Abstract

Congenital anomalies of the kidney and urinary tract (CAKUT) occur in 1 in 500 live births and account for 31% of children with end-stage renal disease (ESRD) in the United States. In this chapter are described congenital renal anomalies in number [unilateral renal agenesis, bilateral renal agenesis/hypoplasia/dysplasia (BRAHD), supernumerary kidney], of rotation (malrotated kidney) and position (simple renal ectopia, crossed fused renal ectopia and horseshoe kidney). A detailed knowledge of these conditions is required due to the high incidence of associated renal and extra renal anomalies requiring appropriate investigations and, in selected cases, prompt treatment; congenital renal anomalies can occasional be part of multi organ syndrome. Understanding the vascular anatomy of anomalies of position of the kidney is of paramount importance when planning surgical intervention as the arterial and venous supply is often not consistent and variable. Finally, some of these conditions such as unilateral renal agenesis require life-long follow-up with annual measurement of blood pressure and urinary protein.

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