Original Article Double obstruction of ureter: A diagnostic challenge Pankaj Halder, Ram Mohan Shukla, Kartik Chandra Mandal, Biswanath Mukhopadhyay, Shibsankar Barman Department of Pediatric Surgery, Nil Ratan Sircar Medical College, Kolkata, West Bengal, India Address for correspondence: Dr. Biswanath Mukhopadhyay, 7E, Dinobandhu Mukherjee Lane, Sibpur, Howrah - 711 102, West Bengal, India. E-mail: [email protected]

ABSTRACT

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Introduction: Isolated obstruction of the ureteropelvic junction and the vesico-ureteric junction are the two most common causes of hydronephrosis in a pediatric population.[1] They do not pose diagnostic difficulties when are present alone but when together can be difficult to diagnose. Here, we discuss the problems we faced when we encountered these two anomalies in the same ureter and the way in which we managed them. Aim: To assess the difficulties in diagnosis of pediatric patients who present with both ureteropelvic junction obstruction (UPJO) and vesico-ureteric junction obstruction (VUJO) in the ipsilateral ureter and their management protocol. Materials and Methods: This is a retrospective study. The study period is from 1 January 2004 to 31 December 2011. Out of 254 children who were diagnosed to have hydronephrosis due to UPJO in our institute, 5 patients (in the age range of 5 to 10 years) had both UPJO and VUJO in the ipsilateral ureter. The problems we faced in diagnosing the two conditions are mentioned with a literature review. Results: Operative intervention was used in four out of five patients; none of the patients had an accurate diagnosis before surgery. All patients were suspected of having double obstruction during pyeloplasty when appropriate size double J stent could not be negotiated through the vesicoureteric junction into the bladder. Postoperative nephrostogram confirmed the diagnosis in all patients. Conclusion: Children with double obstruction of the ipsilateral ureter present as a diagnostic dilemma. Because of the rarity of this condition it can escape the eye of even an astute clinician. Early diagnosis can be made if this condition is kept in mind while treating any hydronephrosis due to UPJO or UVJO.

Website: www.jiaps.com DOI: 10.4103/0971-9261.136457 Quick Response Code:

KEY WORDS: Diagnosis, double obstruction of ureter, pediatric, treatment, UPJO, VUJO

Isolated ureteropelvic junction obstruction (UPJO) and vesico-ureteric junction obstruction (VUJO) are two common causes of hydronephrosis in children.[1] They can be diagnosed easily when are isolated but when together can be difficult to diagnose.

ultrasonography (USG) of the kidney, ureter and bladder region and diuretic renogram. The role of intravenous urography (IVU), micturating cystourethrogram (MCU) and retrograde pyelography is still debatable. The chance of missing associated pathology ranges from 54.55% to 78.57% in different series and whenever the associated pathology is missed it can complicate the postoperative period.[3,4]

The presence of these two anomalies in the ipsilateral ureter is rare and is very difficult to diagnose. [2-4] The established protocol for investigation of UPJO is

Here, we discuss the problems we faced when we encountered these two anomalies in the same ureter and the way in which we managed them.

INTRODUCTION

Cite this article as: Halder P, Shukla RM, Mandal KC, Mukhopadhyay B, Barman S. Double obstruction of ureter: A diagnostic challenge. J Indian Assoc Pediatr Surg 2014;19:129-32. Source of Support: Nil, Conflict of Interest: None declared.

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Halder, et al.: Double obstruction of ureter

MATERIALS AND METHODS This is a retrospective study. The total number of patients with both obstructions at the UPJ and VUJ in the same ureter in our study is 5. These patients were managed in our institute between 1 January 2004 to 31 December 2011. The patients were evaluated according to their demographic profile, side of affected ureter, clinical presentation and with diagnostic studies (USG, diethyl triaminepentaacetic acid (DTPA) scan, IVU, MCU and surgical intervention (Anderson Hynes dismembered pyeloplasty), with the outcome. All the patients with unilateral hydronephrosis due to suspected UPJO were included in the study.

RESULTS In the present study the male:female ratio was 4:1. The mean age of the patients was 7 years (range 5 to 10 years). The left side was affected in three patients and in two the right side was affected [Table 1]. Unfortunately, in none of the patients we were able to diagnose the ipsilateral distal ureteral obstruction in spite of doing all the above-mentioned investigations. Dilated distal ureter was not seen in any of the patients in preoperative USG and even in IVU with 24 hours delayed film.

All these patients underwent an Anderson-Hynes pyeloplasty after routine investigations (USG, DTPA scan and IVU). In four patients, pyeloplasty was done via dorsal lumbotomy approach and in one patient the flank approach was used. Double J stent was used to protect the anastomosis and also to look for any distal obstruction. In all these five patients, we suspected a distal obstruction as we were unable to negotiate an appropriate size DJ stent into the bladder. So, a nephrostomy was done in order to delineate the anatomy of the urinary tract as IVU was showing non-functioning kidney. Images from the ureter in nephrostogram done on the seventh postoperative day showed obstruction at the distal end of the ureter causing dilatation of the ureter with narrowing at the vesicoureteric junction. Ureteric dilatation persisted on the repeat nephrostogram done after 3 weeks in four of the five patients. So, ureteric reimplantation was done in all the four cases with complete VUJO. In one patient (female child) which showed reduced dilatation of the distal ureter on repeat nephrostogram was followed up and the obstruction resolved without any intervention. At present, all the patients are on follow up (mean duration of follow up is 24 months) and are doing well.

Table 1: A summary of the clinical data, preoperative diagnosis, intraoperative findings and management in patients S. No

Age

Sex

Side

Preoperative diagnosis

Intraoperative findings in first surgery

Investigations after first surgery

Secondary surgery needed

Case 1

7 years

M

Left

Left hydronephrosis due to UPJO with no dilatation of distal ureter on USG & nonvisualization of ureter on IVU [Figure 1]

NG (on day 7 & 3rd wk) Left ureteric showing distal ureteral reimplantation obstruction; DR [Figure 2]

Case 2

8 years

M

Left

Anderson Hynes dismembered pyeloplasty was done with inability to place Double J stent of appropriate size into the bladder followed by nephrostomy tube (Foley’s catheter) placement. -do-

Case 3

5 years

M

Right

Case 4

5 years

M

Right

Case 5

10 years

F

Left

Left hydronephrosis due to UPJO with no dilatation of distal ureter on USG & nonvisualization of ureter on IVU [Figure 3] Right hydronephrosis due -doto UPJO with nondilated ureter on USG & nonvisualization of ureter on IVU Right hydronephrosis due -doto UPJO with nondilated ureter on USG & nonvisualization of ureter on IVU Left hydronephrosis due -doto UPJO with nondilated ureter on USG & nonvisualization of ureter on IVU

NG (on day 7 & 3rd wk) showing dilated distal ureter; DR [Figure 4]

Left ureteric reimplantation

NG (on day 7 & 3rd wk) showing dilatation of distal ureter; DR

Right ureteric reimplantation

NG (on day 7 & 3rd wk) confirming distal obstruction of ureter; DR

Right ureteric reimplantation

NG showing distal obstruction of ureter done on day 7 but the dilatation resolved with dye entering into bladder when it was repeated in the 3rd week; DR

No intervention required

HUN: Hydroureteronephrosis, NG: Nephrostogram, DR: Diuretic renogram

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Figure 1: USG and MCU before pyeloplasty in case 1

Figure 2: Preoperative USG & IVU of case 2

Figure 3: Postoperative nephrostogram showing distal obstruction at left vesicoureteric junction in case 1

Figure 4: Postoperative nephrostogram showing dilated distal ureter on the left side suggesting left vesicoureteric junction obstruction

The follow-up plan in our institute is as follows: 1st visit at 2 weeks, 2nd visit after 1 month, 3rd visit after 6 weeks for DJ stent removal, 4th visit after 3 months, then at 6 monthly interval for 1st year and then yearly for at least 3 years.

3% to 25%.[3-9] In our series, the incidence is about 1.97%.

DISCUSSION The coexistence of UPJO and UVJO is so rare that even after getting all the routine investigations done in a case of hydronephrosis or a congenital megaureter it can be missed. This is the reason why a significant number of these cases were diagnosed intraoperatively or postoperatively in the few series dealing with this rare entity published in the literature. In the series by McGrath et al. only 3 of 14 patients with double obstruction were diagnosed preoperatively.[3] It presents as a unique challenge to any clinician because if diagnosed during or after the surgery the parents have to be counseled accordingly which at times is really difficult. The incidence of double obstruction (obstruction at both UPJO and UVJO) varies in different series from

Preoperative diagnosis was done in only 21.43% patients in the study by McGrath et al.[3] in 35.71% patients in the study by Cay et al.[7] in 45.45% in the study by Pesce et al.[4] but none of the patients in our study were diagnosed preoperatively. The reason for missing the dilated ureter in USG, radionuclide scans and IVU may be due to the severe obstruction at one end of the ureter causing the other obstruction to be totally overlooked or it can be missed if we do not keep the possibility of a second obstruction in mind as mentioned in the literature.[3] Obstruction at ureteropelvic junction leading to hydronephrosis is intermittent. The inability to visualize the dilated distal ureter in IVU in a case of UPJO may be due to a very negligible amount of contrast passing through the intermittently obstructing UPJ. This can lead to missing the second obstruction present in the distal ureter as it is not found to be dilated as seen

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in our series. As per McGrath et al.[3] whenever IVU in a case of hydronephrosis due to UPJO is unable to delineate the distal ureter we should go for a retrograde pyelography (RPG) or USG to rule out the presence of a distal ureteral obstruction. Though USG was done in all our cases it failed to suggest any ureteral dilatation. As RPG is not routinely done in our cases of UPJO we missed to make any preoperative diagnosis of double obstruction of ureter. Also there are chances of human error which are there with any investigation leading to a missed diagnosis. Thus, whenever in doubt about a double obstruction of ureter we should go for a RPG if the ureters can be catheterized or for a computerized tomographic urography (CT urography) if the ureters can’t be catheterized.[7] When we encounter both the obstructions together, UPJO should be corrected first as the second obstruction can get resolved spontaneously[3] as was also seen in one of our patients. We routinely put in a nephrostomy tube after pyeloplasty whenever the passage of appropriate size DJ stent into the bladder is not possible. Postoperative nephrostogram helps in confirming the distal obstruction and is also of help in following up patients where the distal obstruction is spontaneously resolved. Children with double obstruction of the ipsilateral ureter present as a diagnostic dilemma. Because of the rarity of this condition it can escape the eye of

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even an astute clinician. Early diagnosis can be made if this condition is kept in mind while treating any hydronephrosis due to UPJO or UVJO. As far as our small series is concerned retrograde pyelogram may not be recommended as an essential preoperative investigation but may be a desirable investigation.

REFERENCES 1. Zhang PL, Peters CA, Rosen S. Ureteropelvic junction obstruction: Morphological and clinical studies. PediatrNephrol 2000;14:820-6. 2. Shokeir AA, Nijman RJ. Primary megaureter: Current trends in diagnosis and treatment. BJU Int 2000;86:861-8. 3. McGrath MA, Estroff J, Lebowitz RL. The coexistence of obstruction at the ureteropelvic and ureterovesical junctions. AJR Am J Roentgenol 1987;149:403-6. 4. Pesce C, Musi L, Campobasso P, Costa L, Fabbro M. Coexisting pelviureteral and vesicoureteral junction obstruction in children. Eur J PediatrSurg 2003;13:367-71. 5. Pfister RC, Hendren WH. Primary megaureter in children and adults.Clinicaland pathophysiologic features of 150 ureters. Urology 1978;12:160-76. 6. Pitts WR Jr, Muecke EL. Congenital megaloureter: A review of 80 patients. JUrol 1974;111:468-73. 7. Cay A, Imamoglu M, Bahat E,Sarihan H. Diagnostic difficulties in children with coexisting pelvi-ureteric and vesico-ureteric junction obstruction. BJUInt 2006;98:177-82. 8. Peters CA, Mandell J, Lebowitz RL,Colodny AH, Bauer SB, Hendren WH, et al. Congenital obstructed megaureters in early infancy: Diagnosis and treatment. J Urol 1989;142:641-5;discussion 667-8. 9. Cozzi F, Madonna L, Maggi E,Piacenti S, Bonanni M, Roggini M, et al. Management of primary megaureter in infancy. J PediatrSurg 1993;28:1031-3.

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Double obstruction of ureter: A diagnostic challenge.

Isolated obstruction of the ureteropelvic junction and the vesico-ureteric junction are the two most common causes of hydronephrosis in a pediatric po...
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