Duplicated and ectopic ureter with ureterocele in an infant patient

Clinical History

A 11 month old male patient came to our hospital suffering from urinary tract infection to receive antibiotic treatment. A renal ultrasound was performed that detected a large ureterocele with renal hydronephrosis.

Imaging Findings

We present the case of an 11 month old male patient, who started 24 hours ago with urinary tract infection. He suffered from anorexia and loose stools for 4 days. He was irritable with fever up to 38°C for 4 days with good response to antipyretic treatment.

An abdominal US showed mild dilation of the right pyelocalyceal system (hydronephrosis grade II / IV), with a tortuous ureter. The entry of the ureter into the bladder showed an ovoid appearance of up to 4 cm, compatible with ureterocele (Fig 1). The content of the urine in the right pyelocalyceal system was quietly hyperechogenic, probably related to the high urine density (not shown). Left kidney was normal. Remaining bladder was normal, with no stones.

A contrast-enhanced abdominal CT showed a complete duplication of the right excretory system following the rule of Weigert-Meyer: The upper pole ureter inserted ectopically medial and caudal to the lower pole ureter in the bladder (Figure 2). The long and tortuous upper pole ureter was dilated, with a caliber up to 1 cm (Figure 3) and drained in an ureterocele up to 4 cm wide (Fig 4). Left kidney was normal.

An upper partial nephrectomy was performed and dilated ureter was ligated. Patient was discharged 48 hours later with good outcome.

Discussion

Ureterocele is a cystic dilatation of terminal ureter, where it drains into the bladder. It prevents urine from flowing freely into the bladder so it builds up in the ureter and stretches its walls, blowing it up like a balloon [1].

In 1954 Ericsson categorized it as simple or ectopic, depending on where the hole was placed in the bladder trigone or in the neck of the bladder or posterior urethra respectively [1]. Stephens classified them depending on size and location of the hole in four categories [2]. Later, Churchill classified it depending on its associated functional risk [2]. Last classification was established by the American Academy of Pediatrics as intravesical (when it drains fully into the bladder) or ectopic (when it does in the bladder neck or urethra) [2].

Ureteroceles occur in approximately 1 out of 500-4000 people. It happens more frequently in females and an ectopic ureterocele is four times more common than inside the bladder [3]. It is more frequently diagnosed in children due to prenatal US exams, and in about 75% of patients it is associated with ureteral duplication, like the case we report. In adults it is usually an incidental diagnosis by ultrasound.

The most frequent presentation is urinary infection or haematuria [2]. Common symptoms are abdominal or back pain, haematuria, dysuria, fever, spasms and pollakiuria. It is often accompanied by hypertension [4]. Stasis and urine infection may predispose to stone formation.

Ureteroceles may be problematic both in diagnosis and treatment. Its diagnosis can be obvious but sometimes it needs a high index of suspicion. As therapeutic approach varies according to symptoms and complications, it is necessary for both urologists and radiologists to be familiar with clinical and radiological presentation to address it as best as possible and avoid using unnecessary diagnostic tests [2].

US is the most frequent diagnostic exam [5]. Besides CT, cystoscopy, pyelography, radionuclide renal scan and cystourethroscopy may be performed.

In our case the abdominal US showed hydronephrosis grade II / IV secondary to a cystic intravesical mass in the posterior bladder. Commonly, hydronephrosis of the upper moiety of a duplex system is present. On the other hand, abdominal CT allowed us to demonstrate ureteral duplication and showed a dilated and tortuous upper pole ureter, with afunctional upper pole.

Several US pitfalls can be found. In bladder overdistension, ureterocele may collapse and is invisible unless ureteral dilatation. However, if bladder is empty, the dilated ureterocele may fill the entire bladder, and ureterocele may be overlooked.

Treatment depends on the functional status of affected and contralateral kidney. Therapeutic antibiotherapy is usually prescribed to prevent further infections before surgical treatment. The goal of treatment is to avoid obstruction. Drains may provide short-term relief of symptoms. Surgical repair is usually curative.

In conclusion, ureterocele is a cystic dilatation of terminal ureter, often associated to ureteral duplication. US is the most frequent initial diagnostic exam for its diagnosis. Surgical treatment is required to avoid obstruction.

Differential Diagnosis List

Final Diagnosis

Right ureteral duplication with dilatation of the upper pole ureter leading to ureterocede.

URL:	https://www.eurorad.org/case/8990
DOI:	10.1594/EURORAD/CASE.8990
ISSN:	1563-4086