摘要
Background/purpose The authors experienced 3 cases of persistent pulmonary hyp ertension of the neonate (PPHN)-associated with duodenal atresia with paradoxic al dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association. Methods The medical c harts of the patients were reviewed retrospectively. Results Case 1 was a 2, 862 -g male infant with a prenatal diagnosis of duodenal atresia. The dilated intes tine was identified as the distal blind end of the duodenum during duodeno-duod enostomy. PPHN developed after surgery, and the patient died despite treatment w ith inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). L ung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment o f pulmonary vessels. Case 2 was a 2, 244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in de ath. Autopsy results showed ACD with misalignment of pulmonary vessels and duode nal atresia associated with dilatation of the distal blind end. Case 3 was a 2, 462-g female infant with a prenatal diagnosis of duodenal atresia associated wi th dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully u nder combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrason ographic findings showed enlarged echogenic fetal lungs as in the first 2 cases. Conclusions The characteristics of this novel association are enlarged echogeni c fetal lungs, duodenal atresia associated with paradoxical dilatation of the di stal blind end, and refractory PPHN resistant to iNO.
Background/purpose The authors experienced 3 cases of persistent pulmonary hyp ertension of the neonate (PPHN)-associated with duodenal atresia with paradoxic al dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association. Methods The medical c harts of the patients were reviewed retrospectively. Results Case 1 was a 2, 862 -g male infant with a prenatal diagnosis of duodenal atresia. The dilated intes tine was identified as the distal blind end of the duodenum during duodeno-duod enostomy. PPHN developed after surgery, and the patient died despite treatment w ith inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). L ung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment o f pulmonary vessels. Case 2 was a 2, 244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in de ath. Autopsy results showed ACD with misalignment of pulmonary vessels and duode nal atresia associated with dilatation of the distal blind end. Case 3 was a 2, 462-g female infant with a prenatal diagnosis of duodenal atresia associated wi th dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully u nder combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrason ographic findings showed enlarged echogenic fetal lungs as in the first 2 cases. Conclusions The characteristics of this novel association are enlarged echogeni c fetal lungs, duodenal atresia associated with paradoxical dilatation of the di stal blind end, and refractory PPHN resistant to iNO.
