DOWN-SYNDROME - IDENTIFICATION AND SURGICAL-MANAGEMENT OF OBSTRUCTIVESLEEP-APNEA

To date, a paucity of information is available on the optimal manageme nt of obstructive sleep apnea in Down syndrome, which may have particu larly important implications in this already vulnerable patient popula tion. The objective of this study was to evaluate prospectively the re sults of a new surgical approach for the treatment of obstructive slee p apnea. Patients with Down syndrome and obstructive sleep apnea under went preoperative and postoperative polysomnography and clinical and r adiologic evaluation to determine prospectively the efficacy of sleep apnea surgery. Statistical testing of apnea index, respiratory disturb ance index, and lowest oxygen saturation were compared by means of pai red t tests. Seven children (five boys, two girls) from 3 to 12 years of age were subjected to a management protocol that included an aggres sive surgical approach to the treatment of obstructive sleep apnea. Cl inical symptoms and signs of obstructive sleep apnea, apnea index, res piratory disturbance index, lowest oxygen saturation, and surgical mor bidity were the main outcome measures. Surgical treatment consisted of a combination of soft-tissue and skeletal alterations including tongu e reduction (n = 6), tongue hyoid advancement (n = 4), uvulopalatophar yngoplasty (n = 7), and maxillary or midface advancement (n = 2). Poly somnography was obtained preoperatively and postoperatively in six pat ients. One patient was intubated preoperatively. Mean preoperative apn ea index and respiratory disturbance index were 34.00 and 52.46 compar ed with mean postoperative values of 1.62 and 6.46, respectively. Clin ically, all patients were improved symptomatically in terms of snoring , noisy breathing, and oxygen requirements. The one patient who had be en intubated preoperatively for respiratory failure was extubated succ essfully but later developed recurrent tricuspid regurgitation and was found to have fixed pulmonary hypertension with cor pulmonale. This p atient represented the only treatment failure and underwent tracheosto my. An aggressive surgical approach aimed at correcting all anatomic a bnormalities associated with upper airway obstruction was applied succ essfully to the treatment of obstructive sleep apnea in Down syndrome. We suggest periodic polysomnography in patients with Down syndrome, e specially if there is unexplained deterioration in mental capacity or other signs and symptoms of obstructive sleep apnea. Surgical treatmen t should address both the soft-tissue abnormalities and the skeletal d eformities such as midface retrusion. Preoperative cardiac ultrasonogr aphy is important to determine the presence of right-sided heart failu re, which may be an indication for cardiac catheterization to determin e pulmonary venous pressures.