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...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.展开更多
Purpose Although intussusception has been reported as quite a rare cause of je junoileal atresia (JIA), pediatric surgeons have noted the frequent presence of intussusception as well as volvulus at surgery. The aim of...Purpose Although intussusception has been reported as quite a rare cause of je junoileal atresia (JIA), pediatric surgeons have noted the frequent presence of intussusception as well as volvulus at surgery. The aim of this study was to inv estigate the contribution of intrauterine intussusception and volvulus to the de velopment of JIA. Methods In 48 newborns (24 boys and 24 girls) treated for JIA at our hospital between 1978 and 2004, the operative and pathologic findings wer e reviewed. Results Intussusceptionwas responsible for gap and cord type atresia in 12 cases (25%). The cord showed an atrophic intestinal lumen in 2 cases. Vo lvulus was observed in 13 cases. Volvulus and intussusception were simultaneousl y noted in 1 case. This suggested that intussusception was the cause of the atre sia, whereas volvulus was a secondary event. Neither intussusception nor volvulu s was observed in high jejunal, apple peel, or multiple atresia. Conclusions Int rauterine volvulus and intussusception were commonly observed in single mid-and low JIA. Thus, intrauterine intussusception may be a common cause of gap and co rd type JIA. Volvulus may not only cause JIA but also result from anatomic chang es after the development of JIA in some cases.展开更多
文摘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.
文摘Purpose Although intussusception has been reported as quite a rare cause of je junoileal atresia (JIA), pediatric surgeons have noted the frequent presence of intussusception as well as volvulus at surgery. The aim of this study was to inv estigate the contribution of intrauterine intussusception and volvulus to the de velopment of JIA. Methods In 48 newborns (24 boys and 24 girls) treated for JIA at our hospital between 1978 and 2004, the operative and pathologic findings wer e reviewed. Results Intussusceptionwas responsible for gap and cord type atresia in 12 cases (25%). The cord showed an atrophic intestinal lumen in 2 cases. Vo lvulus was observed in 13 cases. Volvulus and intussusception were simultaneousl y noted in 1 case. This suggested that intussusception was the cause of the atre sia, whereas volvulus was a secondary event. Neither intussusception nor volvulu s was observed in high jejunal, apple peel, or multiple atresia. Conclusions Int rauterine volvulus and intussusception were commonly observed in single mid-and low JIA. Thus, intrauterine intussusception may be a common cause of gap and co rd type JIA. Volvulus may not only cause JIA but also result from anatomic chang es after the development of JIA in some cases.
