Bilateral Single System Ectopic Ureters with Secondary Calculi in an Adult: Case Report


Dr Gopi Kishore M, Dr Suhasini G, Dr Prasad Pvgs, Dr Sainadh AV    27 January 2018


Ectopic ureter, secondary calculi, megaureter, hydroureteronephrosis

By definition, an ectopic ureter is any ureter, single or duplex, that does not enter the trigonal area of the bladder.1 It is more common among females and is usually associated with double collecting system. About one-fifth of ectopic ureters are associated with single system kidneys and are common in males. A rare entity of bilateral single-system ectopic ureters (BSSEU) occurs and may be associated with a hypoplastic bladder and bilateral renal abnormalities.2

We are presenting a case of BSSEU in an adult male with secondary stones, which was managed endoscopically. 

Case Report

A 50-year-old male presented with obstructive voiding symptoms, increased frequency, dysuria, hematuria and bilateral flank pain since 2 months. General examination was unremarkable. Abdominal examination was normal except mild bladder distension. External genitalia and per rectal examination was normal. All routine investigations including kidney function tests were normal.

Ultrasonography showed bilateral moderate hydroureteronephrosis with lower ureteric calculi. Plain computed tomography-kidney, ureter and bladder (CT-KUB, Fig. 1) revealed bilateral hydroureteronephrosis with multiple calculi in right lower ureter and single calculus in left lower ureter. Intravenous pyelogram (IVP, Fig. 2) revealed bilateral single system gross hydroureteronephrosis with multiple calculi in right ureter and one calculus in left ureter. With provisional diagnosis of bilateral megaureters with secondary stones or bilateral lower ureteric calculi with megaureters, cystourethroscopy was carried out under spinal anesthesia. Cystourethroscopy (Fig. 3) showed right ureteric orifice just distal to bladder neck and left ureteric orifice distal to right ureteric orifice and 1 cm proximal to the veru with absent trigone and good capacity bladder. Bilateral retrograde pyelography showed bilateral single system ectopic megaureters with secondary calculi.

Definitive diagnosis of BSSEU with secondary calculi was made. Bilateral ureteric meatotomy was done up to 1 cm proximal to bladder neck using a Collins knife and stones fragmented with help of nephroscope and lithotripsy. As stone burden was high, fragmented stones in bladder were removed by percutaneous cystolithotripsy and bilateral double-J (DJ) stenting done. Postoperative recovery was uneventful. In postoperative period, the patient was totally continent and able to void freely. DJ stent was removed after 1 month and follow-up ultrasound showed decrease in hydroureteronephrosis and patient is doing well without urinary tract infection (UTI) or flank pain.


Ectopic ureters are more common in females, 80% of them drain a duplicated kidney and are frequently associated with a poorly functioning renal unit.3 Embryologically, ectopic ureters can arise due to abnormal timing or location of the primary ureteral budding from the mesonephric ducts. That temporospatial location will determine both the character of the ureter incorporated into the emerging bladder, as well as the development of the trigone and kidney.2 It is believed that, as single system ectopic ureters (SSEU) are associated with dysplastic kidneys, the affected renal units do not function appreciably.3

Single system ureteral ectopia is due to cranial origin of ureteric bud from mesonephric duct, which results in delay in incorporation into the urogenital sinus and prevents in growth of mesenchyme, which is necessary for development of bladder neck musculature.4 As there is no formation of trigone and base plate, bladder neck is wide, poorly defined and incompetent.

BSSEUs are even rarer compared to SSEU.4 It is possible that during development the abnormal origin of both ureteric buds results in poor mesenchymal induction of the urogenital structures, which results in failure of normal development of the bladder and bladder neck. Both the sphincter and reservoir functions of the bladder will be severely affected.

Overall, female patients are affected twice as commonly as males, although SSEU is reported to be more common in males.3,5 Usually, BSSEU present in infants or children with recurrent UTIs, urinary incontinence and a poor capacity of bladder, which requires ureteric reimplantation with or without bladder augmentation.6,7

In males, the posterior urethra is the most common site for insertion of the ectopic ureter.5,6 Evaluation is usually by ultrasonography, renal nuclear scan, micturating cystourethrography and retrograde pyelography. Intravenous pyelography and magnetic resonance imaging (MRI) may be used occasionally.2,6

Male patients with BSSEU in posterior urethra proximal to external sphincter may be continent with external sphincter control and have a good capacity bladder. Patients with good bladder capacity may require bilateral ureteric reimplantation alone.6,7

Our patient presented with obstructive symptoms due to stone in the right distal ureter obstructing bladder neck. As the patient presented in late adulthood with secondary stones and a good capacity bladder with normal continence, endoscopic management alone was done with ureteral meatotomy, lithotripsy and DJ stenting. Surgical management consisting of transurethral endoscopic incision of the distal ureter has previously been reported by Mathews et al.5 Ureteric reimplantation was not preferred as reflux in late age is not a worrying factor. However, the patient is kept on close follow-up for any late symptoms.

To the best of our knowledge, this is the first case of BSSEUs with secondary ureteric calculi presenting in adulthood in current English literature. As patient presented in late adulthood with only obstructive symptoms, major reconstructive surgery was avoided and patient managed endoscopically.


  1. Glassberg KI, Braren V, Duckett JW, Jacobs EC, King LR, Lebowitz RL, et al. Suggested terminology for duplex systems, ectopic ureters and ureteroceles. J Urol. 1984;132(6):1153-4.
  2. Peters CA, Schlussel RN, Mendelsohn C. Ectopic ureter, ureterocele, and ureteral anomalies. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA (Eds.). Campbell-Walsh Urology. 10th Edition, Philadelphia: Saunders Elsevier; 2011. pp. 3236-66.
  3. Keating MA. Ureteral duplication anomalies: ectopic ureters and ureteral anomalies. In: Belman BA, King LR, Kramer SA (Eds.). Clinical Pediatric Urology. 4th Edition, London: Martin Dunitz; pp. 677-733.
  4. Redman JF, Lightfoot ML, Reddy PP. Bilateral single ureteral ectopia in a boy. Urology. 2002;60(3):514.
  5. Mathews R, Jeffs RD, Maizels M, Palmer LS, Docimo SG. Single system ureteral ectopia in boys associated with bladder outlet obstruction. J Urol. 1999;161(4):1297-300.
  6. Dange AS, Sen S, Zachariah N, Chacko J, Mammen KE. Single-system ureteral ectopia - Associated malformations and management in children lacking an orthotopic ureter. Pediatr Surg Int. 1994;9:377-80.
  7. Kumar A, Goyal NK, Trivedi S, Dwivedi US, Singh PB. Bilateral single system ectopic ureters: case report with literature review. Afr J Paediatr Surg. 2008;5(2):99-101.

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