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Intrarenal Arteriovenous Fistula: Surgical Excision under Selective Renal Hypothermia with Kidney Survival
THE
J Ol:RYAL
OF UROLOGY
Vol. 06,
Copyright© 19[i6 by The Williams & Wilkins Co
1-'rinted in U.S.A..
IKTRARENAL ARTERIOVENOUS FISTULA: SURGICAL EXCIS10i\ UNDER SELECTIVE RE::\:AL HYPOTHERJ\IIA WITH KIDNEY S17RVIVAL JOHN J\L PALMER Fron1 the
AND
JOHN E. CONNOLLY
Stanford Univer8it;u School of Medicine, Palo A/to, Ca/i.forniu
The diagnosis of intrarenal arteriovenous fistula was made by Varela in 1923. 1 Since then 44 additional cases have been reported. Although over four decades have elapsed since Varela's original. description, half of these case reports have been made in the last 3 years. No doubt this apparent inuease in incidence has resulted from the greater use of angiography as a diagnostic tool, as well as from an increased a.wareness of the lesion itself. In only 3 recorded instances has the surgical treatment of intrarenal arteriovenous fistula been other than complete or partial nephrectomy. 1Ve wish to report a fourth such experience in which selective renal hypothermia was employed to permit removal of the fistula with pre3ervation of the kidney. CASE REPORT
R. W. (S.:\ICGH No. 047211), a 26-year-old white man, was admitted to San .:\fateo County General Hospital on February 13, 1962. He had suffered 10 hours of right flank pain and had sought medical advice after the passage of bloody urine with clots. The blood pressure was normal. Abdominal examination reveal.eel muscular resistance with marked right costovertebral angle tenderness. The urine was grossly bloody but otherwise normal on urinalysis. The hemoglobin was 13.5 gm. per cent, and the white cell count was 12,800 with 54 polymorphonuclear and 10 banded forms. Laboratory analysis showed the blood urea nitrogen to be 10 mg., the serum creatinine 1.3 mg., the uric acid 6.5 mg., the calcium 10 mg., and the phosphorus 4 mg. per cent. An ASTO titer was 166 Todd units. A urine culture was sterile. The patient had been hospitalized the previous year for a similar episode when a bloody effiux had been noted from the right ureteral orifice. Although at that time an excretory urogra.m had Accepted for publication December 2, 1965. 1 11' arela, J\!I. C.: Aneurisma arteriovenoso de los vasos renales y Asist.olia consecutiva. Rev. Med. Latino Amer., 14: 3244, 1923.
indicated no almormalitic.o, a similar examination performed on this current admission revealed delayed right nephrogram without visuali;mti,>n of the collecting system. vVhcn cysto.ocopy performecl the, next day, the bladclor ww, found to be filled with coagulated bloocl. Nu intrin:,ic: lesion was evident within the bladder, and blood was noted once again to be coming from the urctc:ral orifice. A right retrograde demonstrated multiple renal pelvic filling defects with irregular deformity and blunting of the calye es (fig. 1, A). No malignant cell~ were found in urine from the right kidney. One week btcr another excretory urogram rc:vcaled a right kidney which lackecl the previously noted filling defects and the mild calyccal dilatation (fig. 1, B). A retrograde femoral arteriogmm was performed on February 23. The renal arterie,'; appeared normal on both sides. However, within the parenchyrna of the right kidney, a dilated collection of small vascular structures was seen to be originating from a lower pole branch of !.he main renal artery (fig. 2). The vascular malformatiou measured 1.5-2 cm. in diameter and io be rapidly drained by a large vein (fig. 2). Because the hematuria soon ceased, and the flank subsided, surgical intervention. was deferred. The patient was readmitted on October eight months after discharge, because of an sode of massive total hematuria with clots which had begun 3 hours earlier. During this admission the patient observed neither renal colic, nor flank pain. Examination of the abdomen revealed no vascular bruit or murmur. vealed for the third time a bloody effiux from the right ureteral orifice, and excretory urography again demonstrated irregular filling defects sug;gesting blood clots within the right collecting system. The hematocrit on admission was 47 per cent. Although the hematuria stopped after hours, it recurred on the seventh hospital day, and the hematocrit subsequently fell. to 32 per cent. On November 9, 1962, the patient wa,'i taken 599
600
PALMER AND CONNOLLY
FIG. 1. A, right retrograde ureteropyelogram (February 14, 1962). Multiple calyceal and pelvic filling defects are present. B, excretory urogram on February 20. Previously noted filling defects have disappea.red. Mild caliectasis is present and right kidney fails to concentrate well at 10 minutes.
FIG. 2. A, retrograde femoral arteriograrn on February 23. Faint opacification is noted within parenchyma of right kidney representing arteriovenous fistula (arrow). B, in later film malformation appears to be originating from lower polar branch of main renal artery. Contrast material is now faintly seen to outline right renal vein (arrow). This can also be seen in A..
INTRARENAL AHTEHIOVENOUS FISTULA
GOJ
.FIG. 3. Photomicrograph of surgical specimen. Thickened vessel to right contains an internal elastic layer and represents arterial component. No communication was demonstrated between this structun· and vein at lower left. N ervc tissue present at top is often found in association with these lesions.
FIG. 4. A, retrograde femoral arteriogram on seventeenth day after resection of arteriovenous fistula. :V[alformation is no longer seen. There is absfmce of nephrngram in medial portion of riglit lower pole and nephrogram is clearly denser in untouched upper portion of kidney. B, IO-minute excretory urogrnrn April 7, 19G5, two and one-lmlf years afterremoval of arteriovenous fistula. There is cnlyccal blunting and irregular deformit3· of renal pelvis nnd lower pole still shows decreased ability to concentrate. There is prompt appearance of contrast on right side however, and kidney appears to lrnvc ndcquatc function. No hypertrophy of left kidney has occurrncl.
602
PALMER AND CONNOLLY
to surgery for definitive renal exploration.* Since resection of the arteriovenous fistula was anticipated, the use of selective renal hypothermia seemed indicated as a proven method of protecting function during total renal ischemia. 2 A right subcostal incision was made; the renal artery and vein were dissected free and were isolated with umbilical tapes. A sterile plastic bagt was inverted over the kidney and was invaginated on itself after loose fixation about the renal pedicle. The bag was filled with a sterile mixture of ice and water. The renal artery and the ureter were occluded separately with vascular clamps, and the kidney was cooled for 20 minutes. At the end of the cooling period the renal core temperature was 3C, and the selective cooling bag was removed. The kidney was then wrapped in moist abdominal packs, and dissection into the renal hilum was begun along the lower pole segmental artery. The vascular malformation was identified in the middle third of the kidney close to the renal hilum. Feeding vessels to the area were divided and secured with 4-0 plain catgut ligatures, and the small malformation was removed. The kidney was closed in the usual fashion. After the clamps had been removed from the renal artery and the ureter, the kidney promptly regained color. The time of total renal ischemia was 123 minutes. The renal core temperature had remained below 20C for the first 60 minutes and had slowly risen to 25C during the second hour. The tissue removed from the kidney was composed of irregular segments of blood vessel wall, and appeared compatible with the diagnosis of vascular malformation, although no communication was demonstrated (fig. 3). The postoperative course was initially uneventful. However, on the seventh postoperative day, the patient experienced massive hematuria and during the next 8 days required 2 liters of blood replacement. On the tenth day after operation, an excretory urogram showed no excretion of dye from the operated kidney. An arteriogram on the seventeenth postoperative day revealed right renal arterial spasm and absence of right lower pole nephrogram but a functioning right kidney (fig. 4, A). The patient was dismissed on the * William Mills, Jr., M.D. and one of the authors
(J.M. P.) performed the operation. 2 Palmer, J.M., Guernsey, J.M. and Connolly, J. E.: An experimental study of selective renal hypothermia. Amer. J. Surg., 106: 224, 1963. t Travenol Laboratories, Inc., Morton Grove, Illinois, U.S.A.
twenty-first postoperative day. His recovery was complicated by another brief bleeding episode 2 weeks after discharge which did not require transfusion. He was last seen on April 7, 1965, two and onehalf years after removal of the renal arteriovenous fistula. Blood pressure: was 112/75 mm. Hg. Urinalysis revealed no protein; urine pH was 5.5; an occasional white blood cell was seen per high power field, and no red blood cells were present. A urine culture was sterile. Although an excretory urogram demonstrated definite right renal calyceal deformity, it also indicated prompt excretion of dye from the operated kidney and no impairment of nephrogram at the site of the nephrotomy (fig. 4, B). The patient was in good health and has suffered no hematuria since December 1962. DISCUSSION
The terms congenital or idiopathic arteriovenous fistula should be reserved for lesions arising in a vascular malformation. Many of these fistulas originate as circoid aneurysms,3 which resemble small hemangioma-like lesions. According to Willis,4 such malformations were included in Albrecht's original designation of hamartoma in 1904. On the other hand, "acquired" intrarenal arteriovenous fistulas have been described in association with neoplasia or as a result of penetrating trauma or surgery. In some instances, arterial aneurysms may erode into renal veins and lead to fistula formation. Although these latter should probably be classified as acquired lesions, it is often difficult to separate them from the congenital variety. When these lesions evolve within renal parenchyma, they may erode the collecting system and may cause hematuria. Pain may develop from intraparenchymal bleeding. If the fistula interferes with the blood supply to a distal segment, hypertension can result from renal ischemia. If the fistula reaches sufficient size, systemic effects of arteriovenous communication may ensue as outlined by Holman. 5 In some instances, these lesions cause no discomfort to the patient.3 • 6 • 7 3 Boijsen, E. and Kohler, R.: Renal arteriovenous fistulae. Acta Radiol., 67: 433, 1962. 4 Willis, R. A.: Pathology of Tumors. St. Louis: C. V. Mosby Co., 1948. 6 Holman, E.: The anatomic and physiologic effects of an arteriovenous fistula. Surgery, 8:
362, 1940. 6
Currie, J. C., Thomas, M. L. and Pinniger,
INTHARENAL AHTERIOVEJ\iOUS FD,;TULA TABLE !
Symptoms & Signs
Xame and Year
Yarela 1 1923 Hieder, 8 1942 Pearse and ;\focMillau,9 1947 ,\dams, 10 1951 Hamilton and associates.11 1953 Pelot and associates," 1ri5+ Schuhe-Bergman, 13 195<1. Yest,1 4 Kirby ;ind associates,1 5 1955 Baron and Koeueman, 16 1955 Slominski-Laws and associates, 17
Etiology
Treatmr:nt
High blood pressure, heart failure Hypertension, heart failnre Hematuria, hypertensi(J]1
CongeJJi1,a.l
Ac1topsy fill(liug
Congcni ta! Cougcnital
Hypertension, heart failure Hypertension, heart failure Hematuria Hematuria Hema,tuna, heart failure Hypertensiou Heart failure
NeopL1stic Traumatic Congenital Postoperative ? Traumatic Traumatic Congenital
K ephrectomy Nephreclomv N ephrectorny N ephrnctomy N ephrect om,,· N ephrectorny N ephrcct omy Nephrcctorny N ephrectomy N ephrectmn,·
Hernaturia, pai11 Hematuria, hear1 failure, pain Hematuria Hematuria, heart failurn Hypertension, heart failure Hematuria Hematuria
l\feoplastic Congenital
N cpbrectomy Nephrectomy
Congcuital Congenital Traumatic Congenital Congenital
Partial nephrectomy N ephrect omy N ephrectumy N eph rnctomy N cphrectomy
1956
i\Iyhre, 18 1956 Bohne and Hendersou ,1 9 HJ57 Edsman, 20 1957 Jouve and associates, 21 1958 .:\Iilloy and associates, 22 1958 Hoffman and Fontoura, 23 1958 Nguyen-Hun ;ind associate;c;, 24 1959
Scheifley and associates, 25 1959 Grace and associates, 26 HJ60 Sauter and Sargent, 27 1960 Abbott and Poutasse, 28 1961 Thomas and associates, 29 1962 ,fantet and associates, 30 HJG2 Boijsen and Kiihler, 3 Boijsen and Kijh]er, 3 1962 Boijsen r111d Kohler,3 19G2 Boijsen and Kiihler, 3 19(52
Hematuria Hypertensio11 .Pain Hypertension, heart failure Hematuria Hematuria, pain Pain, hematuria None Il ypertension
Neoplastic Co11geni1al Congenital Neoplasti,, Congenital Neoplastic, ? Congenital Cougenital Ileirnl biopsy Henal biopsy
Boijsen and Kiihler, 3 1962 Twigg and associates, 31 HJ62
Hematuria IIematuria
? Cougenital Congeni (,al
Hutch and Chisholm, 32 1902 J\langiardi and associates, 33 1963 Blake and associates, 34 1963 Blake and associates, 31 l9G3 Feinberg and Goldberg, 35 1963 ·woodruff and Phalakornkule, 36
Hematuria, pain Pain No added symptoms No added symptoms Hypertension, heart fail me None
CongeuitaJ Postoperative Renal biopsy Heual biopsy Congenital Congenital
N ephrcctomy N ephrectomy N ephrectomy N ephrnctomy N ephrectomy N cphrectomy N ephrectomv N ephrcctomy Partial nephrec,tom:v Branch ·fl rtery 1ig:1 tiou N ephrectomy Fistnla removed through nephrot omv N ephrecturny N ephrectumy NoJJe None N epbrectomy N ephrectomy
None None Pain
CongeJJital Postoperative Congenital
N ephrectomy Nepbrnctorny FistulH, removed
1'11aldonado and associates, 39 Long and associates, 40 1964 Crummy and associates,' 196-1 Crummy and associates, 7 1965 Hiley, 41 HJ65
Heart failure Hypertension Mild hypertension None No added symptoms
Neoplastic Traumatic Congenital Congenital Renal biopsy
1Vilkey and associates,4 2 1965 Wilkey and associates, 42 1965
Hematuria, pain Hypertension, heart failure
Traumatic Congenital
1964
Currie and associates, 0 19G4 Snodgrass and Hohinson, 37 1964 1Vaterhouse and associates, 38 1964
J. L.: Congenital i11trarenal arteriovenous fistula. Brit. J, Surg., 51: 40, 196±. 7 Crnmm~', A. B., Jr., Atkinson, IL J, alld
No treatment, Arterial ligat,io11 wi lh segmental llephrectomy N ephrectomy N ephrectomy
Caruthers, S. B., Jr.: Congeuit,a,l renal n,rterio· venous fistulas. J. Urol., 93: 24, HJG.S. 8 llieder, W. Souderstelling artcrio-vcniiser
604
PALMER AND CONNOLLY
The table lists all reported intrarenal arteriovenous fistulas and includes presenting symptoms, suspected etiology, and treatment of the lesions. The most common presenting symptom was hematuria (18 cases). Thirteen had arterial hypertension, and 11 patients suffered from heart failure. Eight patients had arterial hypertension associated with cardiac decompensation. Of the aneurysmen der Nierengefasse im Rahmen operativer Behandlung schwerer Herz-Kreislaufschaden beim Arteriovenosen Aneurysma. Chirurg., 14: 609, 1942. 9 Pearse, R. and MacMillan, R. L.: Congenital arteriovenous aneurysm of the renal artery. J. Urol., 58: 235, 1947. 10 Adams, H. D.: Congenital arteriovenous and cirsoid aneurysms. Surg., Gynec. & Obst., 92: 693, 1951. 11 Hamilton, G. R., Getz, R. J. and Jerome, S.: Arteriovenous fistula of renal vessels: Case report and review of literature. J. Urol., 69: 203, 1953. 12 Pelot, G., Pesserau, G. and Daftare, B.: Hypertensions malignes et nephropathies unilaterales. Apropos de 4 observations. J. d'Urol., 60: 245, 1954. .. 13 Schulze-Bergman, G.: Uber das arterio-venose Aneurysm der Niere. Ztschr. Urol., 47: 661, 1954. 14 Vest, S. A.: Renal arteriovenous fistula postnephrolithotomy. Urological Correspondence Club letter, December 6, 1954. 15 Kirby, C. K., Nichols, W. G., Garritano, A. P., Wohl, G. T. and Petroluongo, A. L.: Arteriovenous fistula of the renal vessels. A case report. Surgery, 37: 267, 1955. 16 Baron, G. J. and Koeneman, R.H.: Arteriovenous fistula of the renal vessels. Radiology, 64: 85, 1955. 17 Slominski-Laws, M. D., Kiefer, J. H. and Vermeulen, C. W.: Arteriovenous aneurysm of the kidney: Case report. J. Urol., 75: 586, 1956. 18 Myhre, J. R.: Arteriovenous fistula of the renal vessels; a case report. Circulation, 14: 185, 1956. 19 Bohne, A. W. and Henderson, G. L.: Intrarenal arteriovenous aneurysm: Case report. J. Urol., 77: 818, 1957. 20 Edsman, G.: Angionephrography and suprarenal angiography. Acta. Radiol. Stock Suppl., 155: 110, 1957. 21 Jouve, A., Angier, P., Payan, H., Gerard, R., Medvedowsky, J. L. and Guillemaud, J.: Arteriovenous communication of the renal vessels. Presse Med., 66: 1669, 1958. 22 Milloy, F., Jr., Fell, E. H., Dillon, R. F. and Zayas, A. M.: Intrarenal arteriovenous fistula with hypertensive cardiovascular disease. Amer. J. Surg., 96: 3, 1958. 23 Hoffman, H. A. and Fontoura, H.: Arteriovenous aneurysm of the kidney. J. Int. Coll. Surgeons, 29: 729, 1958. 24 Nguyen-Huu, Ngo-Gia-Hy and Bui-MongHung: Aneurysmes arterioveineux des vaisseaux renaux. Presse Med., 67: 1680, 1959. 25 Scheifley, C. H., Daugherty, G. W., Greene, L. F. and Priestley, J. T.: Arteriovenous fistula of the kidney. New observations and report of three cases. Circulation, 19: 622, 1959.
45 cases included in the table, 25 cases were considered congenital in nature. Eleven cases developed after renal trauma; 5 fistulas resulting from needle biopsy of the kidney are included in this group. Six fistulas were found associated with renal carcinoma, and 3 fistulas resulted from previous surgery on the involved kidney. Abdominal bruit or murmur is the only physical finding of specific diagnostic aid for arteriovenous fistula. However, urography and angiography are neces26 Grace, J. T., Staubitz, W., Lessman, F. and Egan, R.: Intrarenal arteriovenous fistula. A.M.A. Arch. Surg., 81: 718, 1960. 27 Sauter, K. E. and Sargent, J. W.: Spontaneous rupture of intrarenal arteriovenous fistula; report of a case ..J. Urol., 83: 17, 1960. 28 Abbott, C. E. B. and Poutasse, E. F.: Renal arteriovenous fistula: occurrence in renal cell carcinoma. Cleveland Clin. Quart., 28: 283, 1961. 29 Thomas, R. G., Grieve, S. and Lewin, B.: Spontaneous renal arteriovenous fistula and contralateral renal artery aneurysm. Brit. J. Radiol., 35: 128, 1962. 30 Jantet, G. H., Foot, E. C. and Kenyon, J. R.: Rupture of intrarenal arteriovenous fistula secondary to carcinoma; a case report. Brit. J. Surg., 49: 404, 1962. 31 Twigg, H. L., Pradhan, R. and Perloff, J. K.: Arteriovenous fistula of the renal vessels: A case report. Amer. J. Roentgenol., 88: 1148, 1962. 32 Hutch, J. A. and Chisholm, E. R.: Intrarenal arteriovenous fistula. J. Urol., 88: 150, 1962. 33 Mangiardi, J. L., Santemma, E. E. and Sullivan, J. J., Jr.: Arteriovenous fistula of the kidney. Amer. Heart J., 65: 549, 1963. 34 Blake, S., Heffernan, S. and McCann, P.: Renal arteriovenous fistula after percutaneous renal biopsy. Brit. Med. J., 1: 1458, 1963. 35 Feinberg, S. B. and Goldberg, M. E.: Arteriovenous communication of the kidney in a patient with hypertension. Report of a case in which diagnosis was suggested by routine pyelography. Radiology, 81: 601, 1963. 36 Woodruff, M. W. and Phalakornkule, S.: Asymptomatic intrarenal arteriovenous fistula. J. Urol., 92: 103, 1964. 37 Snodgrass, W. T. and Robinson, M. J.: Intrarenal arteriovenous fistula: A complication of partial nephrectomy. J. Urol., 91: 135, 1964. 38 Waterhouse, K., Wesolowski, S. A. and McGowan, A. J., Jr.: Intrarenal arteriovenous fistula: Surgical treatment with salvage of the kidney. J. Urol., 92: 256, 1964. 39 Maldonado, J. E., Sheps, S. G., Bernatz, P. E., DeWeerd, J. H. and Harrison, E.G., Jr.: Renal arteriovenous fistula: A reversible cause of hypertension and heart failure. Amer. J. Med., 37: 499, 1964. 40 Long, L., Javid, H. and Julian, 0. C.: Arteriovenous fistula of renal vessels. Ann. Surg., 160: 239, 1964. 41 Riley, J. M.: Renal arte:i;iovenous fistula: A complication of percutaneous renal biopsy. J. Urol., 93: 333, 1965. ,, ' 42 Wilkey, J. L., Rowe, F. H., Brown, J. and Gersack, J.: Intrarenal arteriovenous fistula. J. Urol., 93: 663, 1965.
IN'l'RARENAL AR'l'EHIOVENOUS FIS'l'lTLA
sary for precise localization of the lesion. ,c\s emphasized by Scheifley and associates,'" measurements of cardiac output, circulation time, and venous oxygen saturation are of considerable value in the study of large communications, although not obligatory. The treatment of intrarenal arteriovenous fistula has almost uniformly been nephrectomy. The first instance of renal salvage was reported in 1957 by Eds1nan, 20 who performed partial nephrectomy for this lesion. Boijsen and Kiihler3 reported 5 cascR of intrarenal fistula in 1962, and in two of these the kidney was saved. In one of these patients, partial nephrectorny was clone. In the other, a segmental renal artery was ligated, resulting in avascularity of the middle third of the kidney as demonstrated on subsequent angiography. Twigg and associates utilizing hypotherniia,31 and Y\T aterhouse and associates 38 both succeeded in removing the enlarged fistulous sac and ligating the vascular communications in their cases with no apparent parenchy1nal damage. Riley 41 reported a case treated by distal renal artery ligation with segmental nephrectomy. In our patient, renal survival seems to have been favorably influem:ed by the use of selective renal hypothermia, although its role is admittedly difficult to evaluate. The prolonged time of total
(i0!5
renal i,,chemia apparently resulted in a period uf' acute tubular neeni,,is as evidenced by phy on the tenth day, although obstruction from blood dot was not ruled out. 1-Io,n'ver, tlw right kidney was functioning well by the ~cYenteenUi day. Adequate human datum on the duration of total renal isdiemia compatible with renal srn·vival is difficult to accumulate. 8tarzl 4" had pointed out that periods of ischcmia or exceeding 1 hour generally lead to failure in renal transplantations. Since this case excccclcd that limit by an additional hour, it seems that selective renal hypothermia pla.rnd a role in the salvage of the operated kidney, ciUMMARY
An additional case of intrarenal ar1.erip,·en011s fistula is reported with a review of the litenitLu·c pcrtt1ining to thi,, unu:;ual lesion. The an thorn, case appears to be the fourth in~tance in which surgieal excision of the fotuh wa~ undertaken without removal uf renal pan:n chyma. Selective renal hypothermia ,,aJnable role in the resulting surTival of the operated kidney. Ntar½l, T. E. · Experic11ee in Henal Tnrnsplrrntat.ion. Philadelphia: vV. B. Snnndcrs Com pany, l(JG4.
J Ol:RYAL
OF UROLOGY
Vol. 06,
Copyright© 19[i6 by The Williams & Wilkins Co
1-'rinted in U.S.A..
IKTRARENAL ARTERIOVENOUS FISTULA: SURGICAL EXCIS10i\ UNDER SELECTIVE RE::\:AL HYPOTHERJ\IIA WITH KIDNEY S17RVIVAL JOHN J\L PALMER Fron1 the
AND
JOHN E. CONNOLLY
Stanford Univer8it;u School of Medicine, Palo A/to, Ca/i.forniu
The diagnosis of intrarenal arteriovenous fistula was made by Varela in 1923. 1 Since then 44 additional cases have been reported. Although over four decades have elapsed since Varela's original. description, half of these case reports have been made in the last 3 years. No doubt this apparent inuease in incidence has resulted from the greater use of angiography as a diagnostic tool, as well as from an increased a.wareness of the lesion itself. In only 3 recorded instances has the surgical treatment of intrarenal arteriovenous fistula been other than complete or partial nephrectomy. 1Ve wish to report a fourth such experience in which selective renal hypothermia was employed to permit removal of the fistula with pre3ervation of the kidney. CASE REPORT
R. W. (S.:\ICGH No. 047211), a 26-year-old white man, was admitted to San .:\fateo County General Hospital on February 13, 1962. He had suffered 10 hours of right flank pain and had sought medical advice after the passage of bloody urine with clots. The blood pressure was normal. Abdominal examination reveal.eel muscular resistance with marked right costovertebral angle tenderness. The urine was grossly bloody but otherwise normal on urinalysis. The hemoglobin was 13.5 gm. per cent, and the white cell count was 12,800 with 54 polymorphonuclear and 10 banded forms. Laboratory analysis showed the blood urea nitrogen to be 10 mg., the serum creatinine 1.3 mg., the uric acid 6.5 mg., the calcium 10 mg., and the phosphorus 4 mg. per cent. An ASTO titer was 166 Todd units. A urine culture was sterile. The patient had been hospitalized the previous year for a similar episode when a bloody effiux had been noted from the right ureteral orifice. Although at that time an excretory urogra.m had Accepted for publication December 2, 1965. 1 11' arela, J\!I. C.: Aneurisma arteriovenoso de los vasos renales y Asist.olia consecutiva. Rev. Med. Latino Amer., 14: 3244, 1923.
indicated no almormalitic.o, a similar examination performed on this current admission revealed delayed right nephrogram without visuali;mti,>n of the collecting system. vVhcn cysto.ocopy performecl the, next day, the bladclor ww, found to be filled with coagulated bloocl. Nu intrin:,ic: lesion was evident within the bladder, and blood was noted once again to be coming from the urctc:ral orifice. A right retrograde demonstrated multiple renal pelvic filling defects with irregular deformity and blunting of the calye es (fig. 1, A). No malignant cell~ were found in urine from the right kidney. One week btcr another excretory urogram rc:vcaled a right kidney which lackecl the previously noted filling defects and the mild calyccal dilatation (fig. 1, B). A retrograde femoral arteriogmm was performed on February 23. The renal arterie,'; appeared normal on both sides. However, within the parenchyrna of the right kidney, a dilated collection of small vascular structures was seen to be originating from a lower pole branch of !.he main renal artery (fig. 2). The vascular malformatiou measured 1.5-2 cm. in diameter and io be rapidly drained by a large vein (fig. 2). Because the hematuria soon ceased, and the flank subsided, surgical intervention. was deferred. The patient was readmitted on October eight months after discharge, because of an sode of massive total hematuria with clots which had begun 3 hours earlier. During this admission the patient observed neither renal colic, nor flank pain. Examination of the abdomen revealed no vascular bruit or murmur. vealed for the third time a bloody effiux from the right ureteral orifice, and excretory urography again demonstrated irregular filling defects sug;gesting blood clots within the right collecting system. The hematocrit on admission was 47 per cent. Although the hematuria stopped after hours, it recurred on the seventh hospital day, and the hematocrit subsequently fell. to 32 per cent. On November 9, 1962, the patient wa,'i taken 599
600
PALMER AND CONNOLLY
FIG. 1. A, right retrograde ureteropyelogram (February 14, 1962). Multiple calyceal and pelvic filling defects are present. B, excretory urogram on February 20. Previously noted filling defects have disappea.red. Mild caliectasis is present and right kidney fails to concentrate well at 10 minutes.
FIG. 2. A, retrograde femoral arteriograrn on February 23. Faint opacification is noted within parenchyma of right kidney representing arteriovenous fistula (arrow). B, in later film malformation appears to be originating from lower polar branch of main renal artery. Contrast material is now faintly seen to outline right renal vein (arrow). This can also be seen in A..
INTRARENAL AHTEHIOVENOUS FISTULA
GOJ
.FIG. 3. Photomicrograph of surgical specimen. Thickened vessel to right contains an internal elastic layer and represents arterial component. No communication was demonstrated between this structun· and vein at lower left. N ervc tissue present at top is often found in association with these lesions.
FIG. 4. A, retrograde femoral arteriogram on seventeenth day after resection of arteriovenous fistula. :V[alformation is no longer seen. There is absfmce of nephrngram in medial portion of riglit lower pole and nephrogram is clearly denser in untouched upper portion of kidney. B, IO-minute excretory urogrnrn April 7, 19G5, two and one-lmlf years afterremoval of arteriovenous fistula. There is cnlyccal blunting and irregular deformit3· of renal pelvis nnd lower pole still shows decreased ability to concentrate. There is prompt appearance of contrast on right side however, and kidney appears to lrnvc ndcquatc function. No hypertrophy of left kidney has occurrncl.
602
PALMER AND CONNOLLY
to surgery for definitive renal exploration.* Since resection of the arteriovenous fistula was anticipated, the use of selective renal hypothermia seemed indicated as a proven method of protecting function during total renal ischemia. 2 A right subcostal incision was made; the renal artery and vein were dissected free and were isolated with umbilical tapes. A sterile plastic bagt was inverted over the kidney and was invaginated on itself after loose fixation about the renal pedicle. The bag was filled with a sterile mixture of ice and water. The renal artery and the ureter were occluded separately with vascular clamps, and the kidney was cooled for 20 minutes. At the end of the cooling period the renal core temperature was 3C, and the selective cooling bag was removed. The kidney was then wrapped in moist abdominal packs, and dissection into the renal hilum was begun along the lower pole segmental artery. The vascular malformation was identified in the middle third of the kidney close to the renal hilum. Feeding vessels to the area were divided and secured with 4-0 plain catgut ligatures, and the small malformation was removed. The kidney was closed in the usual fashion. After the clamps had been removed from the renal artery and the ureter, the kidney promptly regained color. The time of total renal ischemia was 123 minutes. The renal core temperature had remained below 20C for the first 60 minutes and had slowly risen to 25C during the second hour. The tissue removed from the kidney was composed of irregular segments of blood vessel wall, and appeared compatible with the diagnosis of vascular malformation, although no communication was demonstrated (fig. 3). The postoperative course was initially uneventful. However, on the seventh postoperative day, the patient experienced massive hematuria and during the next 8 days required 2 liters of blood replacement. On the tenth day after operation, an excretory urogram showed no excretion of dye from the operated kidney. An arteriogram on the seventeenth postoperative day revealed right renal arterial spasm and absence of right lower pole nephrogram but a functioning right kidney (fig. 4, A). The patient was dismissed on the * William Mills, Jr., M.D. and one of the authors
(J.M. P.) performed the operation. 2 Palmer, J.M., Guernsey, J.M. and Connolly, J. E.: An experimental study of selective renal hypothermia. Amer. J. Surg., 106: 224, 1963. t Travenol Laboratories, Inc., Morton Grove, Illinois, U.S.A.
twenty-first postoperative day. His recovery was complicated by another brief bleeding episode 2 weeks after discharge which did not require transfusion. He was last seen on April 7, 1965, two and onehalf years after removal of the renal arteriovenous fistula. Blood pressure: was 112/75 mm. Hg. Urinalysis revealed no protein; urine pH was 5.5; an occasional white blood cell was seen per high power field, and no red blood cells were present. A urine culture was sterile. Although an excretory urogram demonstrated definite right renal calyceal deformity, it also indicated prompt excretion of dye from the operated kidney and no impairment of nephrogram at the site of the nephrotomy (fig. 4, B). The patient was in good health and has suffered no hematuria since December 1962. DISCUSSION
The terms congenital or idiopathic arteriovenous fistula should be reserved for lesions arising in a vascular malformation. Many of these fistulas originate as circoid aneurysms,3 which resemble small hemangioma-like lesions. According to Willis,4 such malformations were included in Albrecht's original designation of hamartoma in 1904. On the other hand, "acquired" intrarenal arteriovenous fistulas have been described in association with neoplasia or as a result of penetrating trauma or surgery. In some instances, arterial aneurysms may erode into renal veins and lead to fistula formation. Although these latter should probably be classified as acquired lesions, it is often difficult to separate them from the congenital variety. When these lesions evolve within renal parenchyma, they may erode the collecting system and may cause hematuria. Pain may develop from intraparenchymal bleeding. If the fistula interferes with the blood supply to a distal segment, hypertension can result from renal ischemia. If the fistula reaches sufficient size, systemic effects of arteriovenous communication may ensue as outlined by Holman. 5 In some instances, these lesions cause no discomfort to the patient.3 • 6 • 7 3 Boijsen, E. and Kohler, R.: Renal arteriovenous fistulae. Acta Radiol., 67: 433, 1962. 4 Willis, R. A.: Pathology of Tumors. St. Louis: C. V. Mosby Co., 1948. 6 Holman, E.: The anatomic and physiologic effects of an arteriovenous fistula. Surgery, 8:
362, 1940. 6
Currie, J. C., Thomas, M. L. and Pinniger,
INTHARENAL AHTERIOVEJ\iOUS FD,;TULA TABLE !
Symptoms & Signs
Xame and Year
Yarela 1 1923 Hieder, 8 1942 Pearse and ;\focMillau,9 1947 ,\dams, 10 1951 Hamilton and associates.11 1953 Pelot and associates," 1ri5+ Schuhe-Bergman, 13 195<1. Yest,1 4 Kirby ;ind associates,1 5 1955 Baron and Koeueman, 16 1955 Slominski-Laws and associates, 17
Etiology
Treatmr:nt
High blood pressure, heart failure Hypertension, heart failnre Hematuria, hypertensi(J]1
CongeJJi1,a.l
Ac1topsy fill(liug
Congcni ta! Cougcnital
Hypertension, heart failure Hypertension, heart failure Hematuria Hematuria Hema,tuna, heart failure Hypertensiou Heart failure
NeopL1stic Traumatic Congenital Postoperative ? Traumatic Traumatic Congenital
K ephrectomy Nephreclomv N ephrectorny N ephrnctomy N ephrect om,,· N ephrectorny N ephrcct omy Nephrcctorny N ephrectomy N ephrectmn,·
Hernaturia, pai11 Hematuria, hear1 failure, pain Hematuria Hematuria, heart failurn Hypertension, heart failure Hematuria Hematuria
l\feoplastic Congenital
N cpbrectomy Nephrectomy
Congcuital Congenital Traumatic Congenital Congenital
Partial nephrectomy N ephrect omy N ephrectumy N eph rnctomy N cphrectomy
1956
i\Iyhre, 18 1956 Bohne and Hendersou ,1 9 HJ57 Edsman, 20 1957 Jouve and associates, 21 1958 .:\Iilloy and associates, 22 1958 Hoffman and Fontoura, 23 1958 Nguyen-Hun ;ind associate;c;, 24 1959
Scheifley and associates, 25 1959 Grace and associates, 26 HJ60 Sauter and Sargent, 27 1960 Abbott and Poutasse, 28 1961 Thomas and associates, 29 1962 ,fantet and associates, 30 HJG2 Boijsen and Kiihler, 3 Boijsen and Kijh]er, 3 1962 Boijsen r111d Kohler,3 19G2 Boijsen and Kiihler, 3 19(52
Hematuria Hypertensio11 .Pain Hypertension, heart failure Hematuria Hematuria, pain Pain, hematuria None Il ypertension
Neoplastic Co11geni1al Congenital Neoplasti,, Congenital Neoplastic, ? Congenital Cougenital Ileirnl biopsy Henal biopsy
Boijsen and Kiihler, 3 1962 Twigg and associates, 31 HJ62
Hematuria IIematuria
? Cougenital Congeni (,al
Hutch and Chisholm, 32 1902 J\langiardi and associates, 33 1963 Blake and associates, 34 1963 Blake and associates, 31 l9G3 Feinberg and Goldberg, 35 1963 ·woodruff and Phalakornkule, 36
Hematuria, pain Pain No added symptoms No added symptoms Hypertension, heart fail me None
CongeuitaJ Postoperative Renal biopsy Heual biopsy Congenital Congenital
N ephrcctomy N ephrectomy N ephrectomy N ephrnctomy N ephrectomy N cphrectomy N ephrectomv N ephrcctomy Partial nephrec,tom:v Branch ·fl rtery 1ig:1 tiou N ephrectomy Fistnla removed through nephrot omv N ephrecturny N ephrectumy NoJJe None N epbrectomy N ephrectomy
None None Pain
CongeJJital Postoperative Congenital
N ephrectomy Nepbrnctorny FistulH, removed
1'11aldonado and associates, 39 Long and associates, 40 1964 Crummy and associates,' 196-1 Crummy and associates, 7 1965 Hiley, 41 HJ65
Heart failure Hypertension Mild hypertension None No added symptoms
Neoplastic Traumatic Congenital Congenital Renal biopsy
1Vilkey and associates,4 2 1965 Wilkey and associates, 42 1965
Hematuria, pain Hypertension, heart failure
Traumatic Congenital
1964
Currie and associates, 0 19G4 Snodgrass and Hohinson, 37 1964 1Vaterhouse and associates, 38 1964
J. L.: Congenital i11trarenal arteriovenous fistula. Brit. J, Surg., 51: 40, 196±. 7 Crnmm~', A. B., Jr., Atkinson, IL J, alld
No treatment, Arterial ligat,io11 wi lh segmental llephrectomy N ephrectomy N ephrectomy
Caruthers, S. B., Jr.: Congeuit,a,l renal n,rterio· venous fistulas. J. Urol., 93: 24, HJG.S. 8 llieder, W. Souderstelling artcrio-vcniiser
604
PALMER AND CONNOLLY
The table lists all reported intrarenal arteriovenous fistulas and includes presenting symptoms, suspected etiology, and treatment of the lesions. The most common presenting symptom was hematuria (18 cases). Thirteen had arterial hypertension, and 11 patients suffered from heart failure. Eight patients had arterial hypertension associated with cardiac decompensation. Of the aneurysmen der Nierengefasse im Rahmen operativer Behandlung schwerer Herz-Kreislaufschaden beim Arteriovenosen Aneurysma. Chirurg., 14: 609, 1942. 9 Pearse, R. and MacMillan, R. L.: Congenital arteriovenous aneurysm of the renal artery. J. Urol., 58: 235, 1947. 10 Adams, H. D.: Congenital arteriovenous and cirsoid aneurysms. Surg., Gynec. & Obst., 92: 693, 1951. 11 Hamilton, G. R., Getz, R. J. and Jerome, S.: Arteriovenous fistula of renal vessels: Case report and review of literature. J. Urol., 69: 203, 1953. 12 Pelot, G., Pesserau, G. and Daftare, B.: Hypertensions malignes et nephropathies unilaterales. Apropos de 4 observations. J. d'Urol., 60: 245, 1954. .. 13 Schulze-Bergman, G.: Uber das arterio-venose Aneurysm der Niere. Ztschr. Urol., 47: 661, 1954. 14 Vest, S. A.: Renal arteriovenous fistula postnephrolithotomy. Urological Correspondence Club letter, December 6, 1954. 15 Kirby, C. K., Nichols, W. G., Garritano, A. P., Wohl, G. T. and Petroluongo, A. L.: Arteriovenous fistula of the renal vessels. A case report. Surgery, 37: 267, 1955. 16 Baron, G. J. and Koeneman, R.H.: Arteriovenous fistula of the renal vessels. Radiology, 64: 85, 1955. 17 Slominski-Laws, M. D., Kiefer, J. H. and Vermeulen, C. W.: Arteriovenous aneurysm of the kidney: Case report. J. Urol., 75: 586, 1956. 18 Myhre, J. R.: Arteriovenous fistula of the renal vessels; a case report. Circulation, 14: 185, 1956. 19 Bohne, A. W. and Henderson, G. L.: Intrarenal arteriovenous aneurysm: Case report. J. Urol., 77: 818, 1957. 20 Edsman, G.: Angionephrography and suprarenal angiography. Acta. Radiol. Stock Suppl., 155: 110, 1957. 21 Jouve, A., Angier, P., Payan, H., Gerard, R., Medvedowsky, J. L. and Guillemaud, J.: Arteriovenous communication of the renal vessels. Presse Med., 66: 1669, 1958. 22 Milloy, F., Jr., Fell, E. H., Dillon, R. F. and Zayas, A. M.: Intrarenal arteriovenous fistula with hypertensive cardiovascular disease. Amer. J. Surg., 96: 3, 1958. 23 Hoffman, H. A. and Fontoura, H.: Arteriovenous aneurysm of the kidney. J. Int. Coll. Surgeons, 29: 729, 1958. 24 Nguyen-Huu, Ngo-Gia-Hy and Bui-MongHung: Aneurysmes arterioveineux des vaisseaux renaux. Presse Med., 67: 1680, 1959. 25 Scheifley, C. H., Daugherty, G. W., Greene, L. F. and Priestley, J. T.: Arteriovenous fistula of the kidney. New observations and report of three cases. Circulation, 19: 622, 1959.
45 cases included in the table, 25 cases were considered congenital in nature. Eleven cases developed after renal trauma; 5 fistulas resulting from needle biopsy of the kidney are included in this group. Six fistulas were found associated with renal carcinoma, and 3 fistulas resulted from previous surgery on the involved kidney. Abdominal bruit or murmur is the only physical finding of specific diagnostic aid for arteriovenous fistula. However, urography and angiography are neces26 Grace, J. T., Staubitz, W., Lessman, F. and Egan, R.: Intrarenal arteriovenous fistula. A.M.A. Arch. Surg., 81: 718, 1960. 27 Sauter, K. E. and Sargent, J. W.: Spontaneous rupture of intrarenal arteriovenous fistula; report of a case ..J. Urol., 83: 17, 1960. 28 Abbott, C. E. B. and Poutasse, E. F.: Renal arteriovenous fistula: occurrence in renal cell carcinoma. Cleveland Clin. Quart., 28: 283, 1961. 29 Thomas, R. G., Grieve, S. and Lewin, B.: Spontaneous renal arteriovenous fistula and contralateral renal artery aneurysm. Brit. J. Radiol., 35: 128, 1962. 30 Jantet, G. H., Foot, E. C. and Kenyon, J. R.: Rupture of intrarenal arteriovenous fistula secondary to carcinoma; a case report. Brit. J. Surg., 49: 404, 1962. 31 Twigg, H. L., Pradhan, R. and Perloff, J. K.: Arteriovenous fistula of the renal vessels: A case report. Amer. J. Roentgenol., 88: 1148, 1962. 32 Hutch, J. A. and Chisholm, E. R.: Intrarenal arteriovenous fistula. J. Urol., 88: 150, 1962. 33 Mangiardi, J. L., Santemma, E. E. and Sullivan, J. J., Jr.: Arteriovenous fistula of the kidney. Amer. Heart J., 65: 549, 1963. 34 Blake, S., Heffernan, S. and McCann, P.: Renal arteriovenous fistula after percutaneous renal biopsy. Brit. Med. J., 1: 1458, 1963. 35 Feinberg, S. B. and Goldberg, M. E.: Arteriovenous communication of the kidney in a patient with hypertension. Report of a case in which diagnosis was suggested by routine pyelography. Radiology, 81: 601, 1963. 36 Woodruff, M. W. and Phalakornkule, S.: Asymptomatic intrarenal arteriovenous fistula. J. Urol., 92: 103, 1964. 37 Snodgrass, W. T. and Robinson, M. J.: Intrarenal arteriovenous fistula: A complication of partial nephrectomy. J. Urol., 91: 135, 1964. 38 Waterhouse, K., Wesolowski, S. A. and McGowan, A. J., Jr.: Intrarenal arteriovenous fistula: Surgical treatment with salvage of the kidney. J. Urol., 92: 256, 1964. 39 Maldonado, J. E., Sheps, S. G., Bernatz, P. E., DeWeerd, J. H. and Harrison, E.G., Jr.: Renal arteriovenous fistula: A reversible cause of hypertension and heart failure. Amer. J. Med., 37: 499, 1964. 40 Long, L., Javid, H. and Julian, 0. C.: Arteriovenous fistula of renal vessels. Ann. Surg., 160: 239, 1964. 41 Riley, J. M.: Renal arte:i;iovenous fistula: A complication of percutaneous renal biopsy. J. Urol., 93: 333, 1965. ,, ' 42 Wilkey, J. L., Rowe, F. H., Brown, J. and Gersack, J.: Intrarenal arteriovenous fistula. J. Urol., 93: 663, 1965.
IN'l'RARENAL AR'l'EHIOVENOUS FIS'l'lTLA
sary for precise localization of the lesion. ,c\s emphasized by Scheifley and associates,'" measurements of cardiac output, circulation time, and venous oxygen saturation are of considerable value in the study of large communications, although not obligatory. The treatment of intrarenal arteriovenous fistula has almost uniformly been nephrectomy. The first instance of renal salvage was reported in 1957 by Eds1nan, 20 who performed partial nephrectomy for this lesion. Boijsen and Kiihler3 reported 5 cascR of intrarenal fistula in 1962, and in two of these the kidney was saved. In one of these patients, partial nephrectorny was clone. In the other, a segmental renal artery was ligated, resulting in avascularity of the middle third of the kidney as demonstrated on subsequent angiography. Twigg and associates utilizing hypotherniia,31 and Y\T aterhouse and associates 38 both succeeded in removing the enlarged fistulous sac and ligating the vascular communications in their cases with no apparent parenchy1nal damage. Riley 41 reported a case treated by distal renal artery ligation with segmental nephrectomy. In our patient, renal survival seems to have been favorably influem:ed by the use of selective renal hypothermia, although its role is admittedly difficult to evaluate. The prolonged time of total
(i0!5
renal i,,chemia apparently resulted in a period uf' acute tubular neeni,,is as evidenced by phy on the tenth day, although obstruction from blood dot was not ruled out. 1-Io,n'ver, tlw right kidney was functioning well by the ~cYenteenUi day. Adequate human datum on the duration of total renal isdiemia compatible with renal srn·vival is difficult to accumulate. 8tarzl 4" had pointed out that periods of ischcmia or exceeding 1 hour generally lead to failure in renal transplantations. Since this case excccclcd that limit by an additional hour, it seems that selective renal hypothermia pla.rnd a role in the salvage of the operated kidney, ciUMMARY
An additional case of intrarenal ar1.erip,·en011s fistula is reported with a review of the litenitLu·c pcrtt1ining to thi,, unu:;ual lesion. The an thorn, case appears to be the fourth in~tance in which surgieal excision of the fotuh wa~ undertaken without removal uf renal pan:n chyma. Selective renal hypothermia ,,aJnable role in the resulting surTival of the operated kidney. Ntar½l, T. E. · Experic11ee in Henal Tnrnsplrrntat.ion. Philadelphia: vV. B. Snnndcrs Com pany, l(JG4.