A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum

Usui, Noriaki; Kamiyama, Masafumi; Kamata, Shinkichi; Yoneda, Akihiro; Tazuke, Yuko; Fukuzawa, Masahiro

doi:10.1016/j.jpedsurg.2004.08.026

« Previous Next »Journal of Pediatric Surgery
Volume 39, Issue 12 , Pages 1808-1811, December 2004

A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum

Presented at the 37th Annual Meeting of the Pacific Association of Pediatric Surgeons, Seoul, Korea, May 16–20, 2004.

Noriaki Usui
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
- Address reprint requests to Noriaki Usui, MD, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan
Masafumi Kamiyama
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
Shinkichi Kamata
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
Akihiro Yoneda
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
Yuko Tazuke
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
Masahiro Fukuzawa
- Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan

Abstract

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.

Keywords: Persistent pulmonary hypertension of the neonate, duodenal atresia, alveolar capillary dysplasia, prenatal diagnosis, prostacyclin