Document Detail

A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum.
MedLine Citation:
PMID:  15616937     Owner:  NLM     Status:  MEDLINE    
BACKGROUND/PURPOSE: 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.
Noriaki Usui; Masafumi Kamiyama; Shinkichi Kamata; Akihiro Yoneda; Yuko Tazuke; Masahiro Fukuzawa
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  39     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2004 Dec 
Date Detail:
Created Date:  2004-12-23     Completed Date:  2005-11-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1808-11     Citation Subset:  IM    
Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Japan.
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MeSH Terms
Capillaries / abnormalities
Dilatation, Pathologic
Duodenal Obstruction / complications*
Duodenum / pathology*
Infant, Newborn
Intestinal Atresia / complications*
Pulmonary Alveoli / blood supply*
Retrospective Studies

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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