Introduction

Pierre Robin sequence (PRS) is a rare (1:8500) congenital anomaly characterized by the triad micrognathia, glossoptosis, and cleft palate, a feature present in more than 90% of cases. The major physiological sequelae after PRS are breathing and feeding problems due to the abnormally small jaw that encourages the tongue to fall back into the pharynx and obstruct the airway. In most neonates with PRS, airway obstruction responds to non-invasive treatments such as prone positioning and nasopharyngeal continuous positive airway pressure. Up to 23% of patients with micrognathia may, however, require interventions beyond these supportive measures, including intubation or tracheostomy. Although tracheostomy in neonatal airway obstruction may be life-saving, it lengthens hospital stays, increases health care costs, and raises problems with care outside the hospital.

The tongue–lip adhesion (TLA) procedure, or glossopexy, was introduced as a surgical option to avoid tracheostomy. In TLA the tongue is anchored to the lower lip and mandible ensuring an anterior lingual position to alleviate the upper airway obstruction. This procedure is typically done in the first months of life and is reversed at around 12 months. Although it is frequently effective in relieving a tongue-based airway obstruction, several investigators consider TLA a temporary procedure because it often requires multiple secondary interventions to achieve airway patency and adequate feeding. TLA may also lead to several complications, including dehiscence, tongue lacerations, injuries to Wharton’s ducts, wound infections, scar deformation, and aspiration pneumonia.

Another surgical procedure for the management of airway problems in infants with micrognathia is jaw advancement by mandibular distraction osteogenesis (MDO). Distraction osteogenesis is a technique that was first introduced for lengthening the long bones in the body. As the mandible is lengthened, the anterior mandibular muscles pull the tongue forward, increasing the airway space and relieving airway obstruction. By acting on bone and soft tissue, MDO can definitively correct micrognathia, eliminating the need for a tracheostomy in 90–95% of cases. Although MDO has advanced craniofacial surgery remarkably, it requires time to complete and may lead to complications resulting from injury to the marginal mandibular branch of the facial nerve and the inferior alveolar nerve and damage to the premolar germs.

No studies published to date have compared the effectiveness of TLA and MDO in managing breathing and feeding in infants with PRS treated in a single institution. Having this information would help in the selection of the surgical procedure most likely to provide the best outcome for infants with PRS.

In this single-centre retrospective study we compared the outcomes of infants with severe breathing difficulties related to PRS resistant to conservative treatment, who underwent TLA or MDO procedures to correct severe airway obstruction. The primary outcome measures were successful weaning from respiratory support and resumption of full oral feeding.