The Rarest of the Rare: Crossed Fused Renal Ectopia of the Superior Ectopia Type.

Leyla Akdogan,Tarkan Ergun,Ali Kemal Oguz,Ihsan Ergun

doi:10.1155/2015/742419

Leyla Akdogan, Tarkan Ergun + Show 2 more

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https://doi.org/10.1155/2015/742419

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Journal: Case reports in nephrology	Publication Date: Jan 1, 2015
Citations: 30	License type: CC BY 3.0

Affiliation: Ufuk University, Başkent University

Abstract

Crossed fused renal ectopia is a rare congenital anomaly of the urinary system where one kidney crosses over to opposite side and the parenchyma of the two kidneys fuse. Herein, we present an atypical CFRE case whose renal anatomy does not exactly match any of the already defined CFRE types. Both of the kidneys are ectopic with the crossed ectopic right kidney lying superiorly and fused to the upper pole of the left kidney. Renal arteries were originating from the common iliac arteries. A focal 90% stenosis was observed on the right main renal artery. The patient is borderline hypertensive.

Highlights

Crossed fused renal ectopia (CFRE) is a markedly rare congenital malformation of the urinary system where one of the kidneys crosses the midline to become located on the opposite side of its ureter entrance to the bladder and the parenchymas of the two kidneys fuse
Crossed renal ectopia is classified into 4 main categories: crossed renal ectopia with or without fusion, unilateral crossed renal ectopia, and bilateral crossed renal ectopia [3]
In the inferior CFRE type, the upper pole of the inferiorly situated crossed ectopic kidney is fused to the lower pole of the superiorly, normally positioned kidney

Summary

Introduction

Crossed fused renal ectopia (CFRE) is a markedly rare congenital malformation of the urinary system where one of the kidneys crosses the midline to become located on the opposite side of its ureter entrance to the bladder and the parenchymas of the two kidneys fuse. CFRE has a reported autopsy incidence of around 1 : 2000 and is the second most frequently observed fusion anomaly of the kidneys following the horseshoe kidney. Resulting from aberrant migration and crossing of the midline of the metanephric blastema and the ureteral bud, CFRE is thought to develop during the fourth to eighth weeks of gestation. Remaining asymptomatic and detected as an incidental finding during imaging studies, six well-defined anatomical variations of CFRE have been reported [1, 2]. We present an atypical CFRE case whose renal anatomy does not exactly match any of the already defined CFRE types

Case Presentation

Findings

Discussion