Ureterocele as a cause of chronic intractable abdominal pain

Abstract

A ureterocele is a submucosal, cystic dilation of the terminal ureter, either congenital or acquired, as it enters the bladder. It is a rare clinical entity that can be entirely asymptomatic and present as an incidental finding or can manifest in the form of distressing symptoms such as unremitting abdominal pain, haematuria, obstructive uropathy, to name a few. The authors present a case of abdominal pain in a 43-year-old woman who was presumptively attributed to various clinical entities and was finally referred to the chronic pain clinic. The patient underwent numerous diagnostic tests, psychological evaluations and therapeutic interventions, including surgeries, over the years that failed to mitigate her symptoms until urologic imaging reported intravesical ureterocele as the underlying disorder. The case report entails the diagnostic challenge faced by the authors along with the clinical characteristics of ureterocele.

Background

A physician usually relies on relevant history, physical examination and imaging studies to formulate a diagnosis or effectuate a treatment algorithm. However, physicians should remember that the tools used for diagnosis have inherent statistical limitations for a particular disease. Furthermore, several pathological entities mimic each other, confounding the clinician’s judgement and exposing the patient to a multitude of diagnostic and therapeutic interventions. Physicians should be vigilant against assigning a specific diagnosis to vague symptomatology and should always be keen to take a step back and reevaluate the clinical scenario, especially if the initial diagnostic or therapeutic intervention fails to yield the desired results. Abdominal pain is an example of such a symptom with an exhausting list of differential diagnoses and is a common report among women of childbearing age. While usually asymptomatic, ureteroceles can have a wide clinical spectrum and should be a part of a broad differential for abdominal pain. Ureterocele—a submucosal, cystic dilation of the terminal ureter—is a rare clinical entity, with an incidence ranging from 1 in 500 (on autopsy) to 1 in 4000 in the general population.¹ Predominantly unilateral, with equal frequency on the right and left, the ureterocele is reportedly bilateral in 10% of cases.² The incidence of ureterocele is reported to be 4–5 times more common in women than in men and primarily in the Caucasian race.³ The case report entails the diagnostic challenge faced by the authors along with the clinical characteristics of ureterocele.

Case presentation

The 43-year-old patient presented to the chronic pain clinic to evaluate right lower quadrant abdominal pain and to specifically undergo an ilioinguinal nerve block. The patient reported that the pain started insidiously without an inciting event 4 years ago, and the symptoms have been persistent. The pain is intermittent, non-radiating and described as cramping, sharp and piercing in nature. The patient reported the pain waxes and wanes in intensity and ranges from 3 to 6/10 on the numeric rating scale (NRS) for pain. The pain was exacerbated by an increase in physical activity and was mitigated by medications. The pain interfering with the patient’s sleep, and the patient frequently developed nausea and vomiting due to severe pain that she described as colicky. The patient reported no diurnal variation to pain and also commented that the pain is not associated with urination, bowel movements, sexual intercourse or menses. The patient further reported that she has 6–8 episodes of increased pain intensity per year that is associated with dysuria. The patient’s medical history was positive for class I obesity with a body mass index of 32 Kg/m, haematuria, recurrent urinary tract infections, renal calculi, depression and anxiety attributed to chronic pain. The patient was married and was a homemaker, with no significant stressors in her life besides chronic abdominal pain. The patient reported that she has developed depression and anxiety due to her symptoms and is currently undergoing cognitive behavioural therapy and psychological counselling for chronic pain. The analgesic regimen consisted of Tramadol 50 mg every 8 hours as needed for pain. The patient was on desvenlafaxine extended—release 150 mg/day and lorazepam 1 mg nightly for anxiety and depression. Failed medical treatments included non-steroidal anti-inflammatory drugs, amitriptyline and gabapentin. The focused examination of the abdomen reported intact surgical scars with pain in the right lower quadrant to deep palpation and a negative Carnett’s sign.

Investigations

During one such episode of increased pain with associated dysuria 2 months previously, the patient underwent a urological assessment. followed by kidney–urinary bladder X rays, ultrasound of the abdomen and CT of the abdomen ruled out acute or chronic pathology, including hydronephrosis and nephrolithiasis. Furthermore, the intravenous pyelography reported no abnormalities with the kidneys, pelvicalyceal system and bladder.

For chronic abdominal pain, the patient underwent cholecystectomy, appendectomy and laparoscopic exploratory surgery to rule out endometriosis. The patient was initially diagnosed with ilioinguinal–iliohypogastric neuralgia based on the distribution of her pain. The patient underwent ultrasound-guided diagnostic ilioinguinal–iliohypogastric nerve blocks on the right side, with 10 mL of ropivacaine 0.2% and 40 mg of triamcinolone, which failed to mitigate her symptoms.

Differential diagnosis

During the follow-up visit, the patient reported developing episodes of dysuria and an increase in the intensity of abdominal pain to 6–8/10 on NRS for pain. The new information provided by the patient and the anecdotal evidence of no response to nerve blocks raised the suspicion that the pain could be urological in origin.

Outcome and follow-up

The patient was advised to follow-up again with the urologist to discern the aetiology further. The pain physician also communicated his rationale with the primary care physician of the patient. Subsequently, the patient underwent MRI of the abdomen and pelvis with contrast that reported the right urinary bladder intravesical ureterocele (figures 1 and 2). The patient underwent a urological endoscopic stenting procedure for obstructive ureterocele followed by transuretheral puncture surgery, shortly thereafter (2 months postdiagnosis). The patient reported gradual resolution of her symptoms. At 6 months follow-up, the patient was satisfied with her outcome, reported complete resolution of her symptoms and was weaned off chronic opioid therapy.

Figure 1.

Cobra head deformity at ureterovesical junction on using gadolinium contrast. Axial T2 MRI image.

Figure 2.

Cobra head deformity at ureterovesical junction on using gadolinium contrast. Coronal T2 MRI image.

Discussion

A ureterocele usually occurs in the lower part of the ureter, where the ureter dilates before opening into the bladder, leading to mechanical or dynamic obstruction. Based on aetiology, the ureterocele is classified as congenital or acquired. The aetiology of congenital ureterocele is hypothesised to be obstruction of the ureteral orifice during embryogenesis, with the incomplete dissolution of the Chwalla membrane, predisposing it to ureterocele formation. The acquired ureterocele is considered to be a sequela of distal obstruction, such as a calculus, which can lead to an increase in intramural pressure and mucosal herniation of the involved ureter. A ureterocele is classified topographically as intravesical or ectopic (the ureterocele extends beyond the trigone, into the urethra). The intravesical ureterocele has a better prognosis than the extravesical ureterocele in terms of better preservation of renal function and less incidence of bladder outlet obstruction.^1–4 The intravesical ureterocele are amenable to minimally invasive treatment options such as transuretheral puncture and transuretheral incision.^{5 6}

Ureteroceles are usually asymptomatic but can have a wide range of the clinical spectrum. Patients may report a history of frequent urinary tract infections, urosepsis, urinary obstruction or retention, haematuria, purulent urine, pyelonephritis and abdominal pain. The most common presentation, accounting for 15%–40% cases, is an accompanying ureteric calculus.⁴ The mechanical obstruction to urine flow due to ureterocele may involve dynamic impediment to urine flow through the ureter, leading to infrequent voiding, increased bladder size and vesicoureteric reflux. The disruption to the normal urodynamics may predispose the patient to stone formation, recurrent obstructive or infective pyelonephritis and repeated expansion and decompression of renal pelvis and capsule. These anomalies may manifest as abdominal, groin or flank pain that could be episodic in nature. Furthermore, frequent urinary obstruction due to ureterocele, if left unchecked, may lead to hydronephrosis, which may manifest as signs of abdominal tenderness to palpation. The patient, in this case, developed recurrent episodes of urinary tract infection or pyelonephritis, leading to unremitting, cyclical abdominal pain, refractory to medical and surgical interventions. The patient was diagnosed with appendicitis, acute and chronic cholecystitis, endometriosis and functional abdominal pain syndrome and underwent multiple surgeries, endoscopic procedures and gynaecological examinations along with psychological evaluations over a period of 4 years before a definitive diagnosis of ureterocele was made.

The ultrasonography (US) of the kidney and bladder is usually the first investigation for ureterocele, and ureterocele is reported as a cyst within cyst on an examination. However, an empty or fully distended bladder can compress on the ureterocele, making it inapparent during the US examination. Furthermore, a small ureterocele may be missed on US or patient’s body habitus (eg, high body mass index) may preclude an optimum US examination. Contrast-enhanced functional studies of renal systems, like intravenous urogram (IVU), voiding urethrocystography and CT scan, have dual benefits of delineating the relevant renal anatomy, defining the size, shape and location of the ureterocele and evaluating the differential renal function. However, the ureterocele, depending on the degree of filling or obstruction, has a variable size and shape and may not be apparent in all phases of the IVU and voiding urethrocystography. Furthermore, in cases of poor or failing renal function and the limited ability of the kidney to excrete the contrast, IVU and CT scan are further limited as a modality to diagnose ureterocele. The CT reports ureterocele as a thin, non-enhancing rim around contrast enhancement within the bladder. However, it is not uncommon to miss ureterocele on a CT scan if it is small or compressed. The patient’s history revealed that the US, IVU or CT scan failed to report the right-sided ureterocele, which could be due to small size or compression of the ureterocele on the examination. The MRI reportedly enables an accurate analysis of the renal and urinary tract, even in the absence of dilatation, promoting a more sensitive and more specific diagnosis of the ureterocele. Moreover, MRI does not expose the patient to harmful radiation and can be employed in patients with declining renal function. The MRI with paramagnetic contrast agent (gadolinium-based contrast agent) offers a better contrast in the image and a higher sensitivity than conventional MRI.^{2 4} The ureterocele, in this report, was apparent on MRI with a gadolinium-based contrast agent (Gadoterate Meglumine).

In conclusion, this case underscores the need for proper evaluation and follow-up of patients with chronic abdominal pain. The pathognomonic signs and symptoms of ureterocele are vague and share a multitude of aetiologies, making it a diagnostic challenge. In our case, there was a delay of 4 years from the onset of symptoms to the definitive management, during which the patient underwent futile procedures, leading to a great deal of stress and psychological trauma. A multidisciplinary collaboration between chronic pain, primary care physician and urologist was required to identify, diagnose and treat the rare diagnosis of ureterocele. The physician should be aware that in a patient with a history of chronic abdominal pain associated with recurrent urological morbidities, ureterocele presents itself as a viable aetiological factor. Early detection and treatment of ureterocele may also prevent irreversible renal injury or decline in renal function.

Learning points.

• Signs and symptoms of ureterocele can be vague and share a multitude of aetiologies, making it a diagnostic challenge.

• Patients with a history of chronic abdominal pain associated with recurrent urological morbidities should have ureterocele as part of the differential diagnosis.

• Early detection and treatment of ureterocele may also prevent irreversible renal injury or decline in renal function.

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