Xanthogranulomatous Cystitis Presenting as Urinary Incontinence and a Vesicovaginal Fistula: a Case Report and Review of the Literature

doi:10.4236/oju.2011.14018

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Open Journal of Urology, 2011, 1, 86-88

doi:10.4236/oju.2011.14018 Published Online November 2011 (http://www.SciRP.org/journal/oju)

Xanthogranulomatous Cystitis Presenting as Urinary

Incontinence and a Vesicovaginal Fistula:

A Case Report and Review of the Literature

Shivon N. Abdullah, Adamantios M. Mellis, Corinne L. Puzio, Daniel J. Culkin*

Department of Urology, University of Oklah om a Health Sciences Cent er , Okl a ho m a Ci t y, USA

E-mail: *[email protected]

Received June 7, 2011; revised July 15, 2011; ac cepted Ju ly 28, 2011

Abstract

Anthogranulomatous cystitis (XC) is an inflammatory condition of the urinary bladder that is benign and

rarely seen. XC is a unique disease in which there have been only 26 cases described in the literature, in-

cluding our case. Patients with XC often present with lower urinary tract symptoms, hematuria, abdominal

pain, or abdominal mass. We present the unusual case of an 81 year old female who presents with urinary

incontinence, which was later diagnosed as a vesicovaginal fistula. After describing the treatment of this pa-

tient, a review of the literature is detailed including presentation, possible etiologies, and treatment of XC.

Keywords: Xanthogranulomatous Cystitis, Inflammation, Bladder, Vesicovaginal Fistula, Urinary

Incontinence

1. Introduction

Xanthogranulomatous cystitis is an uncommon but be-

nign inflammatory condition of the bladder that is chronic

in duration for which the etiology is unknown [1]. XC

was first described by Ota in 1933[2]. Histologically, the

disease is characterized by multinucleated giant cells and

chronic inflammation. The characteristic cell is the

lipid-laden macrophages, known as “foamy histiocytes,”

which stain strongly for CD 68. Although the kidney is

the most common site of xanthogranulomatous disease,

there are currently twenty-five previously described

cases of this disease reported in the bladder [3]. Most of

these cases involve urachal remnants with disease most

often isolated to the dome of the bladder [4]. We add our

case of XC in an 81 year old female with the unusual

initial presentation of urinary incontinence and vesico-

vaginal fistula (VVF) and provide a brief review of the

literature

2. Case Report

An 81 year old Caucasian female of German descent was

referred to clinic with primary complaint of continuous

urinary incontinence which had persisted for several

months and believed to be secondary to stress urinary

incontinence. The patient also had symptoms of urinary

frequency and intermittent gross hematuria. However,

the patient denied having any urinary tract infections.

She also reported a significant unintentional weight loss

in the past year. Past medical history included hyperten-

sion, diabetes mellitus type II, as well as dyslipidemia.

On physical examination, the patient was found to

have a large tumor involving the anterior vaginal wall

with pooling of urine in the vaginal vault, suspicious for

a vesicovaginal fistula. Laboratory analysis revealed nor-

mal renal function, absen ce of hematolo gic abnormalities,

including eosinophilia. Urinalysis and urine culture fai-

led to show to urinary tract infection. Con trast computed

tomography with urogram phase revealed a midline pos-

terior bladder wall lesion and a 4 mm communication

between the posterior bladder wall and the anterior vagi-

nal wall (Figures 1 and 2).

Cystoscopy was performed identifying significant

amount of bullous edema as well as a bulky solid lobu-

lated mass approximately 2 cm in size in the area of the

left posterior bladder neck extending to the trigone of the

bladder. A midline vesicovaginal fistula was identified in

the posterior bladder wall. The lateral walls of the blad-

der, as well as the dome of the bladder were free of

masses or inflammation. Due to concern for malignancy,

endoscopic biopsy and resection were performed of the

S. N. ABDULLAH ET AL.

Figure 1. Contrast CT of the pelvis demonstrates a discer-

nable mass in the area of the bladder trigone extending into

the anterior vaginal wall.

Figure 2. Delayed phase contrast CT of the pelvis demon-

strates vesicovaginal fistula with presence of contrast within

the vaginal vault.

bladder neck lesion. Vaginoscopy was also performed

with biopsies of the anterior vaginal wall and fistulous

tract also obtained. Histologic analysis revealed numer-

ous foamy histiocytes. Immunohistochemical staining de-

monstrated positive staining for CD68 (Figure 3) a kno-

wn marker fo r macrophages.

The patient was treated conservatively with oral anti-

biotics for five months with resulting decrease in in-

flammation; however, urinary incontinence persisted. As

a result, the patient elected to undergo vesicovaginal fis-

tula repair. Intraoperatively, attempted vaginal vesico-

vaginal fistula repair was halted due to extensive fibrosis

at the bladder neck and trigone of the bladder. Then an

abdominal approach to VVF repair was approached.

However, the right ureteral orifice was unable to be can-

nulated, and, on further exploration, the patient was

found to have right hydroureteronephrosis. Furthermore,

extensive fibrosis was present in the pelvis, the bladder

neck and trigone, making vaginal and suprapubic VVF

repair not possible.

Figure 3. CD68 immunohistochemical staining demonstra-

ting numerous characteristic “foamy histiocytes” and mul-

tinucleated giant cells in the submucosa of the bladder

(CD68 immunostain ×400).

With those surgical findings and taking the patient’s

primary complaint of incontinence into consideration, the

decision was made that the patient would best benefit

from a urinary diversion procedure. An ileal conduit uri-

nary diversion was performed. The patient was dis-

charged after five days in the hospital, and now, more

than 3 years later, she is asymptomatic and is pleased

with her continence.

3. Discussion

Xanthogranulomatous cystitis is a rare disease entity and

only 26 cases have been reported and described, include-

ing this case. XC’s etiology is unknown. Some authors

have proposed chronic irritation and inflammation of a

urachal remnant [2], autoimmune etiology as demon-

strated by eosinophilia [5], as well as chronic urinary

tract infections and anaerobic infections [6]. Regardless

of the true etiology, the ag es of patients afflicted with the

disease range from 16-80 years of age, and include both

males and females in equal distribution [4]. Patients

typically present with lower urinary tract symptoms 60%

(15/25) of the time and symptoms include frequency,

urgency, as well as nocturia. Hematuria (microscopic or

gross) is present in ab out a quarter of the patients (6 /25),

whereas abdominal or pelvic pain is present in 40%

(12/25) of patients. Moreover, an abdominal or pelvic

mass is identified in 40% (1 2/25) of patien ts [3-9]. Table

1 provides a summary of these statistics.

When the location of the disease was provided in the

case reports, the majority of cases involved the bladder

dome and was associated with an urachal remnant or

anomaly: 80% (16/20) and 56% (14/25), respectively.

S. N. ABDULLAH ET AL.

Table 1. Symptoms seen in patients affected by xanthogra-

nulomatous cystitis [4,5].

Symptoms Percentage (%)

LUTS 60 (15/25)

Hematuria (gr oss a nd microscopic) 24 (6/25)

Pain (abdominal and pelvic) 40 (12/25)

Mass (abdominal and pelvic) 40 (12/25)

Urachal Remnant Involved 56 (14/25)

Other areas reported to be involved include the posterior

wall (1 case), lateral walls (2 cases), and the anterior wall

(2 cases).

The treatment modality most often employed in cases

of XC is partial cystectomy, as is the case in 18 out of 25

cases reported (72%). Other XC cases have been suc-

cessfully managed with surgical or endoscopic resection

and recurrences have never been reported, even 15 years

after initial resection [1]. Other treatment modalities that

have also been curative are long-course antibiotics and

radical cystectomy [8-9].

Our case is rather unique in that our patient is the old-

est patient (81 years old) reported to be diagnosed with

XC. Furthermore, the patient possesses a unique presen-

tation of XC. This is the first case of XC that has pre-

sented as urinary incontinence, which was diagnosed as a

vesicovaginal fistula. Furthermore, our patient is the first

patient where the disease has involved the bladder

trigone and the bladder neck. Management of this patient

is also distinctive, given her unusual case. The patient’s

XC was primarily treated with long term antibiotics,

while her secondary problem was treated with urinary

diversion with both modalities resulting in successful

treatment of a complicated disease process. As in other

cases, our patient has remained asymptomatic. Although

resection was not undertaken, chronic antibiotic treat-

ment seemed to have decreased the inflammation caused

by the disease, supporting the case for possible bacterial

infection as a contributing etiology [6].

In conclusion, xanthogranulomatous cystitis is a rare

disease that manifests in a variety of ways, including in

our case, as chronic bladder inflammation leading to de-

velopment of a vesicovaginal fistula.

4. References

[1] L. B. Tan, C. P. Chiang, C. H. Huang and C.-H. Chian,

“Xanthogranulomatous Cystitis: A Case Report and Re-

view of the Literature,” International Urology Nephrol-

ogy and Nephrology, Vol. 26, No. 4, 1994, pp. 413-417.

doi:10.1007/BF02768011

[2] N. Ota, “Urachal Tumor of Inflammatory Origin,” Gran-

gigebie, Vol. 7, 1933, p. 1205.

[3] D. E. Chung, L. K. Carr, L. Sugar, M. Hladunewich and

L. A. Deane, “Xanthogranulomatous Cystitis Associated

with Inflammatory Bowel Disease,” Canadian Urologi-

cal Association Journal, Vol. 4, No. 4, 2010, pp. E91-

E93.

[4] A. Fornari, M. Dambros, C. Teloken, A. A. Hartmann, J.

Kolling and R. Seben, “A Case of Xanthogranulomatous

Cystitis,” International Urogynecology Journal, Vol. 18,

No. 10, 2007, pp. 1233-1235.

doi:10.1007/s00192-007-0345-5

[5] M. K. Chung, M. Y. Seol, W. Y. Cho, H. K. Seo and J. S.

Kim, “Xanthogranulomatous Cystitis Associated with

Suture Material,” The Journal of Urology, Vol. 159, No.

3, 1998, pp. 981-982.

doi:10.1016/S0022-5347(01)63794-7

[6] H. L. Tai, C. C. Chen and K. T. Yeh, “Xanthogranulo-

matous Cystitis Associated with Anaerobic Bacterial In-

fection,” The Journal of Urology, Vol. 162, No. 3 Pt 1,

1999, pp. 795-796.

[7] S. K. Singh, A. K. Khandelwal, D. S. Pawar R. Sen and S.

Sharma, “Xanthogranulomatous Cystitis: A Rare Clinical

Entity,” Urology Annals, Vol. 2, No. 3, 2010. pp. 125-

126. doi:10.4103/0974-7796.68863

[8] R. Goel, G. Kadam, A. Devra, S. Patel and P. Modi, “Xan-

thogranulomatous Cystitis,” International Urology Neph-

rology and Nephrology, Vol. 39, No. 2, 2007, pp. 477-

478. doi:10.1007/s11255-006-9059-8

[9] S. Ekici, E. Dogan, S. Ruacan and A. Midi, “Xantho-

granulomatous Cystitis: A Challenging Imitator of Blad-

der Cancer,” The Scientific World Journal, Vol. 10, 2010,

pp. 1160-1173. doi:10.1100/tsw.2010.139

Abbreviations:

XC—xanthogranulomatous cystitis;

VVF—vesicovaginal fistula;

CT—computer tomography.