Introduction

Traditionally, spinal dysraphism or spina bifida had a high mortality rate of 50%–70% by the age of 20 years. In the late 1950s, the refinement of ventriculoperitoneal shunts (VPSs) and advancements in the management of neurological and urological sequelae had dramatically improved survival and quality of life for patients with the disease.¹ VPS placement, the most common procedure used in management of hydrocephalus in this patient population, has a very rare risk of shunt erosion into the viscera. Indeed, there are only 2 cases of VPS erosion into the bladder reported in literature, both of which were managed by suprapubic cystostomy, VPS removal, cystorraphy and antibiotic treatment.² The introduction of clean intermittent catheterization by Lapides et al in 1972, together with anticholinergic medication or intravesical injection of Botox®, has become the basis of conservative management of consequent neurogenic bladder.^3,4 Alternatively, augmentation cystoplasty has become a viable surgical option for patients whose urodynamic parameters and clinical course fail conservative measures. Augmentation cystoplasty is not without its morbidities, however, including urolithiasis, electrolyte abnormalities, bladder perforation, and a mortality rate of 7%–9%.^5,6 In this article, we present a rare case of an adult patient who presented with VPS erosion into her augmented bladder in the setting of a hostile abdomen. The purpose of this report is to describe a minimally invasive endoscopic technique to extract the intravesical portion of an eroded VPS as an alternative to open surgery.

Case Report

A 31-year-old female with a history of lumbosacral myelomeningocele, hydrocephalus, status post VPS placement, syringomyelia, tethered cord, Arnold Chiari II malformation, neurogenic bowel and neurogenic bladder associated with urinary incontinence presented to her primary care physician with discharge from a prior midline incision. The patient had undergone an exploratory laparotomy with augmentation ileocecocystoplasty, creation of a continent catheterizable stoma and a rectus fascial miduretheral sling approximately 1 year prior to presentation. Further history revealed that the patient had been noncompliant with intermittent catheterization and had alternatively been placing a stoma appliance over her urostomy for passive overflow urinary drainage. In addition, the patient had been treated for several urinary tract infections in the interim and had spontaneous nonpurulent serous drainage from her midline incision. Given physical examination and history suggestive of possible fistula formation, a computerized tomography (CT) scan of the abdomen and pelvis was ordered, and the patient was directed to the emergency department.

Imaging revealed a portion of the patient’s VPS had eroded into the augmented bladder (fig. 1, A and B) and identified a fluid collection along the inferior left rectus sheath with a possible fistulous tract to the abdominal wall. Accordingly, an indwelling Foley catheter was placed into the patient’s urostomy, and urgent consultation of the neurosurgery and general surgery services was performed. The neurosurgery team excised the proximal portions of the VPS; however, the intra-abdominal and bladder portions of the VPS remained.

Figure 1. Preoperative axial (A) and coronal (B) sections of CT scan showing VPS tubes inside augmented bladder (arrows).

Given the patient’s hostile abdomen, extensive adhesiolysis and complex abdominal closure after her ileocecocystoplasty, the decision was made to remove the bladder portion of the VPS cystoscopically. The patient was taken to the operating room and a fluoroscopic cystogram was performed to assess for bladder contrast leak and if there was any connection with the cutaneous fistula. The cystogram was negative for intra or extraperitoneal bladder rupture and bladder fistulation. Cystoscopy revealed 2 separate perforations of the VPS through the bowel portion of the augmented bladder (fig. 2). Due to the site of erosion being too distant to reach using rigid cystoscopy, a flexible cystoscope was used. The VPS was captured using a ureteral stent capturing device, pulled outside of the urethral meatus, and held on gentle tension by an assistant surgeon. The flexible cystoscope was re-introduced alongside the externalized VPS. The site of erosion was identified. A 550 μm holmium laser fiber was introduced through the cystoscope and used to gently cut the eroded shunt tubes as deep into the ileal mucosa as possible. The eroded portions of the VPS tubes were then removed. Postoperative CT scan showed no evidence of residual tube portions in the bladder (fig. 3, A and B).

Figure 2. Cystoscopic image of eroding VPS tubes into augmented bladder.

Discussion

The introduction of VPS in 1908 has led to a dramatic improvement in the management of increased intracranial pressure.⁷ However, the procedure has been reported to carry a rare risk of migration into virtually any abdominal organ with few reported cases of migration into the urinary bladder.⁸ The main cause of shunt erosion into the lumen of body cavities has not yet been fully identified, but it is hypothesized that it may be related to sharp stiff ends of some VPSs,⁹ thin visceral wall and strong peristaltic waves in children, perforation following adhesions caused by chronic irritation and inflammation by the shunt, previous surgery, infection, increased intrabdominal pressure, abdominal musculature contraction, silicone or latex allergy, and long abdominal parts of shunt tubes.⁸ We hereby present the rare case of VPS erosion into an augmented bladder of an adult patient. We also reported the endoscopic technique extracting those eroded shunt tubes in order to avoid the rather morbid and complicated abdominal approach.

Figure 3. Postoperative axial (A) and coronal (B) sections of CT scan showing VPS tubes outside side augmented bladder (arrows).

Conclusion

Erosion into abdominopelvic viscera, including urinary bladder, is a rare complication of VPS placement in adults as well as in children. In select cases, resection of the intravesical portion of the penetrating VPS can be performed endoscopically to mitigate the morbidity and mortality of open surgery.