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

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

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.