title: Down syndrome: identification and surgical management of obstructive sleep apnea.
abstract: To date, a paucity of information is available on the optimal management of obstructive sleep apnea in Down syndrome, which may have particularly important implications in this already vulnerable patient population. The objective of this study was to evaluate prospectively the results of a new surgical approach for the treatment of obstructive sleep apnea. Patients with Down syndrome and obstructive sleep apnea underwent preoperative and postoperative polysomnography and clinical and radiologic evaluation to determine prospectively the efficacy of sleep apnea surgery. Statistical testing of apnea index, respiratory disturbance index, and lowest oxygen saturation were compared by means of paired t tests. Seven children (five boys, two girls) from 3 to 12 years of age were subjected to a management protocol that included an aggressive surgical approach to the treatment of obstructive sleep apnea. Clinical symptoms and signs of obstructive sleep apnea, apnea index, respiratory disturbance index, lowest oxygen saturation, and surgical morbidity were the main outcome measures. Surgical treatment consisted of a combination of soft-tissue and skeletal alterations including tongue reduction (n = 6), tongue hyoid advancement (n = 4), uvulopalatopharyngoplasty (n = 7), and maxillary or midface advancement (n = 2). Polysomnography was obtained preoperatively and postoperatively in six patients. One patient was intubated preoperatively. Mean preoperative apnea index and respiratory disturbance index were 34.00 and 52.46 compared with mean postoperative values of 1.62 and 6.46, respectively. Clinically, all patients were improved symptomatically in terms of snoring, noisy breathing, and oxygen requirements. The one patient who had been intubated preoperatively for respiratory failure was extubated successfully but later developed recurrent tricuspid regurgitation and was found to have fixed pulmonary hypertension with cor pulmonale. This patient represented the only treatment failure and underwent tracheostomy. An aggressive surgical approach aimed at correcting all anatomic abnormalities associated with upper airway obstruction was applied successfully to the treatment of obstructive sleep apnea in Down syndrome. We suggest periodic polysomnography in patients with Down syndrome, especially if there is unexplained deterioration in mental capacity or other signs and symptoms of obstructive sleep apnea. Surgical treatment should address both the soft-tissue abnormalities and the skeletal deformities such as midface retrusion. Preoperative cardiac ultrasonography is important to determine the presence of right-sided heart failure, which may be an indication for cardiac catheterization to determine pulmonary venous pressures.
authors.affiliation: Center for Craniofacial Disorders, Scottish Rite Children's Medical Center, Atlanta, Ga., USA.
authors.forename: J F
authors.initials: JF
authors.lastname: Lefaivre
authors.forename: S R
authors.initials: SR
authors.lastname: Cohen
authors.forename: F D
authors.initials: FD
authors.lastname: Burstein
authors.forename: C
authors.initials: C
authors.lastname: Simms
authors.forename: P H
authors.initials: PH
authors.lastname: Scott
authors.forename: G L
authors.initials: GL
authors.lastname: Montgomery
authors.forename: L
authors.initials: L
authors.lastname: Graham
authors.forename: A V
authors.initials: AV
authors.lastname: Kattos
mesh_terms: D002648:Child; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008437:Maxilla / Q000601:surgery; D019340:Osteotomy, Le Fort; D010160:Palate, Soft / Q000601:surgery; D010614:Pharynx / Q000601:surgery; D017286:Polysomnography; D011446:Prospective Studies; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000601:surgery*; D014059:Tongue / Q000601:surgery; D016896:Treatment Outcome; D014609:Uvula / Q000601:surgery