Fistulae between the urinary tract and the vascular system are rare but have increased in frequency with the rapid integration of minimally invasive interventions in the upper urinary tract, such as percutaneous access procedures (Clayman et al, 1984; Segura et al, 1985), and indwelling ureteral stents (Kar et al, 1984; Smith, 1984; Teuton et al, 1987; Sacks and Miller, 1988; Cass and Odland, 1990). These fistulae include communications between the upper urinary tract, including the collecting system or ureter and an artery or vein.
The most common causes of renovascular or pyelovascular fistulae are procedures in which percutaneous renal access is required, such as percutaneous nephrolithotomy (PCNL) (Clayman et al, 1984; Segura et al, 1985; Lang, 1987; Lee et al, 1987). Typically, these fistulae are created upon puncture of an intrarenal vascular structure while creating or dilating the nephrostomy tract. The damaged vessel may bleed on puncture, or may not hemorrhage immediately due to external compression and tamponade from the catheter in the nephrostomy tract. However, upon removal of the catheter, brisk bleeding may be noted into the relatively lower-pressure renal collecting system (Patterson et al, 1985). Alternatively, a long-term indwelling nephrostomy tube may lead to pyelovascular fistula formation. In this setting, a chronic indwelling, large-bore nephrostomy tube may result in erosion into an adjacent renal vessel, with resulting hemorrhage following removal of the tube. Lee and colleagues (1987) reported an 11.2% incidence of bleeding requiring transfusion and 1.2% incidence of bleeding requiring surgical or angiographic intervention following PCNL in 582 patients. Patterson and colleagues (1985) reported a vascular injury in 0.9% of 1032 patients undergoing percutaneous renal stone surgery. Segura and colleagues (1985) reported a 3% transfusion rate in 1000 patients undergoing PCNL. Other causes of renovascular fistula include external penetrating and blunt (Stower et al, 1989) trauma, infection, and open renal surgery, including partial nephrectomy.
Renovascular fistulae may present with life-threatening hemorrhage and hypovolemic shock, or intermittent gross hematuria. Following PCNL, these fistulae may appear upon removal of the nephrostomy tube with brisk bleeding out of the flank from the nephrostomy tube tract in combination with brisk hematuria, or they may present several days to weeks later with only hematuria (Clayman et al, 1984; Patterson et al, 1985).
Treatment of renovascular fistulae is contingent on the presentation, etiology, and hemodynamic stability of the patient. Patients with severe hemorrhage upon removal of the nephrostomy tube can be temporized in some instances by replacing the tube, or, in large mature tracts, by placing a Foley catheter to tamponade the bleeding. In patients with ongoing bleeding, transcatheter angiographic embolization of the lacerated vessel is recommended (Clayman et al, 1984; Patterson et al, 1985). Occasionally, flank exploration is necessary with partial or simple nephrectomy to control hemorrhage.
Rarely reported prior to the advent of indwelling ureteral stents, ureterovascular fistulae are becoming increasingly common. Most reported ureterovascular fistulae are ureteroiliac artery fistulae, although ureteroiliac vein fistulae have been reported as well (Teuton et al, 1987). Ureteroaortic fistulae are extremely rare, with few cases reported in the literature (Holmes et al, 1998; Georgopoulos et al, 2003).
Most cases of ureteroarterial fistulae are reported in patients with a prior history of vascular disease, radiation therapy, and/or pelvic surgery, especially in the setting of indwelling ureteral stents. In fact, ureteroarterial fistulae are highly associated with indwelling stents (Nelson and Fried, 1981; Kar et al, 1984; Smith, 1984; Bhargava and Yusuf, 1987; Sacks and Miller, 1988; Cass and Odland, 1990; Batter et al, 1996; Bergqvist et al, 2001). Of the 37 cases of ureteroarterial fistula reported in the literature by Batter and colleagues (1996) prior to 1996, 24 were associated with a ureteral stent (Table 77–10). All patients in this review had at least one of the risk factors listed in Table 77–10. Pressure necrosis from a chronic, relatively inflexible indwelling stent against a pulsatile iliac artery may be an important factor in the development of ureteroarterial fistulae in some cases (Batter et al, 1996). Fistula formation occurs between the high-pressure vascular lumen and the low-pressure ureter with the development of gross hematuria. A history of radiation therapy or prior pelvic surgery may exacerbate the already compromised and stented ureter, thus increasing the risk of fistula formation (Toolin et al, 1984). Ureteroarterial fistulae are also highly associated with vascular pathology such as iliac artery aneurysms. Atherosclerotic aneurysms may produce perivascular inflammation and fibrosis that entraps the overlying ureter, especially in the region of the iliac vessels. The ureter may become fixed and obstructed and, in such a position, may be subject to chronic pulsations from the underlying abnormal vessel. Placement of a ureteral stent to relieve the obstruction may further compromise the ureteral wall where it crosses over the vessel, resulting in pressure necrosis and eventual fistula formation (Sacks and Miller, 1988; Cass and Odland, 1990). Ureteroarterial fistulae have been reported following balloon dilation of ureteral strictures (Sacks and Miller, 1988; Quillin et al, 1994). Ureteroarterial fistulae may also present in the setting of ileal conduit reconstruction, pelvic malignancy, prior ureterolithotomy, external penetrating trauma, and pregnancy (Reiner et al, 1975; Cass and Odland, 1990; Dervanian et al, 1992; Puppo et al, 1992; Wampler et al, 1992; Batter et al, 1996; DePasquale et al, 2001; Siablis et al, 2002; Takahashi et al, 2004).
Table 77–10 Predisposing Risk Factors in 37 Patients with Ureteroarterial Fistulae
| Prior genitourinary/pelvic surgery | 68% |
| Ureteral stenting | 65% |
| Radiation therapy | 46% |
| Prior vascular surgery | 19% |
| Vascular pathology | 19% |
From Batter SJ, McGovern FJ, Cambria RP. Ureteroarterial fistula: case report and review of the literature. Urology 1996;48:481–9.
Ureterovascular fistulae may present with microscopic hematuria, intermittent gross hematuria, or life-threatening exsanguinating hemorrhage. The key to the diagnosis of ureteroarterial fistulae is a high index of suspicion in an at-risk patient presenting with gross hematuria (Smith, 1984; Dervanian et al, 1992; Batter et al, 1996). These fistulae are rarely considered in the initial differential diagnosis of gross hematuria. Intermittent gross hematuria or the sudden onset of massive hematuria in a patient with an indwelling stent and a history of previous iliac artery surgery or radiation should raise the suspicion of a ureteroarterial fistula (Cass and Odland, 1990).
The routine urologic and radiologic evaluation of hematuria will not generally provide evidence of ureterovascular fistula (Quillin et al, 1994). Even in suspected or proven cases, preoperative radiologic investigations, including nonselective arteriography and pyelography, are often nondiagnostic (Cass and Odland, 1990; Batter et al, 1996). This is especially true in patients with intermittent hematuria in whom there is no active bleeding at the time of the radiographic investigation, presumably due to thrombus over the site of the fistula. Selective or subselective arteriography of the iliac vessels may be more revealing in suspected cases, and provocative maneuvers, such as stent removal, or mechanical friction of the ureteral lumen by manipulation of the stent may be necessary to demonstrate the fistulous connection in patients without active bleeding who are undergoing angiography (Keller et al, 1990; Quillin et al, 1994). However, these adjuvant maneuvers should only be performed with extreme caution in an appropriate setting where immediate angiographic or surgical intervention is possible. In the review by Batter and colleagues (1996) retrograde pyelography was diagnostic for only 6 of 10 patients in whom it was performed, and arteriography was diagnostic for a ureterovascular fistula in only 4 of 14 cases. Indirect evidence of a ureteroarterial fistula can be found on CT, but findings are usually nonspecific and only suspicious in retrospect following a confirmed diagnosis by other means (Baum et al, 1987; Jafri et al, 1987). Nevertheless, in a stable patient with a suspected ureterovascular fistula, a full radiographic evaluation may be pursued, not only for diagnostic purposes but also to evaluate potential reconstructive options (Batter et al, 1996), and, in select cases, to perform therapeutic angiographic embolization procedures.
Because these patients may present in extremis with hypotension and severe hemorrhage, surgical intervention must be considered early, especially because radiographic evaluation may be nondiagnostic (Dervanian et al, 1992). In cases where angiography is pursued for diagnosis, an endovascular stent graft may be placed (Bergqvist et al, 2001; Sherif et al, 2002; Krambeck et al, 2005; Meester et al, 2006; Muraoka et al, 2006; Ishibashi et al, 2007; Araki et al, 2008).
Eventual management of these fistulae must address both the vascular and urinary sides of the fistula. Successful management of the vascular side may involve embolization, endovascular stent graft placement, primary repair (Kar et al, 1984), or even ligation with or without extra-anatomic vascular bypass. Limb salvage is an important consideration in iliac artery fistulae, and therefore vascular surgery consultation is necessary in most cases. Ultimate management of the vascular side of the fistula is dependent on several factors, including the presence of infection or abscess, presence of aneurysm or occlusive disease in the iliac artery, and the availability of collateral circulation to the ipsilateral lower extremity (Batter et al, 1996). Often, vascular occlusion, either angiographically or surgically, is followed by vascular bypass procedures in these cases.
Repair and reconstruction of the urinary tract is complicated in these patients who often have a history of pelvic radiation, malignancy, vascular disease, and/or prior surgery. Preservation of nephrons is a priority in functioning renal units. Urinary reconstruction can be performed by ureteroureterostomy, transureteroureterostomy, cutaneous ureterostomy, or percutaneous nephrostomy with ureteral ligation (Nelson and Fried 1981; Kar et al, 1984; Smith 1984; Batter et al, 1996; Gibbons et al, 1998). Nephrectomy is usually reserved for poorly functioning kidneys, or those patients unfit for urinary reconstruction.
General recommendations to prevent ureteroarterial fistulae include the use of the smallest, softest, and most flexible ureteral stents for the shortest time interval possible in patients at risk for ureterovascular fistula (Cass and Odland, 1990; Puppo et al, 1992).
Key Points: Urovascular Fistulae
Urinary fistulae have been reported between the kidneys and thoracic cavity due to a number of causes, including infection, trauma, and stone disease. Infectious causes include xanthogranulomatous pyelonephritis, TB, and renal abscess (Arida and Verderame, 1977; Blight, 1980; Kyriakopoulos et al, 1991; Haney et al, 1992; Alifano et al, 1999). Fortunately, nephropleural and nephrobronchial fistulae are uncommon. Importantly, percutaneous access to the kidney for endourologic procedures may be complicated by nephropleural fistula. Lallas and colleagues (2004) reported a 1% incidence of nephropleural fistulae in 375 patients undergoing percutaneous access procedures. All of the affected patients had a supracostal access tract performed; none of the patients with a subcostal access tract developed this complication. Presenting symptoms may include cough, a urine-like taste in the mouth, fever, and flank pain. Rarely, recurrent lung abscess may be a manifestation of an occult nephropleural fistula (Caberwal et al, 1977; O’Brien and Ettinger, 1995).
Treatment of nephropleural or nephrobronchial fistulae generally involves percutaneous drainage of any associated abscess (if present), treatment of associated infection, and/or urinary obstruction, and surgical exploration with interposition of healthy tissue. Iatrogenic fistulae due to percutaneous access procedures can be managed nonoperatively in some cases (Lallas et al, 2004). For patients undergoing surgical exploration, a double-lumen endotracheal tube may be useful during surgery to isolate the affected lung and pleural cavity and prevent contamination of the contralateral side (Rao et al, 1981). Nephrectomy is indicated in poorly functioning renal units.
Cutaneous fistulae from the urinary tract may arise from the kidney, ureter, bladder, or urethra. Renocutaneous fistulae may occur as a result of chronic infection, especially in the setting of calculous disease (Haney et al, 1992). Often the associated renal unit is poorly functioning, and thus definitive treatment is provided by nephrectomy. External trauma or iatrogenic surgical trauma, such as percutaneous renal surgery or partial nephrectomy, may also result in a renocutaneous fistula. Prompt treatment with internal ureteric stenting is generally successful by providing unobstructed antegrade urinary drainage. Most ureterocutaneous and vesicocutaneous fistulae are iatrogenic or otherwise purposefully surgically created to facilitate urinary drainage. Other uncommon causes include external penetrating trauma, malignancy, and chronic infection.
For newly diagnosed urocutaneous fistulae, it is imperative to evaluate for distal urinary obstruction. If present, the obstruction should be treated, if possible, or bypassed. Individuals with nonhealing urocutaneous fistulae due to chronic infection should be evaluated for not only an occult source of the infection, but also undergo a nutritional evaluation because these individuals may be catabolic, immunosuppressed, and unable to mobilize adequate metabolic reserves to initiate wound closure. Other considerations in individuals with non-healing urocutaneous fistulae include occult malignancy or an undiscovered foreign body.
Urethrocutaneous fistulae in the male most commonly present as sequelae of hypospadias repair and are covered in Chapter 130.
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