Journal of the Korean Pediatric Society 1989;32(10):1448-1454.
Published online October 31, 1989.
Caudal Regression Syndrome Associated with Potter Syndrome.
Hyun Joo Jeong1, Sung Sook Bang1, Hae Ran Lee1, Choon Myung Ro1, Yeon Lim Suh2
1Department of Pediatrics, Hallym University, College of Medicine, Seoul, Korea
2Department of Pathology, Hallym University, College of Medicine, Seoul, Korea
Potter 증후군을 동반한 Caudal Regression Syndrome 부검 1례
정현주1, 방성숙1, 이혜란1, 노준명1, 서연림2
1한림대학 한강성심병원 소아과학교실
2한림대학 한강성심병원 병리학교실
Received: 8 March 1989   • Accepted: 27 June 1989
Abstract
Caudal egression syndrome is a term applied to a syndrome comprising complete or partial agenesis of sacrum and coccyx, often associated with malformation of the lower extremities, congenital heart disease, urologic abnormalities and malformation of the upper extremities. The primary defect may occur in the mid-posterior axis mesoderm of the embryo prior to the 4th week of development, resulting in other defects of the structure derived from caudal axis. Potter syndrome may be one feature of a more extensive caudal axis anomaly. There was no mention, however, regarding the co-existence of caudal regression syndrome and Potter syndrome. Recently we had a chance to examine a case of caudal regression syndrome associated with Potter syndrome in a 2 hour old male baby. This baby was bom at fullterm by cesarean section with the birth weight of 2.0 kg. He had no self respiration at birth and died immediately. This pregnancy was uncomplicated, but the mother was noticed to be diabetic during the previous pregnancy. Postmortem examination revealed that the baby had caudal regression syndrome with Potter syndrome. And we reported the case with the review of associated literatures.
Key Words: Caudal regression syndrome, Potter syndrome


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