Single unilateral ectopic bifid ureter with contralateral orthotopic quadrufid ureter--a rare combination.

AD Bhandarkar, AM Raju, MS Rao 
 Department of Urology, Kasturba Medical College Hospital, Manipal (D.K.), India., India

Correspondence Address:
A D Bhandarkar
Department of Urology, Kasturba Medical College Hospital, Manipal (D.K.), India.
India

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::   Introduction

Wide range of abnormalities may be associated with single unilateral ureteral ectopia. We report a rare combination of a bifid ureter opening into the urethra with contralateral quadrufid ureter.

::   Case report

A 3-year-old girl with urinary incontinence since birth, was referred from Pediatrics ward for urological evaluation. No obvious congenital anomalies were spotted at birth. There was no history of uirnary infections. On physical examination urine was noted to dribble intermittently from urethral orifice. Urianalysis, haemogram, blood urea, serum creatinine were all within normal limits. Plain X-ray of the lumbar spine revealed L3 hemivertebra. Intravenous urogram revealed normally functioning kidneys and presence of apparently duplicated ureters bilaterally in their upper thirds. At cystoscopy, a normal, orthotopic single right ureteric orifice and absence of the left hemitrigone were noted. A wide, patulous, left ureteric orifice was spotted just distal to bladder neck in the urethra. Right retrograde ureterogram showed a quadrufid proximal half of the ureter [Figure:1]. Left retrograde ureterogram showed a bifid upper third of ureter [Figure:2]. Voiding cystourethrogram failed to show vesicoureteric reflux on either side. Subsequently, exploration of the left ureter, excision of the ectopic ureteral stump and complete closure of its hiatus was done by a simple technique described by Gotoh et al[1] A left ureteric Politano-Ledbetter reimplantation was done. Urinary incontinence totally ceased postoperatively.

::   Discussion

Reports from Japan reveal 70% of ectopic ureters being associated with single collecting systems. This is in contrast to the observations in the western literature of 70 to 80% association with duplex systems[1]. Several authors have commented on the association of ectopic ureters with other anomalies[2],[3],[4]. Presence of L3 hemivertebrae in our case, may be considered as a clinically inconsequential variety of spinal anomaly and variant of VATER syndrome. The most frequently encountered congenital anomalies in this syndrome are anal atresia (87%), vertebral defects (83%), radial dysplasia (50%), congenital heart disease (45%) and lower limb dysplasia, including hypoplasia or absence of either or both tibia and fibula, and absence of metatarsals (25%)[5],[6]. The case reported by Kaufman et al[5] describes the presence of hemivertebrae T12 with other spinal and rib deformities, imperforate anus, perianal fistula with the intravenous urogram showing a non-visualised left kidney. Further investigations to diagnose ectopic ureter were not done.

Renal dysplasia, ectopia, hydronephrosis, and hypoplasia has been described as associated renal anomalies with single ureteral ectopia[3]. Ureteral quadruplication is a rare condition. Only one case of bilateral quadruplication has been reported in the English literature[7], to our knowledge. The embryologic genesis of multiple ureters is best explained by more than one separate ureteral buds arising from the wolffian duct or by fission of a single ureteral bud. The latter explanation seems to be applicable in our case. Smith[8] described 4 types of triplicate ureters and he used term trifid ureter where all 3 ureters joined to drain through a single orifice. The right ureter in the case reported by Soderdahl DW et al[7] was called quadrufid ureter.

Our case showing ipsilateral incomplete duplication and contralateral quadrufid ureter in itself is one of the rare renal/ureteric anomalies associated with ureteral ectopia, which total combination, to the best of our knowledge, has not been previously reported in the English literature. Gotoh et al[1] had seen only 2 cases of ipsilateral incomplete ureteral duplication in their series of single ectopic ureters.

The presence of associated congenital anomalies with single ureteral ectopia suggests simultaneous mesenchymal and epithelial defect which would explain a coexistent defective ureteral but and metanephric development. A single factor, not yet identified and depending upon its severity, may be responsible for the variety of such associated anomalies during early intrauterine development.

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