Arteriovenous Fistula of Renal Vessels: '

Report of a Case Believed to be Congenital and Review of the Literature

LEROY LONG,** M.D., HUSHANG JAVID, M.D., ORMAND C. JULIAN, M.D.

From the Department of Surgery, University of Illinois Research and EducationalHospitals, Chicago, Illinois

AT LEAST 31 case reports of intra- or pre-renal fistula between the artery and veinhave been reported.', 2, 4-15, 17-24, 26-32 Thisreport adds a new case and reviews theclassification, etiology, symptoms, patho-physiology, diagnosis, treatment, and prog-nosis of similar lesions.

Case ReportA white man, 24 years of age, was first ad-

mitted to the University of Illinois Hospital onMay 11, 1960 for evaluation of headaches, blurredvision, and hypertension. At age 14 he sustaineda bullet wound in the left flank with a 22 caliberrifle. Thirteen inches of small bowel were re-sected at the operative repair of this injury. Fiveweeks later a second celiotomy was performed forintestinal obstruction caused by an adhesive band.

One and one half years prior to admission, in1960, he began to experience a frontal headacheand blurred vision. There was also one episodeof gross hematuria, but no other urinary symp-toms. Hypertension was first noted three monthsprior to admission.

Nutrition and development were normal. Bloodpressure in the arms was 220/140 and 300/150in the legs. Keith-Wagner Grade II retinopathywas present. There was moderate cardiac enlarge-ment discernible to percussion and a Grade II/VIsystolic murmur was heard along the left sternalborder. The remainder of the physical examinationwas normal. Auscultation of the abdomen, flank,or back was not mentioned.

Urine contained two plus albumin and numer-ous red and white cells. Serum electrolytes werenormal. Blood urea nitrogen was 30 mg. per cent,creatinine 1.5 mg. per cent and creatinine clear-ance 111 cubic centimeters per minute. Electro-

cardiogram showed left ventricular hypertrophy.Urinary catecholamines in a 24 hour specimenwere normal. Regitine test was equivocal. Entero-cocci were cultured from the urine.

Cardiac contour as seen by x-ray showedprominence of the left ventricle and of the aorta.Anterior-posterior and lateral x-ray films of theabdomen showed a bullet just lateral to the lefttransverse process and posterior to the body ofthe first lumbar vertebra. The earliest film of theintravenous pyelogram series (Fig. 1) was made60 seconds after intravenous injection of the dye.It showed that both kidneys promptly excretedcontrast material. The right renal calyces weresomewhat less well visualized than the left.This finding and the view of the kidneys seenon retrograde pyelogram were thought not to besignificant.

A Howard test showed equal excretion of so-dium from both kidneys, but the volume ofurine excreted by the right was one half that bythe left kidney.

Right renal biopsy on June 2, 1960 showedchanges compatible with early vascular nephrop-athy. Biopsy of the left kidney was not successful.On June 23, 1960 retrograde femoral aortogramwas attempted. This was unsatisfactory, as wasa subsequent translumbar aortogram. Because ofrecurrence of urinary infection with enterococcihe was discharged to be followed in the clinic.

His second hospital admission on February 28,1961 was for the purpose of repeating the aorto-gram to visualize the renal arteries. Physical andlaboratory findings were unchanged except thatthe urine was free of erythrocytes and leukocytes,and urine cultures were negative.

Another intravenous pyelogram was done whichshowed no change. On March 8, 1961 left femoralarteriotomy was performed and a Number 12cardiac catheter passed into the aorta. Four filmswere taken using 70 per cent Diodrast, 25 cubiccentimeters, injected by hand, for each film (Fig.2). These films were taken about 20 minutesapart. A narrow, but normal, left renal artery was

239

* Submitted for publication August 26, 1963.** Present address: 1111 N. Lee, Oklahoma

City, Oklalhoma 73103.

240 LONG, JAVID AND JULIAN

FIG. 1. Intravenous pyelogram with prompt

excretion at 60 seconds. Note poor visualization of

the right kidney and bullet near the left kidney.

visualized. The right renal artery, however, ledto numerous dilated vascular spaces.

At this time a continuous bruit over a smallarea in the right costo-vertebral angle was firstdescribed.

Transabdominal operation on the right kid-ney was carried out on April 5, 1961. There was

a palpable thrill over the lower two thirds of thekidney and pulsations were visible in the mark-edly distended right renal vein. The right renalartery was unremarkable. Nephrectomy was per-formed.

The kidney weighed 180 grams and measured12 x 6 and x 3 centimeters. The intrarenalportion of the artery was dilated and large sac-

culations were present. At least two communica-tions were found between it and the renal vein(Fig. 3, 4). These measured two and five milli-meters in diameter.

A few hyalinized glomeruli were seen in thesections, but most were normal. Some cloudyswelling of the tubules was present, and therewere a few hyaline casts in the tubules. There was

also a moderate chronic inflammatory cell infil-trate in the interstitial tissue.

Recovery was uneventful, however, The BloodPressure Remained at Preoperative Levels. He was

discharged on the tenth postoperative day with-out medication.

One month after nephrectomy he was re-

admitted because of hypertensive crisis. Bloodpressure was 250/150 and pulse 100. He com-

plained of headache and blurred vision. Fundo-

Annals of SurgeryAugust 1964

scopic revealed many retinal hemorrhages andexudates bilaterally, but no papilledema.

The urine contained many hyaline and finelygranular casts. Complete blood count was normalexcept for hematocrit of 28 per cent. The bloodurea nitrogen was 43 mg.%, creatinine 3.9 mg.%o.

He was given Chlorothiazide (Diuril) andReserpine. His blood pressure had decreased to170/110 on the fourth day, and to 140/90 atthe time of discharge.

He has been reexamined at intervals as anoutpatient during the two years since operation.One month after leaving the hospital the retinalexudates disappeared. His treatment has beenchlorthalidone (Hygroton) 200 mg. and guane-thidine sulfate (Ismelin) 12-25 mg. per day.Blood pressures recorded on each visit have beenon the order of 170/118 supine and 138/110standing. He works every day as a postman,carrying a 35-pound mail bag without anydifficulty. He has had no headaches or visualdifficulties.

Discussion

Classification and Etiology. Since periph-eral arteriovenous fistula does not causehypertension, most authors have separatedpost nephrectomy renal vessel fistulae fromfistulae present with the kidney still inplace. Such a fistula can be congenital, trau-matic, or secondary to primary adenocarci-noma. One case secondary to infection hasbeen reported.14

Fistulae following trauma and carcinomaare easily understood. The congenital lesionis, however, less clear. It would appear thatin most such cases the primary lesion is apre-existing aneurysm of the renal arterywhich may be congenital. This erodes therenal vein directly or ruptures and the re-sulting false aneurysm breaks into the vein.Advocates of this theory cite as evidencelong survival before symptoms, and therelatively frequent occurrence of renal ar-tery aneurysm. "

Symptoms. Most authors emphasize hy-pertension, increase in heart size, varyingdegrees of cardiac failure, and a localizedcontinuous bruit. Scheifley25 has gone sofar as to classify these symptoms as repre-senting a renal arteriovenous fistula syn-

Volume 160 ARTERIOVENOUS FISTNumber 2

drome. Most, however, are not so categori-cal. Abbott, for example, does not believethat there is any characteristic form ofhypertension or syndrome associated withrenal arteriovenous fistula.'

In reviewing 29 patients in whom bothetiology and presenting symptoms areknown (Table 1) it becomes apparent thatfistulae occurring suddenly, i.e., traumaticand carcinomatous, are those most likelyto present with an enlarged heart, cardiacfailure, and bruit, while only about one

'UL.A OF RENAL VESSELS 241

third of those of the congenital type havesimilar manifestations. The patient withcongenital fistula is more likely to havehematuria. Hypertension, shock, renal colic,and bruit account for the presenting symp-toms in the remaining cases.

Pathologic Physiology. Post nephrectomyrenal vessel fistula initially acts as anyother peripheral AV fistula. The immediatechanges brought about include fall in sys-tolic and diastolic blood pressure, increasein pulse rate, increase in venous pressure

4...........:

FIG. 2. Retrograde aortogram. Four separate twenty-five cubic centimeter hand injections of 70 percent Diodrast- 20 minutes between each injection. Note the dilated vascular spaces in the right kidneyin all films, suggestion of dye in the inferior vena cava, normal left renal artery, visualization of thecollecting system in later films, and bullet in the region of the left kidney.

-1.NI: ...

I

242 LONG, JAVID AND

FIG. 3. Right kidney with renal artery open.Note two probes (arrows) passing into the dilatedvascular spaces from the renal vein.

FIG. 4. Renal vein with probe passing throughfistula from renal artery.

JULIAN Annals of SurgeryAugust 1964

TABLE 1. Preserzing Symptoms in 29 Patients*

EtiologyPresentingSymptoms Congenital Trauma Carcinoma

Heart, failure, bruit 5 (33%) 7 (70%) 3 (60%)Hematuria 7 (47%) 1 1Hypertension** 1 1 0Shock 0 0 1Renal colic 1 0 0Bruit 1 0 0

15 9 5

* In three patients either etiology or presentingsymptoms not known.

** Hypertension is defined as level over 150/90.

both proximal and distal to the fistula, andincrease in cardiac output.

Later there is an increase in blood vol-ume, gradual dilation of the heart and ves-sels proximal to the fistula, developmentof collateral circulation, hypertrophy of theheart, and return of the systolic blood pres-sure to previous or higher level with thediastolic pressure remaining low.3

It is probable that hypertension in ar-teriovenous fistula of the renal vessels withthe kidney in place is a result of the Gold-blatt phenomenon. This aspect of the prob-lem has been well covered by Milloy.17There is apparently a decrease in totalblood flow, systolic and diastolic bloodpressure and pulse pressure distal to thefistula. The difference between AV fistula

TABLE 2. Sixteen Hypertensive* Patients inWhom Etiology is Known

Etiology

Blood Pressure Congenital Trauma Carcinoma

Drop to normal 6 (86%)** 6 (100%)*** 1after operation

No change after 1 0 2operation

7 6 3

* Hypertension is defined as level over 150/90.** One patient had systolic hypertension only.

* Three patients had systolic hypertension only.

Volume 160 ARTERIOVENOUS FISTULA OF RENAL VESSELS 243Number 2

and the Goldblatt kidney is that the latteris produced by constriction of the artery,the vein not being affected, while in theformer there is decreased distal arterialflow without constriction and increased ve-nous pressure."'

Diagnosis. The two most consistent diag-nostic aids have been the presence of acontinuous bruit in the region of the kid-ney and arteriography.Blood volume, cardiac output and sys-

temic pulse pressure are increased. Circula-tion time is usually decreased. Arterialblood in the renal vein is also diagnostic.Intravenous pyelography and retrogradestudies are sometimes helpful, and mayshow compression of the renal pelvis orcalyces or both. Differential excretion stud-ies have not been helpful.

Treatment. The goal in treatment isconservation of renal tissue. In only twocases,8, 30 however, has any treatment shortof complete nephrectomy been possible. Inthese two, partial nephrectomy was done.Both were congenital lesions. In those sec-ondary to carcinoma it is obvious that totalnephrectomy is the only procedure.

Prognosis. Table 2 indicates that if hyper-tension is present, there is an excellentchance for return to normotensive levels(80%). Interestingly, even though hyper-tension persists, response to medication isbetter once the fistula has been removed.The patient reported is such a case.

Summary

Renal arteriovenous fistulae are classifiedas congenital, secondary to trauma, to carci-noma, or to infection. Congenital lesionsmay arise from pre-existing renal arteryaneurysm which erodes the renal vein.These lesions usually produce hypertension,probably by the Goldblatt phenomenon.Diagnosis can be made from the history ofsudden heart failure or hematuria coupledwith a continuous bruit in the flank or ab-

domen. Confirmation by arteriography isnecessary. Treatment has usually been ne-phrectomy, but renal tissue can be con-served in some instances. Hypertension isusually alleviated following operation.

References1. Abbott, C. E. and E. F. Poutasse: Renal Ar-

teriovenous Fistula: Occurrence in RenalCell Carcinoma: Report of a Case. Cleve.Clin. Quart., 28:283, 1961.

2. Adams, H. D.: Congenital Arteriovenous andCirsoid Aneurysms. Surg., Gynec. and Obst.,92:693, 1951.

3. Allen, E. V., N. W. Barker and E. A. Hines:Peripheral Vascular Diseases. Philadelphia,W. B. Saunders Company, 3rd Edition, 1962.

4. Baron, G. J. and R. H. Koenemann: Arterio-venous Fistula of Renal Vessels: Case Re-port. Radiology, 64:85, 1955.

5. Bohne, A. W. and G. L. Henderson: Intra-renal Arteriovenous Aneurysm. Case Report.J. Urol., 77:818, 1957.

6. Camp, F. B.: Personal communication to E. P.Lasher and F. Glenn. Effects on Kidney andBlood Pressure of Artificial CommunicationBetween Renal Artery and Vein. Arch. Surg.,38:886, 1939.

7. Chevereau-Reignier. Personal Communicationto Authors of Reference 19.

8. Edsman, G.: Angionephrography and Supra-renal Angiography: a Roentgenologic Studyof the Normal Kidney, Expansive Renal andSuprarenal Lesions and Renal Aneurysms.Acta Radiol. Suppl., 155:110, 1957.

9. Grace, J. T., W. Staubitz, F. Lessmann andR. Egan: Intrarenal Arteriovenous Fistula.Arch. Surg., 81:718, 1960.

10. Hamilton, G. R., R. J. Getz and S. Jerome:Arteriovenous Fistula of Renal Vessels: CaseReport and Review of Literature. J. Urol.,69:203, 1953.

11. Hoffman, H. A. and H. Fontoura: Arterio-venous Aneurysm of the Kidney. J. Intemat.Coll. Surg., 29:729, 1958.

12. Hutch, J. A. and E. R. Chisholm: IntrarenalArteriovenous Fistula. J. Urol., 88:150, 1962.

13. Jantet, G. H., E. C. Foot and J. R. Kenyon:Rupture of an Intrarenal Arteriovenous Fis-tula Secondary to Carcinoma: Case Report.Brit. J. Surg., 49:404, 1962.

14. Jouve, A., P. Augier, H. Payan, R. Gerard,J. L. Medvedowsby and J. Guillemaud: LesCommunications Arterio-veineuses Renales.Presse Med., 66:1669, 1958.

244LONG, JAVID AND JULIAN Annals of Surgery244 LN,JVDAugust 196415. Kirby, C. K., W. G. Nichols, A. P. Garritano,

G. T. Wohl and A. L. Pietroluongo: Arterio-venous Fistula or Renal Vessels: Case Re-port. Surg., 37:267, 1955.

16. Lasher, E. P. and F. Glenn: Effects on Kid-ney and Blood Pressure of Artificial Com-munication Between Renal Artery and Vein.Arch. Surg., 38:886, 1939.

17. Milloy, F., Jr., E. H. Fell, R. F. Dillon andA. M. Zavas: Intra-renal Arteriovenous Fis-tula with Hypertensive Cardiovascular Dis-ease. Review of the Literature. Am. J. Surg.,96:3, 1958.

18. Myhre, J. R.: Arteriovenous Fistula of theRenal Vessels: Case Report. Circ., 14:185,1956.

19. Nguyen-Huu, Ngo-Gia-Hy and Bui-Mong-Hung.: Aneurysmes Arterioveineux des Vais-seaux Renaux, Rein en Place, leur role dansL'hypertension Arterielle d'origine Renale.Presse Med., 67:1680, 1959.

20. Pearse, R. and R. L. MacMillan: CongenitalArteriovenous Aneurysm of the Renal Artery.J. Urol., 58:235, 1947.

21. Pelot, G., G. Pessereau and B. Daftari: Hyper-tensions Malignes et Nephropathies Unilate-rales; 'a propos det 4 Observations. J. Urol.Med. Par., 60:245, 1954.

22. Puchlew, Von A.: Ztachr. f. Kreislaufforsch.,34:665, 1942.

23. Rieder, W.: Sonderstellung arterio-ven6seraneurysmen der nierengefasse im rahmenoperativer behandlung schwerer herzkreis-

laufachaden beim arterio-venbsen aneurysma.Der Chirurg., 14:609, 1942.

24. Sauter, K. E. and J. W. Sargent: SpontaneousRupture of Intrarenal Arteriovenous Fistula:Case Report. J. Urol., 83:17, 1960.

25. Scheifley, C. H.: New Clinical Syndrome Pro-ducing Hypertension-Arteriovenous Fistulaof the Kidney. J. Am. Med. Assoc., 174:1625, 1960.

26. Scheifley, C. H., G. W. Daugherty, L. F.Greene and J. T. Priestley: ArteriovenousFistula of the Kidney: New Observationsand Report of Three Cases. Circ., 19:662,1959.

27. Schulze-Bergmann, G.: Ober das arterio-venoseaneurysm der niere. Ztschr. Urol., 47:661,1954.

28. Slominski-Laws, M. D., J. H. Kiefer and C. W.Vermeulen: Arteriovenous Aneurysm of theKidney: Case Report. J. Urol., 75:586, 1956.

29. Thomas, R. G., S. Grieve and B. Lewin: Spon-taneous Renal Arteriovenous Fistula andContralateral Renal Artery Aneurysm. Brit.J. Radiol., 35:128, 1962.

30. Twigg, H. L., R. Pradham and J. K. Perloff:Arteriovenous Fistula of the Renal Vessels.Am. J. Roentgen., 88:1148, 1962.

31. Varela, M. E.: Aneurisma Arteriovenosos delos Vasos Renales y Asistolia Consecutiva.Rev. med. Latino-Am., 14:3244, 1923.

32. Vest, S. A.: Renal Arteriovenous Fistula.Urologists' Correspondence Club Letter.December 6, 1954.