Purpose of our study was to aggregate and analyse rare cases of Ectopic Ureter (EU), their association with other anomalies, clinical features, diagnosis and management. A total of nine patients with rare presentation of EU were evaluated. Combination of endoscopic and imaging modalities was used as required to define the anatomy and devise the best surgical approach in these cases. Among six females and three males with EU, four cases had bilateral EU, four unilateral EU and one case had EU of a solitary kidney. Urinary incontinence was encountered in five cases including one male patient whilst other cases presented with varied clinical features and associated anomalies. Two patients had anorectal malformations, and two had uterine anomalies in the form of bicornuate uterus. Other patients had multiple rare associations such as triplication of ureter, bilateral absence of seminal vesicles with infertility, multicystic dysplastic kidney, ureter draining in uterus, renal failure, absence of bladder trigone, and hypospadias etc. Ureteric reimplantation was performed in four cases, two required ureteroureterostomy, another two had undergone upper pole nephrectomy and in one case renal transplant had been carried out owing to chronic renal failure. EU is among group of those congenital entities which remain shrouded until adulthood, when symptoms become distressing. Fortunately, prognosis is favourable after surgical correction, in spite of its rarity. Complexity arises when other associated anomalies are identified.
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