Congenital Anomalies of the Kidney and Urinary Tract: a Clinical Review

  • Emily Stonebrook
  • Monica Hoff
  • John David SpencerEmail author
Pediatric Nephrology (B Dixon and E Nehus, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Pediatric Nephrology


Purpose of review

This review highlights the most common congenital anomalies of the kidney and urinary tract (CAKUT) that are encountered in pediatric practices. CAKUT are the most common cause of prenatally diagnosed developmental malformations and encompass a spectrum of disorders impacting lower urinary tract development as well as kidney development and function. In pediatric and adolescent populations, developmental abnormalities are the leading cause of end-stage kidney disease. The goal of this review is to provide pediatric providers a framework for appropriate clinical management as well as highlight when referral to subspecialty care is needed.

Recent findings

While the exact etiologies of CAKUT are not completely defined, new evidence demonstrates that genetic and molecular changes impact embryonic kidney and urinary tract development. As a result, phenotypes and clinical outcomes may be affected.


Because pediatric providers provide frontline care to children and adolescents with developmental kidney and urinary tract anomalies, updated knowledge of CAKUT pathogenesis, embryology, clinical management, and patient outcomes is needed. This manuscript reviews CAKUT etiologies and essential diagnostic, prognostic, and management strategies.


Nephrology Urology Congenital kidney anomalies Pediatrics Genetics Embryology 



We acknowledge Ms. Lisa Feurer for assistance for the artwork shown in Fig. 1.

Compliance with Ethical Standards

Conflict of Interest

Emily Stonebrook declares that she has no conflict of interest. Monica Hoff declares that she has no conflict of interest. John David Spencer declares that he has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Emily Stonebrook
    • 1
    • 2
  • Monica Hoff
    • 3
  • John David Spencer
    • 2
    Email author
  1. 1.Pediatric Nephrology Fellowship Program, Division of Pediatric NephrologyNationwide Children’s HospitalColumbusUSA
  2. 2.Division of Pediatric NephrologyNationwide Children’s HospitalColumbusUSA
  3. 3.Pediatrics Residency Program, Department of PediatricsNationwide Children’s HospitalColumbusUSA

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