|Year : 2014 | Volume
| Issue : 3 | Page : 156-161
Management of complicated ureteroceles: Different modalities of treatment and long-term outcome
Sujit K Chowdhary, Deepak K Kandpal, Anupam Sibal, Rajendra N Srivastava
Department of Pediatric Urology and Pediatric Surgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, India
|Date of Web Publication||9-Jul-2014|
Sujit K Chowdhary
Department of Pediatric Urology and Pediatric Surgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi - 110 044
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The presentation and management of ureterocele has been rarely reported from India and is limited to an odd case report. They can be detected antenatally, may have incidental diagnosis or present with consequences of obstructive uropathy. They always present with secondary complications in adulthood, if the diagnosis and treatment is missed in early years of life. The natural history may be particularly disastrous in cases of bilateral obstructing ureterocele. The complications secondary to obstructive ureterocele can be urinary retention, pyonephrosis, urosepsis, stones and even chronic renal failure. Each of these patients needs an individualized treatment plan. In the period 2003-2013, we managed 36 consecutive patients with varied presentation of this entity. All were managed on the basis of a fixed protocol of investigation and operative intervention. Ultrasound, micturating cystourethrogram, isotope renogram were done preoperatively in all the babies. Those with asymptomatic, unobstructed ureteroceles were left without any intervention. Simple or complex intravesical ureteroceles underwent examination under anesthesia and endoscopic deroofing and DJ stenting for 3 weeks. Of a total of 36 children, 6 were presented with acute complications of ureterocele. They have been managed on an individualized optimum management plan. Their management approach with follow-up is being reported as there is no previous reported series on ureterocele in children from our country.
Keywords: Children, complicated ureterocele, endoscopic deroofing
|How to cite this article:|
Chowdhary SK, Kandpal DK, Sibal A, Srivastava RN. Management of complicated ureteroceles: Different modalities of treatment and long-term outcome. J Indian Assoc Pediatr Surg 2014;19:156-61
|How to cite this URL:|
Chowdhary SK, Kandpal DK, Sibal A, Srivastava RN. Management of complicated ureteroceles: Different modalities of treatment and long-term outcome. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2020 Feb 22];19:156-61. Available from: http://www.jiaps.com/text.asp?2014/19/3/156/136471
| Introduction|| |
An ureterocele is defined as a cystic dilatation of terminal ureter with associated tissue defect in the urinary bladder, bladder neck or even extending into the posterior urethra. It is usually associated with variable degree of bladder muscle defect and renal parenchymal abnormality. The incidence of ureterocele is estimated to be 1 in 4000 live births.  The widespread availability of ultrasonography (USG) has allowed the antenatal and asymptomatic ureterocele being detected more frequently. Recurrent urinary tract infection (UTI) is the most common presentation after birth in those cases where diagnosis has been delayed.
In our country where many children remain undiagnosed, presentation with the complications of long-standing ureterocele is not uncommon. While Indian authors have reported that 30% of chronic kidney disease in children in India is secondary to obstructive uropathy that is, a preventable cause of renal failure.  Apart from the odd case report no series has been published with follow-up from our country.  Early detection of ureterocele requires a high index of suspicion, investigations carried out in a methodical approach in specialized centers, before deciding optimum approach to the management of this rare, but complicated condition.
| Case Reports|| |
A 1-year-old boy presented in the casuality with high fever, altered sensorium, pyuria and in azotemia. He had a history of recurrent UTIs. On examination, his bladder was palpable and urine obtained on catheterization was full of pus cells. Ultrasound of KUB region revealed crossed fused renal ectopia, grossly hydronephrotic left kidney with no visible renal parenchyma and dilated ureter until the lower end and mild hydronephrosis of right kidney. Initial ultrasound revealed a solid mass in the urinary bladder of 3.9 cm × 1.9 cm in the region of the left vesicoureteric junction (VUJ). The differential diagnosis considered by the radiologist and pediatrician in the referring hospital was a bladder polyp/tumor [Figure 1]. Despite initial aggressive medical treatment for a few days, the child remained sick. On our evaluation, it appeared to be an infected ureterocele with pyonephrosis.
|Figure 1: Thick walled infected ureterocele misdiagnosed as bladder polyp/tumor on USG in a 5-year-old child presenting with altered sensorium, urosepsis and azotemia (case 1)|
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The child was taken to the operating room and cystoscopic decompression was attempted, but the ureterocele wall was thick walled and inflamed leading to failure of procedure due to excessive bleeding and loss of vision. In a sick child, we decided for open exploration. Surgery revealed an infected left ureterocele, which was adequately decompressed by deroofing. Further investigations after recovery of the child confirmed a poorly functioning single system ectopic kidney on the left subtending an ureterocele, which had led to a disastrous complication.
Six weeks later, he underwent functional and drainage studies. The diethylene triamine pentaacetic acid scan revealed nonfunctioning left kidney and right hydroureteronephrosis secondary to VUJ obstruction. Right VUJ dilatation was done at the time of DJ stent removal from the left side.
The child has remained asymptomatic since that time. No further surgery or left nephrectomy has been done. The right kidney has well preserved function with some delay in drainage not warranting further investigation [Figure 2].
|Figure 2: Delayed drainage of contra lateral kidney on DTPA renal scan. Left non-functioning kidney, (case 1 at 5 year followup)|
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A full-term neonate was delivered with antenatal diagnosis of left sided hydronephrosis. The baby was born by normal delivery and the baby was not able to pass urine after birth. On investigation was found to have full bladder, left hydroureteronephrosis with a prolapsing cecoureterocele [Figure 3]. The baby was catheterized and further investigations were done. Baby was managed by endoscopic deroofing of ureterocele and DJ stenting. The cecoureterocele was incised from the distal most point in the urethra across the bladder neck and into the bladder. The catheter was removed on third postoperative day and a voiding trial was done. There was no voiding dysfunction. The lower pole grade I vesicoureteric reflux is persisting and there is development of reflux in the upper pole moiety. The child is now 3 years on follow-up and completely asymptomatic and has not required any secondary reconstruction yet.
|Figure 3: Cystoscopic appearance of prolapsing cecoureterocele below the bladder neck, presenting with acute urinary retention in a newborn, (case 2)|
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A 5-year-old boy presented with a history of recurrent UTIs and pyuria. There was history of admission in hospitals twice for treatment of UTI in early infancy. Initial ultrasound done in a primary center was reported as normal. On further investigations requested by a physician in the form of intravenous pyelogram he was found to have bilateral single system obstructing extravesical ureteroceles [Figure 4].
|Figure 4: Intravenous pyelogram, delayed 6 hour fi lm in a 5 year old boy: bilateral obstructed extravesical ureteroceles (case 3)|
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Although he had several admissions for infectious complication of ureterocele, serum urea and creatinine were not elevated. He underwent bilateral excision of ureterocele with ureteric reimplantation by robotic approach. Postoperative period was uneventful. Postoperative intravenous pyelogram showed nonobstructed drainage of both kidneys and ureters. The child is on follow-up for 1 year and is completely asymptomatic.
A 6-year-old boy was admitted with pain abdomen, high grade fever and left flank pain. He was started on intravenous antibiotics and investigations started. On investigations, he was found to have obstructing ureterocele with multiple ureteric calculi in the distal ureter. There was no response to conservative management. He underwent endoscopic deroofing, ureterorenoscopy with lithoclast, multiple stones were retrieved, ureterocele decompressed and DJ stenting done. USG done 3 months later revealed a decompressed ureterocele. He remains completely asymptomatic for the last 5 years after removal of DJ stent.
A 4-year-old boy from overseas was referred to us with the history of recurrent UTI. The child was being medically managed for neuropathic bladder and had several admissions in infancy to the hospital. At admission serum urea and creatinine were 163 and 2.7 mg/dl respectively. On detailed evaluation, he had bilateral hydroureteronephrosis secondary to right extravesical and left intravesical obstructed ureteroceles. The child underwent bilateral end ureterostomy as a trial to assess the reversibility of renal damage. Postureterostomy the child was free of any UTI, but at 6 months the renal functions have not improved and he is awaiting renal transplant.
A 1-month-old girl was referred to us with antenatal diagnosis of a cystic mass in the pelvis and vaginal atresia on antenatal ultrasound and magnetic resonance imaging (MRI). Postnatal ultrasound and other investigations done at another hospital could not confirm the diagnosis.
Then she was referred to us from another hospital. An ultrasound examination reported a cystic mass in relation to vagina and nonvisualization of right kidney. At examination under anesthesia and endoscopic examination, it became clear that she had an ureterocele opening in the vagina, which was completely occluding the vaginal inlet. A puncture and contrast study done under image intensifier in the operating room confirmed that ureterocele was subtended by a dilated ureter and contrast going in the pelvis. Endoscopic deroofing of the cyst in the vagina was done. The ureterocele was incised and walls left in situ. Functional imaging revealed a nonfunctioning right kidney. The girl is now 8 years old has occasional dampening of her under garments with a nonfunctioning asymptomatic right kidney [Figure 5]. The parents have elected to watch the situation for present and we have not gone ahead with laparoscopic nephrectomy, which will cure the child of residual dampness.
|Figure 5: Post-operative intravenous pyelogram, delayed 6 hour fi lm showing nonobstructed drainage of both kidneys. (case 3)|
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| Discussion|| |
The clinical symptoms of ureterocele in children are diverse and range from completely asymptomatic to life-threatening symptoms based on the delay in diagnosis, anatomical features related to the variety of ureterocele and presence of single or bilateral kidneys. The symptomatic children may present with urosepsis, septicaemia, stones, renal failure or voiding disturbance.
A high index of suspicion and appropriate investigations are required to diagnose the condition. The choice of management is guided by the presentation, the age of the patient, the type of ureterocele, presence of infection, stones and the function of renal moiety. In this subset of patients with ureterocele who present with complications, no single modality of treatment can be recommended for all. Each patient needs an individualized management plan and close follow-up.
There are many pitfalls in the diagnosis of ureterocele. USG is the initial screening study in children with urological symptoms. Unless the USG is done in a well hydrated, cooperative child with special focus for this anomaly, it is likely to be missed on a casual examination. This is the observation in our group of patients where in the vast majority the initial examination was merely reported as hydronephrosis or hydroureteronephrosis. Rarely, a well distended ureterocele may fill up the entire bladder and the diagnosis of a ureterocele may be missed. Occasionally, in a well distended full bladder the ureterocele may be collapsed and only a dilated ureter is seen. In only one out of these six patients, who had presented with complications of ureterocele, the complete diagnosis could be made by basic USG alone. An intravenous pyelogram, radio isotope renogram, micturating cystourethrogram and in specific cases magnetic resonance urogram are invaluable in the complete understanding of the ureterocele, associated anatomical problem in the bladder or posterior urethra and renal parenchymal anatomy and function. We were able to diagnose the problem with a combination of radiological investigations and cystoscopy in well hydrated state, in all patients before deciding on the plan of management. These pitfalls in diagnosis have been described in literature earlier, but not adequately emphasised in textbooks. 
Cystoscopy can always confirm the diagnosis in doubtful cases. Our protocol for cystoscopy includes taking the child for cystoscopy in a diuretic state under antibiotic cover with calibrated distension of bladder. Rapid distension of bladder may prevent the ureterocele from distending and make it elusive. Forced diuresis with saline bolus and injection frusemide at the time of induction makes the ureterocele prominent and easily identifiable. After identification of the ureterocele in filling state it was punctured by a Deflux needle for retrograde contrast study in relevant cases. Such a study has never failed us in delineating the upper pole ureter and kidney where features of renal duplication were not demonstrated by preoperative imaging.
In general, the traditional aggressive approach in the management of ureterocele has changed to more conservative approach in the last few decades. , Asymptomatic ureteroceles discovered on incidental ultrasound with normal drainage across shown on isotope renogram can be left on observation. The traditional approach involved a two stage heminephrectomy followed by excision of ureterocele and ureteric reimplantation in an infant with poorly functioning upper pole. In those with good function, the approach used to be excision of ureterocele, repair of bladder defect and ureteric reimplantation.  This approach was followed by various combination of endoscopic approach followed by upper or lower surgery either by open surgery or even robotic in this day and age.
The intravesical ureterocele which is obstructed, but without any other secondary complications are ideally managed by endoscopic deroofing and long-term follow-up. This has revealed excellent result in the vast majority without secondary reconstruction. It is the management of the ectopic ureterocele, which is controversial with some authors recommending ultramajor reconstruction in neonatal and early infancy and others recommending initial endoscopic incision or puncture.  Endoscopic incision is the least invasive approach and can successfully decompress the ureterocele and prevent progressive bladder outlet obstruction in about 90% of patients of the simple intravesical ureterocele. 
The above mentioned principals of endoscopic and minimally invasive surgery can be applied to complicated ureteroceles as well. Our aim in treating the complicated ureteroceles was to do a complete decompression and relieve the obstruction, eliminate any potential source of infection and stabilize the renal function with minimal surgical morbidity. This approach allowed the definitive surgery to be delayed to a later date, if required. Three out of six children were managed by endoscopic approach, one by minimally invasive robotic approach and two needed open surgery. The two children out of six, who had open surgery, were in severe urosepsis with thick walled inflamed ureterocele in a sick child and another was in renal failure secondary to repeated urosepsis in a child with bilateral obstructing ureteroceles [Table 1].
|Table 1: Description of complicated ureterocele managed between 2007 and 2014|
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The use of DJ stent and advantages or disadvantages of stenting have not been addressed in the literature. All of our children who underwent endoscopic treatment had DJ stents in place. The DJ stent was left in place for 3-4 weeks. The stenting protects the lower pole ureter and allows the healing after deroofing of the ureterocele to happen around the stent. In intravesical ureterocele the ureteric orifice after deroofing and stent removal 4 weeks later may appear nearly normal after wide deroofing [Figure 6].
|Figure 6: DMSA renal scan: non-functioning asymptomatic right kidney (case 6, at 8 year followup)|
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All six children are on regular follow-up and the follow-up period ranges from 1 to 8 years with a median of 4 years. None of these children have required any additional surgery during the follow-up. All the children who have undergone treatment have not been investigated for presence or absence of vesicoureteric reflux as has been discussed in literature. Our assumption has been that all of them do develop a vesicoureteric reflux after adequate deroofing, but the need for secondary reconstruction will be guided by presence or absence of symptoms rather than the grade of reflux. None of the patients had any voiding dysfunction including the newborn with cecoureterocele who presented with bladder outlet obstruction and urinary retention. This approach has been discussed in literature earlier by Cooper et al. in an editorial review. 
Similarly, the nonfunctioning kidney or nonfunctioning upper pole was not considered as an indication for surgery. The case no 1 who underwent open deroofing has a nonfunctioning ectopic kidney and delayed drainage on the contra lateral side. The child is asymptomatic and is on yearly follow-up with ultrasound examination and blood pressure monitoring. Similarly, in the case no 6 after deroofing of ectopic vaginal ureterocele, there is nonfunctioning kidney, which hasn't required any intervention. Therefore a nonfunctioning kidney or upper moiety by itself does not become an indication for nephrectomy or polar nephrectomy unless secondary complications have set in.  The majority would support the contrary view of secondary surgery in nonfunctioning moiety.
| Conclusion|| |
Ureterocele presenting with complications need an innovative and individualized treatment plan. A screening ultrasound may miss the elusive entity unless specifically searched. Any baby with upper tract dilatation in absence of vesicoureteric reflux warrants a detailed evaluation of the urinary bladder for the obstructing ureterocele. A methodical approach including intravenous pyelogram, isotope renogram, MRI with cystoscopy with image intensifier in the operating room will allow the optimum approach of treatment. Even in patients with initial presentation with complication of ureterocele, approximately 50% can be managed entirely by endoscopic treatment and now an additional significant majority can be dealt with the robotic approach. Long-term follow-up will be required in all these patients to know their natural history after appropriate treatment and relief of obstruction.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]