A 3- year- old boy associated with Opitz syndrome including hypospadia and anorectal anomaly was transferred to Osaka University Hospital with orotracheal intubation because of respiratory distress after respiratory i...A 3- year- old boy associated with Opitz syndrome including hypospadia and anorectal anomaly was transferred to Osaka University Hospital with orotracheal intubation because of respiratory distress after respiratory infection. He had l aryngeal cleft diagnosed in the neonatal period because of symptoms including st ridor and aspiration. After 2 attempts at extubation, tracheostomy was performed . Fiberscopic examination demonstrated obstruction of the airway from the vocal cord to subglottic space by inspired esophageal redundant mucosa. There was no i mprovement observed for 3 months. He underwent anterior repair of cleft, anterio r laryngotracheoplasty with costal cartilage graft, closure of tracheostomy, and endoscopic Nissen fundoplication. The postoperative course during the subsequen t year was uneventful without feeding or speaking disorders. Subglottic airway o bstruction caused by inspired redundant mucosa is a rare complication in larynge al cleft. Anterior laryngotracheoplasty and Nissen fundoplication should be cons idered for this complication, in addition to the usual procedure for cleft repai r.展开更多
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
The use of memory metallic stents for the urinary tract in pediatric patients has not been reported. The authors report on 2 patients with urinary tract diso rders who were successfully treated with a memory metallic ...The use of memory metallic stents for the urinary tract in pediatric patients has not been reported. The authors report on 2 patients with urinary tract diso rders who were successfully treated with a memory metallic stent. A thermoexpand able, nickel- titanium alloy stent was placed at the urethroureteral junction o f a 4- year- old boy with ureteral stenosis associated with cloacal exstrophy for 18 months and at the urethra of a 2- year- old girl with ischuria after a repair of cloacal anomaly for 6 months. Temporary insertion of a memory metallic stent is a safe and effective alternative for organic stricture or functional o bstruction of the urinary tract in pediatric patients.展开更多
文摘A 3- year- old boy associated with Opitz syndrome including hypospadia and anorectal anomaly was transferred to Osaka University Hospital with orotracheal intubation because of respiratory distress after respiratory infection. He had l aryngeal cleft diagnosed in the neonatal period because of symptoms including st ridor and aspiration. After 2 attempts at extubation, tracheostomy was performed . Fiberscopic examination demonstrated obstruction of the airway from the vocal cord to subglottic space by inspired esophageal redundant mucosa. There was no i mprovement observed for 3 months. He underwent anterior repair of cleft, anterio r laryngotracheoplasty with costal cartilage graft, closure of tracheostomy, and endoscopic Nissen fundoplication. The postoperative course during the subsequen t year was uneventful without feeding or speaking disorders. Subglottic airway o bstruction caused by inspired redundant mucosa is a rare complication in larynge al cleft. Anterior laryngotracheoplasty and Nissen fundoplication should be cons idered for this complication, in addition to the usual procedure for cleft repai r.
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The use of memory metallic stents for the urinary tract in pediatric patients has not been reported. The authors report on 2 patients with urinary tract diso rders who were successfully treated with a memory metallic stent. A thermoexpand able, nickel- titanium alloy stent was placed at the urethroureteral junction o f a 4- year- old boy with ureteral stenosis associated with cloacal exstrophy for 18 months and at the urethra of a 2- year- old girl with ischuria after a repair of cloacal anomaly for 6 months. Temporary insertion of a memory metallic stent is a safe and effective alternative for organic stricture or functional o bstruction of the urinary tract in pediatric patients.
