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. 2019;5(3):223-235.
doi: 10.1007/s40746-019-00166-3. Epub 2019 Jun 11.

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

Emily Stonebrook  1   2 , Monica Hoff  3 , John David Spencer  2
Affiliations

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

Emily Stonebrook et al. Curr Treat Options Pediatr. 2019.

Abstract

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.

Summary: Because pediatric providers provide front-line 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.

Keywords: Congenital Kidney Anomalies; Embryology; Genetics; Nephrology; Pediatrics; Urology.

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Conflict of interest statement

Conflict of Interest Emily Stonebrook, Monica Hoff, and John David Spencer declare no conflict of interest.

Figures

Figure 1.
Figure 1.. Embryonic kidney and urinary tract development.
(A) Renal embryogenesis showing development and degeneration of the pronephros (left) and mesonephros (center), with induction of the ureteric bud and metanephric mesenchyme (right). (B) The initiating step in kidney development is the outpouching of the ureteric bud from the Wolffian duct. The stalk of the ureteric bud becomes the ureter (top). The ureteric bud undergoes repetitive branching to form the collecting tubules, major calices, and minor calices (middle). The end of each arched collecting tubule induces clusters of mesenchymal cells in the metanephrogenic blastema to undergo a mesenchymal-to-epithelial transformation that eventually develops into the mature nephron (bottom).
Figure 2.
Figure 2.. Ultrasonography of developmental kidney anomalies
(A) A large left dilated renal pelvis (stars) and thin renal cortical tissue consistent with uretopelvic junction obstruction. (B) A multicystic dysplastic left kidney with multiple parenchymal non-communicating kidney cysts of varying size (*) and hyperechoic, dysplastic kidney tissue. (C) Autosomal dominant polycystic kidney disease with an enlarged kidney showing multiple large cortical cysts (*). (A-C) The white dashed line outlines the kidney parenchyma.
See this image and copyright information in PMC

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