Gangrenous cystitis: An extremely rare infectious condition managed by neobladder - A case report with review of literature.

Gangrenous cystitis (GC) is an extremely rare infectious entity in the modern era. GC in young patients decreases the quality of life as bladder function is totally jeopardized. The management in such cases is challenging. We present a case of GC postpartum managed by Studer neobladder reconstruction restoring the normal voiding pattern and quality of life.

INTRODUCTION

Gangrenous cystitis (GC) is an extremely rare condition with only 33 cases reported in the past 75 years.[1] Majority of these cases were reported in the preantibiotic era. After the advent of antibiotics, its incidence is very rare. The etiology seems to be multifactorial with difficult to diagnose the exact cause.[2] This phenomenon is commonly encountered in elderly patients with spinal cord injury, diabetes, stone disease, or pelvic malignancies.[1] GC due to postpartum etiology has also been reported.[3] Managing and restoring urinary drainage in such cases is very challenging. Here, we describe a case of GC postpartum in a young woman who was managed with ileal neobladder reconstruction.

CASE REPORT

A 24-year-old primigravida presented to our hospital with urinary leak from a cutaneous opening at the lower abdomen. She had undergone vaginal delivery in some community hospital 20 days back which had prolonged duration of labor >20 h. Subsequently, she developed lower abdomen pain and urinary retention 10 days postpartum. She was diagnosed with sepsis and anterior bladder wall gangrene in another hospital. The surgeon performed an emergency debridement and ultimately could not close the bladder due to resultant insufficient bladder tissue. The patient was referred to our center for further management.

At the time of admission, her vitals were stable and nontoxic. There was no previous history of voiding dysfunction. On examination, there was a fistulous opening in the hypogastric region through which Foley bulb was visible with continuous urinary leak. Ultrasonography examination revealed no collection in the abdomen. Bilateral kidneys were normal. Her renal function tests were normal. On diagnostic cystoscopy, urethra, bladder neck, and trigone were normal. Rest of the bladder wall was deficient with free entry into the anterior abdominal wall. She was advised for urinary diversion as a temporizing measure, but she refused. She was managed conservatively in the acute phase for 2 months with diapers.

On readmission, she had a small vesicocutaneous fistula in the anterior abdominal wall [Figure 1]. She underwent a micturition cystogram which showed a thimble bladder with a bladder capacity of about 30 ml along with vesicoureteral reflux [Figure 2a]. She underwent Studer's neobladder reconstruction which was anastomosed to the small native bladder with excision of the epithelized fistulous tract [Figure 3]. The postoperative course was uneventful. Cystogram at 3 weeks showed a healed anastomosis, and voiding trial was successful [Figure 2b–d]. The patient is voiding well with minimal postvoid residual urine at 6-month follow-up.

Figure 1

Vesicocutaneous fistula at the time of presentation through which urine was dribbling

Figure 2

Cystogram: (a) Preoperative period showing very small capacity bladder (30 ml) and bilateral vesicoureteral reflux. (b) Three weeks after surgery showing the neobladder. (c and d) Six months after surgery showing good capacity (300 mL) neobladder

Figure 3

Intraoperative images: (a and b) Small bladder after dissection. (c and d) Studer's neobladder

DISCUSSION

In the past 75 years, only 33 cases were reported worldwide. GC is defined as a massive necrosis or gangrene of the bladder.[1] Originally described by Willis in 1650 as exfoliation of the bladder, this condition is known by various names, such as gangrene of the bladder, exfoliative cystitis, and cystitis gangrenosa dissecans. In 1890, Haultain first proposed that all such cases should be categorized under necrosis of the urinary bladder.[4]

The etiological factors can be classified as indirect and direct.[1] Indirect factors interfere with the blood supply causing vascular impairment and necrosis of the bladder. These could be either pressure from inside such as bladder overdistension due to chronic urine retention or pressure from outside due to various causes such as malposition of the gravid uterus, prolonged labor, pelvic malignancies, and surgical interventions. Ligation, emboli, or thrombophlebitis of major arterial and venous vessels may also lead to this condition. Direct factors potentially by its toxic effect can damage the bladder wall leading to cell death. The inciting factors include intravesical agents, pelvic irradiation, and overwhelming systemic infections. Gas-forming organisms have been implicated in emphysematous cystitis in uncontrolled diabetes mellitus. In majority of cases, the destruction is limited to the mucosa and submucosa. Very rarely, the necrosis extends to the muscularis and serosal layers leading to this devastating complication.[4]

GC has no typical symptoms or clinical findings. It may present with urosepsis depending on the extent of disease. The patient usually presents with symptoms such as lower abdominal pain, dysuria, hematuria, and pyuria.[1] The patient may also present with peritonitis.[5] There are cases of GC causing colovesical fistula.[6] Imaging studies such as ultrasound, computed tomography, cystography, and cystoscopy may help in the diagnosis. Most cases require surgical treatment like debridement of necrosed tissue and drainage along with urinary diversion like bilateral ureterostomies.[5] Partial cystectomies are feasible in healthy trigone to ensure bladder regeneration.[7] In extensive GC, total cystectomy remains the lifesaving procedure.[5]

After debriding, the diseased bladder restoring the urinary tract continuity may be challenging. In 1960 Yeoh and Choo used sigmoid for the reconstruction of the bladder in two cases of GC.[4] There have been cases who have undergone bilateral ureterostomies and enterocystoplasty on later date.[5] In such cases, ileal conduit or neobladder could be an option.

Our case presented with vesicocutaneous fistula after debridement of necrosed bladder in a community hospital. The main challenge was to restore the bladder. She underwent ileal neobladder reconstruction and repair of cutaneous fistula. At present, the patient is continent with passing 300-ml urine with no requirement of Clean Intermittent Catheterisation (CIC) with good quality of life.

CONCLUSION

Puerperal conditions causing GC is extremely rare. After managing acute condition, options such as neobladder may restore urinary continuity and may provide an excellent quality of life.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.