Vol. 116, October Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyrig-ht © 1976 by The Williams & Wilkins Co.
CONGENITAL RENAL ARTERIOVENOUS FISTULA: CASE REPORT, REVIEW OF JAPANESE LITERATURE AND DESCRIPTION OF NON-RADICAL TREATMENT TSUNETADA YAZAKI, MASARU TOMITA, MASAO AKIMOTO, TOSHIMITSU KONJIKI, HIROSHI KAWAI AND TATSUO KUMAZAKI From the Departments of Urology and Radiology, Nippon Medical School, Tokyo, Japan
ABSTRACT
A case of congenital arteriovenous fistula diagnosed by measuring the oxygen saturation difference of renal veins at the time of selective renal angiographic examination and a review of 38 relevant Japanese cases are reported. The English as well as the Japanese literature was reviewed. Treatment has consisted of partial nephrectomy, ligation of the branch artery supplying the fistula and intentional embolization of the arterial branches, feeding the shunt by catheter. CASE REPORT
A renal arteriovenous fistula is a rare disorder despite the increasing number of cases reported since 1960. Approximately 150 cases have been described since the first case reported by Varela in 1923. 1 The increased use of renal angiography on patients with renal hematuria has enabled preoperative angiographic diagnosis and relatively easy localization from the
A 68-year-old woman was seen with gross hematuria and acute right lower quadrant colic. The pain increased in severity during an 18-hour period, requiring intramuscular analgesics. The woman was relieved of the pain after she passed several
A FIG. 1. A, IVP when patient was admitted to hospital revealed no visualization of right pelvis. B, right retrograde pyelogram 2 days later revealed pelviocaliceal dilatation of right kidney.
characteristic signs.2 As a result, unless cardiovascular complications are present, salvage of the residual part of the involved kidney is possible. We report a case and review the English and Japanese literature with emphasis upon the salvage of the ipsilateral renal function. 3 - 5 Accepted for publication January 9, 1976.
large blood clots in the urine. History was non-contributory. Physical examination revealed a woman with pain and anemia. Laboratory findings were normal except for slightly decreased red blood cells. A plain chest x-ray, an electrocardiogram, the blood pressure and the pulse were all normal. There was no abdominal bruit. Cystoscopic examination revealed gradual flow of massive bloody urine from the right
415
416
YAZAKI AND ASSOCIATES
ureteral orifice. Excretory urography (IVP) showed no visualization of the right pelvis with a hazy nephrogram (fig. 1, A). Retrograde pyelography revealed uneven contour of the right renal pelvis and ureter with unequal opacification of contrast
material compatible with the presence of blood clots (fig. 1, B). Selective renal angiography was performed 13 days after hospitalization (fig. 2). A concentric beading appearance was seen at the right main renal artery, which could not be
FIG. 2. Selective right renal arteriogram (posteroanterior projection). A, note inferior phrenic artery arising from right main renal artery and widened pelvioureteral branch supplying area of arteriovenous fistula. B, nephrotic phase.
FIG. 3. A, right retrograde pyelogram 21 days after patient was hospitalized revealed well visualized contour ofright renal calices and pelvis. B, IVP 5 days later revealed delayed visualization of right renal pelvis.
417
CONGENITAL RENAL ARTERIOVENOUS FISTULA
diagnosed roentgenologically as atheromatous change, fibromuscular dysplasia or both. So-called small, widened, tortuous and aneurysmal vessels could be visualized in an area supplied by the dorsal branch at the lower pole of the right kidney and, even in the arterial phase, an abnormal venous drainage was observed from the area that was strongly suspected of arteriovenous fistula. Neither pathologic vessels, pooling nor tapering could be seen otherwise. Two additional catheters were introduced through both femoral veins and were advanced upward until the tips reached both renal veins, respectively. Blood specimens from both renal veins were taken and oxygen saturation difference was measured by a thermistor. Oxygen saturation was 18 per cent higher on the right side simple subtraction. An IVP 26 after hospitalization showed delayed but slight visualization of the right renal pelvis, and shape and contour of the right renal calices and pelvis were normal retrograde pyelography 4 previously 3). Gross hematuria disappeared after 7 of hospitalization. The was discharged from the hospital on September 4, 36 after without surgical treatment because of remarkable improvement in the general condition. observation at the outpatient clinic is so far non-contributory. uuHH00,
REVIEW OF LITERATURE
We collected and reviewed all relevant .Japanese literature on renal arteriovenous fistula. Since Ito and associates de-
scribed the first case in 1964, 38 cases have been reported. 6 Of these 38 cases 20 have been congenital. Age. Patients ranged in age from 19 to 68 years in all cases and from 21 to 68 years in the congenital cases. Mean age was 40 years for all cases and 42 years for the congenital cases (table 1). Sex. Of the 38 pa Lien ts 10 were men, 26 were women and sex was not known in 2. Of the 20 congenital cases 4 were men, 15 were women and the sex was not known in 1 ( table 2). Side of inuoluement. Of the total 38 cases the right side was involved in 20, the left side in 17 and the side was not known in 1. Of the 20 congenital cases the right side was involved in 12 and the left side in 8 (table 3). Localization of the lesion. Of the total cases the upper was involved in 9, the middle in 9 and the lower in 9. The location was not in 11 cases. Of the congenital cases the upper pole was involved in 8, the middle pole in 5 and the lower pole in 4. The location was not described in 3 (table Of the total 38 cases 20 were congenital, being the most frequent type in In 1964 Maldonado and associates collected 35 cases and described 5 (14 per cent) as congenital in origin, 7 while in 1971 McAlhany and associates collected 118 cases and classified 32 (27 per cent) as either congenital or idiopathic. 8 The second most frequent etiology in Japan is neoplasms (table 5). Chief complaints. Gross hematuria was the predominant
L Ai;e distribution
TABLE
Age
TABLE
Total Cases
Congenital Cases
No.1%1
No. (%)
(yrs.)
0-9 10-19 20-29 :io-:ig 40-49
0 1
0 (0) 0 (OJ 5 (25) 6 (:JO)
(0) (2.6)
9 (2:J.4) 10 (27.2) 6 (15.6)
(5)
50-:,9
8 (20 8)
5 (2ii)
60-69 Unknown
:J
2
Totals
(7.8) (2.6)
l
38
(10) (fi)
5. Etiology of renal arteriovenous fistula No.
20 (5:J.2)
Congenital '.\feoplasm Renal biopsy Aneurysm, ruptured Unknown Traumatic Angiography Tuberculosis Not described
8
2 2 2
1 (2.6) 1 (2.6) 6 ( 15.6)
Total
38
TABLE
TABLE
TABLE
6. Chief complaints
Sex
Total Cases
Congenital Cases
No.(%)
No. 1%)
10 (26) 26(68.8) 2 (,5.2)
4 (20) 15 (75) 1 (5)
3. Side of involvement
Right Left Unknown
(7.8) (5.21 (5.2) (5 2) (2.6)
20
TABLE 2.
Men Women Unknown
('ii
Total Cases
Congenital
No. 1%)
No. ('~I
20 (5:J.2) 17(44.2) 1 (2 6)
12 (60) 8 (40) 0 (0)
Cases
Total Cases
Cases
No.(%) Hematuria Flank pain Mass Hypertension Bruit over abdomen Fever Headache Heaviness in the head Abdominal discomfort General malaise Limb fatigue Palpitation Febrile sensation in the left flank Nausea Bladder tamponade
4. Localization
(5.21 17 8) (2.6)
16 (80) :J ( 15) (5) (5) (5)
(fi.2) (7.8)
(5) (5)
(54.6)
21
4 (104)
2
:J 2
:J 1
2
1 1
:1
TABLE
Total Cases
Congenital
(2 6) (2.6) (2.6) (2.6) (5 21 (2.6)
(2.6) (7.8)
1 (5) 2 (101
7. Treatment
Congenital Cases
Total Cases
Congenital Cases
----·-----
Upper pole Middle pole Lmver pole - - - -
-----
No. (0;'. l
No. l''ll
9 U:U)
8 (40)
::\Jephrectom:y
21
(c,5.8)
15 (15)
9 12:141
5 125) 4 (20)
Partial nephrectomy
:J (7 8) lU (~fi.U ( 10.,·;)
:1 ( 15)
---- -------------·
·--
--
------------
l\io. (',)
Non-operative
2 ( 10) 0
418
YAZAKI AND ASSOCIATES
complaint in patients with congenital disease. Flank pain and urinary retention owing to the presence of blood clots are the next 2 most frequent complaints in the congenital cases, while in the non-congenital cases cardiovascular complaints are believed to be predominant. In our collected cases this is not remarkably manifested (table 6). Treatment. Of 38 patients 21 underwent nephrectomy, 10 had non-operative treatment, 3 had partial nephrectomy and 4 are not described. Non-operative treatment is classified into 3 categories, that is medical treatment, followup observation only and patient's refusal. Regarding the congenital type, 15 patients underwent nephrectomy, 2 had partial nephrectomy and 3 were started on non-operative treatment, while in the non-congenital type non-operative treatment is slightly predominant over the nephrectomy by 1 case (table 7). DISCUSSION
The purposes of the treatment are amelioration of the cardiovascular disturbances secondary to renal arteriovenous fistula and therapy for gross hematuria and its subsequent complications. Total nephrectomy was the treatment of choice until 1957 when Edsman reported the first case of partial nephrectomy. • In those days routine and precise angiographic diagnosis of the involved area was not performed, and once the side of the involved kidney was diagnosed the total nephrectomy was relatively easy and the results were better than other methods. As the angiographic diagnosis became popularized and performed with frequency, the precise location of the involved area was not difficult to determine before the operation. Methods for preservation of as much renal parenchyma as possible were sought and applied clinically in the early 1960s. 10 • 11 These methods should be applied to as many patients as possible except for those with renal arteriovenous fistula secondary to hypernephroma. In 1962 Boijsen and Kohler reported the first successful case of ligation of the arterial branches supplying the fistula. 2 In 1973 Bookstein and Goldstein treated their patient with selective arterial embolization of autologous blood clot into a large post-biopsy renal arteriovenous fistula and produced complete closure of the fistula and immediate cessation of hematuria. 12 In this case the patient's condition was too poor to tolerate an operation. The concept of therapeutic arterial embolization was not new at that time but this was the first reported case applied to a renal arteriovenous fistula. In 1973 Rizk and associates reported a similar method for the treatment of post-biopsy renal arteriovenous fistula with intractable gross hematuria by catheter embolization of 4 leadshots and subcutaneous fat into a feeding artery successfully. 13 These authors prescribed 2 possible complications that might militate against catheter embolization of the supplying arterial branches-an approximate 5 to 10 per cent risk of producing infarction of 1 kidney and the resultant infarct could cause and/or deteriorate renal hypertension. Followup observation also is necessary for asymptomatic patients with renal arteriovenous fistula and for patients who refuse surgical treatment. With the growing number of renal biopsies the incidence of post-biopsy renal arteriovenous fistula has been increasing in
recent years. Although this complication is usually asymptomatic and self-limited, 14 followup study should be contemplated when such cases are encountered. Emphasis on followup observation in patients without surgical treatment is the enlargement in size of arteriovenous fistula and cardiovascular manifestations with elapse in time. In 1971 Lang and associates reported that in 1 case an acute traumatic fistula enlarged during a 2-month interval. 15 In 1973 Kostiner and Burnett also reported 1 patient with more than twice the increase in size of a non-traumatic renal arteriovenous fistula during an 8-year interval. 16 After nephrectomy the transverse cardiac diameter decreased by 3 cm. in 24 hours and blood pressure reverted from 180/100 to 150/80 mm. Hg. To the best of our knowledge only 2 such cases have been described in the literature. We propose that every physician who encounters patients with a renal arteriovenous fistula should weigh the decision before undertaking treatment. REFERENCES
1. Varela, M. C.: Aneurisma arteriovenoso de los vasos renales y Asistolia consecutiva. Rev. Med. Latino Amer., 14: 3244, 1923.
2. Boijsen, E. and Kohler, R.: Renal arteriovenous fistulae. Acta Radio!., 57: 433, 1962. 3. Maekawa, M.: Renal arteriovenous fistula: case report, review of literature and new classification. Jap. J. Urol., 59: 837, 1968. 4. Takaha, M., Sonoda, T., Uchida, H. and Ishida, 0.: Congenital renal arteriovenous fistula due to vascular malformation: report of three cases. Jap. J. Urol., 63: 539, 1972. 5. Morita, I. and Hirata, K.: Congenital arteriovenous fistula of kidney. A case report and review of the literature. Nishinihon J. Urol., 35: 553, 1973. 6. Ito, K., Nishimura, Y. and Kitagawa, R.: Renal arteriovenous fistula with chief complaints of hematuria. Jap. J. Urol., 55: 1092, 1964. 7. Maldonado, J. E., Sheps, S. G., Bernatz, P. E., DeWeerd, J. H. and Harrison, E.G., Jr.: Renal arteriovenous fistula: a reversible cause of h:•pertension and heart failure. Amer. J. Med., 37: 499, 1964. 8. McAlhany, J. C., Jr., Black, H. C., Jr., Hanback, L. D., Jr. and Yarbrough, D.R., III: Renal arteriovenous fistula as a cause of hypertension. Amer. J. Surg., 122: 117, 1971. 9. Edsman, G.: Angionephrography and suprarenal angiography. Acta Radio!., suppl. vol. 155, p. 9, 1957. 10. Palmer, J.M. and Connolly, J.E.: Intrarenal arteriovenous fistula: surgical excision under selective renal hypothermia with kidney survival. J. Urol., 96: 599, 1966. 11. Cosgrove, M. D., Mendez, R. and Morrow, J. W.: Branch artery ligation for renal arteriovenous fistula. J. Urol., 110: 632, 1973. 12. Bookstein, J. J. and Goldstein, H. M.: Successful management of postbiopsy arteriovenous fistula with selective arterial embolization. Radiology, 109: 535, 1973. 13. Rizk, G. K., Atallah, N. K. and Bridi, G. I.: Renal arteriovenous fistula treated by catheter embolization. Brit. J. Radio!., 46: 222, 1973. 14. Bennett, A. R. and Wiener, S. N.: Intrarenal arteriovenous fistula and aneurysm. A complication of percutaneous renal biopsy. Amer. J. Roentgen., 95: 372, 1965. 15. Lang, E. K., Trichel, B. E., Turner, R. W., Fontenot, R. A., Johnson, B. and St. Martin, E. C.: Renal arteriography in the assessment of renal trauma. Radiology, 98: 103, 1971. 16. Kostiner, A. I. and Burnett, L. L.: Intrarenal arteriovenous fistula: documented increase in size during an eight-year interval in one case and surgical treatment with renal salvage in another. Radiology, 109: 531, 1973.