Pyloric atresia and epidermolysis bullosa letalis are rare congenital anomalies. The association of the two diseases in siblings has not been reported previously. This paper describes such as association in two newborn infants of caucasian parents with no family history of similar anomalies. Problems in management included severe fluid and electrolyte losses, systemic infection, and prematurity, which led to the death of the two infants. The question is raised whether the two abnormalities are expressions of closely linked genes. The presence of epidermolysis bullosa should alert the pediatrician to the possiblity of a coexisting pyloric atresia.

4 newborn infants requiring prolonged assisted mechanical ventilation (mean 64 days) developed cardiopulmonary complications consisting of: progressive pulmonary air trapping, interstitial, emphysema, pneumothorax, and clinical signs and symptoms of right heart failure. The diagnosis of cor pulmonale was confirmed by cardiac catheterization and cineangiography in 3 of the 4 cases. Each infant had one or more of the following patterns of assisted mechanical ventilation: high peak inspiratory pressure, prolonged positive end expiratory pressure, or prolonged total duration of positive pressure. Three infants died; two had autopsies with evidence of bronchopulmonary dysplasia and cor pulmonale. The surviving infant ventilated for meconium aspiration, now two years old, has persistent pulmonary hyperexpansion and has been readmitted twice for respiratory insufficiency and right heart failure. It is likely that the incidence of cor pulmonale subsequent to pulmonary hyperaeration is higher than currently identified. Limitation of amount and duration of positive pressure may prevent the development of this complication of assisted ventilation.