Overview of congenital anomalies of the kidney and urinary tract (CAKUT)
- Norman D Rosenblum, MD, FRCP
Norman D Rosenblum, MD, FRCP
- Professor of Pediatrics
- University of Toronto
- Section Editors
- Tej K Mattoo, MD, DCH, FRCP
Tej K Mattoo, MD, DCH, FRCP
- Section Editor — Pediatric Nephrology
- Professor of Pediatrics
- Wayne State University School of Medicine
- Laurence S Baskin, MD, FAAP
Laurence S Baskin, MD, FAAP
- Section Editor — Pediatric Urology
- Frank Hinman, Jr., MD, Distinguished Professorship in Pediatric Urology
- Chief Pediatric Urology
- Professor of Urology and Pediatrics
- UCSF Benioff Children’s Hospital
Congenital anomalies of the kidney and urinary tract (CAKUT) constitute approximately 20 to 30 percent of all anomalies identified in the prenatal period . Defects can be bilateral or unilateral, and different defects often coexist in an individual child.
An overview of the congenital anomalies of the kidney and urinary tract (CAKUT) is presented here. The antenatal screening and postnatal evaluation of infants with CAKUT are discussed in greater detail separately. (See "Evaluation of congenital anomalies of the kidney and urinary tract (CAKUT)" and "Congenital ureteropelvic junction obstruction" and "Primary megaureter in infants and children" and "Ectopic ureter" and "Renal ectopic and fusion anomalies" and "Autosomal recessive polycystic kidney disease in children".)
ASSOCIATION WITH END-STAGE RENAL DISEASE (ESRD)
Because CAKUT play a causative role in 30 to 50 percent of cases of end-stage renal disease (ESRD) in children , it is important to diagnose these anomalies and initiate therapy to minimize renal damage, prevent or delay the onset of ESRD, and provide supportive care to avoid complications of ESRD. Patients with malformations with a reduction in kidney numbers or size are most likely to have a poor renal prognosis . (See 'Renal development and CAKUT' below and "Overview of the management of chronic kidney disease in children".)
Normal embryology — Normal embryologic development of the kidney occurs in three stages (figure 1):
●Pronephros – Transient rudimentary and nonfunctioning system that begins in the fourth week of gestation (ie, day 22) and disappears by end of the fourth week (ie, day 28). Degeneration of the pronephros is required for normal kidney development.
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- ASSOCIATION WITH END-STAGE RENAL DISEASE (ESRD)
- Normal embryology
- - Metanephros
- Renal development and CAKUT
- RENAL PARENCHYMAL MALFORMATIONS
- Renal hypoplasia
- Renal dysplasia
- - Presentation and clinical findings
- - Evaluation
- - Outcome and follow-up care
- Multicystic dysplasia
- Renal agenesis
- - Unilateral agenesis
- - History and physical examination
- - Tests
- Chronic kidney disease
- Follow-up care
- - Sports participation
- Renal tubular dysgenesis
- Genetic cystic diseases
- ANOMALIES OF RENAL EMBRYONIC MIGRATION
- ANOMALIES OF THE COLLECTING SYSTEM