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Journal of Pediatric Urology (2014) xx, 1e7
Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction Martin Kaefer a,*, Rosalia Misseri a, Ethan Frank b, Audrey Rhee c, Sang Don Lee d a
Indiana University School of Medicine, Indianapolis, IN, USA Kaiser Permanente, San Diego, CA, USA c Cleveland Clinic, Cleveland, OH, USA d Pusan National University Hospital & College of Medicine, Busan, Republic of Korea b
Received 17 September 2013; accepted 29 January 2014
KEYWORDS Refluxing ureteral reimplantation; Ureterovesical junction obstruction; Neonatal; Ureterostomy
Abstract Objective: An obstructed megaureter can be managed using a number of techniques, with the primary goal being to minimize the potential for further injury to the affected kidney. Classically, these obstructed ureters have been treated using cutaneous ureterostomy. However, this technique has certain limitations including the potential for stenosis. We describe our experience with the refluxing ureteral reimplantation as a novel, yet technically simple, method for temporary internal diversion of the obstructed megaureter. Methods: Treatment consists of transecting the ureter proximal to the obstruction and performing an end-to-side anastomosis with the bladder in a freely refluxing fashion. Patients are placed on antibiotic suppression following surgery. Subsequent open definitive surgery is performed through the same incision site once the child is older than 1 year of age. Results: Sixteen patients identified with severe hydroureteronephrosis were found to have an obstructed megaureter(s) involving 19 ureteral moieties. Patients underwent internal diversion at an average age of 5 months. All patients demonstrated improved drainage of the affected kidney(s) following surgery. Three patients developed a febrile urinary tract infection. Definitive surgical treatment was undertaken in 18 of 19 ureters, and consisted of ureteral reimplantation with tapering or plication (13), ureteral reimplantation without tapering (3), and nephrectomy (2). One patient with multiple other congenital anomalies is not a candidate for further genitourinary reconstruction. Conclusions: Refluxing ureteral reimplantation is a safe and easy method of temporary internal urinary diversion. Simple in principle, the concept of creating a refluxing ureteral
* Corresponding author. Department of Pediatric Urology, Riley Children Hospital Indiana University, 702 Barnhill Drive, Suite 4230, Indianapolis, IN 46202, USA. Tel.: þ1 317 278 1021; fax: þ1 317 274 7481. E-mail address:
[email protected] (M. Kaefer). http://dx.doi.org/10.1016/j.jpurol.2014.01.027 1477-5131/ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
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M. Kaefer et al. reimplantation is no different from that of incising an obstructing ureterocele. This technique allows time for the child to mature, while preserving renal function and awaiting definitive repair. ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction The obstructed ectopic or orthotopic ureter can be treated in many ways. Classically, this condition has been treated with ureterostomy. However cutaneous urostomy has many potential disadvantages. These include the potential for stomal stenosis, risk of infection, and an unsightly stoma [1e7]. In cases of bilateral ureterovesical junction (UVJ) obstruction, bilateral ureterostomy results in the defunctionalization of the bladder. To obviate the disadvantages of cutaneous ureterostomy, we have developed a simple procedure based on modern treatment of obstructing ureteroceles. In the case of the obstructed ureterocele, incision or puncture is often chosen to relieve the blockage. Although the goal with this treatment is to relieve obstruction and not create reflux, on occasion reflux does occur. On these occasions one effectively trades obstruction for reflux, a condition, which is far easier to manage, and carries less risk for continued renal injury. We felt that converting an obstructed ureter to a refluxing intravesical ureter would have similar benefits.
Methods
traced down as far as reasonable without impinging on the bladder neck or sphincter mechanism and secured with a suture ligature. The bladder is filled through a Foley catheter so that one can best approximate the appropriate position to perform the refluxing anastomosis with the bladder. Any clear redundancy of the proximal ureter is excised so as to minimize the chance that distal ureteral tortuousity may restrict antegrade flow. The ureter is anastomosed to the bladder with interrupted 4-0 Vicryl suture at a lateral location (Fig. 1). The bladder is then filled once again through the Foley catheter to document that there is free reflux from the bladder into the dilated system. For the initial four cases, stents were left across the anastomosis for 5 days. Subsequent patients were not managed with stents. Once the child has exceeded an age of 12 months and is deemed to be in stable condition, the definitive repair is performed through a simple extension of the initial incision (the small 2e3 cm incision is extended into a classic Pfannenstiel incision).
Results Over the course of 12 years, 16 patients (F:M Z 8:8) with 19 obstructed ureters were managed using refluxing ureteral
Data acquisition Charts were reviewed and data were collected on mode of presentation, pathology (i.e. etiology of obstruction, function of the affected unit) laterality, age at time of temporizing procedure (i.e. refluxing reimplantation), urine culture results, time to definitive surgical intervention and mode of definitive surgical intervention. Renal status following the relief of obstruction was assessed by postoperative MAG-3 renal scan in most patients. In cases where the relief of the hydroureteronephrosis was profound, we elected to forego the expense and radiation exposure of an additional MAG-3 renal scan and simply followed the patients with renal ultrasound. Urine cultures obtained in the intervening time between the creation of the refluxing reimplant and the definitive surgical repair were recorded. This study was approved by the Indiana University institutional review board (IRB 0908-70),
Technique The procedure involves making a small 2e3 cm transverse incision on the side of the obstructed system. This incision is similar to that made for a ureterostomy. After dissecting through the fascia and separating the rectus muscles in the midline, the perivesicle space is developed and the dilated, obstructed system identified. The ureter is then transected proximal to the site of obstruction. The distal ureter is
Figure 1 Illustration of refluxing reimplantation for single system ureterovesical junction obstruction.
Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
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A logical method for managing neonatal UVJ obstruction Table 1
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Salient characteristics of patient population.
Refluxing reimplant N Z 16 patients (19 ureters) Presentation: prenatal hydronephrosis (11), sepsis (5) Etiology: Ectopic ureter e obstructed (8), POM (7), Ectopic ureter e obstructed and refluxing (3), other (1) Anatomy: unilateral single system (10), bilateral (3), duplex (3) Gender: female (8), male (8) Age at time of procedure (average): 5 months Interval to definitive procedure (average): 16 months
reimplantation. Salient characteristics of this population are shown in Table 1. Patients were identified based on prenatal hydronephrosis (11) or sepsis (5). Anatomically, the systems were described as a unilateral single system (10), bilateral (3), and duplex (3). Etiology of obstruction for these 19 ureters was obstructed ectopic ureter (8), refluxing and obstructed ectopic ureter (3), primary obstructed megaureter (7), and distal ureteral stricture (1). Of the 19 obstructed ureters, eight also demonstrated reflux. When all patients were evaluated as a group, patient age at time of the temporizing refluxing reimplantation averaged 5 months. However, not surprisingly, there was a significant difference in age at time of procedure when patients were stratified based on mode of presentation. Whereas patients who were identified prenatally underwent refluxing reimplantation at an average of 2 months, those who presented with sepsis underwent the procedure at an average age of 10.75 months. The average interval between creation of the refluxing reimplantation and definitive surgery to correct reflux (or otherwise manage the kidney) was 16 months. The refluxing reimplant was successful in relieving obstruction in all cases. Following the procedure, 14 ureters were studied with renal scan, all of which demonstrated a marked improvement in drainage time relative to preoperative studies. In the case of the five other ureters, it was not deemed necessary to obtain a renal scan following relief of the obstruction because there had been such dramatic decrease in the degree of hydroureteronephrosis. During the interval of observation (between undergoing a refluxing reimplantation procedure and definitively addressing the moiety), three patients experienced urinary tract infections. One patient had a double J stent placed for 3 months. This was an unusual case in which the child, subsequent to undergoing refluxing reimplantation, required a long-term full body cast for bilateral hip dysplasia. Not surprisingly, the renal collecting system remained dilated after converting the obstructed system to a refluxing system. Unfortunately, because of the presence of the body cast, a renal scan could not be obtained to definitively document that the system was completely open. At the time of cystoscopy to place a double J stent, the refluxing orifice was noted to be gaping and easily accepted a five French catheter (speaking against any obstruction). One additional patient (a bilateral case) required temporary placement of a suprapubic tube
because of poor bladder emptying and need for management of a urinary tract infection. To date, 18 of the 19 ureters have undergone definitive management. Thirteen ureters required a formal megaureter repair with reimplantation (10 tapered, three plicated). Three ureters have undergone reimplantation without need for tapering or plication. Two patients who initially were found to have poor renal function and did not have any improvement in function after the obstruction had been relieved, underwent nephrectomy (one laparoscopic, one open). The one remaining ureter is in a child with severe cognitive dysfunction, persistent UG sinus, and neuropathic bladder dysfunction. Definitive reimplementation of her ureters is not being considered because of her many other medical problems. In all cases of reimplantation (whether with megaureter tapering or not), renal function has been maintained at preoperative levels and postoperative MAG-3 renal scan (obtained in 13 or 16 patients) demonstrates free flow of urine across the previous site of UVJ obstruction. The three patients who did not undergo postoperative MAG-3 scan demonstrated clear decreases in degree of hydronephrosis. Two illustrative cases help underscore some of the aspects of this management strategy.
Case study 1 A 3-month-old female presented in urosepsis. A left duplex system was identified with upper pole hydronephrosis subtended by a large hydroureter (Fig. 2(a) and (b)). A percutaneous nephrostomy tube was placed and antibiotic therapy initiated (Fig. 2(c)). The child quickly defervesced and recovered from her urinary tract infection. The upper pole ended in an ectopic obstructed ureter. VCUG demonstrated reflux in the left lower pole and right ureter (Fig. 2(d)). One option was to leave the nephrostomy tube in place for an extended period of time. However, to allow the system to heal completely prior to definitive repair, a refluxing reimplantation of the upper pole ureter was performed (Fig. 2(e)). Ten months later, a left duplex reimplant (without tapering) and a simple right ureteral reimplantation were undertaken. Renal scan performed 1 month after surgery revealed excellent function and drainage of the left upper pole system (Fig. 2(f)).
Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
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M. Kaefer et al.
Figure 2 A 3-month-old female presented in urosepsis. (a) Renal ultrasound demonstrating left upper pole hydronephrosis and (b) dilated left upper pole ureter. (c) Nephrostogram at time of nephrostomy tube placement demonstrating obstructed system. (d) VCUG demonstrating reflux in the left lower pole ureter and right ureter. (e) Illustration of refluxing reimplantation of the left upper pole ureter. (f) Renal scan performed 1 month following surgery revealed excellent function and drainage of the left upper pole system.
Case study 2 A newborn full-term boy found prenatally to have significant bilateral hydroureteronephrosis (Fig. 3(a)) with normal amniotic fluid underwent postnatal radiographic evaluation. A positive urine culture obtained at the time of VCUG (Fig. 3(b): no valves or vesicoureteral reflux) and renal scan (Fig. 3(c): marked bilateral obstruction at the UVJ) and the finding of decreased urine output prompted surgical intervention. At the time of cystoscopy, the left system was
found to be in an orthotopic position with distal narrowing (Fig. 3(d)), whereas the right orifice could not be identified and was deemed most likely ectopic. A decision was made to perform bilateral refluxing reimplantation so as not to defunctionalize the bladder (Fig. 3(e)). Exploration confirmed that the right ureter was ectopic. A postoperative renal scan revealed markedly improved drainage and preservation of function (Fig. 3(f)). Definitive surgical intervention in the form of bilateral megaureter tapering occurred at age 23 months. MAG-3 renal scan data obtained
Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
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Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
Figure 3 A newborn full-term boy. (a) Prenatal ultrasound demonstrating significant bilateral hydroureteronephrosis. (b) VCUG demonstrating absence of valves or vesicoureteral reflux. (c) MAG-3 renal scan demonstrating marked obstruction at the UVJ bilaterally. (d) Left retrograde pyelogram demonstrating distal ureteral narrowing. (e) Illustration of bilateral refluxing reimplantation procedure. (f) Postoperative MAG-3 renal scan revealing markedly improved drainage and preservation of function. (g) MAG-3 renal scan data obtained 5 months after definitive surgical repair demonstrates unobstructed drainage.
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6 5 months following definitive surgical repair demonstrate unobstructed drainage (Fig. 3(g)).
Discussion Obstruction at the ureterovesical junction can take on many forms, with ureterocele, primary obstructed ureter, and ectopic ureteral insertion being the most commonly encountered. Specific decisions regarding management are predicated on the etiology of the obstruction, as well as the function of the renal unit in question. Although the absolute point at which surgical intervention is indicated has been strongly debated during the past few decades, most clinicians agree that relief of the obstruction is beneficial in the face of decreased renal function, infection and/or very high grade obstruction. Regardless of the technique utilized to achieve this goal, relief of the obstruction is critical to reduce further renal injury and minimize the chance of developing urosepsis. Many temporary means of relieving the obstruction (with the intent of later definitive reconstruction) have been utilized. Cutaneous diversion proximal to the obstruction (i.e. cutaneous ureterostomy) has been the classic method for managing these patients. Although this approach does achieve the goal of urinary diversion, many aspects of the approach can make it a less than ideal option. The incidence of stomal stenosis ranges between 8% and 22% for megaureters managed in this fashion [1e6]. Febrile urinary tract infections have been reported to occur in as many as 31% of patients with an end ureterostomy, despite the patients being on prophylactic antibiotics [7]. Additionally, some families may find the presence of a cutaneous stoma undesirable. Finally, in bilateral cases, cutaneous ureterostomy will result in bladder defunctionalization and potential loss of long-term bladder capacity. Even if bladder capacity is not lost, the technical challenge of reimplanting two megaureters into a small, defunctionalized bladder can be quite daunting. Percutaneous diversion of urine has a definite place in the initial management of the septic patient with ureteral obstruction. However, long-term management of the urinary tract by this means is cumbersome and, because of the presence of a foreign body, will increase the chance of infection and inflammation of the affected system. In the very specific subset of patients with primary obstructed megaureter, many authors have advocated stenting. The logic behind this choice is predicated on the understanding that the condition of primary obstructed megaureter often spontaneously resolves as maturation proceeds. Farrugia et al. reported on 16 infants with 19 obstructed megaureters who were stented at a median age of 24 weeks, for a median of 6 months. More than half of their patients did not require any further therapy. However, morbidity occurred in one-third of patients and function deteriorated after removal of the stent in two patients, both of whom later required nephrectomy [8]. One additional concern regarding the subpopulation of patients who do not improve with stenting is that the sequela of having a foreign body in the ureter (i.e. post-stent ureteral thickening) proves less than ideal when proceeding with definitive surgical repair. Although this technique seems to have
M. Kaefer et al. great utility, criteria that would help determine the ideal candidate for this form of intervention are needed before advocating its widespread application. Finally, many surgeons have advocated for definitive surgical repair (i.e. megaureter tapering and reimplantation) at the time the pathology is first identified [9e11]. Although excellent results have been achieved, the technical demands of performing a large megaureter repair and reimplanting it into the small neonatal bladder can be quite challenging. Despite meticulous technique, repeat surgery is necessary in 12% of patients younger than 1 year of age [9]. Furthermore, concerns that operating on the neonatal bladder prior to age 1 may have an adverse effect on longterm bladder function, have led some surgeons to try and avoid extensive surgical procedures on the bladder prior to 12 months of age. In light of the potential disadvantages inherent in each of these options, we proposed a novel approach to this clinical problem in 2005 [12]. Our present series focuses on the multiple situations in which this operation can prove useful as well as on the long-term results. We feel that there are significant advantages to this approach when comparing it with cutaneous ureterostomy. Stomal stenosis, seen in between 8% and 22% of patients does not occur when anastomosing two urothelial surfaces in a wide mouthed fashion. The incidence of urinary tract infections is potentially lower with our technique when compared with cutaneous urostomy [7]. In all three of our patients with bilateral obstruction, refluxing reimplantation was able to keep the bladder cycling. All three have undergone successful definitive ureteral reimplantation and are exhibiting no bladder dysfunction. Finally, family satisfaction with this technique is quite high because of the absence of a cutaneous stoma. Since our initial report of this procedure, other surgeons have inquired as to whether a secondary definitive procedure to reimplant the ureter is of absolute necessity. Although nonsurgical management of grade 5 reflux may never result in infection, the presence of a large reservoir of urine that may never completely empty with voiding, posses significant risk of future infection once antibiotic prophylaxis is discontinued. Others have noted that the procedure can be performed in an end-to-side fashion without dividing the ureter. The reason we elected to divide the ureter was to allow for the relief of any severe kinks proximal to the point of obstruction. If one can be confident that significant proximal tortuousity does not pose any threat to the restriction of urine flow, then an end-to-side approach would appear to be a reasonable option. We have considered performing this procedure laparoscopically. Although laparoscopic creation of a refluxing reimplant would be straightforward, the question arises as to what benefit this would provide the neonatal patient. In light of the fact that 1) the initial procedure is an extraperitoneal operation performed through a small 2 cm incision (analogous to a ureterostomy) and 2) the definitive procedure will be performed through a simple extension of the same incision at a later date, it is difficult to imagine that there would be any significant advantage that a laparoscopic procedure might provide in these neonates.
Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027
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A logical method for managing neonatal UVJ obstruction Although we do not advocate this as a first-line treatment for all duplex systems, this procedure can be used advantageously in select cases of upper pole obstruction. In contrast to ureterostomy, refluxing reimplantation requires minimal mobilization of the common shared blood supply. One limitation of the study is that it is a retrospective review and does not compare this population with a cohort that had undergone an alternative diversion procedure (e.g. cutaneous ureterostomy, stenting, percutaneous nephrostomy tube drainage). This manuscript serves to demonstrate that this option is feasible, can serve as an alternative method for relieving neonatal obstruction and is tolerated well by the majority of patients. Future studies comparing this technique with other forms of diversion will be helpful in more clearly defining the role of this procedure in the neonatal population.
Conclusion The refluxing reimplant provides a novel approach to management of the obstructed megaureter. This procedure trades obstruction for reflux while circumventing the potential disadvantages of other techniques for relieving neonatal ureterovesical junction obstruction.
Conflict of interest None.
Funding Pence Charitable Trust.
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Please cite this article in press as: Kaefer M, et al., Refluxing ureteral reimplantation: A logical method for managing neonatal UVJ obstruction, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.01.027