Surgical treatment of Robin sequence and sleep apnea syndrome: Case report and review of the literature

Abstract

Pierre Robin sequence is an etiologically heteroge­ neous condition comprising a variety ofmalformations. Common features include, but are not limited to, mi­ crognathia, glossoptosis, wide If-shaped cleft palate, and upper airway obstruction. Although some cases of the symptom complex had been reported in the 19th century, the French stomatologist Pierre Robin) de­ scribed the essential problems associated with micro­ gnathia and introduced the term glossoptosis in 1923. Dr Robin attributed failure to thrive and even death to. respiratory and nutritional insufficiency, mainly caused by the tongue falling backward and downward in such a way as to obstruct upper airway passage. Palatal clefting was not mentioned in this ini­ tial description of the disease complex. Current knowledge suggeststhat micrognathia is the primary anomaly that causes both cleft palate and up­ per airway obstruction in newborns with Robin se­ quence.' Endoscopic evaluation ofthe pharyngeal air­ way reveals that both the origin and mechanism of airway obstruction inducing repetitive episodes of ob­ structive apnea are multifactorial.' Due to the close relationship ofanatomic abnormalities ofthe mandible and neuromuscular dysfunction of the inspiratory musculature, no single causative factor has been iso­ lated thus far. The effectiveness of a variety of treatment ap­ proaches remains controversial. Methods that have evolved include lateral and prone positioning of the newborn; application of intraoral devices and naso-