title: A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum.
abstract: OBJECTIVE
  The authors experienced 3 cases of persistent pulmonary hypertension of the neonate (PPHN) associated with duodenal atresia with paradoxical 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 charts 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 intestine was identified as the distal blind end of the duodenum during duodeno- duodenostomy. PPHN developed after surgery, and the patient died despite treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Lung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment of 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 death. Autopsy results showed ACD with misalignment of pulmonary vessels and duodenal 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 with dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully under combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrasonographic findings showed enlarged echogenic fetal lungs as in the first 2 cases.
  
  
  CONCLUSIONS
  The characteristics of this novel association are enlarged echogenic fetal lungs, duodenal atresia associated with paradoxical dilatation of the distal blind end, and refractory PPHN resistant to iNO.
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Japan.
authors.forename: Noriaki
authors.initials: N
authors.lastname: Usui
authors.forename: Masafumi
authors.initials: M
authors.lastname: Kamiyama
authors.forename: Shinkichi
authors.initials: S
authors.lastname: Kamata
authors.forename: Akihiro
authors.initials: A
authors.lastname: Yoneda
authors.forename: Yuko
authors.initials: Y
authors.lastname: Tazuke
authors.forename: Masahiro
authors.initials: M
authors.lastname: Fukuzawa
mesh_terms: D002196:Capillaries / Q000002:abnormalities; D004108:Dilatation, Pathologic; D004380:Duodenal Obstruction / Q000150:complications*; D004386:Duodenum / Q000473:pathology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007409:Intestinal Atresia / Q000150:complications*; D008297:Male; D011650:Pulmonary Alveoli / Q000098:blood supply*; D012189:Retrospective Studies