Encrusted Cystitis: A Rare Morbid Disease

ABSTRACT

Encrusted cystitis (EC) is a rare clinical entity, with the first description dating back to 1914 by Francois J. This variety of debilitating and rare cystitis is caused by urea-splitting Corynebacterium urealyticum, especially Corynebacterium type 2B2 (CBGD2). Patients with prior urinary trauma, a history of intravesical bacillus Calmette–Guérin instillation, or chemotherapy are at higher risk for developing EC. Clinical presentation varies from dysuria, pain, hematuria, or passage of mucus or calcified mucopurulent stones. Diagnosis of Encrusted cystitis is done after excluding the other pathologies and is confirmed on isolation of Corynebacterium Type 2B2 organism and its characteristic features on radiotherapy. Medical management involves glycopeptide antibiotics and acidification of urine, either topical or systemic. Surgical treatment is done in rare cases with failed medical management or spontaneous bladder rupture. We report a rare case of morbid EC who required cystectomy and diversion.

INTRODUCTION

Encrusted cystitis (EC) is a rare inflammatory bladder condition characterized by the formation of calcified plaques on the bladder mucosa, secondary to the chronic infection caused by urea-splitting bacteria, such as Corynebacterium urealyticum.[1] These are slow-growing Gram-positive organisms and produce urease, which hydrolyzes urea into ammonia, which in turn raises the urine pH and promotes the formation of calcium and magnesium precipitation as struvite and apatite crystals. This leads to encrustations on the bladder wall and the formation of microlithiasis. This organism is usually found as a skin commensal in almost 12% of healthy individuals and is often resistant to a wide range of antibiotics.[2,3] Other organisms that have been associated with this condition are Proteus, Klebsiella, and Pseudomonas. This condition is managed effectively with antibiotics such as Glycopeptides and acidification of urine with the use of Thomas solution (27 g each of sodium gluconate, citric acid, and malic acid, in 1 L of distilled water) or Suby solution (32.3 g citric acid, 4.4 g sodium carbonate, 3.8 g magnesium oxide, and 1 L distilled water).[3] EC may or may not coexist with encrusted pyelitis, so it is essential to evaluate the renal pelvis for concomitant problems.

We report a rare case of EC with recurrent retention of urine following lithuria that crippled the patient. Although treated with culture-sensitive antibiotics, she did not respond to the same and was then subjected to cystectomy and diversion.

CASE REPORT

A 34-year-old female presented to our outpatient department with a history of pain in the lower abdomen, increased urinary frequency, and lithuria with frequent urinary retention due to stones. Her urine examination showed acidic urine with a positive culture (Escherichia coli and Enterococcus faecalis) sensitive to aminoglycosides, benzylpenicillin, and nitrofurantoin. Culture-sensitive antibiotic treatment was instituted with no improvement in the patient’s symptoms.

On ultrasound, it showed bladder wall calcification as well as multiple bladder calculi with normal kidneys. The patient was subjected to a computed tomography (CT) scan, which showed diffuse, extensive, irregular calcification and thickening of the bladder wall with calcific intraluminal extensions with reduced bladder capacity [Figure 1].

Figure 1.

Computed tomography scan image showing intense mural calcification of bladder and calcified intraluminal stones

The patient was then evaluated for tuberculosis and schistosomiasis, which was also negative. Over time, the patient developed multiple episodes of urinary retention needing catheterization with recurrent catheter blockage secondary to crystalluria. On cystoscopy, the whole of the bladder mucosa was bluish in color with calcific plaques all over the bladder walls. A biopsy of the bladder wall was taken under anesthesia, which revealed necrotic tissue with nonspecific cystitis.

After exhausting all options at conservative treatment, a decision was taken to do cystectomy and urinary diversion (ileal conduit). The cystectomy specimen revealed thick calcification extending into the muscle [Figure 2].

Figure 2.

Resected cystectomy cut specimen showing small capacity rigid calcified bladder with intraluminal calcified material

Histopathology of the resected specimen revealed ulceration and denudation of urothelium with extensive infiltration of lamina propria by inflammatory cell infiltrates rich in plasma cells, histiocytes, and eosinophils, as well as calcified necrotic debris permeating through the muscle planes and surrounding nerve bundles and muscle fibers [Figures 3 and 4]. Postcystectomy, 3 months of follow-up, the patient was doing well.

Figure 3.

Photomicrograph showing large calcific deposits superficially covering the bladder wall as well as infiltrating it (×100)

Figure 4.

Photomicrograph showing intense lymphoplasmacytic infiltrate admixed with eosinophils and histiocytes present throughout the wall (×400)

DISCUSSION

Fortunately, very few cases of EC have been reported in the literature, with the first case dating back to 1914 by Françoise.[3,4] Encrusted cystitis is such a morbid disease that Françoise commented on the disease as nothing short of misery, he quoted, “EC may not be a death sentence, but it is a torture for life.”

Predisposing conditions for EC include immunocompromised patients, patients with prior urinary trauma, neurogenic bladders, history of bacillus Calmette–Guérin installation or intravesical chemotherapy, or patients with long-term catheterization.[5,6] Clinical presentations are often variable – ranging from urinary symptoms like dysuria, pain, and hematuria to intermittent urinary obstruction secondary to recurrent passage of mucus or calcified mucopurulent stones.[6] Our patient developed persistent lithuria and that was associated with recurrent episodes of retention of urine. Moreover, every episode of retention needed catheterisation which got blocked frequently by the crystals, thus increasing the agony for the patient.

Diagnosis of EC is often missed, and it is diagnosed often late until the sequel of this disease causes significant morbidity. Exclusion of common conditions, isolation of Corynebacterium, and radiology can facilitate reaching the diagnosis. Often, a CT scan clinches the diagnosis with intense intramural calcification.[7] In our case, we did an ultrasound to start with, which showed multiple calcifications within the bladder with a normal upper tract. However, the definitive diagnosis was clinched on contrast-enhanced CT, which showed bladder wall mural calcification with a rigid bladder.

Treatment of EC with a positive culture of corynebacterium is initially done with glycopeptide antibiotics such as vancomycin and teicoplanin, and acidification of urine, topical and systemic.[8] However, isolation of the causative organism, i.e., C. urealyticum, is difficult at times and possible in only 15.6% of cases.[6] Surgical treatment is done in rare cases with failed medical management or spontaneous bladder rupture due to sepsis.[9]

Our case was presented with recurrent urinary tract infection and was treated with a wide range of culture-sensitive (E. coli and E. faecalis) antibiotics. We did not isolate C. urealyticum in any of the cultures. With time, the patient started with recurrent lithuria with multiple episodes of urinary retention for which nothing was helping, as the catheter used to get blocked by the debris. Hence, she was evaluated, and the options were explained. She was then subjected to cystectomy and ileal conduit.

To conclude, EC is a rare disorder, and a high degree of suspicion should be there to diagnose this debilitating disease. Other disorders such as tuberculosis or malignancy should be excluded with relevant investigations. Early treatment with glycopeptide antibiotics such as vancomycin and teicoplanin with acidification of the urine may help control the condition. However, in the case of advanced disease with a small-capacity calcified bladder not responding to conservative treatment, surgical options should be discussed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.