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Arteriovenous Aneurysm of the Kidney: Case Report
THE JOURKAL OF' UROLOGY
Vol. 75, No. 4, April 1956
Printed in U.S.A.
ARTERIOVENO1.7S ANEURYSM OF THE KIDNEY: CASE REPORT MIMI D. SLOMINSKI-LAWS, JOSEPH H. KIEFER
AND
C. W. VERMEULEN*
From the Departments of Pathology and Urology, University of Illinois College of ivledicine, Chicago, Ill.
The purpose of our paper is to present the eighth proven case of arteriovenous aneurysm involving the renal vessels. The lesion, in this case, we believe to be the result of a congenital malformation. CASE REPORT
A 19-year-old white girl entered the Illinois Research and Educational Hospitals on October 13, 1952. Her chief complaints on admission were swelling of the ankles and dyspnea on exertion. Her family physician and a cardiology consultant referred her to the hospital with the diagnosis of congenital heart disease, most probably an interauricular septal defect. The patient had apparently been normal at birth. She had had diphtheria at the age of 7 years but had no history of rheumatic fever, joint swellings or growing pains. When she was 11 years of age she had been told that she had a heart murmur, although it apparently did not interfere with her routine activities, inasmuch as she continued to take gymnastics at school. During the 5 years prior to her hospital admission she had had increasing ankle edema which was worse during the year before admission. Together with the increase in ankle edema she had become aware of an increased shortness of breath on exertion. There was no cough, hemoptysis or cyanosis. The patient had no symptoms referable to the urinary tract. On physical examination the patient appeared well developed and ·well nourishei. She was in no acute pain or distress. She was moderately hirsute. There were no distended veins noted externally. Her blood pressure was 120/80 with a regular cardiac rhythm. A grade 3 systolic murmur was heard best at the third interspace to the left of the midsternal line. The heart was moderately enlargei to the left on percussion. The blood pressure in the left leg was 140/80; in the right leg it was 140/106. In the area of the left flank, just above the iliac crest, there was a definite systolic-diastolic machinery-like murmur or bruit heard on auscultation. There was also a palpable thrill at this point. Routine blood counts and urinalyses were within normal limits. The nonprotein nitrogen, serum albumin, globulin, sodium, potassium and chlorides were also within normal limits. A urea clearance test was 36 cc per minute. The basal metabolism rate was reported as minus 21 per cent oxygen uptake. However, subsequent radio-iodine uptake studies showed the patient to be euthyroid. A routine chest x-ray disclosed gross enlargement of the left ventricle with prominence of the pulmonary conus. Fluoroscopy and oblique chest films revealed dilatation of the pulmonary artery and the aorta as well as the left and the right ventricles. The electrocardiogram showed left ventricular preponderance. The vital capacity ·was 3.25 L. sitting and 2.90 L. lying. The venous pressure Accepted for publication October 24, 1955. * Present address: The University of Chicago School of Medicine. 586
ANEURYSM OF KIDNEY
587
Fm. 1. A, excretory urogram. Note slight mottling and calyceal distortion on left. B, aortogram. Splenic artery is ~een passing across left kidney at junction of its upper and middle third. Main portion of arteriovenous aneurysm of left kidney is seen to lie in that area below splenic artery and above needle passing into aorta.
was 120 mm. of saline. The decholin circulation time and the ether circulation time were within the normal range. A ballistocardiogram was interpreted as showing evidence of a high cardiac output. Films of the lumbar and dorsal spine were negative. Excretory urography revealed distortion of the renal pelvis and the superior infundibulum of the left kidney with multiple radiolucent areas in that region which suggested dilated vascular structures (fig. 1, A). A retrograde pyelogram confirmed these abnormalities of the left kidney pelvis and infundibulum. A preoperative aortogram (fig. 1, B) showed a widely dilated left renal vein with many tortuous, vascular structures visualized in the region of the left kidney pelvis. These dilated structures were believed to represent an arteriovenous aneurysm involving the left renal artery and vein. In addition, the right renal artery and the splenic artery were visualized and appeared normal. At cystoscopy, ureteral catheters were inserted and urine was collected from each kidney separately for a differential evaluation of kidney function. Sodium and potassium concentrations were determined on the urine samples and a differential phenolsulfonphthalein test was run. Values for these tests were as follows: In the left kidney the dye appeared in 5 minutes while on the right it appeared in a shorter time~3 minutes. The urine from the left kidney had a concentration of sodium of 76 mEq./L, and 24 mEq./L of potassium; while the right kidney urine had a sodium concentration of 84 mEq./L and a potassium concentration of 38 mEq./L. The preoperative diagnosis was arteriovenous fistula involving the left kidney associated with mild cardiac failure of the high output type. On November 10, 1952, exploration of the left renal area was accomplished through a left flank incision with resection of the twelfth rib. The retroperitoneal space was exposed posteriorly while the peritoneal sac was exposed in the anterior
588
M. D. SLOMINSKI-LAWS, J. H. KIEFER AND C. W. VERMEULEN
Fm. 2. Gross appearance of left kidney removed at operation
r
portion of the wound and opened, utilizing a double approach to the kidney area. At the hilus of the left kidney the main renal vein was found to be markedly dilated and on palpation of this vein a striking vibratory thrill was felt. The two main branches of the renal vein were also immensely dilated and through the thin walls of these vessels a rapid flow of blood could be Geen. Temporary occlusions of the arterial inflow to the kidney resulted in complete and immediate cessation of the palpable thrill. Accordingly, a left nephrectomy was accomplished without incident. The patient had an uneventful recovery and was discharged on the thirteenth postoperative day. The gross specimen consisted of an intact left kidney (fig. 2) which had a smooth external surface with normal contour.* The renal artery divided into three branches at the hilum, two passing anterior to the pelvis and one behind. Following formalin fixation and injection of the kidney with a plastic material, the renal sinus was exposed and the arterial branches dissected. The three primary branches were found to give rise to the interlobar vessels in the usual manner. However, in addition to the interlobar branches, which passed laterally from their origin, there could be seen numerous tortuous arterial trunks of about the same size which passed medially from their origin to encircle the renal pelvis from both sides, in many instances causing indentation of the pelvis. Similar tortuous venous branches were also seen draining into the renal vein. In several * We wish to acknowledge our debt to C. A. Krakower for his kind assistance and advice in the preparation of this pathological report.
ANEURYSM OF KIDNEY
589
places, direct connections between the renal artery and vein could be traced through these tortuous arterial and venous channels. Other tortuous vessels disappeared into the tissue filling the renal sinus anterior to the pelvis and into the medullary parenchymal tissue that lay, for the most part, anterior to the hilus. When the organ was cut transversely, in the hilar region, the vessels described were found to communicate with a large multiloculated aneurysmal sac (fig. 3, A). This saccular portion was studied by sectioning the kidney and by probing. It comprised, together with the communicating arterial and venous channels, a varicose aneurysm which occupied approximately the central onethird of the kidney. In regions distant from the aneurysm the cortex measured
Fm. 3. A, coronal section through middle of left kidney, which shows anatomic location of arteriovenous aneurysm that occupies pelvis and compresses overlying renal substance. B, closeup of coronal section through middle of left kidney showing thick walls of arteriovenous aneurysm and marked compression of renal parenchyma
590
M. D. SLOMINSKI-LAWS, ,J. H. KIEFER AND C. W. VERMEULEN
Fm. 4. Low power photomicrograph of coronal section through left kidney. Walls of aneurysm are eccentrically thickened making arterial and venous portions indistinguishable from each other histologically.
approximately 5 mm. in thickness and was clearly delineated from the subjacent medullary tissue. However, in the areas overlying the aneurysm, the cortical tissue was thinned to approximately 2 mm. (fig. 3, B). Microscopic study included sections stained for elastic tissue with WeigertVan Giesen technique, connective tissue with JVIallory's trichrome stain and routine hematoxylin and eosin (fig 4). There was marked vascular congestion and dilatation of vessels in the cortical areas with a few scattered scars and patchy increase in fibrous connective tissue. The interlobar vessels were also dilated. These arteries, as well as the ones comprising the aneurysm, had greatly thickened intimal linings and some showed frayed elastic membranes. There were places where the vessel walls were tremendously thinned out and in one continuous area the elastic membrane disappeared completely and the wall resembled that of the adjacent veins. Many of the latter showed focal areas of intimal thickening, as a result of blood flow through these channels at high pressure. In many sections through the walls of the aneurysm it was extremely difficult to differentiate artery from vein even with special staining techniques. An arteriovenous aneurysm is characterized by an abnormal communication between an artery and vein. The usual cause is trauma which may be operatiYe or otherwise. Infection or ulceration of vessel walls may precipitate the de,'elopment of such an arteriovenous fistula. An aneurysm of the artery occasionally may precede the formation of fistula with adjacent veins. The arteriovenous fistulas may consist of direct communication between artery and vein or, as in
ANEURYSM OF KIDNEY
591
varicose aneurysm, of an arterial or venous sac at the communicating site. Because of the constant flow of blood under high pressure from the arterial side into the venous channels, the venous walls become eccentrically thickened and the varicose sac enlarges at the expense of surrounding tissues. A congenital arteriovenous aneurysm is very rare but cases have been reported. This case appears to fall into this category. We believe that the following three points help substantiate this view: 1) No history of trauma could be elicited; 2) symptoms of cardiac decompensation of the high output type occurred at a very early age; and 3) the aneurysm was located within the renal parenchyma. The literature dealing with renal arteriovenous aneurysms was reviewed by Hamilton, et al.1 Four reported cases2 - 4 are similar to the one described here although one had a history of injury 5 to the involved lumbar area. The fifth case1 arose secondary to an adenocarcinoma of the kidney. The sixth case 6 had an origin which was either traumatic or congenital. The seventh case7 arose following nephrectomy for pyonephrosis. The present case had no hypertension; three of the previous patients did, and three did not. Our case showed mild cardiac decompensation as did six of the other cases reported. SUMMARY
The eighth case of arteriovenous aneurysm of the renal vessels is reported. This lesion was believed to be congenital in nature. The patient was cured by nephrectomy. 1 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. 2 Varela, M. E.: Aneurisma arteriovenoso de los vasos renales y asistolia consecutiva. Rev. Med. Latino-Am., 14: 3244-3266, 1923. 3 Hollingsworth, E.W.: Arteriovenous fistula of renal vessels. Am. J. Med. Sci., 188: 399403, 1934. 4 Pearse, R. and MacMillan, R. L.: Congenital aneurysm of renal artery. J. Urol., 68: 235-238, 1947. 5 Rieder, W.: Sonderstellung arterio-venoser Aneurysmen der Nierengefasse im Rahmen operativer Behandlung schwerer Herz-Kreislaufschaden beim arterio-venosen Aneurysma. Der Chirurg., 14: 609-618, 1942. 6 Kirby, C. K., Nichols, W. G., Garritano, A. P., Wohl, G. T. and Pietroluongo, A. L.: Arteriovenous fistula of renal vessels. Surg., 37: 267-271, 1955. 7 Schwartz, J. W., Borski, A. A. and Jahnke, E. J.: Renal arteriovenous fistula. Surg., 37: 951-954, 1955.
Vol. 75, No. 4, April 1956
Printed in U.S.A.
ARTERIOVENO1.7S ANEURYSM OF THE KIDNEY: CASE REPORT MIMI D. SLOMINSKI-LAWS, JOSEPH H. KIEFER
AND
C. W. VERMEULEN*
From the Departments of Pathology and Urology, University of Illinois College of ivledicine, Chicago, Ill.
The purpose of our paper is to present the eighth proven case of arteriovenous aneurysm involving the renal vessels. The lesion, in this case, we believe to be the result of a congenital malformation. CASE REPORT
A 19-year-old white girl entered the Illinois Research and Educational Hospitals on October 13, 1952. Her chief complaints on admission were swelling of the ankles and dyspnea on exertion. Her family physician and a cardiology consultant referred her to the hospital with the diagnosis of congenital heart disease, most probably an interauricular septal defect. The patient had apparently been normal at birth. She had had diphtheria at the age of 7 years but had no history of rheumatic fever, joint swellings or growing pains. When she was 11 years of age she had been told that she had a heart murmur, although it apparently did not interfere with her routine activities, inasmuch as she continued to take gymnastics at school. During the 5 years prior to her hospital admission she had had increasing ankle edema which was worse during the year before admission. Together with the increase in ankle edema she had become aware of an increased shortness of breath on exertion. There was no cough, hemoptysis or cyanosis. The patient had no symptoms referable to the urinary tract. On physical examination the patient appeared well developed and ·well nourishei. She was in no acute pain or distress. She was moderately hirsute. There were no distended veins noted externally. Her blood pressure was 120/80 with a regular cardiac rhythm. A grade 3 systolic murmur was heard best at the third interspace to the left of the midsternal line. The heart was moderately enlargei to the left on percussion. The blood pressure in the left leg was 140/80; in the right leg it was 140/106. In the area of the left flank, just above the iliac crest, there was a definite systolic-diastolic machinery-like murmur or bruit heard on auscultation. There was also a palpable thrill at this point. Routine blood counts and urinalyses were within normal limits. The nonprotein nitrogen, serum albumin, globulin, sodium, potassium and chlorides were also within normal limits. A urea clearance test was 36 cc per minute. The basal metabolism rate was reported as minus 21 per cent oxygen uptake. However, subsequent radio-iodine uptake studies showed the patient to be euthyroid. A routine chest x-ray disclosed gross enlargement of the left ventricle with prominence of the pulmonary conus. Fluoroscopy and oblique chest films revealed dilatation of the pulmonary artery and the aorta as well as the left and the right ventricles. The electrocardiogram showed left ventricular preponderance. The vital capacity ·was 3.25 L. sitting and 2.90 L. lying. The venous pressure Accepted for publication October 24, 1955. * Present address: The University of Chicago School of Medicine. 586
ANEURYSM OF KIDNEY
587
Fm. 1. A, excretory urogram. Note slight mottling and calyceal distortion on left. B, aortogram. Splenic artery is ~een passing across left kidney at junction of its upper and middle third. Main portion of arteriovenous aneurysm of left kidney is seen to lie in that area below splenic artery and above needle passing into aorta.
was 120 mm. of saline. The decholin circulation time and the ether circulation time were within the normal range. A ballistocardiogram was interpreted as showing evidence of a high cardiac output. Films of the lumbar and dorsal spine were negative. Excretory urography revealed distortion of the renal pelvis and the superior infundibulum of the left kidney with multiple radiolucent areas in that region which suggested dilated vascular structures (fig. 1, A). A retrograde pyelogram confirmed these abnormalities of the left kidney pelvis and infundibulum. A preoperative aortogram (fig. 1, B) showed a widely dilated left renal vein with many tortuous, vascular structures visualized in the region of the left kidney pelvis. These dilated structures were believed to represent an arteriovenous aneurysm involving the left renal artery and vein. In addition, the right renal artery and the splenic artery were visualized and appeared normal. At cystoscopy, ureteral catheters were inserted and urine was collected from each kidney separately for a differential evaluation of kidney function. Sodium and potassium concentrations were determined on the urine samples and a differential phenolsulfonphthalein test was run. Values for these tests were as follows: In the left kidney the dye appeared in 5 minutes while on the right it appeared in a shorter time~3 minutes. The urine from the left kidney had a concentration of sodium of 76 mEq./L, and 24 mEq./L of potassium; while the right kidney urine had a sodium concentration of 84 mEq./L and a potassium concentration of 38 mEq./L. The preoperative diagnosis was arteriovenous fistula involving the left kidney associated with mild cardiac failure of the high output type. On November 10, 1952, exploration of the left renal area was accomplished through a left flank incision with resection of the twelfth rib. The retroperitoneal space was exposed posteriorly while the peritoneal sac was exposed in the anterior
588
M. D. SLOMINSKI-LAWS, J. H. KIEFER AND C. W. VERMEULEN
Fm. 2. Gross appearance of left kidney removed at operation
r
portion of the wound and opened, utilizing a double approach to the kidney area. At the hilus of the left kidney the main renal vein was found to be markedly dilated and on palpation of this vein a striking vibratory thrill was felt. The two main branches of the renal vein were also immensely dilated and through the thin walls of these vessels a rapid flow of blood could be Geen. Temporary occlusions of the arterial inflow to the kidney resulted in complete and immediate cessation of the palpable thrill. Accordingly, a left nephrectomy was accomplished without incident. The patient had an uneventful recovery and was discharged on the thirteenth postoperative day. The gross specimen consisted of an intact left kidney (fig. 2) which had a smooth external surface with normal contour.* The renal artery divided into three branches at the hilum, two passing anterior to the pelvis and one behind. Following formalin fixation and injection of the kidney with a plastic material, the renal sinus was exposed and the arterial branches dissected. The three primary branches were found to give rise to the interlobar vessels in the usual manner. However, in addition to the interlobar branches, which passed laterally from their origin, there could be seen numerous tortuous arterial trunks of about the same size which passed medially from their origin to encircle the renal pelvis from both sides, in many instances causing indentation of the pelvis. Similar tortuous venous branches were also seen draining into the renal vein. In several * We wish to acknowledge our debt to C. A. Krakower for his kind assistance and advice in the preparation of this pathological report.
ANEURYSM OF KIDNEY
589
places, direct connections between the renal artery and vein could be traced through these tortuous arterial and venous channels. Other tortuous vessels disappeared into the tissue filling the renal sinus anterior to the pelvis and into the medullary parenchymal tissue that lay, for the most part, anterior to the hilus. When the organ was cut transversely, in the hilar region, the vessels described were found to communicate with a large multiloculated aneurysmal sac (fig. 3, A). This saccular portion was studied by sectioning the kidney and by probing. It comprised, together with the communicating arterial and venous channels, a varicose aneurysm which occupied approximately the central onethird of the kidney. In regions distant from the aneurysm the cortex measured
Fm. 3. A, coronal section through middle of left kidney, which shows anatomic location of arteriovenous aneurysm that occupies pelvis and compresses overlying renal substance. B, closeup of coronal section through middle of left kidney showing thick walls of arteriovenous aneurysm and marked compression of renal parenchyma
590
M. D. SLOMINSKI-LAWS, ,J. H. KIEFER AND C. W. VERMEULEN
Fm. 4. Low power photomicrograph of coronal section through left kidney. Walls of aneurysm are eccentrically thickened making arterial and venous portions indistinguishable from each other histologically.
approximately 5 mm. in thickness and was clearly delineated from the subjacent medullary tissue. However, in the areas overlying the aneurysm, the cortical tissue was thinned to approximately 2 mm. (fig. 3, B). Microscopic study included sections stained for elastic tissue with WeigertVan Giesen technique, connective tissue with JVIallory's trichrome stain and routine hematoxylin and eosin (fig 4). There was marked vascular congestion and dilatation of vessels in the cortical areas with a few scattered scars and patchy increase in fibrous connective tissue. The interlobar vessels were also dilated. These arteries, as well as the ones comprising the aneurysm, had greatly thickened intimal linings and some showed frayed elastic membranes. There were places where the vessel walls were tremendously thinned out and in one continuous area the elastic membrane disappeared completely and the wall resembled that of the adjacent veins. Many of the latter showed focal areas of intimal thickening, as a result of blood flow through these channels at high pressure. In many sections through the walls of the aneurysm it was extremely difficult to differentiate artery from vein even with special staining techniques. An arteriovenous aneurysm is characterized by an abnormal communication between an artery and vein. The usual cause is trauma which may be operatiYe or otherwise. Infection or ulceration of vessel walls may precipitate the de,'elopment of such an arteriovenous fistula. An aneurysm of the artery occasionally may precede the formation of fistula with adjacent veins. The arteriovenous fistulas may consist of direct communication between artery and vein or, as in
ANEURYSM OF KIDNEY
591
varicose aneurysm, of an arterial or venous sac at the communicating site. Because of the constant flow of blood under high pressure from the arterial side into the venous channels, the venous walls become eccentrically thickened and the varicose sac enlarges at the expense of surrounding tissues. A congenital arteriovenous aneurysm is very rare but cases have been reported. This case appears to fall into this category. We believe that the following three points help substantiate this view: 1) No history of trauma could be elicited; 2) symptoms of cardiac decompensation of the high output type occurred at a very early age; and 3) the aneurysm was located within the renal parenchyma. The literature dealing with renal arteriovenous aneurysms was reviewed by Hamilton, et al.1 Four reported cases2 - 4 are similar to the one described here although one had a history of injury 5 to the involved lumbar area. The fifth case1 arose secondary to an adenocarcinoma of the kidney. The sixth case 6 had an origin which was either traumatic or congenital. The seventh case7 arose following nephrectomy for pyonephrosis. The present case had no hypertension; three of the previous patients did, and three did not. Our case showed mild cardiac decompensation as did six of the other cases reported. SUMMARY
The eighth case of arteriovenous aneurysm of the renal vessels is reported. This lesion was believed to be congenital in nature. The patient was cured by nephrectomy. 1 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. 2 Varela, M. E.: Aneurisma arteriovenoso de los vasos renales y asistolia consecutiva. Rev. Med. Latino-Am., 14: 3244-3266, 1923. 3 Hollingsworth, E.W.: Arteriovenous fistula of renal vessels. Am. J. Med. Sci., 188: 399403, 1934. 4 Pearse, R. and MacMillan, R. L.: Congenital aneurysm of renal artery. J. Urol., 68: 235-238, 1947. 5 Rieder, W.: Sonderstellung arterio-venoser Aneurysmen der Nierengefasse im Rahmen operativer Behandlung schwerer Herz-Kreislaufschaden beim arterio-venosen Aneurysma. Der Chirurg., 14: 609-618, 1942. 6 Kirby, C. K., Nichols, W. G., Garritano, A. P., Wohl, G. T. and Pietroluongo, A. L.: Arteriovenous fistula of renal vessels. Surg., 37: 267-271, 1955. 7 Schwartz, J. W., Borski, A. A. and Jahnke, E. J.: Renal arteriovenous fistula. Surg., 37: 951-954, 1955.