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Spontaneous rupture of a renal artery aneurysm in polyarteritis nodosa: critical review of the literature and report of a case
Spontaneous Rupture of a Renal Artery Aneurysm in Polyarteritis Nodosa: Critical Review of the Literature and Report of a Case DAVIDL. SMITH,
M.D., RICHARD WERNICK, M.D.
Portland, Oregon
p
olyarteritis nodosa (PAN) is a systemic inflammatory disorder affecting small and medium-sized vessels. Renal involvement from aneurysm formation is common, but perirenal bleeding is unusual. Fewer than 50 cases of PAN with perirenal hemorrhage from a ruptured renal aneurysm have been reported in the literature [1]. When it occurs, both surgical and medical intervention are usually required to control bleeding and the underlying vasculitic process [1,2]. We describe a patient with PAN who developed a large retroperitoneal hemorrhage from a ruptured renal cortex aneurysm. Gelfoam ® (absorbable gelatin sponge, Upjohn Company, Kalamazoo, Michigan) was embolized selectively during arteriography and successfully controlled the aneurysmal bleeding. We review the literature for previous cases of renal aneurysm rupture caused by PAN and discuss the use of Gelfoam embolization as an additional therapeutic modality to control bleeding. CASE REPORT
A 25-year-old white man presented to his rural community hospital emergency room on February 25, 1988, with a four-month history of a 25-pound weight loss, two months of intermittent left upper quadrant pain postprandially, and bizarre ideation, which included cough productive of "bugs" and the presence of "worms" in his nose, eyes, and skin. He admitted to intermittent intravenous amphetamine abuse over the preceding year. Physical examination revealed a chronically ill-appearing and emaciated man with a blood pressure of 150/100 mm Hg and a tender abdominal left upper quadrant. An extensive evaluation was performed during a subsequent two-week hospitalization. Abnormalities that were found included a white blood cell count of 22,000/mm 3, with 78% polymorphonuclear leukocytes and 6% lymphocytes, albumin 20.1 g/L (normal, 40 to 60 g/L), aspartate aminotransferase 82 U/L (normal, 0 to 35 U/L), alanine aminotransferase 98 U/L (normal, 0 to 35 U/L), positive hepatitis B surface antigen, and abdominal computerized tomography (CT) scan revealing low attenuation in the posterior upper pole of the right kidney, suggesting a previous cortical infarct. The patient was anergic to purified protein derivative and three controls. Tests that were normal or negative included chest radiograph, urinalysis, serum amylase, blood lead level, antinuclear antibody, antibody to huFrom the Division of General Medicine, Ambulatory Care and Medical Service, Section of Arthritis and Rheumatic Diseases, Oregon Health Sciences University (DLS, RW), Veterans Administration Medical Center (DLS), Portland, Oregon. Requests for reprints should be addressed to David L. Smith, M.D., Ambulatory Care and Medical Service (1 ]C-OPC), Veterans Administration Medical Center, P.O. Box ]036, Portland, Oregon 97207. Manuscript submitted April ] 2, 1989. and accepted May ] 2. ] 989.
464
man immunodeficiency virus, upper and lower gastrointestinal endoscopy, right upper quadrant ultrasonography, barium enema, bone marrow biopsy, cranial CT scan, and cerebral spinal fluid examination. During his hospital course, he developed right foot drop and lost an additional eight pounds. He was persistently hypertensive up to a level of 180/110 mm Hg, and bizarre ideation persisted. On March 17, six days after discharge, he developed sudden intense right upper quadrant pain with radiation to the back, and he collapsed. In the emergency room, blood pressure was 88/72 mm Hg supine, decreasing to 60/40 mm Hg sitting, which prompted syncope. Pulse was 124/minute, and he had right upper quadrant and right flank tenderness. His hematocrit was 26%, and his white blood cell count was 36,000/ mm 3. Abdominal CT scan revealed a large right perirenal hematoma that had not been present on the initial scan 10 days earlier ( F i g u r e I). He was given three units of packed red blood cells. On March 18, he was transferred to Providence Medical Center, where he was noted to have a blood pressure of 130/70 mm Hg, atrial fibrillation with a ventricular rate of 150/minute, and moderate left wrist drop. Hemoglobin of 10 g/ dL on admission rapidly declined to 6.6 g/dL, and an additional six units of packed red blood cells were transfused on March 18 and 19. Selective abdominal arteriography revealed multiple aneurysms up to 1 cm in diameter in both renal systems as well as in the right hepatic arterial system (Figure 2). Extravasation was noted along the lateral border of the right kidney and appeared to emanate from an aneurysm just below the right lateral renal cortex. A tertiary artery supplying both the lateral aspect of the right kidney and the bleeding aneurysm was catheterized, resulting in the formation of a clot at the origin of the involved artery. A Gelfoam torpedo was placed at the site of the occluded artery to maintain the obstructive process. Right renal arteriogram was repeated after embolization and revealed no further opacification of arterial structures at the site of the previous hemorrhage ( F i g u r e 3). Methylprednisolone 1 g/day and cyclophosphamide 200 rag/day were begun intravenously. On March 19, findings on a repeat CT scan were unchanged. On March 20, hemoglobin had declined to 8.9 g/dL, and an additional two units of red cells were transfused. The patient's condition remained hemodynamically stable, and on March 23, increased strength in the right foot was noted and the cyclophosphamide dose was decreased to 125 mg/day. Mentation improved slowly, and hypertension was controlled with a combination of nifedipine 30 mg/day, captopril 150 mg/day, and propranolol 80 rag/day. On March 26, the patient was discharged after receiving a regimen of prednisone
October1989 The American Journal of Medicine Volume87
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK
Figure 1. CT scan demonstrates a large right perirenal hematoma (arrow), resulting in compression and displacement of the kidney. There is residual contrast in the gut from the previously performed barium enema.
80 mg/day and cyclophosphamide 125 mg/day, and the aforementioned antihypertensive regimen. At the eight-month follow-up, the patient's mentation has returned to normal, weight has increased 30 pounds, and wrist drop has resolved. He is able to dorsiflex his right foot, although not against gravity. Cyclophosphamide has been discontinued because of poor compliance, and the patient continues to receive prednisone 20 rag/day. His hypertension has improved, and he requires enalapril alone to maintain normotension.
Figure 2. Right renal arteriogram reveals multiple aneurysms and extravasation of contrast along the lateral renal border, originating from a ruptured aneurysm just below the cortex at that site. The kidney is compressed and displaced medially by the hematoma seen in Figure 1.
COMMENTS
In 1908, the first description of spontaneous renal hemorrhage from PAN appeared in the literature [3]. Over the last 80 years, approximately 50 additional cases have been reported. In 1933, Polkey and Vynalek [4] reviewed 112 cases of renal hematoma formation. PAN-induced hemorrhage was found in only seven cases (5%). Other causes of spontaneous renal hemorrhage and their relative frequencies are listed in Table I [5,6]. We retrospectively analyzed 28 cases of PAN with renal hemorrhage due to aneurysm or artery rupture in the English literature over the last 50 years [1,2,728]. We evaluated only those reports with a clear case description, confirmed diagnosis, and documented hemorrhage. Results are depicted in Table II. The renal hemorrhage typically is due to arterial aneurysreal rupture, more rarely from renal artery dissection [24] or rupture [25]. The preponderance of cases involve young men with a history of hypertension who present with sudden, severe flank or abdominal pain and are found to have an abdominal mass and anemia. A fulminant course with vascular collapse often follows. Our patient demonstrated the salient characteristics of PAN complicated by perirenal hemorrhage from aneurysmal rupture. He was a young man without a prior diagnosis of PAN who developed severe, sudden abdominal pain, hypotension, and anemia. Although extreme tenderness limited the abdominal examination, CT scan revealed a large retroperitoneal hemorrhage. In cases with this presentation, emergent diagnostic
Figure 3. Right renal digital subtraction arteriogram performed after Gelfoam embolization demonstrates no opacification of arterial structure, at the site of previous hemorrhage.
testing is required to differentiate PAN-associated perirenal bleeding from other conditions with a similar presentation (Table I). In fact, in the pre-arteriogram era, the vast majority of cases were diagnosed at surgery or autopsy. In the absence of arteriography, clues to a vascular etiology include a poorly visualizing inOctober 1989 The American Journal of Medicine Volume 87
465
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK
TABLE I Leading Causes of Spontaneous Perirenal Hemorrhage* Novlcki etal[5] (n = 194) (%)
McDougaletal[6] (n = 78) (%)
16 16 12 15 5 4 4
58 5 l0 5 5 13 --
Tumor Vascular, nonarteritic Infection Nephritis Blood dyscrasias PAN Hydronephrosis "Adapted from [5] and [6].
TABLE II Presenting Manifestations of Patients with PAN and a Ruptured Renal Aneurysm Characteristic
Number*
Percent
Male Age 25-40 History of hypertension Acute flank/abdominal pain Prior systemic symptoms of PAN Hypotension Flank/abdominal mass Anemia
24128 19/28 19/28 27/28 10/28 14/27 18/27 23/24
86 68 68 96 36 52 67 96
Number of patients with manifestation/number assessable for manifestation m literature reviewed.
TABLE III Course of Patients with PAN and a Ruptured Renal Aneurysm
Survivalwith surgery Survivalwith medical management Recurrent bleeding*
Number
Percent
7/13
54 40 18
6/15 5/28
Three patients had been treated surgically, and two patients had combined medical and surgical treatment prior to recurrence.
travenous pyelogram coupled with a normal retrograde pyelogram [26]. With the advent of arteriography, cases are more promptly diagnosed. The arteriographic findings of PAN have been well described [29]. The finding of multiple small aneurysms involving the renal vasculature is virtually diagnostic, with a specificity approaching 100% [30]. When active bleeding occurs, the site of hemorrhage can also be isolated. Abdominal CT scanning with contrast medium has been described in patients with PAN [27]. It can detect the site of perirenal or abdominal hemorrhage. Although it helps distinguish neoplastic or infiltrative disorders from PAN, its sensitivity and specificity are unknown. Moreover, it is rarely possible to detect intrarenal aneurysms because of their small diameter [27]. In this setting, its utility may be limited to detailmg the site and follow-up of hemorrhage. The optimal management of PAN-induced renal aneurysm rupture is unclear. Supportive therapy accompanied by prompt arteriographic recognition of the condition, with visualization of the bleeding site, is fundamental. In previous reports, both surgery to di-
466
October 1989 The American Journal of Medicine Volume 87
rectly control the hemorrhage and medical therapy for the underlying vasculitis have been employed [1,26,28], but mortality is approximately 50% with either form of management (Table III). Recent use of more aggressive immunosuppressive therapy that includes cyclophosphamide in addition to glucocorticoids may improve survival rates for PAN [31], but experience in this setting is limited [1]. In addition, aggressive treatment of hypertension is mandatory since elevated pressure against a weakened arterial wall may promote aneurysmal rupture [1]. As in our case, the majority of patients with PAN and renal bleeding have pre-existing hypertension. Therapeutic arterial embolization with Gelfoam during arteriography has gained recognition as an alternative to surgery as a technique to control hemorrhage [32], and has been employed in gastrointestinal, traumatic, gynecologic, and tumor-related hemorrhage [32-34]. Gelfoam blocks or strips are selectively embolized after the arteriographic catheter is positioned and the bleeding point is identified. Successful Gelfoam embolization has also been used in an unusuel case of Wegener's granulomatosis in which a subcapsular renal hematoma from a bleeding aneurysm was demonstrated [35]. Overall, Gelfoam selective embolization has been shown to control hemorrhage in 93% of cases, with a procedure-related mortality of 3.6% [32]. Complications following embolization include inadvertent distal infarction [32]. To our knowledge, our case is the first in which Gelfoam was selectively embolized to control aneurysreal bleeding from PAN. Its use, coupled with aggressive medical management of the vasculitis and hypertension, obviated the need for surgical intervention in our patient. Considering the high surgical mortality noted in patients with PAN who develop aneurysmal hemorrhage, we believe that Gelfoam embolization should be considered when a single active renal bleeding site can be demonstrated arteriographical]y. In summary, perirenal hemorrhage should be considered when a patient presents with acute flank and abdominal pain, an accompanying mass, and hypotension or flank tenderness. PAN should be considered as a potential cause of the renal hemorrhage. Prompt arteriography and transcatheter selective embolization of Gelfoam, combined with aggressive medical therapy for PAN and hypertension, should be considered as an alternative to surgery in patients with PAN in whom hemorrhage from a ruptured renal aneurysm is identified. ACKNOWLEDGMENT We wish to thank George Burgermeister, M,D., for his expert arteriography and helpful discussion.
REFERENCES 1. Miller CM, Reiber K, WaxmanJ, etal: Massiveintraabdominal bleed!ngin polyarteritis nodosa. Mt Sinai J Med 1987; 54: 512-515. 2. Homer BA, Hunt OW, Kincaid OW, DeWeerdJH: Perirenal hematoma secondary to intrarenal microaneurysms of periarteritis nodosa demonstrated radiographically. Mayo Clin Proc 1966; 41: 169-178. 3. Schmidt JE: 0ber periarteriitis nodosa. Beitr Path Anat 1908; 43: 455-469. 4. Polkey HJ, Vynalek WJ: Spontaneous nontraumatic perirenal and renal hematomas: an experimental and clinical study. Arch Surg 1933; 26: 196-218. 5. Novicki DE, Turlington JT, Ball TP: The evaluation and management of spontaneous perirenal hemorrhage, J Urol 1980; 123: 764-765. 6. McDougal WC, Kursh ED, Persky L: Spontaneous rupture of the kidney with perirenal hematoma. J Urol 1975; 114: 18]-182.
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK 7. Hardeman EF: Massive bilateral perirenal hemorrhage in periarteritis nodosa. Bull Charlotte Mem Hosp 1944: 1: 35-40, 8. Joachim GR, Becker EL: Spontaneous rupture of the kidney. Arch Intern Med 1965; 115: 176-183. 9. EkengrenA, RiegerA: Spontaneousrupture of the kidney in periarteritis nodosa. Acta Chir Scand 1961; 121: 158-160. 10. Evens RG. Oobry CA, Eckert JF: An exercise in radiologic-patholog~ccorrelation. Radiology 1968; 9]: 1028-1032. 11. Litvak AS, Lucas BA, McRoberts JW: Urologic manifestations of polyarteritis nodosa. J Urol 1976; 115: 572-574. 12. Fleming RJ, Stern LZ: Multiple intraparenchymal renal aneurysmsin polyarteritis nodosa. Radiology 1965; 84: 100-103. 13. McClure PH, Westcott JL: Periarteritis nodosa with perirenal hemorrhage: a case report with angiographic findings. J Urol 1969; 102: 126-129. 14. Wever GK, Perry IH: Periarteritis nodosa: report ol a case with perirenal hemorrhage. JAMA 1935; 104: 1390-1395. 15. Bron KM, Stilley JW, Shapiro AP: Renal arteriography enhanced by tolazoline: value in the diagnosisof polyarteritis nodosa complicated by perinephric hematoma. Radiology 1971; 99: 295-301. 16. Capps JH, Klein RM: Polyarteritis nodosa as a causeof perirenal and retroperio toneal hemorrhage. Radiology 1970; 94: 143-146. 17. Peterson C, Willerson JT, Doppman JL, Decker JL: Polyarteritis nodosa with bilateral renal artery aneurysms and perirenal hematomas: angiographic and nephrotomographic features. Br J Radiol 1970; 43:62-71. 18. Gambill EE, De Weerd JH, Dockerty MB: Periarteritis nodosa complicated with bilateral spontaneous massive perirenal hemorrhage. Minn Med 1968; 51:767769. 19. HendersonFW, Jordan WP: Spontaneousrupture of the kidney due to polyarteritis nodosa: a case report. J Urol 1967; 97: 811-814. 20. Ostrum BJ, Soder PD: Periarteritis nodosacomplicated by spontaneousperinephric hematoma. AJR 1960; 84: 849-860. 21. Cabel E, Holtz S: Polyarteritis as a cause of intestinal hemorrhage. Gastroen-
terology 1971; 61: 99-105. 22. Fort CA: Spontaneousperirenal hematoma secondary to periarteritis nodosa. J Urol 1948; 59: 307-311. 23. McGrae JD: Perirenal hematoma secondary to polyarteritis nodosa. Arch Intern Med 1959; 104: 421-426. 24. Hekali PE, Pajari RI, KivisaariML, et al: Bilateral renal artery dissection: unusual complications of polyarteritis nodosa. Eur J Radiol 1984; 4: 6-8. 25. GerstenbergTH, Nielsen ML, Lindenberg J, Rasmussen BB: Spontaneousnonaneurysmal rupture of the renal pedicle artery due to acute vasculitis in polyarteritis nodosa--with survival. Acta Chir Scand 1978; 144: 549-551. 26. Tocci PE, Lankford RW, Lynne CM: Spontaneousrupture of the kidney secondary to polyarteritis nodosa. J Urol 1975; 113: 860-863. 27. Hekali P, Kivisaari L, StandertskjOId-NordenstamCG. Palari R, Turto H: Renal complications of poiyarteritis nodosa: CT findings. J Comput AssistTomogr 1985; 9: 333-338. 28. SmailowitzZ, KanetiJ, SoberI: Spontaneousperirenal hematoma: a complication of polyarteritis nodosa. J Urol 1979: 121: 82-83. 29. Gron KM, Stroh CA, ShapiroAP: The diagnostic value of angiographic observations in polyarteritis nodosa, Arch Intern Med 1965; 116: 450-459. 30. Albert DA. Rimon O, SilversteinMD: The diagnosisof polyarteritis nodosa: 1. A literature-based decision analysis approach. Arthritis Rheum 1988; 31: 1] 171127. 31. Fauci AS, Katz P, Haynes BF, Wolf SM: Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 1979; 301: 235-238. 32. Jander PH, RussinovichNA: Transcathetergelfoam embolization in abdominal, retroperitoneal, and pelvic hemorrhage. Radiology 1980; 136: 337-344. 33. Spigos DG, Tan WS, JonassonO: Therapeutic emobolization. Techniques and clinical application. Surg Annu 1981; 13: 361-381. 34. Soimakallio S, Lahtinen J, Tanska S: Arterial embolization in the treatment of malignant tumours. Ann Chir Gynaecol 1981; 70:112-I 15. 35. Baker SB, RobinsonDR: Unusual renal manifestation of Wegener's granulomatosis. Am J Med 1978; 64: 883-889.
October 1989
The American Journal of Medicine
Volume 87
467
M.D., RICHARD WERNICK, M.D.
Portland, Oregon
p
olyarteritis nodosa (PAN) is a systemic inflammatory disorder affecting small and medium-sized vessels. Renal involvement from aneurysm formation is common, but perirenal bleeding is unusual. Fewer than 50 cases of PAN with perirenal hemorrhage from a ruptured renal aneurysm have been reported in the literature [1]. When it occurs, both surgical and medical intervention are usually required to control bleeding and the underlying vasculitic process [1,2]. We describe a patient with PAN who developed a large retroperitoneal hemorrhage from a ruptured renal cortex aneurysm. Gelfoam ® (absorbable gelatin sponge, Upjohn Company, Kalamazoo, Michigan) was embolized selectively during arteriography and successfully controlled the aneurysmal bleeding. We review the literature for previous cases of renal aneurysm rupture caused by PAN and discuss the use of Gelfoam embolization as an additional therapeutic modality to control bleeding. CASE REPORT
A 25-year-old white man presented to his rural community hospital emergency room on February 25, 1988, with a four-month history of a 25-pound weight loss, two months of intermittent left upper quadrant pain postprandially, and bizarre ideation, which included cough productive of "bugs" and the presence of "worms" in his nose, eyes, and skin. He admitted to intermittent intravenous amphetamine abuse over the preceding year. Physical examination revealed a chronically ill-appearing and emaciated man with a blood pressure of 150/100 mm Hg and a tender abdominal left upper quadrant. An extensive evaluation was performed during a subsequent two-week hospitalization. Abnormalities that were found included a white blood cell count of 22,000/mm 3, with 78% polymorphonuclear leukocytes and 6% lymphocytes, albumin 20.1 g/L (normal, 40 to 60 g/L), aspartate aminotransferase 82 U/L (normal, 0 to 35 U/L), alanine aminotransferase 98 U/L (normal, 0 to 35 U/L), positive hepatitis B surface antigen, and abdominal computerized tomography (CT) scan revealing low attenuation in the posterior upper pole of the right kidney, suggesting a previous cortical infarct. The patient was anergic to purified protein derivative and three controls. Tests that were normal or negative included chest radiograph, urinalysis, serum amylase, blood lead level, antinuclear antibody, antibody to huFrom the Division of General Medicine, Ambulatory Care and Medical Service, Section of Arthritis and Rheumatic Diseases, Oregon Health Sciences University (DLS, RW), Veterans Administration Medical Center (DLS), Portland, Oregon. Requests for reprints should be addressed to David L. Smith, M.D., Ambulatory Care and Medical Service (1 ]C-OPC), Veterans Administration Medical Center, P.O. Box ]036, Portland, Oregon 97207. Manuscript submitted April ] 2, 1989. and accepted May ] 2. ] 989.
464
man immunodeficiency virus, upper and lower gastrointestinal endoscopy, right upper quadrant ultrasonography, barium enema, bone marrow biopsy, cranial CT scan, and cerebral spinal fluid examination. During his hospital course, he developed right foot drop and lost an additional eight pounds. He was persistently hypertensive up to a level of 180/110 mm Hg, and bizarre ideation persisted. On March 17, six days after discharge, he developed sudden intense right upper quadrant pain with radiation to the back, and he collapsed. In the emergency room, blood pressure was 88/72 mm Hg supine, decreasing to 60/40 mm Hg sitting, which prompted syncope. Pulse was 124/minute, and he had right upper quadrant and right flank tenderness. His hematocrit was 26%, and his white blood cell count was 36,000/ mm 3. Abdominal CT scan revealed a large right perirenal hematoma that had not been present on the initial scan 10 days earlier ( F i g u r e I). He was given three units of packed red blood cells. On March 18, he was transferred to Providence Medical Center, where he was noted to have a blood pressure of 130/70 mm Hg, atrial fibrillation with a ventricular rate of 150/minute, and moderate left wrist drop. Hemoglobin of 10 g/ dL on admission rapidly declined to 6.6 g/dL, and an additional six units of packed red blood cells were transfused on March 18 and 19. Selective abdominal arteriography revealed multiple aneurysms up to 1 cm in diameter in both renal systems as well as in the right hepatic arterial system (Figure 2). Extravasation was noted along the lateral border of the right kidney and appeared to emanate from an aneurysm just below the right lateral renal cortex. A tertiary artery supplying both the lateral aspect of the right kidney and the bleeding aneurysm was catheterized, resulting in the formation of a clot at the origin of the involved artery. A Gelfoam torpedo was placed at the site of the occluded artery to maintain the obstructive process. Right renal arteriogram was repeated after embolization and revealed no further opacification of arterial structures at the site of the previous hemorrhage ( F i g u r e 3). Methylprednisolone 1 g/day and cyclophosphamide 200 rag/day were begun intravenously. On March 19, findings on a repeat CT scan were unchanged. On March 20, hemoglobin had declined to 8.9 g/dL, and an additional two units of red cells were transfused. The patient's condition remained hemodynamically stable, and on March 23, increased strength in the right foot was noted and the cyclophosphamide dose was decreased to 125 mg/day. Mentation improved slowly, and hypertension was controlled with a combination of nifedipine 30 mg/day, captopril 150 mg/day, and propranolol 80 rag/day. On March 26, the patient was discharged after receiving a regimen of prednisone
October1989 The American Journal of Medicine Volume87
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK
Figure 1. CT scan demonstrates a large right perirenal hematoma (arrow), resulting in compression and displacement of the kidney. There is residual contrast in the gut from the previously performed barium enema.
80 mg/day and cyclophosphamide 125 mg/day, and the aforementioned antihypertensive regimen. At the eight-month follow-up, the patient's mentation has returned to normal, weight has increased 30 pounds, and wrist drop has resolved. He is able to dorsiflex his right foot, although not against gravity. Cyclophosphamide has been discontinued because of poor compliance, and the patient continues to receive prednisone 20 rag/day. His hypertension has improved, and he requires enalapril alone to maintain normotension.
Figure 2. Right renal arteriogram reveals multiple aneurysms and extravasation of contrast along the lateral renal border, originating from a ruptured aneurysm just below the cortex at that site. The kidney is compressed and displaced medially by the hematoma seen in Figure 1.
COMMENTS
In 1908, the first description of spontaneous renal hemorrhage from PAN appeared in the literature [3]. Over the last 80 years, approximately 50 additional cases have been reported. In 1933, Polkey and Vynalek [4] reviewed 112 cases of renal hematoma formation. PAN-induced hemorrhage was found in only seven cases (5%). Other causes of spontaneous renal hemorrhage and their relative frequencies are listed in Table I [5,6]. We retrospectively analyzed 28 cases of PAN with renal hemorrhage due to aneurysm or artery rupture in the English literature over the last 50 years [1,2,728]. We evaluated only those reports with a clear case description, confirmed diagnosis, and documented hemorrhage. Results are depicted in Table II. The renal hemorrhage typically is due to arterial aneurysreal rupture, more rarely from renal artery dissection [24] or rupture [25]. The preponderance of cases involve young men with a history of hypertension who present with sudden, severe flank or abdominal pain and are found to have an abdominal mass and anemia. A fulminant course with vascular collapse often follows. Our patient demonstrated the salient characteristics of PAN complicated by perirenal hemorrhage from aneurysmal rupture. He was a young man without a prior diagnosis of PAN who developed severe, sudden abdominal pain, hypotension, and anemia. Although extreme tenderness limited the abdominal examination, CT scan revealed a large retroperitoneal hemorrhage. In cases with this presentation, emergent diagnostic
Figure 3. Right renal digital subtraction arteriogram performed after Gelfoam embolization demonstrates no opacification of arterial structure, at the site of previous hemorrhage.
testing is required to differentiate PAN-associated perirenal bleeding from other conditions with a similar presentation (Table I). In fact, in the pre-arteriogram era, the vast majority of cases were diagnosed at surgery or autopsy. In the absence of arteriography, clues to a vascular etiology include a poorly visualizing inOctober 1989 The American Journal of Medicine Volume 87
465
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK
TABLE I Leading Causes of Spontaneous Perirenal Hemorrhage* Novlcki etal[5] (n = 194) (%)
McDougaletal[6] (n = 78) (%)
16 16 12 15 5 4 4
58 5 l0 5 5 13 --
Tumor Vascular, nonarteritic Infection Nephritis Blood dyscrasias PAN Hydronephrosis "Adapted from [5] and [6].
TABLE II Presenting Manifestations of Patients with PAN and a Ruptured Renal Aneurysm Characteristic
Number*
Percent
Male Age 25-40 History of hypertension Acute flank/abdominal pain Prior systemic symptoms of PAN Hypotension Flank/abdominal mass Anemia
24128 19/28 19/28 27/28 10/28 14/27 18/27 23/24
86 68 68 96 36 52 67 96
Number of patients with manifestation/number assessable for manifestation m literature reviewed.
TABLE III Course of Patients with PAN and a Ruptured Renal Aneurysm
Survivalwith surgery Survivalwith medical management Recurrent bleeding*
Number
Percent
7/13
54 40 18
6/15 5/28
Three patients had been treated surgically, and two patients had combined medical and surgical treatment prior to recurrence.
travenous pyelogram coupled with a normal retrograde pyelogram [26]. With the advent of arteriography, cases are more promptly diagnosed. The arteriographic findings of PAN have been well described [29]. The finding of multiple small aneurysms involving the renal vasculature is virtually diagnostic, with a specificity approaching 100% [30]. When active bleeding occurs, the site of hemorrhage can also be isolated. Abdominal CT scanning with contrast medium has been described in patients with PAN [27]. It can detect the site of perirenal or abdominal hemorrhage. Although it helps distinguish neoplastic or infiltrative disorders from PAN, its sensitivity and specificity are unknown. Moreover, it is rarely possible to detect intrarenal aneurysms because of their small diameter [27]. In this setting, its utility may be limited to detailmg the site and follow-up of hemorrhage. The optimal management of PAN-induced renal aneurysm rupture is unclear. Supportive therapy accompanied by prompt arteriographic recognition of the condition, with visualization of the bleeding site, is fundamental. In previous reports, both surgery to di-
466
October 1989 The American Journal of Medicine Volume 87
rectly control the hemorrhage and medical therapy for the underlying vasculitis have been employed [1,26,28], but mortality is approximately 50% with either form of management (Table III). Recent use of more aggressive immunosuppressive therapy that includes cyclophosphamide in addition to glucocorticoids may improve survival rates for PAN [31], but experience in this setting is limited [1]. In addition, aggressive treatment of hypertension is mandatory since elevated pressure against a weakened arterial wall may promote aneurysmal rupture [1]. As in our case, the majority of patients with PAN and renal bleeding have pre-existing hypertension. Therapeutic arterial embolization with Gelfoam during arteriography has gained recognition as an alternative to surgery as a technique to control hemorrhage [32], and has been employed in gastrointestinal, traumatic, gynecologic, and tumor-related hemorrhage [32-34]. Gelfoam blocks or strips are selectively embolized after the arteriographic catheter is positioned and the bleeding point is identified. Successful Gelfoam embolization has also been used in an unusuel case of Wegener's granulomatosis in which a subcapsular renal hematoma from a bleeding aneurysm was demonstrated [35]. Overall, Gelfoam selective embolization has been shown to control hemorrhage in 93% of cases, with a procedure-related mortality of 3.6% [32]. Complications following embolization include inadvertent distal infarction [32]. To our knowledge, our case is the first in which Gelfoam was selectively embolized to control aneurysreal bleeding from PAN. Its use, coupled with aggressive medical management of the vasculitis and hypertension, obviated the need for surgical intervention in our patient. Considering the high surgical mortality noted in patients with PAN who develop aneurysmal hemorrhage, we believe that Gelfoam embolization should be considered when a single active renal bleeding site can be demonstrated arteriographical]y. In summary, perirenal hemorrhage should be considered when a patient presents with acute flank and abdominal pain, an accompanying mass, and hypotension or flank tenderness. PAN should be considered as a potential cause of the renal hemorrhage. Prompt arteriography and transcatheter selective embolization of Gelfoam, combined with aggressive medical therapy for PAN and hypertension, should be considered as an alternative to surgery in patients with PAN in whom hemorrhage from a ruptured renal aneurysm is identified. ACKNOWLEDGMENT We wish to thank George Burgermeister, M,D., for his expert arteriography and helpful discussion.
REFERENCES 1. Miller CM, Reiber K, WaxmanJ, etal: Massiveintraabdominal bleed!ngin polyarteritis nodosa. Mt Sinai J Med 1987; 54: 512-515. 2. Homer BA, Hunt OW, Kincaid OW, DeWeerdJH: Perirenal hematoma secondary to intrarenal microaneurysms of periarteritis nodosa demonstrated radiographically. Mayo Clin Proc 1966; 41: 169-178. 3. Schmidt JE: 0ber periarteriitis nodosa. Beitr Path Anat 1908; 43: 455-469. 4. Polkey HJ, Vynalek WJ: Spontaneous nontraumatic perirenal and renal hematomas: an experimental and clinical study. Arch Surg 1933; 26: 196-218. 5. Novicki DE, Turlington JT, Ball TP: The evaluation and management of spontaneous perirenal hemorrhage, J Urol 1980; 123: 764-765. 6. McDougal WC, Kursh ED, Persky L: Spontaneous rupture of the kidney with perirenal hematoma. J Urol 1975; 114: 18]-182.
RUPTURE OF RENAL ARTERY ANEURYSM IN PAN / SMITH AND WERNICK 7. Hardeman EF: Massive bilateral perirenal hemorrhage in periarteritis nodosa. Bull Charlotte Mem Hosp 1944: 1: 35-40, 8. Joachim GR, Becker EL: Spontaneous rupture of the kidney. Arch Intern Med 1965; 115: 176-183. 9. EkengrenA, RiegerA: Spontaneousrupture of the kidney in periarteritis nodosa. Acta Chir Scand 1961; 121: 158-160. 10. Evens RG. Oobry CA, Eckert JF: An exercise in radiologic-patholog~ccorrelation. Radiology 1968; 9]: 1028-1032. 11. Litvak AS, Lucas BA, McRoberts JW: Urologic manifestations of polyarteritis nodosa. J Urol 1976; 115: 572-574. 12. Fleming RJ, Stern LZ: Multiple intraparenchymal renal aneurysmsin polyarteritis nodosa. Radiology 1965; 84: 100-103. 13. McClure PH, Westcott JL: Periarteritis nodosa with perirenal hemorrhage: a case report with angiographic findings. J Urol 1969; 102: 126-129. 14. Wever GK, Perry IH: Periarteritis nodosa: report ol a case with perirenal hemorrhage. JAMA 1935; 104: 1390-1395. 15. Bron KM, Stilley JW, Shapiro AP: Renal arteriography enhanced by tolazoline: value in the diagnosisof polyarteritis nodosa complicated by perinephric hematoma. Radiology 1971; 99: 295-301. 16. Capps JH, Klein RM: Polyarteritis nodosa as a causeof perirenal and retroperio toneal hemorrhage. Radiology 1970; 94: 143-146. 17. Peterson C, Willerson JT, Doppman JL, Decker JL: Polyarteritis nodosa with bilateral renal artery aneurysms and perirenal hematomas: angiographic and nephrotomographic features. Br J Radiol 1970; 43:62-71. 18. Gambill EE, De Weerd JH, Dockerty MB: Periarteritis nodosa complicated with bilateral spontaneous massive perirenal hemorrhage. Minn Med 1968; 51:767769. 19. HendersonFW, Jordan WP: Spontaneousrupture of the kidney due to polyarteritis nodosa: a case report. J Urol 1967; 97: 811-814. 20. Ostrum BJ, Soder PD: Periarteritis nodosacomplicated by spontaneousperinephric hematoma. AJR 1960; 84: 849-860. 21. Cabel E, Holtz S: Polyarteritis as a cause of intestinal hemorrhage. Gastroen-
terology 1971; 61: 99-105. 22. Fort CA: Spontaneousperirenal hematoma secondary to periarteritis nodosa. J Urol 1948; 59: 307-311. 23. McGrae JD: Perirenal hematoma secondary to polyarteritis nodosa. Arch Intern Med 1959; 104: 421-426. 24. Hekali PE, Pajari RI, KivisaariML, et al: Bilateral renal artery dissection: unusual complications of polyarteritis nodosa. Eur J Radiol 1984; 4: 6-8. 25. GerstenbergTH, Nielsen ML, Lindenberg J, Rasmussen BB: Spontaneousnonaneurysmal rupture of the renal pedicle artery due to acute vasculitis in polyarteritis nodosa--with survival. Acta Chir Scand 1978; 144: 549-551. 26. Tocci PE, Lankford RW, Lynne CM: Spontaneousrupture of the kidney secondary to polyarteritis nodosa. J Urol 1975; 113: 860-863. 27. Hekali P, Kivisaari L, StandertskjOId-NordenstamCG. Palari R, Turto H: Renal complications of poiyarteritis nodosa: CT findings. J Comput AssistTomogr 1985; 9: 333-338. 28. SmailowitzZ, KanetiJ, SoberI: Spontaneousperirenal hematoma: a complication of polyarteritis nodosa. J Urol 1979: 121: 82-83. 29. Gron KM, Stroh CA, ShapiroAP: The diagnostic value of angiographic observations in polyarteritis nodosa, Arch Intern Med 1965; 116: 450-459. 30. Albert DA. Rimon O, SilversteinMD: The diagnosisof polyarteritis nodosa: 1. A literature-based decision analysis approach. Arthritis Rheum 1988; 31: 1] 171127. 31. Fauci AS, Katz P, Haynes BF, Wolf SM: Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 1979; 301: 235-238. 32. Jander PH, RussinovichNA: Transcathetergelfoam embolization in abdominal, retroperitoneal, and pelvic hemorrhage. Radiology 1980; 136: 337-344. 33. Spigos DG, Tan WS, JonassonO: Therapeutic emobolization. Techniques and clinical application. Surg Annu 1981; 13: 361-381. 34. Soimakallio S, Lahtinen J, Tanska S: Arterial embolization in the treatment of malignant tumours. Ann Chir Gynaecol 1981; 70:112-I 15. 35. Baker SB, RobinsonDR: Unusual renal manifestation of Wegener's granulomatosis. Am J Med 1978; 64: 883-889.
October 1989
The American Journal of Medicine
Volume 87
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