Triplication of Ureter: A Rare Case.

Anatomical fusion abnormalities of the urinary system and ureters are not uncommon. However, triplication of the ureter is very rare. A boy with this abnormality is being presented in view of its rarity. The importance of a thorough preoperative evaluation is being projected that may help us to choose the best management avenue. Copyright:

INTRODUCTION

A rare anomaly first described by Wrany in 1870, there have been only sporadic case reports regarding ureteric triplication in children. Smith, in 1946, classified ureteric triplication into four types, based on eleven cases.[1] We hereby present a case of antenatally diagnosed left hydroureteronephrosis, which was eventually diagnosed as ureteric triplication during work-up, and highlight the importance of thorough preoperative evaluation in the management of these atypical and rare cases.

CASE REPORT

A 2-year-old male child was antenatally diagnosed with left hydroureteronephrosis, but parents did not seek medical care until the child developed pyuria at the age of 6 months. There was no history of fever, pain abdomen, or crying during micturition. The urinary stream was normal with no history of dribbling. Physical examination revealed no positive findings. Urine analysis revealed pus cells, and there was a culture-proven urinary tract infection (UTI) that was adequately treated with oral antibiotics.

The child was further investigated for the cause of hydroureteronephrosis and UTI. Ultrasonography by an experienced sonologist suggested a possibility of a left-sided duplex system with hydronephrotic moieties. Two ureters were seen fusing to form a dilated ureter close to pelvicalyceal systems, insertion of which was likely ectopic into the bladder neck. Micturating cystourethrogram was normal. A magnetic resonance urogram (MRU) was done, findings of which are depicted in Figure 1. Renal dynamic scan using LLEC revealed a left duplex system with hydronephrotic, functioning moieties; no obstruction was reported.

Figure 1

Magnetic resonance urogram: Reformatted projection image showing triplication of the left ureter with their respective moieties uniting to form a single dilated ureter, that tapers to enter into the bladder neck region. The right column of images shows the axial sections at various levels

With the above findings and discrepancy, it was planned to explore and perform ureteric reimplantation of the left-sided ectopic ureter. Upon exploration, the intraoperative findings corroborated with the MRU. Intraoperatively disproportionately dilated terminal left ureter with a shouldering at junction of lower 1/3rd and upper 2/3rd of the ureter with an ectopic insertion into the bladder neck distally and medially to the orthotopic location [Figure 2a]. The upper and middle moiety's ureter was fused early and ran in a common sheath but remained separate in the common sheath joining with the third further down with three openings visible at the level of confluence of these ureters with the common channel that was disproportionately dilated as shown in figure [Figure 2a]. A plan was thus formulated to perform an excisional tapering ureteroplasty after ligating the ureter just proximal to its ectopic insertion [Figure 2b]. After opening the dilated terminal bulbous ureter in preparation for excisional tapering, three lumens were found proximally, as described before. After doing an excisional ureteroplasty [Figure 2c], a Leadbetter–Politano type ureteric reimplantation was performed [Figure 2d]. The child was discharged after an uneventful postoperative recovery period. After more than a year of the procedure, the child is asymptomatic and is free from UTI.

Figure 2

Schematic representation of intraoperative findings (a) insertion of dilated, ectopic ureter into bladder neck (b) division of left ureter just proximal to ectopic insertion (c) A Excisional tapering of the terminal dilated ureter (d) ready for reimplantation

A close watch on the poorly functioning moiety, which in retrospect might have been missed on the renal dynamic scan, has been planned. In the follow-up, complications in this moiety may merit partial nephrectomy although after 15 months of the intervention the child has remained asymptomatic and upper tracts did not deteriorate further.

DISCUSSION

After 150 years, since the first case description by Wrany and approximately a hundred case reports later,[2] the embryology and the pathogenesis of this particular anomaly remain unclear. The postulated theories for duplicated and triplicated systems are the same.[34] Multiple ureteric buds arising from the mesonephric duct with or without fusion may contribute. It is worth noting that the Weigert-Meyer law is applicable to most of the duplicated ureteric systems; this may not be the case in triplication of ureters. Ireland et al.[3] postulated that this is possible because of the early splitting of the ureteric bud. Smith's classification[1] divides the triplicate ureter into four types: Triple ureters (complete triplication): Three separate ureters and three separate ureteral orifices, with no interconnection between ureters.

Triple ureters (complete triplication): Three separate ureters and three separate ureteral orifices, with no interconnection between ureters

Double ureter with one bifid: Three ureters arise from the kidney, but two of these join before reaching the bladder, in which two ureteral orifices are found

Trifid ureter: Three ureters arise from the kidney. Two of these later joins, and the ureter formed by this union joins the third ureter before reaching the bladder, in which there is only one ureteral orifice

Double ureter with inverted “Y” bifurcation: Two ureters arise from the kidney; one of these bifurcates before reaching the bladder, where three ureteral orifices are present.

Clinical significance in ureteric triplication is the same as that of ureteric duplication. Associated anomalies are often present, including, but not limited to ureter duplication of the opposite side, ureterocoele, fusion anomalies of kidney, and vesicoureteral reflux.[5678] Our case is a Type 3, trifid ureter.

In view of rare incidence, variable presentation, and associated anomalies, there is no set protocol for management. In the index case, the terminal ureter was repositioned in the bladder, and a close watch on the superior most poorly functioning moiety was planned. In the follow-up, complications in this moiety may merit partial nephrectomy.

We understood from this case that a thorough radiological and functional assessment by a pediatric radiologist is mandatory. Whenever in doubt, other complementary imaging options should be utilized. Complete preoperative evaluation helps in appropriate decision making and salvage of renal moieties. Knowledge of the abnormal anatomy helps the surgeon to create a road map for surgical intervention and minimize intraoperative surprises.

CONCLUSION

Triplication of the ureter is a rare anomaly. Multiple investigative modalities may be required to confirm the diagnosis. The management depends on the exact anatomy, associated anomalies, and functional status of each moiety. The importance of long-term follow-up cannot be overemphasized.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.