Challenging cases in urology: Hematuria in a man with prune belly syndrome

“Challenging Cases in Urology” is a new Urology Times section in which residents from the nation’s leading urology programs present their toughest cases and how they ultimately managed them. Cases inform readers of the problem-solving process and provide a lesson from the authors’ experience.  

Dr. Singla is a urology resident and Dr. Lotan is professor of urology and chief of urologic oncology at the University of Texas Southwestern, Dallas.

History

A 48-year-old male is referred from an outside provider for evaluation of gross hematuria for several weeks. He denies any dysuria or subjective difficulty with voiding. His medical history is remarkable for prune belly syndrome, atrial fibrillation, hypertension, and type II diabetes mellitus. Records regarding his childhood surgical history are unfortunately scant, though notable for a remote vesicostomy that was subsequently closed, an enterocystoplasty, and abdominoplasty.

More recently, he had undergone a right simple nephrectomy 5 years prior for a non-functional kidney in the setting of recurrent urinary tract infections. The patient reports a long history of intermittent management with a urethral catheter, although he notes that he currently evacuates his bladder via spontaneous voiding without difficulty. He denies any family history of malignancies and notes that he did smoke approximately a half-pack per day of cigarettes for 2 years, from 1984 to 1986.

Examination

On physical examination, the patient’s body mass index is 32 kg/m², and he is noted to have a well-healed, large, left-sided paramedian scar from the pubic bone to above the umbilicus; a midline scar at his prior vesicostomy site; and laxity of his abdominal wall with redundancy of his overlying skin. Testes are palpable bilaterally. Laboratory evaluation reveals creatinine of 1.5 mg/dL (calculated GFR of 50 mL/min/1.73 m²); hemoglobin and hematocrit are 9.9 g/dL and 30.1%, respectively. Electrolytes, platelets, coagulation profile, liver function tests, and remainder of lab results are within normal range.

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To his initial appointment, the patient brings MR images of his abdomen and pelvis, displayed in the figure. The imaging reveals a 5.6 x 4.3 x 4.5-cm mass at the anterior bladder dome with inflammation extending to the perivesical fat. No pelvic lymphadenopathy or sites of metastatic disease are found. Severe left-sided hydoureteronephrosis extends to the level of an abnormally conformed bladder, consistent with a patulous collecting system, with ureteral insertion into the dome. His abdominal wall musculature appears thin, and his prostate appears diminutive with an apparent atrophic left seminal vesicle.

Figure. MRI of the abdomen and pelvis reveal a solitary left kidney with severe hydroureteronephrosis seen in axial (A) and coronal T2-weighted (B) views. A 5.6 x 4.3 x 4.5-cm mass is seen at the anterior bladder dome (C) with inflammation extending to the perivesical fat on T1-weighted imaging in axial view (yellow circle). No pelvic lymphadenopathy or sites of metastatic disease are found. (Figure courtesy of Nirmish Singla, MD, and Yair Lotan, MD)

Next: What is your diagnosis?

Diagnosis and discussion

He undergoes a transurethral resection of the tumor that reveals muscle-invasive urothelial carcinoma with >90% squamous differentiation and no lymphovascular invasion. The bladder appears to have been augmented with small bowel, and the tumor is located within the native portion of the bladder.

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The concern for a potentially increased risk of intravesical malignancy following augmentation enterocystoplasty has generated considerable interest. At present, however, we lack definitive evidence supporting this association. In 2010, Higuchi et al reported that bladder augmentation was not an independent risk factor in the development of bladder cancer in a relatively sizable cohort of patients with congenital bladder abnormalities (J Urol 2010; 184:2492-6), though their study is limited by its single-institution, retrospective nature.

Given the controversy surrounding this topic, there are currently no guidelines to routinely screen asymptomatic patients with prior bladder augmentation for intravesical malignancy. Nonetheless, many urologists would still elect to pursue some means of monitoring, whether through urine studies, imaging, cystoscopic evaluation, or some combination thereof.

The present case further highlights the issue of transitional care, commonly faced by pediatric urologists. Indeed, there are several challenges inherent in transitioning pediatric urology patients to adult providers-a process that must be carefully orchestrated. Some obstacles to this transition include locating adult urologists with special expertise, maintaining comprehensive documentation regarding childhood medical and surgical care, coordinating care with other adult specialties such as nephrology, and navigating the adult health care environment (Semin Pediatr Surg 2015; 24:73-8).

In our patient’s case, unfortunately much was unknown regarding his prior history, such as the original indications for bladder augmentation, whether ureteral reimplantation was pursued, and the type and length of bowel segment used, which are all important to know prior to offering surgical intervention.

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There are additional challenges worth consideration in the subsequent management of our patient’s malignancy. First, the role of neoadjuvant chemotherapy remains unclear in the setting of variant histology, as endorsed by the 2017 AUA guideline on muscle-invasive bladder cancer (J Urol 2017; 198:552-9). Several operative challenges during radical cystectomy can be anticipated in his case as well. Given his multiple prior abdominal surgeries including bowel resection, extensive scarring can be anticipated, and preoperative consultation with a general surgeon may be worthwhile for intraoperative assistance with adhesiolysis and dissection.

Next: "Deciding on a type of diversion poses another challenge"

Deciding on a type of diversion poses another challenge. Given his patulous ureter and unclear degree of bowel resection for prior reconstructions, the possibility of utilizing a cutaneous ureterostomy is worth consideration. Although long-term outcomes of ureterostomies in adults are not well known, the likelihood of stenosis is conceivably low in his case, while obviating the need to resect further bowel. Finally, given his history of prune belly syndrome, fascial closure must be meticulous, and the increased risk of parastomal hernia warrants discussion as well.

Given his predominantly squamous histology and baseline renal dysfunction in the setting of a solitary kidney, the decision was ultimately made to proceed with upfront radical cystectomy. A midline incision was initially made. Several dense adhesions were encountered and lysed with the assistance of our general surgery colleagues, including adhesions between the small bowel and augmented bladder. The patulous left ureter was located coursing into the augmented bladder segment, and following its ligation, appeared to have a diameter larger than that of the small bowel lumen.

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A large bladder mass was palpable at the anterior portion of the bladder. Once the bladder was successfully removed en bloc with the prostate, the left ureter was noted to have been tunneled under the sigmoid mesentery proximally. There were significant adhesions proximally with the bowel and mesentery that made the dissection of the left ureter challenging, and it was felt that an attempt to free it to allow for a cutaneous ureterostomy would be precarious and possibly injure its blood supply. As such, a decision was made to bridge the gap between the ureter and skin using an ileal conduit, which was anastomosed to the solitary left ureter in an end-to-end fashion.

Postoperatively, the patient had a relatively uncomplicated course, and his final pathology revealed pT3bN0 high-grade urothelial carcinoma with squamous and sarcomatoid differentiation arising in the dome, anterior, and posterior walls of the bladder with invasion into the perivesical soft tissues. Lymphovascular invasion was present, and all margins were negative. He is currently under consideration for adjuvant systemic immune therapy trials with our medical oncology colleagues.

Conclusion

In summary, we present the unique case of a 48-year-old male with prune belly syndrome, solitary left kidney, and prior enterocystoplasty, who was found to have muscle-invasive bladder cancer with extensive squamous differentiation. Our case highlights multiple complexities in his management, including the role for multimodal care, operative challenges necessitating consultation with other surgical colleagues, and the importance of having multiple strategies in the operating room should the initial plans prove less desirable.

Our case also highlights the controversy surrounding the development of and screening for intravesical malignancy in patients with prior enterocystoplasty, for which a stronger level of clinical evidence is necessary. Finally, our case more broadly underscores the challenges inherent in transitional care within urology.

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