Abstract
Alveolar capillary dysplasia (ACD) is a uniformly lethal lung disorder found in newborns and characterized by severe pulmonary hypertension and hypoxemia.Histologically, it is identified by inadequately capillarized alveoli.We experienced a case of ACD.The case patient was a 2670g-boy, the product of a nonconsanguineous marriage, who was born by vaginal delivery at 40 weeks of gestation.Shortly after birth, he suffered from respiratory distress and cyanosis, and chest x-ray demonstrated bilateral pneumothrax.Echo-cardiography revealed the right to left shunt at the level of foramen ovale and ductus arteriosus.With the difference in oxygen saturation between pre-and post-ductus, the diagnosis of persistent pulmonary hypertension was made.He required mechanical ventilation and medical treatment such as administration of cathecholamine, nitroglycerine, and tolazoline. Although these treatment was initially effective and resulted in weaning oxygen off on seven days of his age, oxygenation was seriously deteriorated on 12 days of age.He required mechanical ventilation with FiO2 of 1.0, peak inspiratory pressure of 50cmH2O, and a maximal positive end-expiratory pressure of 8cmH2O. Despite the administration of prostaglandinI2 for PPHN, his oxygenation was not improved at all.Eventually, the PaCO2 level was elevated and debris was attached to the tracheal tube extensively.The pathological findings of the debris was confirmed as necrotizing tracheobronchitis (NTB).Ventilation insufficiency was deteriorated progressively and he was expired.Postmortem examination revealed ACD and NTB.(JJPP 2003: 14: 156-160)
