,18 ,39,66,91, 92 . Similar to the studies on mortality, several authors have shown that rates of NDI are significantly higher in children with syndromes or associated abnormalities (23%‐66%) when compared with nonsyndromic patients (7.6%‐10%) and are higher than the general population 15,18 , 39 .…”

“…Seventeen studies reported that most of their study population was managed successfully with only conservative measures *. Most of the studies include a mixed population of patients (isolated PRS, syndromic PRS, unique PRS, or other), but 4 studies reported more surgical intervention was needed in the syndromic or unique PRS population, 5,20 ,30, 38 and 2 studies reported more success with conservative management in the isolated PRS population 5,39 . However, in contrast to this, Evans et al 15 reported no statistical difference in the frequency of surgery between syndromic and nonsyndromic patients ( P =.…”

“…Fifteen studies were found that used PSG in neonates with TBAO to document the presence and severity of obstructive breathing during sleep and to measure outcomes after surgical intervention ‖ . Three studies used PSG in patients before and after TLA and suggested improvement in postoperative PSG, but quantitative data were not provided 39,55 , 57 . Cohen et al 71 used PSG after a variety of surgical interventions and showed that the average RDI improved from severe to mild, and the average oxygen saturation nadir rose from 61% to 92%.…”

“…When cohorts of PRS patients have been evaluated, rates of NDI range from 11.5% to 23.8% 15,18 ,39,66,91, 92 . Similar to the studies on mortality, several authors have shown that rates of NDI are significantly higher in children with syndromes or associated abnormalities (23%‐66%) when compared with nonsyndromic patients (7.6%‐10%) and are higher than the general population 15,18 , 39 . Drescher et al 92 showed that infants with isolated upper airway obstruction had cognitive development that fell within the normal reference range, but PRS patients consistently demonstrated worse cognitive development than controls in sequential processing ( P =.…”

“…* Most of the studies include a mixed population of patients (isolated PRS, syndromic PRS, unique PRS, or other), but 4 studies reported more surgical intervention was needed in the syndromic or unique PRS population, 5,20,30,38 and 2 studies reported more success with conservative management in the isolated PRS population. 5,39 However, in contrast to this, Evans et al 15 reported no statistical difference in the frequency of surgery between syndromic and nonsyndromic patients (P = .264). Buchenau et al 25 reported success in a prospective randomized controlled trial of 11 patients using a palatal obturator with oropharyngeal extensions, but this single prospective randomized controlled trial is limited by its small treatment population and potential for a crossover effect.…”