Association of Congenital Anomalies of the Kidney and Urinary Tract with Those of Other Organ Systems

Abstract

About one-third to half of patients with congenital anomalies of the kidney and urinary tract (CAKUT) have one or more associated anomalies of other organ systems including cardiovascular, gastrointestinal, central nervous, skeletal, and genito-reproductive systems as well as abnormalities of the lung and face, chromosomal aberrations and multiple congenital anomalies syndromes. Awareness of this association is essential in the management of affected patients, especially when the anomaly is not evident on routine physical examination and when early intervention can contribute to prevention of chronic kidney disease (CKD). CAKUT should be suspected in patients with multiple congenital anomalies particularly in those with chromosomal aberrations, congenital anomalies of other organ systems, and in newborns and children with single umbilical artery, febrile urinary tract infection, significant abnormalities of the ear lobes, and supernumerary nipples. Chromosomal studies should be considered in any fetus with ultrasonographic evidence of CAKUT associated with other congenital anomalies.