THE JOURNAL O.F UROLOGY
Vu!. 76, No. 5, November 1956 Printed in U.S.A.
ANEURYSM OF THE RENAL ARTERY SAMUEL MALISOFF
AND
M. CERRUTI
From the Department of Urology, Beth David Hospital, New York, N. Y.
Aneurysm of the renal artery is a very well known entity which has already been described in excellent papers. It is our purpose to present another case. This lesion is not a frequent pathological finding: 12 cases in 100,421 autopsies (Abeshouse), and only in 21.7 per cent of the cases previously reported the diagnosis was made preopera tively. Age and sex are not important etiological factors in its incidence; the lesion has been found at any age, from 9 months to 82 years and with no predominance in one sex or the other. The primary factor in its pathogenesis is a ·weakening of the arterial wall, mainly of the media. The etiological factor leading to this process is either congenital or acquired. In the first type the similarity ,Yith the congenital aneurysm of the arteries of the brain has been pointed out. Acquired factors are classified as: 1) Degenera ti ve: Atheroma tosis or arteriosclerosis; 2) Inflammatory: Embolus in subacute bacterial endocarditis, syphilis, rheumatic endarteritis, periarteritis nodosa, tuberculosis; 3) Traumatic: Blunt trauma, considered as a precipitating factor acting over an already weakened arterial wall. Stab and gunshot wounds are also to be mentioned. The aneurysm is a localized dilatation of the artery; if in the wall of the aneurysm one or more layers of the artery are present, it is called true aneurysm. Sometimes, either spontaneously or by trauma, a rupture of the aneurysm takes place, or the rupture of a normal or diseased artery, and if the hemorrhage is spontaneously controlled, a so-called false aneurysm may be formed. In this case the wall of the sac is formed by the same extravasated clotted blood and by surrounding tissues, but no elements of a normal artery are found. Calcification is a common finding in the true aneurysm, a fact which is rarely observed in the false aneurysm. In the true aneurysm the saccular, fusiform, dissecting and arteriovenous types have been described. The aneurysm. may increase progressively in size and its complications depend on its volume and location. The more frequent complication is the extravasation of blood; this bleeding may erupt into the renal pelvis, a calyx, or be intracapsular or intraparenchymal. The obstruction of the arterial flow may produce infarction or thrombosis, atrophy or necrosis; the Goldblatt type of ischemic kidney with hypertension has been mentioned. Accepted for publication May 17, 1956. 542
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SYMPTOMATOLOGY
Pain and hematuria are the more frequent symptoms. The pain is not always present, and is variable in its characteristics. Sometimes it is mild and dull and may be also severe and colicky, intermittent, in recurrent attacks. It may be localized in the lumbar region, flank, upper abdomen or lower back; may or may not be associated with other symptoms. Hematuria is a very important sign; it may be minimal, moderate or severe; the microscopic findings may vary from a few red blood cells in the urine, to the acute, massive, sometimes fatal hemorrhage. The clinical picture would vary accordingly. The hematuria has been attributed to erosion or perforation into a calyx or renal pelvis; it also may be due to infarction of the kidney. Generally the hematuria is present in episodes. On palpation of the abdomen a pulsating mass may be felt, sometimes accon1panied by a systolic bruit. The association with hypertension is described in 20 per cent of the cases. Berneike and Pollock found 11 cases out of 56 but in only three cases the blood pressure has been restored to normal after nephrectomy. Other authors also agree that in many cases no definite relationship between renal aneurysm and hypertension was proved. RADIOLOGY
This is the clue in the diagnosis. Generally the sac appears as a circular, calcified ring which as a rule is incomplete (signet ring, wreath-like, etc.). The center is radiolucent representing the lumen of the vessel. If large enough, the kidney, pelvis or calyces may be deformed or displaced. Located usually anterior to the renal pelvis and medial to the upper third of the kidney, its position can be accurately obtained by anterior-posterior and lateral films, and also by intravenous and retrograde pyelogram; but a certain diagnosis is given only by aortography, which shows the relationship between the mass and the renal artery or its branches. The diagnosis is definite when the radiolucent center is filled with the opaque medium. Combined with perirenal insuffiation, the best diagnostic results can be obtained. In spite of its importance in diagnosis, aortography has not become a routine method. In fact, the chances to obtain a positive result are minimal and so far, the opacification of the ring has seldom been demonstrated successfully in the reported cases in the literature. Differential diagnosis has to be done to eliminate renal lithiasis, tumors, tuberculosis, cysts or hematoma of the kidney. Extrarenal conditions, as calcified mesenteric lymph nodes, cholelithiasis, aneurysm of the splenic, hepatic or pancreatic arteries should also be ruled out. The prognosis is bad when the aneurysm is allowed to follow its normal evolution. Death has been reported in all cases where surgery was not performed. When nephrectomy was undertaken, the mortality rate was 6 per cent; with other types of surgical treatment the mortality rate reaches 5;3 per cent (Atkinson).
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S. MALISOFF AND M. CERRUTI TREATMENT
Nephrectomy is the procedure which yields the lowest mortality rates. Some other types of more conservative operations have been performed, such as excision of the aneurysm, resection of the sac with repair of the defect, denervation of the renal pedicle, sympathectomy, release of adhesions, etc. CASE REPORT
B. Z., aged 31 years, male, married, history No. 54-6552, Beth David Hospital. The patient was admitted to Beth David Hospital on November 16, 1954 complaining of recurrent abdominal pain and hematuria. The first episode was in 1946. He was admitted to another hospital with gross hematuria, pain in both flanks, especially the right. Pain was felt also in the lower abdomen, which was severe, steady, and apparently was associated with difficulty in urination. He signed out 15 days after admission, with no definite diagnosis because the studies were inconclusive. During the admission, patient did not require blood transfusion. The blood and urine cultures were negative and he had a fluctuating temperature, at times reaching 106F. At the time of discharge, he was symptom-free and the urine revealed 3-4 red blood cells, was negative for sugar and albumin. In 1950, the second attack of pain occurred, localized over the right costovertebral angle and right flank, severe, steady, nonradiating, of over 10 days' duration. He was kept under medical observation but not hospitalized, for 2 months, and discharged without pain and with no red blood cells in the urine X-ray revealed an enlarged right kidney but no other abnormality (fig. 1, A). It is worthwhile mentioning that in 1943, while in the Army the patient was
Fm. 1. A, excretory urograph;1· (1950) shows slightly enlarged right kidney with blunting of minor calyces and no delineation of infundibulum. B, excretory urography (1954) shows definite evidence of calcified ring shadm1· in region of right renal pelvis.
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in an airplane crash, although no dcfiuite contusion over lumbar region \Ya,-, noted. Repeated medical examinations by the Army after this episode ,rnre negative The one completed early in HJ-lJi included x-ray studies. In Kovember l\lo"½, the patient was hospitalized at Beth David Hospital for complete study due to the presence of pus cells in the mine. The past and family histories ,rnre noncontributory. Physical examination on admission was completely negative. Blood pressure wac; I ::ion 5. Careful examination of the abdomen revealed 110 tendcrHe,m, no masses, 110 tenderness in lumbar regions and auscultation ,nts negative. Cystoscopy re,·ealed a dilatable, mild urethral stricture. An intran)nous injection of indigo carmine returned in a normal amount from both nretPral orifices. Au x-rny of the chest was negative. Excretory urography rt)vealed a calcified ring shadow in the right upper quadrant located at the level of the midportio11 of the right. kidney (fig. 1, B). The contrast medium ,,-as very well conceHtratccl bilaterally and promptly excreted. There was poor elimination OH the lateral aspect of the right renal pelvis, marked middle pole calycdasis, and mild lower pole caJyectasis. J.. gallbladder series was negative. Laboratory data A urine culture (three specirnens) revealed no growt.h. B. prnteus ,Yas found in another specimen. Guinea pig inoculation ,ms negative for tuben:ulosis. Urinalysis: Specific gravity l .020; albumin, ,mgar and acetone, negatirn; ,d1itc blood rclls 2 3; red blood cells ~3-S; Ziehl-Neelscn stain, no acid fast bacilli found.. Blood cnuni: red blood cells 4,GI0,000, hemoglobin 101 per ce11e; whit.e blood cells 8200; polymorphonuclears 60; lymphocytes monocytes :i; eosinophils :3; blood urea nitrogen 1.20 per cent; sugar ll/5 mg. per cPnt; chlm·ides ; Kolmer-Mazzini, ;i50 mg. per cent; sodium 1:39 m/Eq.; potassiurn 4.5 negative. Skiu testi'i for echinococcosis, negative. Preoperative (and postoperative) diagnosis: aneurysm of the right reual A right nephredomy was accomplished December 31, 1954.. "~n oblique lumbar imision ,ms made and three-fourths of the t,Yelfth rib excised. The perirenal area ,,·as entered. The ureter \Yas visualized, doubly clamped and ligated. The kidney appeared to he normal in outline and appearance, but palpation disclosed a hard mass in the region of the pelvis (fig. 2). The pedicle ,ms doubly ligated and clamped and the kidney excised. T,Yo transfixation sutures ,rnre used to ligate the p(,dide. T,Yo Penrose drains were insert.Pd, one in the region of tbe upper pole and one in the region of the ligated lower ureter and brought out of the upper anglll of the wound. The ,nmncl was then clm:ed in layers, using interrupted l'hromic Ko. 1 for muscle, interrupted chromic No. 1 for fascia. and int.errupi,ed silk Stewart suturec; for 8kin. The postopnratiye recovery and course wet'c uneventful and the patieiit ,yas di,wharged at the thirteenth postoperative day. Pathological rqiort: Thn grm,s specimen was a kidnPy slightly larger than Hurmal. The fibrow, capc;ul(, had b(,Cll stripped, exposing a surface \Yhich wa::-; :,;lightly corrngatcd. Thel'e wew several scattered, depressed scars, the largest up to l crn.
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S. MA.LlSOFF' AND M. CERRU'l'I
Fw. 2. Appcarnnce of kidney at time of ncphrcctom)·
in diameter at the pole opposite that which eontains the aneurysm to be described. One pole of the kidney ,vas thickened in the region of the hilum. The stumps of renal arteries were visible on the hilar surface. On section there was a large thrombosed aneurysm embedded in the substan<'e of the kidney adjacent to the hilum and projecting toward one pole and also compressing the surrounding kidney parenchyma (fig. 8). This aneurysm ,ms the size of a walnut and up to :1 cm. in diameter; its wall thin and fibrous and 1-1.5 n1m. in thickness. The lumen of the aneurysm ,vas filled ,Yith an old compacted, laminated thrombus, the cut surface of ,vhich ,Yas grey-brown in color and marbled. The aneurysm was firmly adherent to the adjacent kidney parenchyma, compressing and distorting and producing pressure atrophy of the overlying parenchyma. There were several compressed pyramids and a distorted calyx adjacent to it. The aneurysm extended to within 8 mm. of the parenchymal surface on one side and 1;3 mm. on the opposite side. Its tough, fibrom; wall ,rn::, slightly calcified. The aneurysm also abutted against and distorted the adjacent pelvis, which partly covered the aneurysmal -wall along a portion of the periphery. The aneurysm was continuous with a ::,hort ::,egment of a branch of the renal artery. The most central portion of the thrombus within the aneurysm was freshly dotted. There ,vas slight hemorrhage in the pelvis.
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Fm. 3. Demmrntrntion of thrnmboseo imeurc·sm when kidney was bisec1ed
Examination of tlw microsropic 8ection (fig. 4) revealed theaneury8mal wall to lw composed of a thick layt>r of smooth rnusde and fibrous tissue which ,Yas partly l'alf'ified and hyalinized. In the section stained for elastic tissue the internal elastic layer was iudistinctly delineated and partly destroyed. The lumen of the aneurysm wa8 filled with a lami11ated, fairly homogeneous mass of thrombotic material, the peripheral portions of "·hich were hyalinized. The old inspis8atcd, compacted portion of the thrombu8 "·as transformed into connective tissue 1,·hich for the most part was hyalinized but which also ro11tai11cd deposits of {'holest1.erin. The bulk of the thrombus ,yas transformed into a fairly homogeneous, .structureless, agglutinated mass of eosinophilic material in which the individual components of thP blood were no longer evident. The most central portion of the thrombus included a rccognizablP fibrin meshwork, the interstices of ,Yhich coutaiued some residual, partly intact erythrncyte8. The thickened wall of tfo· aneurysm had adjacent. to it a compressed artery, the latter showing fibrous thickening of the intima and disapp1.earancc of abont half the circumference of the internal and also external elastic lamina. There were foci of inflammatory round cell infiltration in the ad\'eutitia and also there was a thin layer of hemorrhage between the wall of the aneurysm and the adjaeent. compressed kidney parenehyma. The section also revealed compression and distortion of the pelvic mucosa with thinning of the mucosal lining oYer the aneurysm. The pmenchyma adjarent to the aneury8m re\'ealed evideuce of pressure atrophy -with distortion of the parenchymal elements including tubulPs and glomeruli. Some of the latter were completely hyalinized and shrunken. The section also revealed a small artery with a markedly thickened wall and narrowed lumen, with hemorrhage in the inner portion of the wall. Diagnosis: Kidney with 1.embed
548
S. MALISOFF AND M. CERRUTI
Fm. 4. Microscopic section of thrombosed aneurysm of branch of right renal artery
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COMMENTS
One episode of pain and hematuria, and 4 years later a second episode of pain were the essential clinical features of the case, leading to further investigation of the genitourinary tract. It is of interest to point out that the x-rays taken in 1950 were negative despite
ANEURYSM OF RENAL ARTERY
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the fact that symptoms started in 1946, and can be explained on the basis that calcification in the aneurysm had not yet developed. The lesion was revealed by x-ray for the first time in 1954. The accident reported in 1943 should be kept in mind as a possible etiological factor. The diagnosis was based upon radiologic findings. Tuberculosis was eliminated from the differential diagnosis, because of lack of history, negative urine culture and guinea pig inoculation and normal chest film. Renal lithiasis was ruled out because the history and type of pain were not typical; tumor, because of the long standing history; echinococcosis, because of negative skin tests and absence of other evidence. The differential diagnosis from other cysts or a calcified aneurysm of another artery was considered and eliminatAr1 hecause of the almost typical x-ray findings of aneurysm of the renal artery. In this case no further diagnostic procedures such as aortography or presacral insuffiation were believed necessary. The clotted blood filling the aneurysmal sac, as specified in the pathological report, would have prevented a successful opacification of the ring by the contrast medium. The clinical diagnostic evidence we had was sufficient; in any case, surgery was clearly indicated. During the operation the single finding was a hard mass felt in the region of the renal pelvis. N ephrectomy was done and the pathological report confirmed the preoperative diagnosis of aneurysm of the renal artery, emphasizing the fact that it was a true aneurysm of one of the branches of the renal artery. SUMMARY
A case report of aneurysm of the renal artery has been presented, with a brief discussion on etiology, pathology, symptomatology and treatment. The importance of radiologic aid in the preoperative diagnosis is emphasized. Nephrectomy is the only satisfactory treatment.
5 W. 86th St., New York 24, N. Y. REFERENCES ABESHOUSE, B. S.: Aneurysm of the renal artery. Report of two cases and review of the literature. Urol. & Cutan. Rev., 55: 451, 1951. ATKINSON, R. L.: Aneurysm of the renal artery. J. Urol., 72: 117, 1954. BEGNER, J. A.: Aortography in renal aneurysm. J. Urol., 73: 720, 1955. BERNEIKE, R. R. AND PoLLOCK, H. M.: True renal artery aneurysm. New Eng. J. lVIed., 243: 12, 1950. LEGER, J. L., M1cHEN, J., BuRGE01s, P. AND BELISLE, M.: Aneurysm of the renal artery; presentation of a case. Union Medicale de Canada, 83: 634, 1954. LowsLEY, 0. S. AND CANNON, E. M.: Aneurysm of the renal artery. J.A.M.A., 121: 1137, 1943. VoN RoNNAN, J. R.: The roentgen diagnosis of calcified aneurysm of the splenic and renal arteries. Acta Radiologica, 39: 386, 1953.