All issues > Volume 32(10); 1989

Original Article

J Korean Pediatr Soc. 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

Received: March 8, 1989;  Accepted: June 27, 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.

Keywords :Caudal regression syndrome, Potter syndrome