The objective of this study was to evaluate the long-term success of mandibular distraction osteogenesis in patients with mandibular airway obstruction syndrome (MAOS), defined as obstructive sleep apnea, swallowing abnormalities, and failure to thrive in the presence of micrognathia, glossoptosis, gastroesophageal or laryngeal reflux, and microaspiration. This is a cross-sectional study at the International Craniofacial Institute in the Medical City Hospital, Dallas, TX. From January 1997 to July 2008, a group of 81 patients were examined for MAOS using medical history and physical examination. They all met the criteria for a multidisciplinary team evaluation consisting of upper airway endoscopy, swallowing evaluations, standard overnight polysomnography, and radiologic evaluations of the airway and craniofacial structures. In the 81 patients examined, Pierre Robin Sequence was diagnosed in 65; micrognathia and glossoptosis without cleft palate, in 13; and micrognathia as a component of a craniofacial syndrome (Treacher Collins syndrome), in 3. A total of 45 patients were documented to have gastroesophageal or laryngeal reflux.Only 41 patients had yet to receive any treatment at the time of evaluation; 40 patients presented with a tracheotomy.Of the 41 untreated patients, 14 responded to conservative treatment; Pierre Robin syndrome was diagnosed in all of them, and they did not require mandibular distraction or other surgical procedure to improve the MAOS.Bilateral distraction osteogenesis of the mandible was performed in 67 patients; 27 were younger than 6 months at the beginning of the distraction, and 40 were older than 6 months (mean age at distraction, 1.2 yr). Of this group, 26 patients did not have any prior surgical treatment, and 41 patients already had a tracheotomy. The distraction devices used were internal in 33 (49.26%) and external in 34 (50.74%) of 67 patients. The distraction protocol consisted of a 24-hour latency period and then a 1-mm/d activation period. The mean activation period was 19.4 days (range, 10-27 d), the mean consolidation period was 73 days, and the mean length of distraction was 22 mm (range, 10-32 mm). Tracheostomy was prevented in 25 (96%) of 26 patients, and decannulation after distraction was possible in 38 (92%) of 41 patients. Success, defined as decannulation within 1 year of the start of distraction or prevention of tracheostomy in a patient otherwise deemed as a candidate, was found in 63 (94%) of 67 patients. Oral feedings have been resumed in 61 (91%) of 67 patients.Complications observed were mostly related to pin site infections requiring antibiotics. Device failure was experienced in 2 (3%) of 66 internal devices and in 7 (10.2%) of 68 external devices.Distraction osteogenesis of the mandible provides an excellent treatment for mandibular airway obstruction in patients younger than 6 years who do not respond to conservative measures and allows for early decannulation in patients who previously underwent tracheotomy.