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Renal Arteriovenous Fistulas
Vol. 109, May Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1973 by The Williams & Wilkins Co.
RENAL ARTERIOVENOUS FISTULAS B. D. NELSON, S. A. BROSMAN*
AND
W. E. GOODWIN
From the Departments of Surgery/Urology, UCLA School of Medicine, Los Angeles and Harbor General Hospital, Torrance, California
the cardiomegaly (fig. 1, C). The man has no physical limitation and is regularly employed. Case 2. D. M., a 46-year-old woman, was admitted to Harbor General Hospital for evaluation of intermittent episodes of gross, painless hematuria first noted 15 years earlier. On admission to the hospital the patient's blood pressure was 200/110 mm. Hg and the pulse was 106. On physical examination a grade III/VI bruit was heard over the right costovertebral angle. An excretory urogram (IVP) revealed a large filling defect in the right renal pelvis. Cystoscopy showed a normal bladder. Retrograde pyelography showed caliceal distortion and displacement of the pelvis by an intrarenal mass (fig. 2, A). A retrograde femoral arteriogram demonstrated a massive A- V malformation involving the right renal artery, vein and its tributaries (fig. 2, B). A renal scan revealed no loss of renal function. Bilateral renal vein renin studies were within normal range (right vein 200 ng. per 100 ml., left vein 235 ng. per 100 ml. and inferior vena cava 210 ng. per 100 ml.). At operation a large cirsoid A-V malformation was found to involve the upper and middle segments of the kidney. The lesion coursed over and around the renal pelvis. The lower pole of the kidney revealed multiple cysts adherent to adjacent fibrous tissue and bowel. These cysts appeared to have been associated with a previous inflammatory process. A nephrectomy was performed because of extensive disease in the lower pole as well as the massive intrarenal vascular malformation. A barium injection of the operative specimen shows the extent of the malformation (fig. 2, C). Convalescence was uneventful but the blood pressure has remained mildly elevated even on antihypertensive medication. Case 3. J. V., a 44-year-old male physician, was admitted to UCLA Medical Center for evaluation of cardiomegaly and refractory congestive heart failure 2 months in duration. He had a history of hypertension without apparent etiology. Physical examination revealed a blood pressure of 120/90 while on medication and a pulse of 105. The heart was enlarged and a grade II/VI systolic ejection murmur was audible at the left sternal border. A prominent high-pitched blowing murmur was heard in the left flank. The remainder of the examination was normal. An IVP showed a mass lesion in the left kidney deforming the renal pelvis. A renal angiogram revealed a large left renal A-V fistula (fig. 3, A). On exploration of the kidney there was a dense fibrous reaction the upper and the
The application of sophisticated angiographic procedures has led to a greater awareness of renal arteriovenous (A-V) fistulas. Therefore, many features of the entity are better understood, the diagnosis is being considered earlier and a variety of operative techniques have been presented as forms of therapy. Herein we describe 4 illustrative cases and review the features of renal A-V fistulas. CASE REPORTS
Case 1. F. H., a 28-year-old man, was admitted to Harbor General Hospital with acute congestive heart failure progressing steadily for more than 1 month in duration. Two years previously he sustained a stab wound to the abdomen and underwent exploratory laparotomy and suture of stomach perforations at another hospital. No other injuries were reported. Chest x-ray on admission to the hospital revealed a massively enlarged heart (fig. 1, A). The blood pressure was 165/95 and the patient was dyspneic at rest. Chest examination revealed cardiomegaly and the liver was palpable 2 fingerbreadths beneath the right costal margin. There was no tenderness or masses on abdominal examination but auscultation revealed a prominent bruit over the epigastrium and left costovertebral angle area. The congestive heart failure was treated with bed rest, diuretics and fluid restriction and the acute episode gradually subsided. A diagnosis of left renal A-V fistula was made on the basis of the history and clinical findings and was confirmed by a retrograde femoral arteriogram, showing the site of the fistula at the origin of the left renal artery (fig. 1, B). A renal scan revealed evidence of a small left kidney with decreased function. Surgical exploration was done. Because the retroperitoneum surrounding the vessels and the left kidney was involved in an extensive fibrotic process a reconstructive procedure was not considered possible and a left nephrectomy was performed. The renal artery was ligated and divided at its entrance from the aorta. The histopathology showed severe chronic interstitial nephritis. Postoperatively, the blood pressure was 130/80 and the patient has remained normotensive. Followup chest films are normal with complete resolution of Accepted for publication September 29, 1972. Read at annual meeting of Western Section, American Urological Association, Vancouver, British Columbia, Canada, July 2-7, 1972. * Requests for reprints: Harbor General Hospital, Tona.nee, California 90509. 779
780
NELSON, BROSMAN AND GOODWIN
Fm. 1. Case 1. A, preoperative chest x-ray demonstrates enlarged cardiac silhouette. B, aortogram shows early filling of renal veins and vena cava. C, chest x-ray 2 months postoperatively shows decrease in cardiac size.
adrenal gland. A huge A- V fistula was found in the vascular pedicle, extending into the upper and mid portion of the kidney. Nephrectomy was thought to be the most efficacious form of therapy. Careful dissection of the specimen demonstrated at least 2 A-V fistulas (fig. 3, B). Postoperatively, the heart size gradually returned to normal but the patient has remained hypertensive requiring medication. Case 4. H. M., a 57-year-old woman, was admitted to Harbor General Hospital for evaluation of a right abdominal mass, hematuria and general debilitation. She had seen several physicians during the previous 2 years for evaluation of malaise, recurrent urinary tract infection and progressive dyspnea. The abdominal mass had been noted for approximately 2 months. Blood pressure at hospitalization was 140/75 and pulse was 104. She was dyspneic, pale and obviously debilitated. Scattered rales were heard in the
chest and a grade III/V murmur was present on cardiac auscultation. A prominent right abdominal mass measured 12 cm. There was a secondary 4 cm. mass attached to the larger lesion. A loud bruit was audible over the entire abdomen particularly over the mass. The patient had 2 plus pitting edema of the lower extremities. Chest x-ray revealed vascular congestion but no cardiomegaly. IVP demonstrated a large mass with poor visualization of the right kidney. There was caliceal distortion and filling defects in the pelvis and upper ureter. At the time of cystoscopy blood was seen coming from the right ureteral orifice. Angiography was consistent with renal carcinoma and hepatic metastases. Immediate filling of the renal vein and vena cava was compatible with an A-V fistula (fig. 4). Because of the systemic effects of the tumor a nephrectomy was performed. Metastatic nodules were present in the liver and the tumor had spread
RENAL ARTERIOVENOUS FISTULAS
781
FIG. 2. Case 2. A, bilateral retrograde pyelogram discloses caliceal distortion and displacement of right renal pelvis by intrarenal mass. B, selective angiogram of right kidney demonstrates large cirsoid vascular malformation occupying upper and middle portions of kidney. Pelvis (p) is displaced medially. C, barium injection of vascular malformation following nephrectomy.
to the hilar lymph nodes. PostopEcratively, the patient showed improvement in her cardiovascular status and was discharged from the hospital. DISCUSSION
The first reported case of renal A-V fistula was described in 1923 by Varela.' Reviewing the literature in 1964 Maldonado found 35 cases of fistulas associated with functioning ipsilateral kidneys.2 Since then the number of reported cases has steadily grown with McAlhany and associates describing a total of 118 fistulas up to 1971. 3 1 Varela, M. E.: Aneurisma arteriovenoso de los vaso renales y asistolia consecutiva. Rev. Med. Latino-Amer., 14: 3244, 1923. 'Maldonado, J. E., Sheps, S. Bernatz, P. E., DeWeerd, J. H. and Harrison, E. G., · Renal arteriovenous fistula. A reversible cause of hypertension and heart failure. Amer. J. Med., 37: 1964. 3 1wcrt,111a11 J.C., Jr., Black, H. Jr., Hanback, L. D. HI: Renal mteriovenous Amer. J. Surg., 122:
Etiology. There are 3 types of renal A-V fistulas: 1) congenital, 2) idiopathic and 3) acquired. The
vascular malformations having a cirsoid appearance with multiple interconnecting fistulas probably represent a true congenital abnormality. Idiopathic fistulas are single, they are not cirsoid and they have no apparent cause. The acquired fistulas can be subdivided according to their etiology into 4 groups: 1) traumatic, 2) surgical, 3) neoplastic and 4) inflammatory. The most common cause of traumatic fistulas are those from penetrating wounds, such as from knives and gunshots. Rarely is blunt trauma a cause. 4 The diagnosis is not usually made for months or years following the injury until the patient presents with symptoms of heart failure. Some patients acquire fistulas as a result of previous surgical procedures such as nephrectoD. G. and Milliken, J.C.: Renal arteriovenous to blunt trauma presenting as hypertension. Brit. J. Surg., 55: 471, 1968.
fistula
782
NELSON, BROSMAN AND GOODWIN
FIG. 3. Case 3. A, renal angiogram shows large A-V fistula occupying middle of left kidney. Arrow points to site of A-V communication. B, post-nephrectomy injection of vascular supply reveals multiple sites of fistula formation and large venous cavity.
my, partial nephrectomy and nephrolithotomy. s-a In 1962 Boijsen and Kohler reported on the development of fistulas resulting from percutaneous renal biopsies. 7 In 1965 Kaufman and associates predicted that this complication of needle biopsy would be recognized more frequently in the future.• Ekelund and Lindholm found fistulas in 7 of 41 patients, an incidence of 15 per cent. 10 They noted that the incidence depends on the time interval between biopsy and angiography. Angiography performed within a month of the renal biopsy will reveal a higher incidence. Of the 7 cases described by Ekelund and Lindholm, 5 fistulas closed spontaneously within 18 months of biopsy. They also noted that fistulas are more likely to develop in patients with hypertension. Renal neoplasms are a rare cause of significant A- V fistulas. In 1967 Wise found 7 reported cases of massive renal A-V fistulas owing to renal cell carcinoma and added 3 of his own. 11 He made a 'Muller, W. H., Jr. and Goodwin, W. E.: Renal arteriovenous fistula following nephrectomy. Ann. Surg., 144: 240, 1956. "Snodgrass, W. T. and Robinson, M. J.: lntrarenal arteriovenous fistula: a complication of partial nephrectomy. J. Urol., 91: 135, 1964. 7 Boijsen, E. and Kohler, R.: Renal arteriovenous fistulae. Acta Radio!., 57: 433, 1962. • Kelly, D. G.: Renal arteriovenous fistula. A review of four cases and review of the literature. Brit. J. Urol., 39: 162, 1967. "Kaufman, J. J., Gordon, A. and Maxwell, M. H.: lntrarenal arteriovenous fistula following needle biopsy of the kidney. Calif. Med., 103: 350, 1965. 10 Ekelund, L. and Lindholm, T.: Arteriovenous fistulae following percutaneous renal biopsy. Acta Radio!., 11: 38, 1971. 11 Wise, G. J ., Bosniak, M. A. and Hudson, P. B.: Arteriovenous fistula associated with renal cell car-
FIG. 4. Case 4. Selective angiogram depicts early filling of right renal vein and vena cava within 1 second of arterial injection.
distinction between simple A-V shunting so common in renal tumors and fistulas large enough to cause cardiac and hemodynamic changes. In his 3 patients the inferior vena cava filled with dye within 1 second of renal artery injection on angiography. cinoma of the kidney. Report of three cases with cardiovascular findings. Brit. J. Urol., 39: 170, 1967.
783
RENAL ARTERIOVENOUS FISTULA'.S
Only 4 instances of A-V fistula have been attributed to inflammatory causes.2· 3 In 2 patients granulomatous changes were demonstrated in the vessel walls. Diagnosis. The diagnosis of renal A-V fistula is often suggested by the patient's history and the physical findings and confirmed by radiologic atients will e a study. Three-four bruit an ha of them will have coDges:t;ive heart failure and/or cardiomegaly and/or diastolic hypertension. 3 -Hematuria is present in about a third of the patients while a palpable mass is rarely present except when there is tumor. After a percutaneous needle biopsy of the kidney, routine auscultation for a bruit has made possible the recognition of early, small A-V fistulas before any significant signs or symptoms have developed. An IVP will frequently show a space-occupying lesion of the involved kidney. The definitive procedure is the aortogram, with selective views to demonstrate the radiologic anatomy of the fistula. Properly done studies will show if the fistula is intrarenal or extrarenal, the number of main renal arteries and feeding vessels and whether there are multiple fistulas and associated renal disease. Other valuable diagnostic studies include renal scans, renograms, dye dilution procedures, renal vein renins, measurement of urinary electrolytes and renal function studies. Treatment. The treatment of renal A-V fistulas is related to their etiology and symptomatology. The A-V fistula which develops after a renal biopsy is usually small and generally disappears within 1 to 18 months. 9 • 10 , 12· 1 5 If the lesion pers~sts and angiography shows that the lesion is enlarging and there are signs of cardiac decompensation, then an operation is indicated. Surgical treatment is necessary for patients with symptomatic disease. In Maldonado's report of 35 patients with a renal A-V fistula associated with a functioning ipsilateral kidney, 30 patients underwent nephrectomies, 3 had partial nephrectomies and 1 had ligation of the branch renal artery only. 2 In the past few years there have been scattered case reports of successful repair of renal A-V fistulas without nephrectomy. If the lesion is isolated to one area of the kidney, a partial nephrectomy should be considered. 9 In 12 DeBeukelaer, M. M., Schreiber, M. H., Dodge, W. F. and Travis, L. B.: Intrarenal arteriovenous fistulas following needle biopsy of the kidney. J. Pediat., 78: 266, 1971. 13 Bennett, A. R. and Wiener, S. N.: Intrarenal arteriovenous fistula and aneurysm. A complication of percutaneous renal biopsy. Amer. J. Roentgen., 95: 372, 1965. 14 Herschman, A., Klein, M. J. and Blumberg, A.G.: Spontaneous disappearance of iatrogenic renal arteriovenous fistula: report of a case. J. Urol., 105: 4, 1971. 1 'Nilsson, C. G. and Ross, R. J.: Bilateral renal a!teriovenous fistulas and decreased blood pressure following renal biopsies. J. Urol., 97: 176, 1967.
1962 Boijsen and Kohler reported on a case in which the arterial supply to the fistula was ligated with apparent cure. 7 Tunner and associates described a case in which the arterial feeder was isolated, ligated and divided, interposing a piece of fat between the cut ends with a successful result. 16 Tynes and associates reported on the surgical treatment in 5 patients with 2 undergoing nephrectomies, 1 a ligation and 2 ligation and excision of the fistulous mass. 17 In 1970 Merkel reported on the closure of a fistula by opening the aneurysmal sac connecting the artery and vein and ligating the communication with a row of fine arterial silk. 18 Renal A-V fistulas with functioning ipsilateral kidneys are commonly associated with diastolic hypertension in contrast to post-nephrectomy A-V fistulas when there is systolic hypertension, a low diastolic blood pressure and a widened pulse pressure. 3 The exact cause of the hypertension is not clea:r, although localized ischemia may be responsible for the diastolic hypertension operative via the angiotensin-renin system. Maldonado reported on .a patient in whom there was definite evidence of renal ischemia as determined by renography, renal function studies and histologic changes consistent with ischemia. 2 Secrest studied the effects of renal A-V fistulas in dogs and found that only systolic hypertension developed which was not associated with any increase in plasma renin activity or measurable changes in renal function. 19 Accurate renal vein renin determinations are difficult to obtain because the fistula allows such a large quantity of renal arterial blood to flow directly into the vein that an increase in renin from a perfused ischemic area would be significantly diluted. In more than 60 per cent of those patients with a fistula and associated hypertension, nephrectomy will relieve the hypertension. 3 Two of our 3 patients with hypertension have remained hypertensive. SUMMARY
A-V fistulas involving the renal vasculature are being reported with increasing frequency. Congenital, idiopathic and acquired fistulas have been described. Diagnosis is suggested by history and physical examination and confirmed by renal angiography. Percutaneous renal biopsy is the cause of an increasing number of fistulas. Most of these fis1•Tunner, W. S., Middleton, R. G., Watson, R. W. and Marshall, V. F.: Repair of an intrarenal arteriovenous fistula with preservation of the kidney. J. Urol., 103: 286, 1970. "Tynes, W. V., Devine, C. J., Jr., Devine, P. C. and Poutasse, E. F.: Surgical treatment of renal arteriovenous fistulas: report of 5 cases. J. Urol., 103: 692, 1970. 18 Merkel, F. K. and Sako, Y.: Surgical treatment for traumatic renal arteriovenous fistulas. Arch. Surg., 101: 438, 1970. "Secrest, A. J.: Experimental renal arteriovenous fistula. J. Urol., 102: 552, 1969.
784
NELSON, BROSMAN AND GOODWIN
tulas are small, clinically insignificant and resolve spontaneously. Those fistulas, whatever the cause, which produce enough hemodynamic changes to cause
symptoms are treated surgically. The most common operation has been nephrectomy, although improved techniques in renal vascular surgery offer the hope of greater renal salvage.
THE JOURNAL OF UROLOGY
Copyright © 1973 by The Williams & Wilkins Co.
RENAL ARTERIOVENOUS FISTULAS B. D. NELSON, S. A. BROSMAN*
AND
W. E. GOODWIN
From the Departments of Surgery/Urology, UCLA School of Medicine, Los Angeles and Harbor General Hospital, Torrance, California
the cardiomegaly (fig. 1, C). The man has no physical limitation and is regularly employed. Case 2. D. M., a 46-year-old woman, was admitted to Harbor General Hospital for evaluation of intermittent episodes of gross, painless hematuria first noted 15 years earlier. On admission to the hospital the patient's blood pressure was 200/110 mm. Hg and the pulse was 106. On physical examination a grade III/VI bruit was heard over the right costovertebral angle. An excretory urogram (IVP) revealed a large filling defect in the right renal pelvis. Cystoscopy showed a normal bladder. Retrograde pyelography showed caliceal distortion and displacement of the pelvis by an intrarenal mass (fig. 2, A). A retrograde femoral arteriogram demonstrated a massive A- V malformation involving the right renal artery, vein and its tributaries (fig. 2, B). A renal scan revealed no loss of renal function. Bilateral renal vein renin studies were within normal range (right vein 200 ng. per 100 ml., left vein 235 ng. per 100 ml. and inferior vena cava 210 ng. per 100 ml.). At operation a large cirsoid A-V malformation was found to involve the upper and middle segments of the kidney. The lesion coursed over and around the renal pelvis. The lower pole of the kidney revealed multiple cysts adherent to adjacent fibrous tissue and bowel. These cysts appeared to have been associated with a previous inflammatory process. A nephrectomy was performed because of extensive disease in the lower pole as well as the massive intrarenal vascular malformation. A barium injection of the operative specimen shows the extent of the malformation (fig. 2, C). Convalescence was uneventful but the blood pressure has remained mildly elevated even on antihypertensive medication. Case 3. J. V., a 44-year-old male physician, was admitted to UCLA Medical Center for evaluation of cardiomegaly and refractory congestive heart failure 2 months in duration. He had a history of hypertension without apparent etiology. Physical examination revealed a blood pressure of 120/90 while on medication and a pulse of 105. The heart was enlarged and a grade II/VI systolic ejection murmur was audible at the left sternal border. A prominent high-pitched blowing murmur was heard in the left flank. The remainder of the examination was normal. An IVP showed a mass lesion in the left kidney deforming the renal pelvis. A renal angiogram revealed a large left renal A-V fistula (fig. 3, A). On exploration of the kidney there was a dense fibrous reaction the upper and the
The application of sophisticated angiographic procedures has led to a greater awareness of renal arteriovenous (A-V) fistulas. Therefore, many features of the entity are better understood, the diagnosis is being considered earlier and a variety of operative techniques have been presented as forms of therapy. Herein we describe 4 illustrative cases and review the features of renal A-V fistulas. CASE REPORTS
Case 1. F. H., a 28-year-old man, was admitted to Harbor General Hospital with acute congestive heart failure progressing steadily for more than 1 month in duration. Two years previously he sustained a stab wound to the abdomen and underwent exploratory laparotomy and suture of stomach perforations at another hospital. No other injuries were reported. Chest x-ray on admission to the hospital revealed a massively enlarged heart (fig. 1, A). The blood pressure was 165/95 and the patient was dyspneic at rest. Chest examination revealed cardiomegaly and the liver was palpable 2 fingerbreadths beneath the right costal margin. There was no tenderness or masses on abdominal examination but auscultation revealed a prominent bruit over the epigastrium and left costovertebral angle area. The congestive heart failure was treated with bed rest, diuretics and fluid restriction and the acute episode gradually subsided. A diagnosis of left renal A-V fistula was made on the basis of the history and clinical findings and was confirmed by a retrograde femoral arteriogram, showing the site of the fistula at the origin of the left renal artery (fig. 1, B). A renal scan revealed evidence of a small left kidney with decreased function. Surgical exploration was done. Because the retroperitoneum surrounding the vessels and the left kidney was involved in an extensive fibrotic process a reconstructive procedure was not considered possible and a left nephrectomy was performed. The renal artery was ligated and divided at its entrance from the aorta. The histopathology showed severe chronic interstitial nephritis. Postoperatively, the blood pressure was 130/80 and the patient has remained normotensive. Followup chest films are normal with complete resolution of Accepted for publication September 29, 1972. Read at annual meeting of Western Section, American Urological Association, Vancouver, British Columbia, Canada, July 2-7, 1972. * Requests for reprints: Harbor General Hospital, Tona.nee, California 90509. 779
780
NELSON, BROSMAN AND GOODWIN
Fm. 1. Case 1. A, preoperative chest x-ray demonstrates enlarged cardiac silhouette. B, aortogram shows early filling of renal veins and vena cava. C, chest x-ray 2 months postoperatively shows decrease in cardiac size.
adrenal gland. A huge A- V fistula was found in the vascular pedicle, extending into the upper and mid portion of the kidney. Nephrectomy was thought to be the most efficacious form of therapy. Careful dissection of the specimen demonstrated at least 2 A-V fistulas (fig. 3, B). Postoperatively, the heart size gradually returned to normal but the patient has remained hypertensive requiring medication. Case 4. H. M., a 57-year-old woman, was admitted to Harbor General Hospital for evaluation of a right abdominal mass, hematuria and general debilitation. She had seen several physicians during the previous 2 years for evaluation of malaise, recurrent urinary tract infection and progressive dyspnea. The abdominal mass had been noted for approximately 2 months. Blood pressure at hospitalization was 140/75 and pulse was 104. She was dyspneic, pale and obviously debilitated. Scattered rales were heard in the
chest and a grade III/V murmur was present on cardiac auscultation. A prominent right abdominal mass measured 12 cm. There was a secondary 4 cm. mass attached to the larger lesion. A loud bruit was audible over the entire abdomen particularly over the mass. The patient had 2 plus pitting edema of the lower extremities. Chest x-ray revealed vascular congestion but no cardiomegaly. IVP demonstrated a large mass with poor visualization of the right kidney. There was caliceal distortion and filling defects in the pelvis and upper ureter. At the time of cystoscopy blood was seen coming from the right ureteral orifice. Angiography was consistent with renal carcinoma and hepatic metastases. Immediate filling of the renal vein and vena cava was compatible with an A-V fistula (fig. 4). Because of the systemic effects of the tumor a nephrectomy was performed. Metastatic nodules were present in the liver and the tumor had spread
RENAL ARTERIOVENOUS FISTULAS
781
FIG. 2. Case 2. A, bilateral retrograde pyelogram discloses caliceal distortion and displacement of right renal pelvis by intrarenal mass. B, selective angiogram of right kidney demonstrates large cirsoid vascular malformation occupying upper and middle portions of kidney. Pelvis (p) is displaced medially. C, barium injection of vascular malformation following nephrectomy.
to the hilar lymph nodes. PostopEcratively, the patient showed improvement in her cardiovascular status and was discharged from the hospital. DISCUSSION
The first reported case of renal A-V fistula was described in 1923 by Varela.' Reviewing the literature in 1964 Maldonado found 35 cases of fistulas associated with functioning ipsilateral kidneys.2 Since then the number of reported cases has steadily grown with McAlhany and associates describing a total of 118 fistulas up to 1971. 3 1 Varela, M. E.: Aneurisma arteriovenoso de los vaso renales y asistolia consecutiva. Rev. Med. Latino-Amer., 14: 3244, 1923. 'Maldonado, J. E., Sheps, S. Bernatz, P. E., DeWeerd, J. H. and Harrison, E. G., · Renal arteriovenous fistula. A reversible cause of hypertension and heart failure. Amer. J. Med., 37: 1964. 3 1wcrt,111a11 J.C., Jr., Black, H. Jr., Hanback, L. D. HI: Renal mteriovenous Amer. J. Surg., 122:
Etiology. There are 3 types of renal A-V fistulas: 1) congenital, 2) idiopathic and 3) acquired. The
vascular malformations having a cirsoid appearance with multiple interconnecting fistulas probably represent a true congenital abnormality. Idiopathic fistulas are single, they are not cirsoid and they have no apparent cause. The acquired fistulas can be subdivided according to their etiology into 4 groups: 1) traumatic, 2) surgical, 3) neoplastic and 4) inflammatory. The most common cause of traumatic fistulas are those from penetrating wounds, such as from knives and gunshots. Rarely is blunt trauma a cause. 4 The diagnosis is not usually made for months or years following the injury until the patient presents with symptoms of heart failure. Some patients acquire fistulas as a result of previous surgical procedures such as nephrectoD. G. and Milliken, J.C.: Renal arteriovenous to blunt trauma presenting as hypertension. Brit. J. Surg., 55: 471, 1968.
fistula
782
NELSON, BROSMAN AND GOODWIN
FIG. 3. Case 3. A, renal angiogram shows large A-V fistula occupying middle of left kidney. Arrow points to site of A-V communication. B, post-nephrectomy injection of vascular supply reveals multiple sites of fistula formation and large venous cavity.
my, partial nephrectomy and nephrolithotomy. s-a In 1962 Boijsen and Kohler reported on the development of fistulas resulting from percutaneous renal biopsies. 7 In 1965 Kaufman and associates predicted that this complication of needle biopsy would be recognized more frequently in the future.• Ekelund and Lindholm found fistulas in 7 of 41 patients, an incidence of 15 per cent. 10 They noted that the incidence depends on the time interval between biopsy and angiography. Angiography performed within a month of the renal biopsy will reveal a higher incidence. Of the 7 cases described by Ekelund and Lindholm, 5 fistulas closed spontaneously within 18 months of biopsy. They also noted that fistulas are more likely to develop in patients with hypertension. Renal neoplasms are a rare cause of significant A- V fistulas. In 1967 Wise found 7 reported cases of massive renal A-V fistulas owing to renal cell carcinoma and added 3 of his own. 11 He made a 'Muller, W. H., Jr. and Goodwin, W. E.: Renal arteriovenous fistula following nephrectomy. Ann. Surg., 144: 240, 1956. "Snodgrass, W. T. and Robinson, M. J.: lntrarenal arteriovenous fistula: a complication of partial nephrectomy. J. Urol., 91: 135, 1964. 7 Boijsen, E. and Kohler, R.: Renal arteriovenous fistulae. Acta Radio!., 57: 433, 1962. • Kelly, D. G.: Renal arteriovenous fistula. A review of four cases and review of the literature. Brit. J. Urol., 39: 162, 1967. "Kaufman, J. J., Gordon, A. and Maxwell, M. H.: lntrarenal arteriovenous fistula following needle biopsy of the kidney. Calif. Med., 103: 350, 1965. 10 Ekelund, L. and Lindholm, T.: Arteriovenous fistulae following percutaneous renal biopsy. Acta Radio!., 11: 38, 1971. 11 Wise, G. J ., Bosniak, M. A. and Hudson, P. B.: Arteriovenous fistula associated with renal cell car-
FIG. 4. Case 4. Selective angiogram depicts early filling of right renal vein and vena cava within 1 second of arterial injection.
distinction between simple A-V shunting so common in renal tumors and fistulas large enough to cause cardiac and hemodynamic changes. In his 3 patients the inferior vena cava filled with dye within 1 second of renal artery injection on angiography. cinoma of the kidney. Report of three cases with cardiovascular findings. Brit. J. Urol., 39: 170, 1967.
783
RENAL ARTERIOVENOUS FISTULA'.S
Only 4 instances of A-V fistula have been attributed to inflammatory causes.2· 3 In 2 patients granulomatous changes were demonstrated in the vessel walls. Diagnosis. The diagnosis of renal A-V fistula is often suggested by the patient's history and the physical findings and confirmed by radiologic atients will e a study. Three-four bruit an ha of them will have coDges:t;ive heart failure and/or cardiomegaly and/or diastolic hypertension. 3 -Hematuria is present in about a third of the patients while a palpable mass is rarely present except when there is tumor. After a percutaneous needle biopsy of the kidney, routine auscultation for a bruit has made possible the recognition of early, small A-V fistulas before any significant signs or symptoms have developed. An IVP will frequently show a space-occupying lesion of the involved kidney. The definitive procedure is the aortogram, with selective views to demonstrate the radiologic anatomy of the fistula. Properly done studies will show if the fistula is intrarenal or extrarenal, the number of main renal arteries and feeding vessels and whether there are multiple fistulas and associated renal disease. Other valuable diagnostic studies include renal scans, renograms, dye dilution procedures, renal vein renins, measurement of urinary electrolytes and renal function studies. Treatment. The treatment of renal A-V fistulas is related to their etiology and symptomatology. The A-V fistula which develops after a renal biopsy is usually small and generally disappears within 1 to 18 months. 9 • 10 , 12· 1 5 If the lesion pers~sts and angiography shows that the lesion is enlarging and there are signs of cardiac decompensation, then an operation is indicated. Surgical treatment is necessary for patients with symptomatic disease. In Maldonado's report of 35 patients with a renal A-V fistula associated with a functioning ipsilateral kidney, 30 patients underwent nephrectomies, 3 had partial nephrectomies and 1 had ligation of the branch renal artery only. 2 In the past few years there have been scattered case reports of successful repair of renal A-V fistulas without nephrectomy. If the lesion is isolated to one area of the kidney, a partial nephrectomy should be considered. 9 In 12 DeBeukelaer, M. M., Schreiber, M. H., Dodge, W. F. and Travis, L. B.: Intrarenal arteriovenous fistulas following needle biopsy of the kidney. J. Pediat., 78: 266, 1971. 13 Bennett, A. R. and Wiener, S. N.: Intrarenal arteriovenous fistula and aneurysm. A complication of percutaneous renal biopsy. Amer. J. Roentgen., 95: 372, 1965. 14 Herschman, A., Klein, M. J. and Blumberg, A.G.: Spontaneous disappearance of iatrogenic renal arteriovenous fistula: report of a case. J. Urol., 105: 4, 1971. 1 'Nilsson, C. G. and Ross, R. J.: Bilateral renal a!teriovenous fistulas and decreased blood pressure following renal biopsies. J. Urol., 97: 176, 1967.
1962 Boijsen and Kohler reported on a case in which the arterial supply to the fistula was ligated with apparent cure. 7 Tunner and associates described a case in which the arterial feeder was isolated, ligated and divided, interposing a piece of fat between the cut ends with a successful result. 16 Tynes and associates reported on the surgical treatment in 5 patients with 2 undergoing nephrectomies, 1 a ligation and 2 ligation and excision of the fistulous mass. 17 In 1970 Merkel reported on the closure of a fistula by opening the aneurysmal sac connecting the artery and vein and ligating the communication with a row of fine arterial silk. 18 Renal A-V fistulas with functioning ipsilateral kidneys are commonly associated with diastolic hypertension in contrast to post-nephrectomy A-V fistulas when there is systolic hypertension, a low diastolic blood pressure and a widened pulse pressure. 3 The exact cause of the hypertension is not clea:r, although localized ischemia may be responsible for the diastolic hypertension operative via the angiotensin-renin system. Maldonado reported on .a patient in whom there was definite evidence of renal ischemia as determined by renography, renal function studies and histologic changes consistent with ischemia. 2 Secrest studied the effects of renal A-V fistulas in dogs and found that only systolic hypertension developed which was not associated with any increase in plasma renin activity or measurable changes in renal function. 19 Accurate renal vein renin determinations are difficult to obtain because the fistula allows such a large quantity of renal arterial blood to flow directly into the vein that an increase in renin from a perfused ischemic area would be significantly diluted. In more than 60 per cent of those patients with a fistula and associated hypertension, nephrectomy will relieve the hypertension. 3 Two of our 3 patients with hypertension have remained hypertensive. SUMMARY
A-V fistulas involving the renal vasculature are being reported with increasing frequency. Congenital, idiopathic and acquired fistulas have been described. Diagnosis is suggested by history and physical examination and confirmed by renal angiography. Percutaneous renal biopsy is the cause of an increasing number of fistulas. Most of these fis1•Tunner, W. S., Middleton, R. G., Watson, R. W. and Marshall, V. F.: Repair of an intrarenal arteriovenous fistula with preservation of the kidney. J. Urol., 103: 286, 1970. "Tynes, W. V., Devine, C. J., Jr., Devine, P. C. and Poutasse, E. F.: Surgical treatment of renal arteriovenous fistulas: report of 5 cases. J. Urol., 103: 692, 1970. 18 Merkel, F. K. and Sako, Y.: Surgical treatment for traumatic renal arteriovenous fistulas. Arch. Surg., 101: 438, 1970. "Secrest, A. J.: Experimental renal arteriovenous fistula. J. Urol., 102: 552, 1969.
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tulas are small, clinically insignificant and resolve spontaneously. Those fistulas, whatever the cause, which produce enough hemodynamic changes to cause
symptoms are treated surgically. The most common operation has been nephrectomy, although improved techniques in renal vascular surgery offer the hope of greater renal salvage.