Crossed fused renal ectopia in children: a review of clinical profile, surgical challenges, and outcome.

Abstract

INTRODUCTION\nCrossed fused renal ectopia is a rare congenital malformation, wherein both kidneys are present unilaterally, with the ureter of the crossed kidney opening into the bladder on the contralateral side. It has varied presentation from incidental detection to renal impairment. In this largest series of crossed fused renal ectopia, the authors assessed the clinical profile of these children and also attempted to shed light on the challenges in the surgical management.\n\n\nMETHODS\nIt is a retrospective study conducted from January 2009 to July 2018, among patients evaluated for crossed fused ectopia, in the Department of Pediatric Surgery, Christian Medical College, Vellore. Electronic medical records of 36 children were reviewed. Imaging modalities and operative interventions were recorded. Results were analyzed.\n\n\nRESULTS\nA total of 36 children were found to have crossed fused ectopia, with male preponderance. Most children presented within one year of age with urinary tract infection being the commonest cause. The most common associated anomaly was anorectal malformation. Ultrasound abdomen and pelvis, micturating cystourethrogram, and radionucleotide scans were the imaging modalities preferred for diagnosis and follow-up. Left-to-right ectopia was more common with inferior ectopic variant being the most common. The predominant urological problems include pelviureteric junction obstruction, vesicoureteric junction obstruction, and vescioureteric reflux. Ureteric re-implant was the most common surgery performed followed by pyeloplasty. Pelvi-pelvostomy, uretero-ureterostomy, bladder augment with Mitrofanoff, and other procedures were performed for select cases.\n\n\nCONCLUSION\nCrossed fused renal ectopia is a challenging entity which requires individualized management plans based on the predominant urological anomaly and the functional status. Surgical options are diverse and are guided toward the symptomatic urological problem with focus on preserving the renal function. The long-term prognosis is good in these children.