Correspondence *The Division of Pediatric Urology, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, CA 94305-5118, USA

Email lindashortliffe@stanford.edu

The case

A 17-year-old male college student, with a history of bladder augmentation 7 years earlier, presented to the emergency department of another institution with the acute onset of abdominal pain, nausea, emesis, anorexia, and fever. He was born with bladder exstrophy–epispadias syndrome and had undergone neonatal closure of the bladder. Because of low bladder capacity and intractable urinary incontinence, he had previously undergone multiple procedures including epispadias repairs, bladder-neck reconstruction, and, at the age of 10 years, an ILEOCYSTOPLASTY with YOUNG–DEES–LEADBETTER URETHROPLASTY and catheterizable appendicovesicostomy. He had also received a bladder-neck collagen injection to improve continence. Following these procedures, the patient had improved bladder capacity and urinary continence, but still required 2–3 pads daily. He occasionally voided spontaneously with a weak stream, and performed self-catheterization 2–3 times daily.

The patient admitted that he had stopped catheterizing 2 weeks before his symptoms began, and relied upon spontaneous voiding to empty his bladder. He had voided decreasing volumes during this time. On the night before admission, he had an onset of nausea and emesis that worsened on the following morning. As this patient had previously been warned about the complications of bladder augmentation, he became concerned about possible bladder augmentation perforation and went to his local emergency department. His medical history was confirmed by telephone, and management and evaluation for potential perforation was performed. CT with intravesical contrast revealed contrast extravasation into the left pelvic gutter (Figure 1).

Figure 1 CT cystogram.

(A) Arrow A points to ureter. The initial leak of contrast into the left intraperitoneal space around the left pelvic gutter can be seen (arrow B). This is a classic finding in bladder perforation. (B) Increased extravasation of contrast into the pelvic gutter (arrows).

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The emergency-department physician requested that the patient be evacuated by air for treatment. Before the transfer to our institution, the patient had blood and urine cultures taken and was given broad-spectrum antibiotics, including antimicrobial coverage for anaerobic organisms (ampicillin, gentamicin and metronidazole). His umbilical APPENDICOVESICOSTOMY was placed to allow gravity drainage for ongoing decompression.

The patient was in mild distress on arrival, but alert and oriented. He was febrile, with generalized abdominal pain and guarding, but no distinct rebound tenderness. On surgical exploration, a large volume of cloudy fluid was found to be loculated in the left pelvic gutter; this was cultured. A 2-cm rupture was found in the posterolateral portion of the augmented bladder in the area of the apparent scar from the cystostomy of his original ileocystoplasty operation. The bladder was repaired with 3-0 vicryl suture in two layers; one catheter was left in the appendicovesicostomy, and another left in a separate cystostomy through a different opening. A Jackson–Pratt drain was placed in the left abdominal gutter.

The patient was continued on parenteral broad-spectrum antibiotics postoperatively. Intraoperative cultures grew Streptococcus sanguis viridans from urine and peritoneal-fluid collection and Acetobacter from blood samples. On the basis of culture sensitivities, the patient was switched to intravenous moxifloxacin for 48 h on postoperative day 6. Nine days after surgery, the patient was discharged on oral moxifloxacin 400 mg twice daily for 14 days. A cystogram on postoperative day 23 showed no extravasation, so the appendicovesicostomy catheter was removed, followed by the suprapubic catheter on postoperative day 27, after the patient committed to catheterizing four times a day. Oxybutynin was also resumed.

Discussion of diagnosis

ENTEROCYSTOPLASTY and intestinal BLADDER REPLACEMENT are standard urologic procedures employed in patients with abnormally small bladders or with bladder loss resulting from various conditions, including neurogenic bladder (spina bifida), exstrophy/epispadias complex, surgical excision of urinary-tract tumors, and obstructions caused by posterior urethral valves.¹ Enterocystoplasty involves using various segments of bowel to enhance the size of the native bladder. It is usually performed for actual or functional low bladder capacity that might also be associated with intractable detrusor instability, poor bladder compliance, and high intravesical pressures that can cause damage to the upper urinary tract. Some of these patients experience severe incontinence, debilitating urgency and enuresis. Complicating factors include urinary tract infections, vesicoureteral reflux, and impaired renal growth or renal insufficiency.

Enterocystoplasty rupture is a life-threatening complication that occurs in 5–13% of these patients.^{1, 2, 3} While it has been reported that perforations within the first year of the operation sometimes occur along lines of surgical anastomosis, the augmenting bowel segments appear to be more susceptible in delayed perforations. Rupture does not seem to be more common in any particular enteric segment.^{1, 4}

There are multiple risk factors for enterocystoplasty rupture. These include total urinary continence with high bladder outlet resistance, dysfunctional native bladder, and ischemia, whether resulting from detubularization or accentuated by high intravesical pressures caused by persistent poor drainage (either due to noncompliance with timely self-catheterization or to formation of a mucus plug).^{1, 5} Because the augmented bladder spontaneously empties poorly, or not at all, it is essential that regular clean intermittent catheterization (CIC) be performed.

The typical presentation is with a sudden onset of abdominal pain, distension, rebound tenderness, oliguria or anuria. Other, accompanying symptoms include nausea, emesis, fever, and sepsis. Some patients present with referred shoulder pain from intraperitoneal urine irritation of the diaphragm. Patients might have flank pain from ureteral obstruction and electrolyte abnormalities from peritoneal urine reabsorption. Neurologically impaired patients might not exhibit classic symptoms, especially with regards to pain, but instead present with vague signs and sepsis.^{1, 5, 6} In enterocystoplasty, patients who complain of abdominal pain, whether with signs of acute abdomen or more vague symptoms, a high index of suspicion is necessary for correct diagnosis and prevention of mortality. It is also important that patients who undergo enterocystoplasty understand the possible complications and their symptoms.

Diagnosis is confirmed by urinary-fluid extravasation seen on cystogram. When the patient is stable, the preferred imaging to make the diagnosis is CT cystography.^{7, 8} In addition to being easy to perform, it is more accurate than conventional cystography.^{8, 9} It accurately diagnoses 85–100% of bladder perforations,⁷ but failure to demonstrate perforation should not preclude immediate surgical intervention if such treatment is clinically indicated.²

We believe that poor patient compliance with CIC was the cause of the patient's bladder perforation. Before undergoing enterocystoplasty, eligible patients must be willing to perform CIC to ensure adequate emptying of the bladder and maintain low intravesical pressures. This is especially necessary if the patient undergoes bladder-neck closure or lacks spontaneous and/or complete voiding ability. Regular CIC will reduce the risk of a nontraumatic spontaneous perforation developing from high pressures caused by intravesical volume retention. Intermittent catheterization is also recommended just before sports activities, jogging, and long car journeys, as abrupt contact or falling on the distended augmented bladder can also precipitate rupture. Other etiologies suggested to predispose augmented bladders to rupture include shear force related to adhesions, transmural infection of the bowel segment, and catheterization trauma.^{4, 10}

Treatment and management

Patients require immediate evaluation, bladder decompression (usually with an indwelling catheter), blood and urine cultures, and initiation of broad-spectrum intravenous antibiotics, most often followed by surgical repair. In patients with enterocystoplasty who perform intermittent catheterization, bacteriuria with multiple organisms is a common phenomenon.^{11, 12} Escherichia coli is the most common cause of bacteriuria and urinary-tract infection, but other organisms that have been reported as causes include Klebsiella, Pseudomonas and Enterococcus species, group D Streptococcus, and Serratia marcescens.^{6, 11, 12} In some cases, more unusual, fastidious or microaerophilic organisms can also be to blame. Peritonitis is particularly deadly in patients who have ventriculoperitoneal shunts, in whom retrograde bacterial infection can be lethal. Broad antibiotic treatment, therefore, is important while urine and blood cultures are pending, in order to decrease the risk of sepsis and persistent peritonitis. Treatment with a broad-spectrum penicillin (e.g. ampicillin), aminoglycoside, and an antimicrobial to which anaerobes would be sensitive (e.g. metronidazole) is prudent.

Although recent publications have shown that some stable patients have been successfully managed nonoperatively,^{6, 13, 14} appropriate patient selection and close clinical monitoring with serial abdominal examination and frequent reassessment for potential surgical intervention are recommended when this route is taken. It should be emphasized that the standard therapy is surgical exploration.

Conclusion

This case demonstrates the importance of rapid diagnosis of bladder perforation in enterocystoplasty patients. Patients with abdominal pain, who have undergone enterocystoplasty in the past, must be considered as having a potential perforation until evaluation eliminates this possibility. A high level of patient and physician suspicion of this diagnosis, on the basis of medical history and evaluation by CT cystography, are important. Bladder replacement and enterocystoplasty are commonly performed and are procedures well known to urologists, but those physicians who initially evaluate such patients might be unaware of the signs and symptoms of potential complications. For this reason, in addition to raising the suspicion of diagnosis in physicians, patient education about possible problems, their symptoms, and the need for immediate medical attention is paramount. Since the patient's mental status at presentation might not be optimally intact, we recommend that at-risk patients wear a medic alert bracelet that will help inform the emergency-room physicians of their condition. This might prevent a delay in diagnosis and avert the risk of untimely death, especially in patients with neurologic deficiency who might falsely appear stable.

Patients must be aggressively and rapidly stabilized, evaluated, and managed. They should be treated with broad-spectrum antibiotics, and assessed using an imaging modality—preferably CT cystography, as conventional fluoroscopic cystography is less accurate. Radiologic confirmation should be followed by immediate surgical intervention to repair the perforation, but false-negative cystography can occur. To decrease the risk of death, patient education is essential. Although enterocystoplasty rupture is a potentially fatal complication, the outcome can be favorable if managed promptly and adequately.

References

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Subject areas under which this article appears: Trauma and reconstruction | Pediatric urology