0022-534 7/79/1211-0082$02.00/0 THE JOURNAL OF UROLOGY
Vol. 121, January Printed in U.S.A.
Copyright © 1979 by The Williams & Wilkins Co.
Case Reports SPONTANEOUS PERIRENAL HEMATOMA: A COMPLICATION OF POLYARTERITIS NODOSA Z. SMAILOWITZ,* J. KANETI
From the Department of Urology, The Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheba, Israel
Polyarteritis nodosa is a rare cause of perirenal hematoma owing to rupture of 1 of the aneurysms. Eleven cases have been reported previously in the English literature, including 5 with a preoperative angiographic diagnosis. Our case, which was diagnosed angiographically before a successful conservative operation, is the first report of a patient who survived 3 years. The pathology is discussed. the left kidney was made through a flank incision. At operation 1,500 cc clotted blood was found in the perinephric space, principally around the lower pole extending to the entrance of the pelvic cavity. The lower pole of the kidney was dark with a 0.5 cm. rupture on its surface. No active bleeding occurred at operation. A biopsy was taken near the site of rupture and the wound was closed with drainage of the perinephric space. Postoperatively, the patient received steroid therapy, 100 mg. phenaglycodol daily, immunosuppressive drugs, 50 mg. cyclophosphamide 3 times daily and antihypertensive treatment. The biopsy specimen showed extensive vasculitis indicative of polyarteritis nodosa. The patient was discharged from the hospital 16 days postoperatively with a maintenance dose of corticosteroids and antihypertensive drugs. At followup 3 years later he was well. An IVP revealed 2 well functioning kidneys (fig. 3) and the lower pole of the left kidney showed minimal scarring. The patient is still continuing steroid therapy and antihypertensive drugs. The average blood pressure is 140/80.
The urologist is likely to be involved in the treatment of polyarteritis nodosa principally when an aneurysm ruptures, producing perirenal bleeding. This is a particularly fulminating form of the disease that may affect many different tissues and, therefore, has protean clinical manifestations. CASE REPORT
G. K., a 26-year-old man, was seen at another hospital with severe left flank and left upper quadrant pain. Examination revealed tenderness in the same areas. Blood pressure was 140/90 and pulse was 120. Hemoglobin was 12.6 gm. per cent and the patient was afebrile. A decrease in the blood pressure to 90/50 was noted 2 hours later and the hemoglobin decreased to 10.5 gm. per cent. Repeated examination revealed a left flank mass. The patient received 2 pints of whole blood and the blood pressure increased to 120/80. An emergency excretory urogram (IVP) showed a large mass arising from the lower pole of the left kidney thought to be a hematoma from a tumor. The patient was admitted to our hospital later on the same day. He was anemic with a hemoglobin of9.5 gm. per cent and a white blood count of 7,100. Platelet count was 256,000 with a bleeding time of 2 minutes 30 seconds and a clotting time of 3 minutes. The prothrombin time was 86 per cent. Blood pressure was 190/130 and pulse was 175. An electrocardiogram showed sinus tachycardia. The urine contained 1 plus protein, no sugar, and 4 to 6 white and 10 to 12 red blood cells per high power field. The blood urea was 42 mg. per cent, creatinine 1.1 mg. per cent and blood sugar 93 mg. per cent. Liver function tests, serum protein and electrolytes were within normal limits. A phentolamine test was negative. An aortogram revealed multiple small aneurysms in the kidneys, liver, spleen and mesenteric arteries. Selective renal arteriograms showed microaneurysms arising from the intralobular and arcuate renal arteries (figs. 1 and 2). One of the aneurysms in the lower pole of the left kidney had ruptured, causing a large perirenal hematoma that extended to the iliac bone. Diagnosis was polyarteritis nodosa with a left perirenal hematoma. An emergency exploration of
COMMENTS AND DISCUSSION
Polyarteritis nodosa was first described in 1852 by Rokitansky, and Kussmaul and Maier completed the description in 1866. 1 The pathophysiology of polyarteritis nodosa is poorly understood. It appears to be owing to a deposition of immune complexes in the walls of the medium and small arteries causing necrosis, inflammation, incomplete ruptures, thrombosis and aneurysmal formations. Arkin divided the disease into 4 stages of arterial change: 1) hyaline necrosis of the media; 2) inflammation involving the intima and adventitia, with weakening of the wall, incomplete ruptures, bulging and aneurysmal dilatations; 3) thrombosis with infarctive necrosis of granulation tissue and 4) healing. 2 The destroyed wall was replaced by scar tissue. In any patient different arteries and, in fact, separate branches of the same artery may be seen in various stages of arteritis. The nature of the disease with its multiple organ and system involvement precludes a classic symptom pattern to establish a clinical diagnosis. Confirmation of the specific vascular lesion by biopsy is disappointing. Only 20 to 35 per cent positive results are obtained from skeletal muscle biopsy and 20 per cent from skin biopsy in patients with polyarteritis nodosa. Several authors, in analyzing the incidence of organ involvement in this disease, stress that the kidney is involved in 80 to 100 per
Accepted for publication April 21, 1978. * ~equests for reprints: Department of Urology, The Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheba, Israel. 82
SPONTANEOUS PERIRENAL HEMATOMA
FIG. 1. Preoperative selective arteriograms of A, right and B, left kJdneys show multiple. microaneurysms. Note ruptured aneurysmal d1latat10n above mass m left lower pole of left kidney.
cent of the cases. The liver is involved in approximately 50 to 60 per cent of the cases and the spleen in about 45 per cent. Judging by the available reports in the last decade angiographic examinations are of high diagnostic value in polyarteritis nodosa.:i-5 The presence on angiography of multiple microaneurysms, like clusters of grapes, differing in size, distributed in the intralobular and arcuate renal arteries, particularly in the bifurcations and in other organs from the abdominal cavity, is pathognomonic of this disease. Spontaneous perirenal hematoma as a complication of polyarteritis nodosa was first reported in 1908 by Schmidt. 6 It is not rare for this complication to be the presenting sign. An extensive review of the literature reveals that 34 similar cases appeared in the medical literature but only 11 documented cases have been reported in the English literature. 3 • 7 · 8 This frequently fatal disease is seldom recognized preoperatively. Smith and Hinman indicated that only 2 of 8 cases associated with rupture and perirenal hematoma owing to polyarteritis nodosa had been diagnosed before operation or autopsy. 9 The duration of illness of this fulminating form of disease ranges from 1 day to about 1½ years. Only the patients reported on Fort, 10 and Litvak and associates 8 lived approximately a year after the development of a perirenal hematoma. A plain film usually demonstrates an enlarged renal outline and an IVP adds some degree of displacement of the kidney and ureter or a non-functioning kidney, without demonstrating the nature of the lesion. Angiography is diagnostic and shows the extent of the disease and possibly the site of the rupture, as well as the state of the renal parenchyma and the condition of the other kidney and other organs. Thus, an appropriate operation can be planned. An operation is contraindicated in cases associated with bilateral intrarenal aneurysms or if the aneurysms appear elsewhere in the body, suggesting generalized disease. However, a ruptured aneurysm from polyarteritis nodosa with a perirenal hematoma requires an emergency operation. In such cases all attempts should be made to avoid nephrectomy and to conserve renal tissue. Prof. M. Hirsch aided in the diagnosis of this case. REFERENCES 1. Kussmaul, A. and Maier, R.: Uber eine bisher nicht beschrie-
FIG. 2. P~eoI?erative celiac arteriogram shows multiple microaneurysms m liver and spleen. Note good function of both kidneys. 2.
bene eigenthumliche Arterienerkrankung (Periarteritis Nodosa) die mit Morbus Brightil und rapid fortschreitender allgemeiener muskell ahmung einkergeht. Deutch. Arch. Klin. Med., l: 484, 1866. Arkin, A.: A clinical and pathological study of periarteritis nodosa. A report of five cases, one histologically healed. Amer. J. Path., 6: 401, 1930. Ostrum, B. J. and Soder, P. D.: Periarteritis nodosa complicated by spontaneous perinephric hematoma; roentgenographic findings in three cases and a review of the literature. Amer. J. Roentgen., 84: 849, 1960. Chudiicek, Z.: Angiographic diagnosis ofpolyarteritis nodosa of the liver, kidney and mesentery. Brit. J. Radiol., 40: 864, 1967. McClure, P. H. and Westcott, J. L.: Periarteritis nodosa with perirenal hemorrhage: a case report with angiographic findings. J. Urol., 102: 126, 1969. Schmidt, J. E.: Uber periarteriitis nodosa. Beitr. Path. Anat., 43: 455, 1908. McDougal, W. S., Kursh, E. D. and Persky, L.: Spontaneous rupture of the kidney with perirenal hematoma. J. Urol., 114: 181, 1975. Litvak, A. S., Lucas, B. A. and McRoberts, J. W,: Urologic manifestations ofpolyarteritis nodosa. J. Urol., 115: 572, 1976. Smith, J, N. and Hinman, F., Jr.: Intrarenal arterial aneurysms. J. UroL, 97: 990, 1967. F'o!'t, C, A.: Spontan.eous perirenal hematoma secondary to periarte:ritis nodosc~. J. 1Jrol.; 59: 3Q?, 1913.