Treatment of Upper Airway Obstruction and Feeding Problems in Robin-Like Phenotype

Abstract

obin sequence (RS) was first delineated by Fairbairn in 1846 and Shukowsky in 1911; it was more formally investigated by the French stomatologist Pierre Robin in 1934, who later became the source of the eponym for this condition. Robin described it as consisting of a hypoplastic or retropositioned mandible and glossoptosis, resulting in respiratory distress, with or without a cleft palate. It fulfills criteria for a sequence, in which one or all anomalies found are secondary to another anomaly. The incidence varies between one in 8500 and one in 14 000 live births. The main functional problems associated with RS are upper airway obstruction (UAO) and failure to thrive (the latter resulting from feeding problems, UAO alone, or both problems). Feeding problems are often long-lasting, resulting in gastrostomy rates of 50% in large series. Interventions for RS should thus be assessed by their ability to improve polysomnography (PSG) results and weight gain. Although reported as used in infants with RS by 74% of US pediatric otolaryngology fellowship programs in 1994, only 39% believed PSG to be indicated for most patients with this condition, and just one in 5 performed serial PSG to test for treatment effectiveness. However, it is difficult to document the effect of treatments for RS on UAO without performing PSG, especially because anatomy does not correlate well with function. For this Medical Progress article, we searched PubMed for studies on the effectiveness of interventions suggested to improve UAO and weight gain in infants with RS (Table). It does not cover surgical techniques for which evidence in the literature is extremely scant or objective documentation of their effectiveness lacking.