Congenital bilateral agenesis of diaphragm: Report of a case

Shih-Min Wang, Chyi-Her Lin, Yuh-Jyh Lin, H. B. Yang, M. H. Wu

研究成果: Article

6 引文 (Scopus)

摘要

Bilateral agenesis of the diaphragm is a rare, life-threatening malformation. Infants with this defect rarely survive to have surgical intervention. We report a 32-week premature female infant who was born to a 36-year-old mother via vaginal delivery. The pregnancy course was complicated by hypertension and polyhydramnios. Cytogenetic study showed a normal 46 XX female karyotype. She had cyanosis, respiratory distress and scaphoid abdomen at birth A roentgenograph confirmed the diagnosis of diaphragmatic hernia. Surgery was performed at 21 h of age. Bilateral agenesis of diaphragm, herniation of abdominal organs and oesophagus and pulmonary hypoplasia were noted. Furthermore, stomach and spleen were adherent to the mediastinum and vertebrae. The patient developed hypotension and persistent hypoxaemia and expired at age of 26 h. Autopsy revealed bilateral agenesis of diaphragm, hypoplasia of lungs, and pancreas fibrosis with mild hypoplasia of islets of Langerhans. Conclusion: Bilateral agenesis of diaphragm associated with pancreas fibrosis is a rare entity, and its clinical significance needs further investigation.

原文English
頁(從 - 到)572-574
頁數3
期刊European Journal of Pediatrics
156
發行號7
DOIs
出版狀態Published - 1997 七月 23

指紋

Diaphragm
Pancreas
Fibrosis
Polyhydramnios
Lung
Cyanosis
Diaphragmatic Hernia
Mediastinum
Karyotype
Islets of Langerhans
Premature Infants
Cytogenetics
Abdomen
Hypotension
Esophagus
Autopsy
Stomach
Spine
Spleen
Mothers

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health

引用此文

Wang, Shih-Min ; Lin, Chyi-Her ; Lin, Yuh-Jyh ; Yang, H. B. ; Wu, M. H. / Congenital bilateral agenesis of diaphragm : Report of a case. 於: European Journal of Pediatrics. 1997 ; 卷 156, 編號 7. 頁 572-574.
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Congenital bilateral agenesis of diaphragm : Report of a case. / Wang, Shih-Min; Lin, Chyi-Her; Lin, Yuh-Jyh; Yang, H. B.; Wu, M. H.

於: European Journal of Pediatrics, 卷 156, 編號 7, 23.07.1997, p. 572-574.

研究成果: Article

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