BJU International 2002 90 (4cr), 472

Identical twins with a left dysplastic kidney and ectopic ureter entering the female genital tract

J.S. Kalsi, P. Menezes, M. Arya, M. Dillon* and P.M. Cuckow

The Institute of Urology and Nephrology, and * Great Ormond Street Hospital for Children, London, UK

Case report

Comment  References  Authors 

Identical twin girls (5 years old, monochorionic pregnancy confirmed on late first trimester ultrasonography, US) presented with recurrent UTIs and incontinence. Both twins had no other physical problems or learning difficulties. In both cases US (Fig. 1a,b) and DMSA scintigraphy showed dysplastic left kidneys with left renal function of < 10%. The US appearances were also suggestive of left ureterocele in both twins. At cystoscopy in the first twin, no left ureteric orifice was apparent. Laparoscopy confirmed a dysplastic left kidney and vaginoscopy revealed a ureteric orifice opening into the cervix. A laparoscopic left nephroureterectomy was undertaken. In the second twin, cystoscopy again revealed no left ureteric orifice, and laparoscopy again confirmed a small dysplastic left kidney with an ectopic ureter. On this occasion the ureter terminated in the vagina directly (confirmed on vaginoscopy). A laparoscopic left nephroureterectomy was undertaken. Both girls made an uneventful recovery and are now completely continent.


Case report  References  Authors 

This is the first report of ectopic ureters occurring in twins. Ectopic ureters terminating in the female genital tract are caused by an abnormally lateral origin of the ureteric bud from the mesonephric duct in the 3- to 4-week embryo. The ureter remains intimately involved with mesonephric duct remnants, which acquire a communication with Müllerian structures [1]. The more remote the ectopic orifice from its normal position, the more severe is the associated renal lesion. Dysplasia is inevitably present when the ectopic ureter opens outside the urinary tract (e.g. vagina, vestibule, Gartner's duct, seminal tract) [2]. Additionally, there is a higher incidence of reflux and renal agenesis in single-system ectopic ureters than in ectopic ureters of a duplex system [3]. Although ectopic ureters opening into the vagina have been described, reports of them opening into the cervix are rare [1,4]. Urinary incontinence and UTI are the most frequent presentations of an ectopic ureter in children [5]. Careful endoscopic examination of the urethra and (in girls) of the vagina will identify the ectopic ureteric orifice in most cases [6]. An ectopic ureter can be confused with an ectopic ureterocele on US. The correct diagnosis, as in the present case, is vital to ensure appropriate treatment [6]. Laparoscopic nephroureterectomy is the recommended treatment for ectopic ureter and dysplastic kidney [7,8]. Renal dysplasia can be inherited in an autosomal dominant manner with incomplete penetrance [9]. In the present case the parents had no renal abnormalities on US and there was no family history of renal dysplasia or any other urogenital abnormality. These rare findings in monozygotic twins opens the question of whether there is a common environmental or as yet unknown genetic cause.


Case report  Comment  Authors 


Case report  Comment 

J.S. Kalsi, MBBS(Hons), BSc(Hons), MRCSEd, MRCS(Eng), Research Registrar.

P. Menezes, MBBS, MS, FRCS, Specialist Registrar.

M. Arya, MBChB, FRCS, Research Registrar.

M. Dillon, MBBS, MD, MRCP, Professor of Nephrology.

P.M. Cuckow, MBBS, FRCS(Paeds), Consultant Paediatric Urologist.

Correspondence: J.S. Kalsi, The Institute of Urology, 48 Riding Street, London W1P 7 PN, UK.


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To cite this article
Kalsi, J.S., Menezes, P., Arya, M., Dillon, M. & Cuckow, P.M. Identical twins with a left dysplastic kidney and ectopic ureter entering the female genital tract.  BJU International 2002 90 (4cr), 472
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Kalsi, J
Menezes, P
Arya, M
Dillon, M
Cuckow, P