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Congenital anomalies of the kidneys

Last updated: March 26, 2020

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Congenital anomalies of the kidneys and urinary tract (CAKUT) are one of the most common malformations diagnosed in newborns. The pathogenesis of CAKUT is multifactorial; both specific genes and environmental factors (e.g., in utero exposure to ACE inhibitors) have been implicated in the development of CAKUT. Even though most cases are initially asymptomatic, up to 50% of children with end-stage renal disease have an underlying CAKUT. For this reason, early identification of these malformations is essential in order to prevent renal damage.

Anomalies of the collecting system

Malformation of the renal parenchyma

Renal dysgenesis

Congenital solitary kidney

Anomalies of kidney migration




Floating kidney


The differential diagnoses listed here are not exhaustive.

  1. Rosenblum ND, Mattoo TK, Baskin LS, Kim MS. Overview of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/overview-of-congenital-anomalies-of-the-kidney-and-urinary-tract-cakut.Last updated: August 29, 2016. Accessed: September 18, 2017.
  2. Shaikh N, Hoberman A. Urinary Tract Infections in Infants Older than One Month and Young Children: Acute Management, Imaging, and Prognosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/urinary-tract-infections-in-infants-older-than-one-month-and-young-children-acute-management-imaging-and-prognosis.Last updated: March 27, 2018. Accessed: April 4, 2018.
  3. Deem SG, Schwartz BF. Nephroptosis. Nephroptosis. New York, NY: WebMD. http://emedicine.medscape.com/article/1458935-overview. Updated: April 8, 2015. Accessed: June 28, 2017.