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This case report describes 3 patients with Down syndrome and obstructive breathing problems, ages: 5 months, 15 months, and 22 years. The youngest one had normal cardiopulmonary function at birth, but soon developed a pulmonary hypertension. The next had a severe atrioventricular defect and additional pulmonary hypertension and there was little hope for her to survive heart surgery. The oldest one had had apneas since childhood with increasing severity, but was regarded as having normal heart function. All 3 were operated to relieve their breathing obstruction. The 5-month-old boy improved only slightly after an initial UPPP and had to be tracheotomized, which solved his problems. The tracheotomy could be removed when he was one year. The 15-month-old girl was cured of her breathing problems through an A + T and survived her heart surgery one month later. Tonsillectomy and UPPP was performed on the oldest patient. Following surgery, he had an episode of life-threatening bleeding and developed a DIC syndrome, and was critically ill for 18 days. After he recovered, his sleep apnea had improved. Once a myxoedema was diagnosed and treated, he made further progress. These cases stress the significance of early recognition of sleep apnea in children with Down syndrome and the importance of a careful preoperative investigation in collaboration with cardiologists.

A prospective study of 34 consecutive patients with the sleep apnea syndrome was undertaken to evaluate the effectiveness and complications of, and predictors of, results in a conservative type of uvulopalatopharyngoplasty (UPPP). At six months, 65% of the patients had a 50% or greater reduction in the number of apneas and hypopneas per hour (responders). The responders to UPPP were less severely affected preoperatively regarding apneas and hypopneas, the mean number of which (+/- SD) were 32.7 +/- 20.8 compared with 64.6 +/- 26.0. They also had a lower body mass index before UPPP (31.3 +/- 4.1 vs 36.0 +/- 7.0 kg/m2). Preoperative computed tomography and cephalometry indicated that the nonresponders had narrower upper airways. It is concluded that UPPP is the treatment of choice for mild to moderate sleep apnea syndrome, but not for severely affected, heavily overweight patients with an increased tongue width.

Two 12-year-old boys with severe sleep apnea syndrome but normal-sized tonsils were satisfactorily corrected by tonsillectomy and uvulopalatopharyngoplasty. One of the boys had muscular hypotony as contributing cause of the condition. The other had mandibular hypoplasia in combination with a long soft palate. Thorough preoperative anamnesis and examination in a sleep laboratory are necessary to determine which cases will benefit from surgery in spite of normal-sized tonsils and which surgical procedure will be most helpful.