Obstructive sleep apnea in Apert's and Pfeiffer's syndromes: more than a craniofacial abnormality.

Nine acrocephalosyndactyly type I patients (Apert's syndrome) and three acrocephalosyndactyly type V patients (Pfeiffer's syndrome) were evaluated for the relative importance of upper and lower airway abnormalities in the generation of obstructive sleep apnea. All patients were found to have a combination of upper and lower abnormalities. The influence of lower pathology was greater in the infants, and the influence of upper airway, specifically pharyngeal, was greater in the adults. A comparison between preoperative and postoperative polysomnography revealed little improvement with standard craniofacial advancements. Furthermore, three patients are described who succumbed to pulmonary death despite tracheostomy. Conservative treatment with prone or lateral positioning and medical pulmonary regimens is advocated. Finally, the pathogenesis of this diffuse airway pathology is discussed.

Respiratory complications in children with spondyloepiphyseal dysplasia congenita.

Mild respiratory distress at birth is a common occurrence in infants with spondyloepiphyseal dysplasia congenita (SEDC) while severe respiratory insufficiency and death have been reported only rarely. We describe three infants with SEDC who experienced severe respiratory complications and required tracheostomies. Two of these infants have also needed long-term continuous positive airway pressure to maintain adequate ventilation. The features and clinical course of these children are described, previous reports of respiratory complications in SEDC are summarized, mechanisms resulting in respiratory problems in SEDC are postulated, and comparison is made with mechanisms giving rise to respiratory risks in other skeletal dysplasias.

Reduction of nosocomial infection during pediatric intensive care by protective isolation.

To determine whether simple protective isolation reduces the incidence of nosocomial bacterial and fungal infection during pediatric intensive care, we randomly assigned 70 children who were not immuno-suppressed and who required mechanical ventilatory support and three or more days of intensive care to receive standard care (n = 38) or protective isolation (n = 32) with use of disposable, non-waven, polypropylene gowns and nonsterile latex gloves. Risk factors predisposing patients to infection were comparable in the two groups. Nosocomial colonization occurred later among isolated patients (median, vs. 7 days; P less than 0.01) and was associated with subsequent infection in 12 patients, as compared with 12 patients given standard care (P = 0.01). Among patients who were isolated, the interval before the first infection was significantly longer than (median, 20 vs. 8 days; P = 0.04), the daily infection rate was 2.2 times lower than (95 percent confidence interval, 1.2 to 4.0; P = 0.007), and there were fewer days with fewer (13 percent vs. 21 percent; P = 0.001). The benefit of isolation was most notable after seven days of intensive care. Isolation was well tolerated by patients and their families. Regular monitoring showed that the children in each group were touched and handled comparably often by hospital personnel and family members. We conclude that the use of disposable, high-barrier gowns and gloves for the care of selected, high-risk children who require prolonged intensive care significantly reduces the incidence of nosocomial infection, is well tolerated, and does not compromise the delivery of care.