Surgical Management and Outcomes of Pierre Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion

Abstract

There is a paucity of literature directly comparing tongue-lip adhesion versus mandibular distraction osteogenesis in surgical treatment of patients with Pierre Robin sequence. This study comprehensively reviews the literature for evaluating airway and feeding outcomes following mandibular distraction osteogenesis and tongue-lip adhesion. A search was performed using the MEDLINE and Embase databases for publications between 1960 and June of 2017. English-language, original studies subjects were included. Extracted data included prevention of tracheostomy (primary airway outcome) and ability to feed exclusively by mouth (primary feeding outcome). A total of 67 studies were included. Ninety-five percent of subjects (657 of 693) treated with mandibular distraction osteogenesis avoided tracheostomy, compared to 89% of subjects (289 of 323) treated with tongue-lip adhesion. Eighty-seven percent of subjects (323 of 370) treated with mandibular distraction osteogenesis achieved full oral feeds at latest follow-up. Seventy percent of subjects (110 of 157) treated with tongue-lip adhesion achieved full oral feeds at latest follow-up. The incidence of second intervention for recurrent obstruction ranged from 4 to 6 percent in mandibular distraction osteogenesis studies, compared to a range of 22 to 45 percent in tongue-lip adhesion studies. Variability of patient selection, surgical techniques, outcomes measurement methods, and follow-up length across studies precluded meta-analysis of the data. Both mandibular distraction osteogenesis and tongue-lip adhesion are effective alternatives to tracheostomy for patients who fail conservative management and improve feeding. Mandibular distraction osteogenesis may be superior to tongue-lip adhesion in long-term resolution of airway obstruction and avoidance of gastrostomy, but is associated with notable complications.

Long Term Speech and Feeding Outcomes in Patients With Pierre Robin Sequence

“…[28][29][30] Though a number of decision algorithms have been published, there is a paucity of supporting evidence for these tools. 4 One previous study systematically reviewed all feeding and airway outcomes for children with PRS treated with MDO or TLA. These authors found comparable rates of tracheostomy avoidance; results that are supported by the data presented herein.…”

“…15,16 The selection of MDO versus TLA is controversial, and to date, no randomized trials exist to guide management. 4 Although MDO and TLA have shown comparable outcomes with respect to tracheostomy avoidance 4 and feeding, 17 there remains a paucity of evidence on the effect of TLA and MDO on speech. Although cleft-related speech disorders have been well studied in children with PRS, 18,19 we are only aware of 1 other study that has explicitly examined the effects of surgical airway interventions on consonant production errors (LeBlanc and Golding-Kushner, 1992).…”

“…1,2 In approximately half of cases, PRS may also be associated with a genetic syndrome, and up to 90% of infants may also have a concurrent cleft palate. 3,4 A small jaw and ptotic tongue result in posterior displacement of the tongue against the posterior pharyngeal wall and subsequent airway obstruction in PRS. Airway obstruction also contributes to poor feeding in infants with PRS, often necessitating the insertion of a nasogastric or gastrostomy tube (g-tube).…”

Phenotypes, Developmental Basis, and Genetics of Pierre Robin Complex

“…While a tracheostomy involves many quality of life considerations and appears to have a higher mortality associated in syndromic PR patients [ 47 ], mandibular distraction osteogenesis (MDO) requires two operations, i.e., one to create mandibular osteomies and apply distraction devices, and a second to remove the devices after completion of distraction and consolidation. Feeding issues may be addressed by glossopexy (tongue–lip adhesion) or MDO [ 48 ]. Due to the individuality of each PR case presentation, no one treatment is best suited to all patients, and each possible intervention is accompanied by benefits and risks that must be carefully evaluated by a multidisciplinary team.…”

Upper Airway Anomalies

“…Tongue-lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) have both been described. Studies have shown that MDO is associated with less recurrent obstruction and better oral feeding results [11,12]. However, patients who fail these surgical interventions will ultimately require tracheostomy.…”