Calcified ureterocoele causing renal tract obstruction in an adult

The ureterocele is an intravesical cystic dilatation of the terminal ureter, often due to a congenital weakness in the lower ureteric wall.  Adult orthotopic ureterocoeles, although more common in women, remain a rare clinical entity. We believe this is the first report of a calcified ureterocoele, containing no calculi, and causing significant renal tract obstruction in an adult. We report the case of an elderly Caucasian female patient, presenting with a calcified, right sided, simple orthotopic ureterocoele, mimicking a right vesicoureteric junction (VUJ) calculus.

An 84-year old female presented to the emergency department with a short history of worsening right-sided abdominal pain. Cognitive function was well preserved despite significant medical comorbidities, including a previous cerebrovascular accident with residual right hemiplegia, atrial fibrillation (for which she was taking warfarin), giant cell arteritis, ischaemic heart disease and congestive cardiac failure. On general examination, the patient appeared cushingoid and was drowsy, confused, tachycardic and hypotensive. Abdominal examination revealed tenderness in the right loin and right iliac fossa, with no evidence of peritonism. Blood tests revealed acute renal failure (Ur 14.2, Cr 145), raised inflammatory markers (CRP >250) and a raised serum lactate (6.7). The patient was also in fast atrial fibrillation, with rate-related ischaemia evident on ECG.

The patient responded well to appropriate fluid resuscitation and intravenous antibiotics. Ultrasound of the renal tract reported right hydronephrosis, with a calculus at the right vesicoureteric junction. Subsequent CT scan of the abdomen & pelvis reported a calculus at the right VUJ and moderate right hydronephrosis and hydroureter (Figure 1a/1b).

Although no calculus was identifiable on limited IVU, hydronephrosis and hydroureter in a single collecting system with obstruction at the level of the right VUJ was reported. The ‘cobra head’ sign pathognomic of a ureterocele was not evident.

Once clinically stable and fit for general anaesthetic, retrograde ureteric stenting was arranged. During the procedure, a ureterocoele was noted at the right ureteric orifice. The site and appearance of the left ureteric orifice was cystoscopically normal. Transurethral de-roofing of the right ureterocoele was performed. No calculus was present in the ureterocele, or at the vesicoureteric junction. A ureteric stent was inserted without complication, and the patient recovered well from the procedure. Renal function normalised (Creatinine 79, Urea 7) and the patient was discharged three days after the procedure. The stent was removed without incident 4 weeks post-procedure, and the patient remains well.

The ureterocoele is a congenital abnormality, commonly now identified with antenatal screening. Ureterocoeles may be either intravesical or ectopic in location, and there is a great clinical spectrum of symptoms and functional consequences. The precise aetiology of ureteroceles is uncertain – it is most widely accepted that obstruction of the ureteral orifice during embryogenesis, with incomplete dissolution of the Chwalla membrane, predisposes to ureterocoele formation. Occasionally, ureterocoeles are discovered in adulthood – these are typically intravesical, and associated with a single collecting system[1]. Presentation may include flank/back pain, recurrent urinary tract infections, urosepsis, voiding difficulty or loin to groin pain. In addition, they may be detected incidentally at endoscopy, or radiologically[1-5].  Stones are known to form in ureterocoeles, thought to be secondary to ureteral atony and urinary stasis – it is reported that stone formation in ureterocoeles in more common in males than females [1,5].

In our patient, the decision for retrograde stenting instead of nephrostomy and antegrade stenting was based on availability of resources at the time for urgent intervention.  If antegrade stenting were performed, the ureterocoele may not have been identified and treated, as was the outcome in our patient. Endoscopic management is the gold standard therapy for management for symptomatic adult ureterocoele [2,6].  Timely diagnosis and endoscopic de-roofing of obstructing ureterocoeles may avert recurrent presentations with infection and obstruction, and thereby significantly reduce subsequent morbidity. This is especially important in patients such as ours, with multiple other co-morbidities, which may be exacerbated by episodes of sepsis.

Adult ureterocoele, although an uncommon clinical entity, should be considered in the differential diagnoses of patients presenting with urinary symptoms. Cystoscopic examination in those presenting with refractory and/or severe urinary symptoms may allow correct and timely recognition and management of previously unidentified ureterocoeles.

We believe this is the first reported case of a ureterocoele mimicking a ureteric calculs. Our patient was admitted acutely unwell, with urinary sepsis and radiological findings suggestive of obstructing ureteric stone. The cystoscopic finding of a calcified ureterocoele, with no obstructing calculus, was unexpected. After definitive management of the ureterocoele, the patient recovered rapidly, and remains well.

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