Single-Setting Laparoscopic Treatment of Complete Bilateral Ureteral Duplication in Adult Women

Introduction

Complete duplication of the ureter (CUD) is one of the most common congenital malformations of the urinary tract. The reported incidence is 1 in 125, or 0.8%. ¹ This condition is more common in women (85%), and it is bilateral in 10% of cases. ² Ectopic ureter insertion may be located anywhere on the ectopic pathway, which is different in each sex. In female patients, the orifice is most frequently located at the urethra (35%), vaginal vestibule (34%), or the vagina (25%). ³ CUD frequently occurs in association with a dysplastic upper-pole renal moiety with minimal residual function where the orthotopic lower ureter enters the bladder and the upper ureter is ectopic. The farther this ureteral orifice is located, the greater the degree of renal maldevelopment. ⁴

As with localization, clinical presentation varies in each case. Girls may be partially asymptomatic or they usually complain of vaginal discharge and flank pain. Symptomatic CUD (sCUD) is characterized by recurrent urinary tract infections (UTIs) or urinary incontinence (UI, generally congenital and incomplete). ⁵ The standard surgical treatment of sCUD is upper pole heminephrectomy with partial ureterectomy. ¹ To date, several studies have investigated the role of laparoscopic upper-pole partial nephrectomy (LUPPN) for sCUD and hypofunctional renal moiety in adult patients.^6–8

We present a series of 12 LUPPNs performed in 6 female patients to highlight the feasibility and effectiveness of a single-setting treatment of bilateral sCUD. The technical challenges include bleeding control, dissection of the ectopic from the orthotopic ureter, and management of the ectopic ureteral stump (EUS). This report details step by step the technique, the feasibility, and the clinical outcome of single-setting bilateral laparoscopic upper pole partial nephrectomy (SSBLUPPN) for bilateral sCUD in adult women.

Materials and Methods

From February 2002 to November 2009, 6 female patients underwent SSBLUPPN for bilateral sCUD. Mean patient age was 38 years (range 36–40). Every patient complained a history of continuous UI characterized by constant dribbling with a normal pattern of voiding, and all patients but 2 had a history of recurrent UTIs. Serum creatinine measurements were obtained preoperatively (range 0.7–1 mg/dL). An initial evaluation by abdominal ultrasonography (AUS) showed in all the cases a hydronephrotic upper pole of both kidneys with an abnormal dilatation of the proximal ureter but a normal aspect of the distal tract. A further investigation by magnetic resonance imaging of the urinary tract demonstrated an enlarged ectopic ureter (megaureter) originating from the upper pole of the kidney and draining in the vagina. This condition was bilateral in all the cases (Fig. 3C, F). Finally, all the patients underwent a renal scan with DTC 99 and a complete urodynamic evaluation. Ureteral reflux was excluded before surgery. All the procedures were performed in a single setting by a transperitoneal route.

Once anesthesia has been provided, the patient undergoes cystoscopy to bilaterally place an open-ended 6 Fr ureteral catheter into the normal lower-pole ureter for helping its intraoperative identification and reducing the risk of iatrogenic injuries. Ureteral catheters are inserted on a hydrophilic guidewire with a flexible tip under fluoroscopic control to avoid injuries to the ureteral mucosa. Then, the patient is positioned on a flank with a break in the table on flexion (1 in Fig. 1). The controlateral arm is secured to the operating table. Peritoneal access is obtained through an open technique. Four port sites are placed under direct endoscopic vision with a 10-mm Hasson trocar at the umbilicus (camera), a 10-mm port below the costal margin in the midclavicular line (MCL), and a third 5- or 10-mm port in the MCL 4–5 cm distant from the transverse umbilical line. An optional fourth port (5 mm) is placed over the midline (ML) 4 cm above the Hasson trocar (Fig. 2). In all the cases, we planned a single-setting treatment (2 and 3 in Fig. 1). Thus, after completing a unilateral LUPPN (first step), the patient is redraped and positioned on the opposite flank. Then, the procedure is controlaterally performed (second step). For the laparoscopic dissection, we used the Harmonic Scalpel (Ultracision^®).

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FIG. 1.

(1) Patient Positioning. (2, 3) Disposition of the operative ports. (4) Cosmetic results at 6 months follow-up after single-setting bilateral laparoscopic upper pole partial nephrectomy (SSBLUPPN).

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FIG. 2.

Trocar disposition for SSBLUPPN. MCL, midclavicular line; ML, midline.

The colon is medially reflected by an incision along the line of Toldt to enter the retroperitoneum. The ectopic ureter is easily identified for its typical enlarged aspect (megaureter), whereas the orthotopic ureter is usually medially located (Fig. 3A). The ovaric vein is located laterally to them and, if necessary, it can be isolated and ligated. A careful dissection of the orthotopic ureter is performed. The kidney and the upper-pole hypofunctional segment are identified. The inferior ureter frequently runs close to the upper-pole ureter. During this step, the ureteral catheter is helpful to differentiate between the upper-pole ureter and the lower-pole ureter. The hilar dissection is the most technically demanding time of the procedure, and it must be carefully performed. Once the main renal artery is identified, the upper-pole ureter is mobilized proximally toward the renal hilum. The upper-pole ureter is located posteriorly to the renal hilar vessels, and it requires complete mobilization. When the hilum is completely dissected away from the upper-pole ureter, the vessels supplying the upper pole are clipped and divided (Fig. 3C). During these steps, injuries to the blood supply of the normal ureter must be avoided. Preoperative imaging is helpful to identify a clear cleavage space between the hypofunctional upper-pole and the normal kidney. However, an anatomical line between the devascularized upper-pole segment and the remaining normal parenchyma is usually present. If absent, the renal capsule between the upper-pole segment and the remnant parenchyma can be scored with the bipolar cautery. The harmonic scalpel is used to fully transect the upper pole moiety (Fig. 3C). After the resection of the upper pole, the ectopic megaureter is dissected inferiorly and isolated at the level of the iliac vessels. Distally, it is secured with Hem-O-Lok^® and sectioned with cold scissors (Fig. 3B). Then, the specimen is carefully passed behind the renal pedicle, avoiding traction (Fig. 3D) and it is entrapped in the endobag. Once hemostasis is achieved, application of tissue sealants (Floseal^®) on kidney surface is provided. If concern exists regarding the integrity of the lower pole calices, methylene blue is injected via the ureteral catheters, or a retrograde pyelogram is obtained before closing the operative ports. If no signs of urinary leakage are present, the specimen is extracted through a 10-mm port site using a standard retrieval bag. A drain is left adjacent to the surgical field, and the port sites are closed.

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FIG. 3.

(A) Anatomical landmarks in the retroperitoneal space. EU, ectopic ureter; NU, normal ureter. (B) Isolation and section of the EU at the level of the iliac vessels. (C, F). Magnetic resonance imaging of a bilateral complete duplication of the ureter. (D) Isolation of the upper pole of the kidney with Ultracision^®. UP, upper pole. (E) UP with the EU removed from the kidney surface.

Results

All the procedures were successfully performed with no conversion to open surgery. Mean operative time of BLUPPN was 244 minutes (range 200–288 minutes). Mean operative times of the first-step and second-step procedures were 118 (100–136) and 126 (105–147) minutes, respectively. Mean blood loss was 236 cc (100–372 cc). No intraoperative major complications occurred in all the cases. Despite chronic inflammation in all the resected specimens, patients showed no clinical signs of infection postoperatively. Mean hospital stay was 5 days (range 4–6 days). Ureteral catheters were removed at the end of the procedure if an intraoperative retrograde pyelogram showed no signs of urinary leakage. During follow-up, biochemical analyses assessing the renal function were performed at 3 months; a Uro-CT scan was performed at 6 months (Fig. 4A, B), and AUS was performed at 1 and 2 years, postoperatively. At 3 months, a normal creatinine (range 0.8–1.1 mg/dL) value was found in every patient. At a mean follow-up of 36 months (24–48 months), all patients were symptom free; they showed no hydronephrosis and a complete remission of UI and UTIs. Thus, no evidence of dilated EUS was found at AUS follow-up in any of the cases.

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FIG. 4.

(A, B) CT scan at 6 months follow-up showing no hydronephrosis. (C) Site of specimen retrieval (arrow) and final aspect after a single-setting procedure.

Discussion

When an ectopic ureter is associated to a hypofunctional upper pole, open heminephrectomy with partial ureterectomy is recommended as the standard treatment. ⁶ In case of early diagnosis of sCUD with preserved upper-pole function, more conservative surgical techniques can be attempted. ⁹ Laparoscopic Heminephrectomy for the treatment of ectopic ureter in adult patients was first described by Wang et al. In another study, Abouassaly et al. provided surgical and functional outcomes of LUPPN for unilateral duplicating renal collecting system. ⁶

Several series have highlighted the feasibility of single-setting bilateral nephron sparing surgery.^10,11 According to Reisiger et al., this approach is safe and can expedite resolution of pathologic findings without increased morbidity only if the primary procedure has been expeditiously completed. ¹⁰ In case of intraoperative complications during the first step of SSBLUPPN, we suggest completing a unilateral LUPPN and delaying the controlateral treatment in a second setting at 3–6 months. However, this may temporarily cause lack of regression of symptoms.

If a SSBLUPPN is performed, management of both EUSs remains controversial. ¹² According to Ade-Ajayi et al., a long EUS after heminephrectomy in children may later lead to the development of persistent UTIs due to stasis of infected urine, or UI in only 8% of cases. ¹³ The recurrence of symptoms related to EUS, also known as “residual stump syndrome,” was first described in children by Pace and Pinto ¹⁴ However, when the ureterectomy is performed at the pelvic brim, symptoms related to EUS seem to be rare. ¹⁵ Most of pediatric urologists believe that the risks of injuring the healthy orthotopic ureter during laparoscopic heminephrectomy for sCUD may well be higher than the benefits of performing a complete ureterectomy. ¹⁵ We believe that these observations may be extended also to adult patients.

With regard to the best management of EUSs, we suggest that a bilateral ureterectomy as low as possible to prevent a refluxing diverticulum and a possible source of UTI would be a safer alternative treatment. A preoperative complete urodynamic assessment is necessary to exclude ureteral reflux or a different etiology of the UI. Independently from surgical experience, female pelvic anatomy in a patient with sCUD and a past history of UTIs makes the isolation of the normal ureter from the ectopic one a difficult surgical challenge. In the bilateral CUD, reducing the size of EUSs rather than completing their diversion from the vaginal vault may be a reasonable option. According to surgeon experience, EUS may be left open or closed. Belman et al. ¹⁶ proposed that EUS should be ligated if UR is diagnosed in an ectopic ureter before surgery. Persad et al. ¹⁷ found that two of eight ureters left open had closed at the time of re-exploration for complications.

A complete excision of EUS could be necessary only in case of sCUD with concomitant ureteral cancer. To date, only one case of ureteral cancer associated to CUD has been described in an adult patient. ¹⁸ A complete distal ureterectomy might offer the advantage of specimen extraction through the vaginal route, especially in case of dysplastic upper-poles >4 cm. However, in our series, mean specimen size was <4 cm (range 2.7–3.6 cm); and we found it easier and less traumatic to extract the endobag through the main port site (Fig. 4C).

The main objectives of sCUD treatment should be a complete recovery of symptoms and the full preservation of the renal function. We are aware that long-term follow-up is necessary to make conclusions about the need for performing a complete distal ureterectomy. However, in our series, the success rate at 36 months follow-up was 100%. None of the patients treated experienced recurrence of symptoms. With regard to the technical challenges of SSBLUPPN, we found the use of the Harmonic Scalpel (Ultracision^®) helpful in all the main steps of the procedure.

Conclusions

SSBLUPPN for bilateral sCUD is feasible and associated with minimal morbidity. The tricks of the trade involve the placement of a catheter in both normal ureters at the beginning of the procedure, full mobilization of the upper-pole of the kidney with its ectopic ureter, and a precise identification of the blood supply to the upper pole. A single-setting treatment of complete ureteral duplication is a technically demanding procedure, and it should be performed only in centers with large experience in laparoscopic renal surgery.