0022-5347/05/1734-1357/0 THE JOURNAL OF UROLOGY® Copyright © 2005 by AMERICAN UROLOGICAL ASSOCIATION
Vol. 173, 1357–1360, April 2005 Printed in U.S.A.
DOI: 10.1097/01.ju.0000152317.72166.df
REFLUXING URETERAL REIMPLANT AS TEMPORARY TREATMENT OF OBSTRUCTIVE MEGAURETER IN NEONATE AND INFANT SANG DON LEE, CEM AKBAL
AND
MARTIN KAEFER*
From the Department of Urology, Riley Hospital for Children, Indiana University, Indiana, and College of Medicine, Pusan National University, Busan, Korea (SDL)
ABSTRACT
Purpose: An obstructive megaureter identified in the neonatal period can be managed using a number of techniques, with the primary goal being to minimize the potential for further injury to the affected kidney. We describe our experience with refluxing ureteral reimplantation as a novel method for temporizing the obstructive megaureter. Materials and Methods: Three patients identified prenatally with severe hydroureteronephrosis were confirmed following delivery to have an obstructive ectopic ureter. Unilateral obstruction was identified in 2 patients (1 female, 1 male). The third patient was a female with bilateral single system ectopic ureters. Treatment consisted of anastomosing the ureter proximal to the obstruction to the dome of the bladder in a freely high grade refluxing fashion. All of the patients were placed on antibiotic suppression after surgery. Results: All patients demonstrated improved drainage of the affected kidney(s) following surgery. One female patient with unilateral obstruction had a poorly functioning kidney that showed no improvement of renal function 6 months following refluxing reimplantation, and laparoscopic nephrectomy was performed. The male patient with unilateral obstruction had adequate function with a significantly decreased ureteral diameter 1 year following refluxing ureteral reimplant, and a ureteral reimplantation without tapering was performed. The female patient with bilateral obstruction had 1 breakthrough urinary tract infection 6 months after surgery and now awaits second stage repair. Conclusions: Refluxing ureteral reimplantation is a safe, easy, beneficial and well tolerated means of temporizing the obstructive megaureter. This technique allows time for the child to mature, while accurately establishing renal function and preparing for a definitive surgical solution. KEY WORDS: infant, newborn; ureter; ureteral diseases; ureteral obstruction; disease management
MATERIALS AND METHODS
lowing delivery. In 2 patients (a 9-day-old male and a 5-month-old female) the obstruction was unilateral. The remaining patient (an 8-day-old female) had bilateral single system ectopic ureters with obstruction and vesicoureteral reflux. All patients were started on prophylactic antibiotics following delivery. After undergoing renal scintigraphy to establish the diagnosis of obstruction the patients underwent diagnostic cystoscopy and retrograde pyelography. In each case a stenotic distal ureteral segment was identified. Thereafter, the bladder was approached through a Pfannenstiel incision, and the ureter was identified extravesically as it coursed below the obliterated umbilical artery. The ureter was transected proximal to the stenotic segment and mobilized only enough to allow for a tension-free refluxing vesicoureteral anastomosis along the lateral aspect of the bladder. All of the patients were placed on antibiotic suppression after surgery. Patients were followed with ultrasound and renal scan to confirm resolution of obstruction and determine renal function once the obstruction had been relieved. Two patients have reached an age suitable for definitive treatment of the involved renal unit, while the remaining patient awaits definitive surgical correction. Followup subsequent to refluxing ureteral reimplantation ranged from 5 to 21 months (average 11.3).
Three patients with a history of fetal hydroureteronephrosis were discovered to have an obstructive megaureter fol-
CASE HISTORIES
Management of the obstructive megaureter is one of the most difficult problems in urological surgery, particularly in the neonate and young infant.1 An obstructive megaureter identified in the neonatal period can be managed using a number of operative techniques with the primary goal being to minimize the potential for further injury to the affected kidney. However, the commonly used methods of providing drainage have their limitations. The nephrostomy tube is cumbersome and difficult to handle, and is a poor long-term solution.2, 3 Cutaneous ureterostomy is prone to stenosis and pyelonephritis.3, 4 Megaureter tapering and standard ureteral reimplantation have technical limitations in the first year of life because of small bladder capacity.1–5 A method for internal drainage would provide a more acceptable means of relieving obstruction in neonates and infants, especially with uncontrolled urinary tract infection and/or deteriorating renal function, until a time at which these patients could undergo a more definitive surgical repair. We describe our experience with refluxing ureteral reimplantation as a beneficial means of temporizing the obstructive megaureter secondary to a stenotic ureterovesical segment.
Submitted for publication July 6, 2004. Case 1. A 5-month-old female with prenatally detected * Correspondence: Department of Pediatric Urology, Riley Hospi- hydroureteronephrosis was referred after experiencing retal for Children, Indiana University, 702 Barnhill Dr., Indianapolis, Indiana 46202 (telephone: 317-274-8896, FAX: 317-274-7481; e-mail: current urinary tract infections. Mercaptoacetyltriglycine (MAG-3) renal scan revealed that the right kidney
[email protected]). 1357
1358
REFLUXING URETERAL REIMPLANT TO OBSTRUCTIVE MEGAURETER
uted only 10% to 15% of overall renal function. Ultrasound suggested that there may have been a small ureterocele as the etiology of the obstruction. However, at the time of cystoscopy it became clear that the etiology was an obstructive ectopic ureter. Since the patient was already under anesthesia it was decided to proceed with intravesical drainage rather than subject her to a second anesthesia for placement of a percutaneous nephrostomy tube. Following creation of a refluxing ureteral reimplant the child was placed on antibiotic suppression. Postoperative renal scan demonstrated resolution of the obstruction but no significant improvement in renal function. Postoperative voiding cystourethrogram (VCUG) showed high grade reflux on the operated side. Six months following refluxing ureteral reimplantation the parents elected to have the child undergo laparoscopic nephrectomy (fig. 1). Case 2. A 9-day-old male presented with prenatal findings of marked left hydroureteronephrosis. The renal scan revealed an obvious obstructive pattern with the affected side contributing 30% of overall renal function. At age 6 weeks retrograde pyelography demonstrated a stenotic ureteral segment, and refluxing ureteral reimplant was performed. The patient was placed on antibiotic suppression after surgery. Followup studies showed adequate renal function with high grade reflux and a significantly decreased ureteral di-
FIG. 1. Patient 1. A, severe hydronephrosis of right side on preoperative ultrasound after birth. B, poor renal function and obstructive renogram pattern of affected side on MAG-3 renal scan at age 5 months. C, nonobstructive pattern of renogram with no improvement of renal function on MAG-3 renal scan 6 months after first stage repair.
ameter on the affected side. At age 18 months the patient underwent Cohen cross-trigonal ureteral reimplantation without the need for ureteral tapering. Followup studies after second stage repair revealed adequate renal function and good drainage of urine from the affected side (fig. 2). Case 3. An 8-day-old female presented with prenatally detected progressive bilateral hydroureteronephrosis. MAG-3 renal scan demonstrated poor bilateral renal function with obstruction, while VCUG showed bilateral low grade vesicoureteral reflux. Refluxing ureteral reimplant was performed at age 3 weeks. The patient was placed on antibiotic suppression after surgery. Followup studies after surgery revealed improved renal function with a significantly decreased ureteral diameter and bilateral high grade reflux. The patient had 1 breakthrough urinary tract infection 6 months after surgery while receiving antibiotic prophylaxis. She is doing well and awaits future second stage repair in the form of either bilateral megaureter reimplantation with or without ureteral tapering, or unilateral tapered megaureter reimplantation with transureteroureterostomy at age 18 to 24 months (fig. 3). DISCUSSION
The ultrasonic identification of increasing numbers of perinatal dilatations of the renal pelvis and ureter has raised serious questions regarding their natural history, evaluation and management. Patients found postnatally to have severe obstruction have the potential for development of significant renal impairment. Neonates and infants with distal ureteral obstruction, especially those who require surgery due to breakthrough urinary tract infections or severe impairment of renal function, present a severe therapeutic challenge to the pediatric urologist. There is a long existing controversy as to whether to perform primary reconstruction or to provide urinary drainage initially with the subsequent intent of providing a longterm solution.4 The primary treatment goal in patients with high grade distal ureteral obstruction is to preserve upper urinary tract function. However, the most commonly used methods of providing drainage have limitations. A brief period of nephrostomy drainage before surgical remodeling of the lower ureter can allow for ureteral decompression while providing access to the kidney and ureter for functional assessment.5 However, any form of prolonged intubation has obvious drawbacks, including difficulty in handling and securing the tubes and inevitable urinary tract infection.2 Furthermore, placement of a nephrostomy tube in a young patient has been reported to have a significant complication rate (4% to 8%), including severe hemorrhage requiring blood transfusion, septicemia and inadvertent puncturing of the pleura or intraabdominal viscera such as liver, colon and spleen.6 Distal cutaneous ureterostomy has been considered a potentially useful procedure for prolonged temporary diversion.4, 7, 8 Proponents of this method state that it can be maintained as long as necessary to evaluate functional and radiological improvement before restitution of an intact urinary tract. However, stomal stenosis is a potential concern with this technique.3 Stenosis is usually due to an inadequate terminal lumen circumference.4 Finally, many parents express significant anxiety regarding the maintenance of a cutaneous stoma. Primary reconstruction in the form of megaureter tapering and ureteral reimplantation would appear to be an attractive option. However, this approach has technical limitations in the first year of life.9 The limitations of previously described methods for treatment of distal ureteral obstruction led us to develop our approach. Reasoning that another form of distal ureteral obstruction, namely an obstructing ureterocele, is commonly
REFLUXING URETERAL REIMPLANT TO OBSTRUCTIVE MEGAURETER
1359
FIG. 2. Patient 2. A and B, left severe dilated ureter with ureteral tortuosity on ultrasound of abdomen and obvious obstructive pattern of renogram on MAG-3 renal scan 4 weeks after birth. C, left severe hydroureteronephrosis with ureteral kinking on intraoperative retrograde ureteropyelography. D, left vesicoureteral reflux on VCUG 6 months after first stage repair. E, nonobstructive pattern of renogram on MAG-3 renal scan.
FIG. 3. Patient 3. Bilateral (A, right and B, left) severe hydronephrosis on preoperative ultrasound of abdomen. C, bilateral obstructive pattern of renogram on preoperative MAG-3 renal scan. D, nonobstructive pattern of renogram on postoperative MAG-3 renal scan. Marked decreased bilateral hydronephrosis (E, right kidney with bladder empty and F, left kidney with bladder empty) on ultrasound of abdomen 6 months after first stage repair.
temporized using internal urinary drainage (ie incision of ureterocele), we decided to create a similar situation for the obstructive ureter with a distal stenotic segment. While a possible result of this procedure is reflux, it is commonly believed that relieving the obstruction will reduce the caliber of the ureter and make eventual tapering either easier or unnecessary. From our point of view, as with other methods of diversion, our method relieves the obstruction while allowing the child to mature and the clinician to make a clearer assessment of
renal function. Although basic in concept, our method avoids the disadvantages of other techniques while trading obstruction for the more manageable problem of vesicoureteral reflux. Also, internal drainage is more acceptable to the parent, since there is no alteration of the external anatomy as in the form of a stoma or nephrostomy tube. An additional advantage resides in the fact that by delaying the definitive surgical repair of the megaureter there is potential for the ureter to decrease in diameter, rendering the subsequent megaureter repair more feasible. Therefore, through the same inci-
1360
REFLUXING URETERAL REIMPLANT TO OBSTRUCTIVE MEGAURETER
sion ureteral reimplantation could be achieved without the necessity of ureteral tapering (as seen in case 2). Belman addressed this point and suggested that an alternative treatment for bilateral distal obstruction is ureterovesicostomy (ie cutting back on the narrowed ureteral segments to create gaping refluxing orifices within the bladder) along with cutaneous vesicostomy.10 This technique can yield good results when the ureteral meatus of a primary obstructive megaureter is in an orthotopic location. The location of the obstruction(s) in our patients did not allow for a simple cutback. In case 1 the ureter was in an ectopic location and incising it would have injured the bladder neck. In case 2 the stricture was 3 cm superior to the insertion of the ureter, and, therefore, would not have been reachable with this technique. In case 3 both ureters were in an ectopic location, and incising them would have risked cutting through the continence mechanism of the bladder neck. Management of the female child with bilateral single system ectopic ureters is one of the most challenging problems in pediatric urology. In case of bilateral single ectopic ureters subsequent malformation of the bladder trigone and bladder neck may result in additional voiding dysfunction, and ureteral reimplantation alone may not solve the urological problems.11 It has been theorized that for the bladder to develop normally, bladder cycling must occur. As a result of the urine being diverted away from the bladder, normal bladder cycling does not occur and development may be impaired. As opposed to treating this condition with cutaneous diversion (in which case the bladder remains defunctionalized), using our technique in the neonate optimizes the chance for bladder cycling by theoretically providing a potential stimulus for bladder growth. In our patient bladder size has increased with time.12 This finding will hopefully translate into greater ease of subsequent reimplantation as well as improved chances for urinary continence. CONCLUSONS
Refluxing ureteral reimplantation for temporary urinary diversion in neonates and infants with severe hydroureteronephrosis, especially with uncontrolled urinary tract infection and/or compromised renal function, provides effective renal drainage, allows for 1-stage reconstruction 12 to 18 months after the first stage of the operation, and avoids inconvenience to the child and family. Despite the diminishing indications for temporary urinary diversion, this technique might have a useful role in the treatment of properly selected patients with lower urinary tract anomalies, including obstructive megaureter secondary to a fixed distal ureteral obstruction. REFERENCES
1. Perovic, S.: Surgical treatment of megaureters using detrusor tunneling extravesical ureteroneocystostomy. J Urol, 152: 622, 1994
2. Leadbetter, G. W., Jr.: Skin ureterostomy with subsequent ureteral reconstruction. J Urol, 107: 462, 1972 3. MacGregor, P. S., Kay, R. and Straffon, R. A.: Cutaneous ureterostomy in children: long-term followup. J Urol, 134: 518, 1985 4. Mor, Y., Ramon, J., Raviv, G., Hertz, M., Goldwasser, B. and Jonas, P.: Low loop cutaneous ureterostomy and subsequent reconstruction: 20 years of experience. J Urol, 147: 1595, 1992 5. Hanna, M. K. and Jeffs, R. D.: Primary obstructive megaureters in children. Urology, 6: 419, 1975 6. Wah, T. M., Weston, M. J. and Irving, H. C.: Percutaneous nephrostomy insertion: outcome data from a prospective multi-operator study at a UK training centre. Clin Radiol, 59: 255, 2004 7. Rosen, M. A., Roth, D. R. and Gonzales, E. T., Jr.: Current indications for cutaneous ureterostomy. Urology, 43: 92, 1994 8. MacGregor, P. S., Kay, R. and Straffon, R. A.: Cutaneous ureterostomy in children long-term followup. J Urol, 134: 518, 1985 9. Hendren, H. W.: Complication of megaureter repair in children. J Urol, 113: 238, 1975 10. Belman, A. B.: Temporary diversion. In: Clinical Pediatric Urology, 2nd ed. Edited by P. P. Kelalis, L. R. King and A. B. Belman. Philadelphia: W. B. Saunders Co., vol. 1, chapt. 17, pp. 590 –593, 1985 11. Heuser, M., Zoller, G., Seseke, F., Zappel, H. and Ringert, R. H.: Bladder dysfunction in children with bilateral single ectopic ureters. J Pediatr Surg, 37: E15, 2002 12. Grady, R. W. and Mitchell, M. E.: Complete primary repair of exstrophy. J Urol, 162: 1415, 1999 EDITORIAL COMMENT The authors describe an alternative technique that can be used in select cases of isolated ureteral ectopia, especially if associated with obstruction. I agree that exchanging obstruction for reflux is logical, and, indeed, preferable to cutaneous ureterostomy. The latter guarantees bacteriuria and the inevitable nuisance calls from family and physicians each time the child has a fever or is fussy, and where they believe the urine must be the culprit. Still, in cases with poor renal function my inclination would be to place a temporary percutaneous nephrostomy first and then reassess the renal function. This approach would potentially avoid unnecessary anesthesia and lower tract surgery in an infant, especially in the event that nephrectomy ultimately was a more preferable therapeutic option. We have been impressed with the technique described by Caulk in 1923, which Belman popularized in the 1970s. This minimally invasive approach where one incises the obstructed ureter endoscopically should be strongly considered over open surgery as a temporizing alternative whenever possible. However, I recently used the technique described by the authors (admittedly after reviewing the first draft of their article) in a female infant who had a multicystic dysplastic kidney on one side and an ectopic ureter (distal to the bladder neck) subtending the sole functioning kidney on the contralateral side. Given the small bladder, I reimplanted the ureter to the dome, which took roughly 30 minutes. Like the authors, we are awaiting long-term followup. Martin A. Koyle Children’s Hospital University of Colorado School of Medicine Denver, Colorado