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The Korean Journal of Pathology 1984;18(3): 304-308.
Imperforate cloacal membrane 을 동반한 이중유출로 우심실 -1예 보고-
A Case of Double Outlet Right Ventricle and Imperforate Cloacal Membrane
Double outlet right ventricle This diverse group of cardiac malformations are characterized by the relationship of the great arteries to the ventricular septal deect and the presence of absence of valve or subvalvular pulmonic stenosis. The terminal protion of the hindgut is called the cloaca. The cloaca is devided by a coronal sheet or wedge of mesenchyme, the urorectal septum, which develops in the angle between the allantois and the hindgut at 4 weeks. By the end of the sixth week, the urorectal se[ptum has fused with the cloacal membrane deviding the cloaca into urogenital sinus and the rectum. If the urorectal septum does not develop and the cloacal membrane persist, and anomaly that the urogenital system and the digestive system are connected with each other is developed. We report an autopsy case of double outlet right ventricle and imperforated cloacal membrane with cystic dilatation. This case was accompanied by atrial septal defect, patent ductus arteriosus, agenesis of the right ear, left lower set ear with hypogenesis, lordosis, focal defect of the anterior abdominal wall defect just below umbilicus. The aorta was dextroposed and it arouse from the right ventricle, right to the pulmonary artery. The pulmonary artery was in normal position and the pulmonary stenosis was not found. The atrial septal defect and patent ductus arteriosus were also found. The baby had rudimentary phallus without opening. The Abdomen was markedly dilated due to a cystic mass in the abdominal cavity. Both ureters and the large intestine drained into the cystic structure and the distal part of the left ureter was dilated. There were neither urethral opening nor anal opening connected to the outside.
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