“Urological Claudication”: Blind ending ureteric remnant in a bifid ureteric system causing neurological claudication—Case report with brief review of the literature

Abstract

Background

A blind ending ureteric remnant in a duplicated urinary system is extremely rare, and most of these anomalies go unrecognized as they are asymptomatic. We describe a unique case of a blind ending ureteric remnant presenting with neurological claudication in the lower limb secondary to sacral nerve root radiculopathy which was managed by robotic excision.

Case presentation

A 61-year-old male presented with radiating pain in the left gluteal region for the past 2 years, which persisted despite multiple conservative treatments. Extensive evaluation for the pain revealed a blind ending ureteric remnant in a partially duplicated system, which contained multiple calculi and caused the compression of the adjacent sacral nerve roots. The patient was subsequently taken up for excision of the remnant, which was performed robotically, after which the symptoms resolved completely.

Conclusion

Blind ending ureteric duplications are rare anomalies that mostly go unrecognized as they are asymptomatic. Rarely, these anomalies may produce clinically relevant symptoms and result in diagnostic dilemmas, meriting intervention.

1 Background

Anatomical variations in the urinary tract are common and most of these do not have any major clinical implications. A blind ending ureteric remnant in a duplex urinary system is one such extremely rare anatomical variation which is detected incidentally in majority of the cases. We hereby describe a unique case of neurological claudication caused by a blind ending ureteric bud remnant in a bifid ureteric system managed with robotic excision, resulting in complete resolution of the symptoms.

2 Case presentation

A 61-year-old male without any co-morbidities or relevant medical or family history presented with complaints of long standing sharp intermittent left-sided gluteal pain radiating to the leg, worsened by activity and erect posture which had aggravated over the past 2 years. The patient also had a history of multiple consultations with various specialists over the years for these symptoms with no significant relief. The patient had no urinary or bowel-related symptoms and examination revealed a mild reduction in the muscle power during extension of the hip and flexion of the knee in the left lower limb with diffuse, appreciable loss of sensations. The patient was referred to the department of urology as the magnetic resonance imaging of the spine revealed a cystic lesion in the left presacral region causing compression of the adjacent sacral nerve roots with suspected communication with the urinary bladder (Fig. 1A). The imaging findings were further discussed with the radiologist, which led to the suspicion of a rudimentary ureteric bud in a duplex system on the left side causing compression on the exiting sacral nerve roots (Fig. 1B, C). The retrograde pyelography on the left side revealed an incompletely duplex ureteric system with union just proximal to the vesico-ureteric junction and a rudimentary blind ending ureteric bud that was lodged in the pelvis on the left side. The blind end of the ureteric remnant also contained fine debris suggestive of multiple small calculi (Fig. 1D). The patient was then taken up for robot-assisted laparoscopic excision of the ureteric remnant (Fig. 2). Intraoperatively, the patient experienced jerks on the ipsilateral lower limb during the activation of the electrosurgical device while dissecting around the blind ending remnant, reconfirming its proximity to the sacral nerve roots. The patient was discharged on the postoperative day one, and at 6 months of follow-up, the patient was completely free of all neurological symptoms in the left lower limb.

Fig. 1

Imaging evaluation. A—Axial T2-weighted image showing a hyper-intense cystic lesion (Marked by *) on the left side of the pelvis in close proximity to the sacral neurovasculature with hypo-intense-dependent calculi. B Coronal T2-weighted image show hyper-intense lesion (marked by X) on the left side of pelvis lodged between the rectum and lateral wall of pelvis. C Coronal T2 fat saturation image reveals compression of exiting sacral nerve roots [S1, S2 and S3] (marked with blue arrows) by the cystic structure in the pelvis (marked by red arrow). D Left-sided retrograde pyelogram revealing partially bifid ureteric system on the left side with union just proximal to the vesico-ureteric junction and blind ending remnant lodged in the pelvis (marked by X)

Fig. 2

Intraoperative findings. A Initial intraoperative pelvis survey reveals cystic lesion on the left side of the pelvis lodged between the pelvic wall and rectum (marked by #) with the draining ureter (marked by arrow heads). Also note the urinary bladder (marked by X) and normal ureter (Marked by *). B Dissection of the blind ending remnant with identification of supplying blind ureter (marked by *) and the normal ureter (marked by arrow heads). C Complete excision of the remnant by securing blood supply. D Post-operative specimen

3 Discussion

The ureteric bud is an embryological precursor of the renal collecting system and arises from the Mesonephric/Wolffian duct (MND) around the 28th day of gestation and interacts with the metanephric blastema to give rise to the pelvi-calyceal system [1]. Ureteral duplication results from the aberrant branching of the ureteric bud and the blind ending ureteric remnants are observed if there is a failure of the ureteric bud to interact with the metanephric blastema [1]. Most of the blind ending mesonephric duct remnants are asymptomatic and clinically irrelevant [2]. Rarely, these ureteric remnants can present with clinically attributable symptoms if there is associated infection, inflammation, stones or vesico-ureteric reflux, when they merit management ranging from stone clearance [3] to excision of the remnant [4] or even nephroureterectomy for the non-functional renal units [5]. In addition, the MND also serves as the precursor for the development of male internal sexual organs, which explains their association with anomalies such as renal agenesis [6], uterine/vaginal anomalies [7] and ectopic drainage into organs of the reproductive tract [8] observed in patients with maldevelopment of the MND. The clinical presentation and associated anomalies of the ureteric bud remnants are summarized in Table 1. Coleman and colleagues classified the developmental anomalies of MND into a 3-tier system based on their experience of 19 cases over a period of 7 years, and designated Type 1 as those anomalies in which the ureteral bud arises from the orthotopic site, the Type 2 were those when the ureteral bud structures inserted at the ectopic sites (vagina, bladder neck, etc.) and the Type 3 were complex MND anomalies [9]. Further, Ney and Friedenberg divided the blind ending ureter in duplicated systems into 3 types, the proximal blind ending variant being the most common, with others being the distal blind ending and double-blind ending variant with communication to the other normal ureter from the middle of the blind ending remnant [10]. Extremely rare is the 4th type, which is a double-blind ending remnant without communication with the normal ureter [11]. The most commonly accepted theory for the formation of a duplicated blind ending ureteric system is the failure of the ureteric bud to establish a contact with the metanephric blastema, leading to an aborted development [6]. However, the distal blind ending and double-blind variants cannot be explained by this theory. Rebecca L. Hulett and Hasan T. Ozgur postulated that the distal and the double-blind ending variants result from an ischemic event of the distal ureter leading to its atresia with or without the anomalous bud blastema interaction [11]. The formation of multiple calculi as in our case can be explained by the triple threat of urinary stasis, recurrent subclinical infection and reduced peristalsis. Nevertheless, the presentation in our case is unique and we could not find any previously published literature, with a similar presentation. Blind ending ureteral duplications warrant surgical excision when they are symptomatic or when complicated by recurrent infection, stone formation or malignancy. In our case, the remnant caused lumbosacral radiculopathy in the form of neurological lower limb claudication on the left side, which seems to be the first reported case in the published literature. Robotic systems offer advantages such as better vision, easy maneuverability in the pelvis, finer dissection possibilities, lesser postoperative pain and early recovery, making it ideal for pelvic surgeries.

Table 1 Summary of published literature on blind ending ureteric remnants and their associations

4 Conclusion

Blind ending ureteric duplications are rare anomalies and most of them go unrecognized as they are asymptomatic and carry minimal clinical relevance. Rarely, these anomalies result in clinical symptoms, causing diagnostic dilemmas and meriting surgical intervention.