Mandibular Distraction Osteogenesis as a Primary Intervention in Infants With Pierre Robin Sequence

Abstract

Background Pierre Robin sequence (PRS) is a triad of congenital facial abnormalities that can present as a syndrome (syndromic PRS [sPRS]) or an isolated entity (isolated PRS [iPRS]). Patients with PRS can develop airway and feeding problems that may result in failure to thrive. Mandibular distraction osteogenesis (MDO) is a method for improving the functional issues associated with breathing and feeding. There is a Paucity of literature evaluating the outcomes of MDO between sPRS and iPRS patients. Methods An institutional review board–approved retrospective review of PRS patients managed by a single surgeon and treated with MDO between January 2015 and December 2019 at a tertiary referral hospital was performed. The patients were stratified into iPRS or sPRS based on gene testing. Airway outcome measures included avoidance of tracheostomy, relief of sleep apnea, and oxygen saturation improvement. Primary feeding measures included achievement of full oral feeds and growth/weight gain. Statistical analysis included t tests and χ2 tests where appropriate using SPSS. Results Over the study period, of the 29 infants with PRS, 55% identified as iPRS and 45% as sPRS. There were no significant differences in the patient characteristics, apnea-hypoxia index (22.27 ± 12.27) and laryngeal view (3 ± 0.79) pre-MDO. After MDO, 83% of the subjects achieved a positive feeding outcome and 86% achieved a positive airway outcome with no statistical significance between sPRS and iPRS (P = 0.4369). There was a statistically significant change post-MDO in apnea-hypoxia index (5.24 ± 4.50, P = 0.02) and laryngeal view (1.59 ± 1.00, P = 0.01). Conclusions Our recent experience would lead us to believe that sPRS patients have greater morbidities and challenging clinical developments that, when properly evaluated, can be managed by MDO. There is a potential role for MDO in reducing the need for traditional surgical interventions for respiratory and feeding problems in both iPRS and sPRS patients.

Fluoroscopic‐Assisted Tongue Suspension: Advancement and Innovation in the Management of Complex Pediatric Obstructive Sleep Apnea

“…36 Mandibular distraction has been the gold standard for the management of OSA in children with Pierre Robin sequence, secondary to the underlying retrognathia as the primary cause of obstruction. 37 Although preliminary results of hypoglossal nerve stimulation show promise in the pediatric population, it is still not FDA-approved for use in children and its scope of application is only targeting children with Trisomy 21 and severe OSA above ten years old. 19 TBS is an alternative intervention that has been uncommonly described in children with OSA and glossoptosis.…”

Treated Pierre Robin Sequence Using Placed Allogenic Acellular Bone Matrix and Mandibular Distraction Osteogenesis in the Neonate

“…McCarthy et al ( 12 ) firstly reported gradual mandibular elongation in cases with congenital hypoplasia in 1992. Later on, prospective research of Soto et al ( 13 ) evaluated 29 cases with tongue-based upper airway obstruction treated with mandibular DO. Decannulation or extubation was performed postoperatively in all cases.…”

Study on the Effect of Bilateral Mandible Distraction Osteogenesis About the Nutrition Status of Infants With Pierre-Robin Sequence

“…The study on the nutritional status mainly focused on the changes in weight and body length followed by the airway improvement. More people believed that the improvement of nutritional status is a result of the alleviation of airway problems (24)(25)(26). However, some scholars believe that nutritional changes should be studied independently with airway changes, because breathing and swallowing difficulties in PRS patients may not only be related to anatomical variation (such as micrognathia and glossoptosis etc.…”