- Email: [email protected]
Spontaneous rupture of the kidney
Spontaneous Rupture of the Kidney A Cause of Atraumatic
Retroperitoneal
Bleeding
J. S. Howalt, MD, Boston, Massachusetts J. W. Squires, MD, Boston, Massachusetts
Retroperitoneal bleeding frequently presents a diagnostic and theraputic dilemma. This is particularly evident when the patient has not sustained traumatic injury. Under these conditions, a variety of causes have been described. The purpose of this report is to call attention to atraumatic rupture of the kidney as a source of retroperitoneal bleeding. Although the condition has been described in the past, the majority of reports appear in the earlier literature. A diagnostic approach will be presented based on our experience with three cases. Guidelines for therapy are suggested. The scarcity of recent well detailed cases prompted this review. Case Reports Case I. The patient (SF, NECH 7%24-43), a fifty-eight year old white male physician, was abruptly seized with severe left flank pain while sitting quietly at home. He was immediately admitted to another hospital wheie examination revealed mild hypertension, obesity, and a tender mass in the left flank. The presence of a mass in the left flank was confirmed by a plain x-ray film of the abdomen. Additional diagnostic work-up included retrograde pyelography, intravenous pyelogram, and repeated abdominal films, which revealed no abnormality except for the continued suspicion of a retroperitoneal mass. The pL?ient had a past history of a cerebrovascular accident twelve years prior to admission from which he made a complete recoveiy, returning to active practice. He had also been treated for marked diastolic and systolic hypertension attributed in large measure to obesity. Additional complaints include one episode of right ienal colic, diverticulosis coli, and hyperuricemia. After a course of observation, the patient was transferred to the New England Medical Center for further study. From the New England Medical Center and the Boston Veterans Administration Hospital, Boston, Massachusetts. Reprint requests should be addressed to Dr Squires, Boston Veterans Administration Hospital, Surgical Service, 150 South Huntington Avenue, Boston, Massachusetts 02130. Presented at the Fifty-Second Annual Meeting of the New England Surgical Society, Portsmouth, New Hampshire, September 30. October 1 and2, 1971.
484
On admission, the only complaint was vague discomfort in the left flank. Physical examination revealed marked obesity. The blood pressure was 180/110 mm Hg and the pulse was 120 per minute and regular. There was distinct tenderness in the left flank with involuntary guarding lateral to the rectus sheath on the left side. Needle aspiration revealed a small amount of old, unclotted blood. Pertinent laboratory studies revealed hemoglobin of 12.6 gm per 100 ml, white blood cell count of 18,300 per mm3, and a normal urine sediment. Mild azotemia was present with a blood urea nitrogen level of 54 mg per 100 ml; and a creatinine value of 2.4 mg per 100 ml. Abdominal aortography with a selective left renal arteriogram was immediately obtained. (Figure 1.) The left renal artery appeared to be displaced downward and distorted by a l&ge perinephric shadow. No intrinsic lesion of the kidney was seen. The aorta and its principal branches were considered to be normal. During the next forty-eight hours the patient continued to exhibit tenderness over the left flank associated with a falling hemoglobin and hematocrit as well as persistent tachycardia. On the second hospital day, emergency abdominal exploration was performed. A large retroperitoneal hematoma was approached by reflection of the descending colon and splenic flexure towards the midline. The source of bleeding appeared to be the lower pole of the left kidney which contained a small area of clot resting in a depression in the cortex measuring approximately 2.5 by 2.5 cm. The remainder of the kidney appeared grossly normal. Because of the pre-existing renal disease and the absence of significant pathology in the remaining kidney parenchyma, the small lower pole clot was excised and oversewn. Postoperatively a transient period of oliguria developed which was corrected by the administration of fluids. He was discharged with no further evidence of bleeding. Microscopic examination of the specimen obtained from the lower pole revealed ischemic necrosis of the cortex with hemorrhage. Marked changes of arterial and arteriolar nephrosclerosis were present. Case II. The patient (SA, NECH 76-60-83), a seventyfive year old man, was admitted five days after the abrupt
The American Journal of Surgery
Spontaneous
onset of severe right flank pain, which had prompted admission to another hospital. For many years the patient had been treated for gout, prostatic hypertrophy, chronic obstructive pulmonary disease, and hypertension. Physical examination initially was unrevealing except for the presence of severe pain. Laboratory studies revealed the following: hemoglobin, 11.7 gm per 100 ml; white blood cell count 15,000 per mm3; and normal urine sediment. Intravenous pyelogram showed nonfunction of the right kidney. A tentative diagnosis of right ureteral calculi was made. On the third hospital day the hemoglobin was 8.6 gm per 100 ml. The following day the patient continued to exhibit signs of anemia as well as a bilateral bronchopneumonia which precluded further diagnostic studies. Because of difficulties in cross matching blood, he was transferred to the New England Medical Center Hospitals for further diagnosis and therapy. Upon admission to this hospital, the blood pressure was 250/100 mm Hg, and the pulse 68 per minute and regular. The patient was complaining of severe right flank and subscapular pain. Abdominal examination was again unremarkable. Results of laboratory studies were unchanged from those of the preceding day with continued evidence of anemia. On the day after admission, aortography and inferior vena cava studies were carried out. The right kidney was displaced laterally with stretching of the right renal artery. The inferior vena cava was displaced toward the left by a mass adjacent to the right kidney. Abdominal aspiration revealed nonclotting blood, and prompted emergency laparotomy after the contrast studies. A large hematoma was present in the retroperitoneal space, surrounding the right kidney. There was a tear in the superior pole of the right kidney. No other source of bleeding could be identified. Biopsy of the kidney parenchyma in the area of the superior pole revealed fresh focal hemorrhagic necrosis with patchy tubular ischemic necrosis and severe occlusive vascular disease. The hematoma was evacuated and the area oversewn. No active bleeding was observed at the time of surgery. Immediately after surgery, the patient appeared to be doing well with adequate urine output and stable vital signs. Six hours postoperatively, however, respiratory and cardiac arrest abruptly developed and he could not be resuscitated. An autopsy was not performed. Case III. The patient (PW, NECH 79-97-29), a fifty-two year old white woman, was admitted for the first time to the New England Medical Center Hospitals for evaluation of relapsing polychondritis of eight years’ duration, for which steroids and salicylates had been intermittently administered. At the time of admission she was receiving prednisone, 30 mg per day. During the weeks immediately preceding hospitalization, the patient complained of laryngitis,’ myalgias, and vague arthralgias. Physical examination was unrevealing except for a blood pressure of 180/109 mm Hg, an apical systolic murmur and a healed incision from a prior hysterectomy. Laboratory work-up revealed, the following: hematocrit, 36 per cent; white blood cell count 16,900 per mms; many red blood cells and white cells as well as some large granular cells in the urine sediment;
Volume
123,
April 1972
Rupture of Kidney
Flgure 1. Case I. Left renal arteriogram showing artery displaced downward and distorted by large perinephric shadow.
blood urea nitrogen 9 mg per 100 ml; and creatinine, 0.8 mg per 100 ml. A clean catch urine culture revealed coliform organisms in a concentration of 10,000 to 30,000 colonies per ml; and Diptheroids, Staphylococcus albus, and Enterococcus in a concentration greater than 100,000 colonies per ml. The antinuclear antibody was positive to a dilution of 1.64. The remainder of results of the initial laboratory work-up were within normal limits. On the first hospital day, the patient suddenly noted the onset of severe pain in the left flank which radiated to the anterior abdominal wall and which was associated with a fall in blood pressure to 70/50 mm Hg. Intensive monitoring and resuscitative measures were promptly instituted followed by an emergency intravenous pyelogram. This study showed no visualization of the left kidney until seven hours after the injection, when it was seen to be displaced laterally by a soft tissue mass. The calyces appeared normal. Later that same day emergency left renal and adrenal arteriography was performed. This revealed stretching and displacement of the renal and adrenal arteries by a large left paravertebral mass. No definite intrinsic abnormalities were noted within the left kidney. The patient was carefully observed during the next several days with a diagnosis of spontaneous hemorrhage from the left kidney. During this time the urine sediment gradually reverted to normal, the blood urea nitrogen and creatinine values remained unchanged. The hematocrit fell to 23.1 per cent on the day of the bleeding, but gradually returned to 35.4 per cent by the fourth hospital day with appropriate replacement therapy. A repeat intravenous pyelogram on the tenth hospital day showed prompt excretion of the dye by both kidneys but with continued displacement by the mass on the left side. The patient was discharged home on
485
Howalt and Squires
Figure 2.
Diagnostic outline of retroperitoneal Meedlng.
the fourteenth day, without complaint and on a regimen of steroids, antihypertensives, and antimetabolites. Comments A painful tender flank mass associated with blood loss is the common clinical feature of retroperitoneal bleeding. Immediate consideration should be devoted to deciding whether or not a history of trauma is present or, in fact, the bleeding has arisen atraumatically. The proper management of traumatic retroperitoneal bleeding remains a matter of debate and will not be further discussed. Atraumatic retroperitoneal collections most commonly, in older patients, arise from rupture of abdominal aortic aneurysms or related vascular pathology. A second general group includes bleeding secondary to the administration of drugs or resulting from hemorrhagic diathiasis. A final category includes spontaneous bleeding from viscera, which for all practical purposes means the kidney or adrenal gland. In either instance there may be preexisting disease or the hemorrhage may arise de novo from previously normal parenchyma. Bleeding from the adrenal gland has recently been reviewed by Lawson et al [l]. Spontaneous disruption of the kidney has been described for many years. Such patients usually present the evidence of bleeding although rarely urine is sequestered in the retroperitoneum [2]. Very infrequently perirenal collections of air are generated [3] by the presence of gas-producing organisms in the upper urinary tract which escape to the adjacent tissue planes through disruptions of the collecting system. It is not always easy to document a history of trauma. Mechanical injury to the bony pelvis and/or damage to the great vessels and viscera constitute the common sources of bleeding in these circumstances. None of our patients could recall any traumatic episodes, although emphasis was placed on the
486
significance of this information. In two instances, aspiration of the flank confirmed the presence of blood. In the two cases in which tissue specimens were obtained, evidence of occlusive vascular disease was obtained. The only preoperative clue to the correct tissue diagnosis was the long-standing history of hypertension supported by blood pressure measurements subsequent to admission. A summary of these considerations is outlined in Figure 2. Spontaneous rupture of the kidney with perirenal hematoma was originally described by Wunderlich [4] in 1856. For a period of time the condition was associated with his name, a custom which has been abandoned in modern times. It is not known if this original case was truly idiopathic or secondary. An exhaustive review of the problem covering the preceding seventy-six years was presented by Polkey and Vynalek [5] in 1933. This report is difficult to interpret, however, because the authors include examples of subscapular hematomas as well as renal and perirenal cysts. In more recent years a number of reports described spontaneous bleeding from the kidney. Unfortunately many of them are not sufficiently detailed to accurately determine predisposing causes. In the majority, however, as in the patients described here, renal pathology is present. A frequently cited example is the patient presented by Irwin [6] in 1943. Retroperitoneal and abdominal bleeding was encountered in a young male patient undergoing exploration for suspected perforation of a duodenal ulcer. The source of bleeding proved to be the right kidney upon which appeared a break in the cortex. There was no history of trauma. However, microscopic examination revealed “chronic pyelonephritis,” placing this case in the category of spontaneous secondary hemorrhage. A large variety of pathologic processes have been implicated in spontaneous secondary bleeding from the kidney. These include two reports associated with pregnancy. One of these involved rupture of a hydronephrotic kidney in the immediate postpartum period along with thrombosis of the ovarian vein [7]. Cohen and Pearlman [8] described a woman in the sixth month of pregnancy who underwent emergency nephrectomy for bleeding from an angiomyolipoma (renal hamartoma). There was no history of trauma. In 1946 Ekman [9] reviewed seventeen cases of “spontaneous perirenal hematoma” in the European literature. Thirteen patients presented with documented renal disease including glomerulonephritis, hydronephrosis, periarteritis nodosa, neoplasms, hemophilia, and arteriosclerosis. In three patients no tissue was obtained, the area being drained. In one patient the kidney appeared normal, and in one final
The American Journal of Surgery
Spontaneous
patient the site of bleeding was thought to have originated in the adrenal gland. Isolated case reports have appeared over the last twenty-five years describing patients with specific underlying causes. These have included hydronephrosis [lo], aneurysms of the interlobular arteries and arteritis [II], periarteritis nodosa [12], hypertension [13], congenital hydronephrosis [14], and in a series by Uson et al [15] anaplastic carcinoma, polycythemia secondary to the tetralogy of Fallot, liposarcoma, and pyelonephritis. There are scattered reports that appear to document true atraumatic primary renal bleeding. These include two patients described by Uson et al [15] and one patient by Martin [14]. Levy and Hudson [16] noted the problems of a forty-four year old woman who presented with sudden flank pain and a mass; the abdomen was explored, showing the kidney to be surrounded by blood. No definite lesion could be identified by the pathologist although the authors note the “suspicion” of a cyst wall in one of the microscopic sections. Murphy and Harney [17] reported a twenty-four year old man with a similar clinical picture and no history of trauma. A 5 cm long fissure was present on the posterior surface of the right kidney which was removed. A massive retroperitoneal hemorrhage appeared to have arisen from this source. Microscopic examination revealed a normal renal parenchyma except for the cortical disruption. Despite these isolated reports, primary bleeding from a normal kidney must be exceedingly rare. Awareness of these facts is essential for proper evaluation of a patient presenting with a retroperitoneal collection of blood in the absence of trauma. Clinical examination will reveal hypertension or other types of primary vascular disease. Routine roentgenographic diagnostic procedures will help to establish the presence of known predisposing causes including hydronephrosis, tumors, cysts, and congenital abnormalities. Hematologic study will aid in uncovering a primary bleeding disorder as well as the effects of anticoagulant therapy. The management of these problems will obviously depend upon the nature of the suspected pathology and the urgency of the bleeding. An unanswered question in the current literature is the proper course to follow when presented with the clinical picture of retroperitoneal bleeding with no history of trauma and no demonstrable disease on roentgenographic examination except for nonvisualization or displacement of the kidney shadow. The most difficult differential problem is to exclude the possibility of a bleeding aortic aneurysm. In each of our patients the bleeding was occurring at a rate slow enough to provide time for observation as well as diagnostic maneuvers. More urgent problems would
Volume 123, April 1972
Rupture of Kidney
clearly require operative intervention at an early time. The normal appearance of the aorta on aortography, although not completely excluding the possibility of an aneurysm, was also helpful in this clinical setting of slow-hemorrhage. The presence of hypertension wab the only clue to underlying renal disease except in the first patient who exhibited mild azotemia. Surgical intervention was carried out because of evidence of continued bleeding at a slow rate. The case reports reviewed herein indicate that such patients, in general, have been managed by nephrectomy. It is our opinion that a conservative approach is indicated for spontaneous retroperitoneal bleeding if the appropriate diagnostic studies fail to demonstrate vascular or renal disease. In the patients who underwent operation, the bleeding had ceased by the time surgery was performed. The third patient benefited from the experience of the other two and conservative management was fully justified. It must be emphasized, however, that this nonoperative approach is indicated only when radiologic studies have failed to demonstrate such known predisposing renal causes of bleeding such as tumors, cysts, and hydronephrosis as well as extrarenal pathology. Inability to demonstrate the renal shadow probably reflects a more generalized process within the renal parenchyma as demonstrated in our patients. The fact that this is a reversible phenomenon can be seen in the patient who did not undergo surgery. The basic pathology in all these patients appears to be hypertensive vascular disease involving the smaller vessels of the kidney. This is evident in both specimens obtained for microscopic examination. The bilateral nature of these changes makes nephrectomy unappealing and further justifies a conservative approach or the minimal procedure necessary to control bleeding if surgery becomes necessary. Summary Retroperitoneal bleeding presents a familiar clinical picture to the practicing surgeon. The majority of such cases arise from traumatic injury to retroperitoneal structures or to complications associated with abdominal aneurysms. This report describes three patients -who spontaneously presented the symptoms and signs of retroperitoneal bleeding without any antecedent cause. In two instances exploratory laparotomy demonstrated a bleeding point in the kidney which appeared to be secondary to spontaneous disruption of the renal cortex. Microscopic findings revealed only nephrosclerosis. Both operative cases were managed by evacuation of the clot and oversewing of the bleeding point. One patient survived and one died. The third patient was treated
487
Howalt and Squires
with volume replacement and made an uneventful recovery. The management of such cases and a review of the earlier literature are presented. References 1. Lawson DW et al: Massive retroperitoneal adrenal hemorrhage. Surg Gynec Obstet 129: 989, 1969. 2. Valtonen EJ: Spontaneous rupture of an apparently normal kidney; some criticism concerning this diagnosis. Brit J Ural 38: 484, 1966. 3. Yu SF: Spontaneous renal and perirenal emphysema. Brit J Radio/ 39: 466, 1966. 4. Wunderlich CRA: Hundbuch der Pathologie und Therapie, ed 2. Stuttgart, Ebner & Seubert, 1856. 5. Polkey HS, Vynalek WJ: Spontaneous non-traumatic perirenal and renal hematomas. Arch Surg 26: 196, 1933. 6. Irwin RG: Spontaneous rupture of kidney: case report. US Naval Med Bull 41: 818, 1943. 7. Bridge RAC: Spontaneous rupture of the kidney secondary to ovarian vein obstruction. Amer Surg 35: 67, 1989. 8. Cohen SG, Pearlman CK: Spontaneous rupture of the kidney in pregnancy. J Ural 100: 365, 1968. 9. Ekman H: Displacement of the kidney consequent to spontaneous perirenal hematoma. Acta Chir Stand 93: 531, 1946. 10. Shaw RE: Spontaneous rupture of kidney. Brit J Surg 45: 68, 1957. 11. Joachim GR, Becker EL: Spontaneous rupture of the kidney. Arch Int Med 115: 176,1965. 12. Horner BA, et al: Perirenal hematoma secondary to intrarenal microaneurysms of periarteritis nodosa demonstrated radiographically. Proc Mayo C/in 41: 169, 1966. 13. Link GS: Spontaneous bilateral perirenal hematoma. J Ural 69: 13, 1953. 14. Martin KW: Spontaneous circumrenal hematoma. Brit Med Jl: 1118,1949. 15. Uson AC, et al: Nontraumatic perirenal hematomas: a report based on 7 cases. J Ural 81: 388.1959. 16. Levy SI, Hudson N: A case of spontaneous perirenal hematoma. Brit J Ural 23: 168, 1951. 17. Murphy GE, Harney C: Spontaneous rupture of the normal kidney parenchyma. Ann Surg 134: 127, 1951.
Discussion Ernest K. Landsteiner (Providence, RI): The term spontaneous conjures up the notion that traumatic rupture can occur in a completely healthy kidney. I believe that, although this may occur, it is indeed very rare. On the other hand, rupture of a previously diseased kidney is not so uncommon, although it is still not an everyday occurrence. This paper serves to remind us of this possibility and this entity should be thought of when one is confronted with a patient with flank pain, a flank mass, and signs of internal bleeding. Predisposing causes, as reviewed by Drs Howalt and Squires, include occlusive vascular disease, hypertension and vascular anomalies and aneurysms, periarteritis calculus disease and hydronodosa, pyelonephritis, nephrosis, renal cyst, tumor (particularly hamartoma), nephritis, and hydronephrosis associated with pregnancy. Renal hamartomas are more prone to spontaneous rupture, as are renal artery aneurysms associated with pregnancy. The triad of x-ray findings in spontaneous rupture are:
488
large renal mass, nonvisualization on intravenous pyelography, and an essentially normal retrograde pyelogram with displacement. Awareness of this entity and its particular association with vascular disease will help to assure correct diagnosis and treatment. Frank C. Wheelock, Jr (Boston, Mass): We have encountered one episode of this entity which we did not handle quite as well as did Drs Howalt and Squires. This patient presented with a large hematoma, having had bleeding for four or five days. We operated, thinking it was a ruptured aneurysm, and found a large hematoma around the kidney which felt as though it contained a tumor. We therefore removed the entire mass and, on opening it, were quite surprised that there was just a hematoma formed by a small spontaneous rupture of the kidney. I would like to ask the authors if they agree that sometimes it is hard to rule out the presence of a tumor in the midst of a large hematoma. If one is faced with such a problem, I presume that one should not dissect into the hematoma to look for a tumor because, if one were found, a chance of cure would be obviated by the spilling of the cancer cells. Richard E. Wilson (Boston, Mass): Just for completeness, I want to point out that in our transplant series at the Peter Bent Brigham Hospital we have had six patients with spontaneous rupture of the transplant and massive bleeding. In all cases but one, we have been able to save the kidney. Usually, there is a laceration on the convex surface of the kidney, but sometimes the laceration starts at the hilum and extends toward one of the poles. Suture of the laceration with Gelfoams packing and replacement of blood lost has made preservation of kidneys possible. Gerald 0. Strauch (Stamford, Conn): It is interesting to hear this report at a time when one hears of an increasing number of cases of hemorrhage from apparent spontaneous rupture of small branches of the celiac and mesenteric vascular beds. It is fascinating to speculate as to the cause. James W. Squires (closing): From the left side, we approach the lesion by reflection of the spleen, swinging the tail of the pancreas toward the midline, in the manner in which one might approach pheochromocytoma on that side. On the right side, we excise the peritoneal reflection along the colon, swinging that structure away from the duodenum, towards the midline. I think there are, undoubtedly, a number of ways to approach the problem, depending upon the patient. Obviously, I think the transabdominal approach is indicated. In regard to Dr Wheelock’s comment, if one had the opportunity, as we did, to obtain an arteriogram and selective renal studies, the possibility of ‘stumbling unbeknownst on a tumor would be diminished. I think that this fear is very real, but we did not have the problem because of the arteriographic studies. Doctor Wilson’s comment is interesting. In the transplant situation, I would think that in addition to the diagnoses presented here, thrombosis of the renal vein and acute graft rejection would have to be included in the differential diagnosis.
The American Journal of Surgery
Retroperitoneal
Bleeding
J. S. Howalt, MD, Boston, Massachusetts J. W. Squires, MD, Boston, Massachusetts
Retroperitoneal bleeding frequently presents a diagnostic and theraputic dilemma. This is particularly evident when the patient has not sustained traumatic injury. Under these conditions, a variety of causes have been described. The purpose of this report is to call attention to atraumatic rupture of the kidney as a source of retroperitoneal bleeding. Although the condition has been described in the past, the majority of reports appear in the earlier literature. A diagnostic approach will be presented based on our experience with three cases. Guidelines for therapy are suggested. The scarcity of recent well detailed cases prompted this review. Case Reports Case I. The patient (SF, NECH 7%24-43), a fifty-eight year old white male physician, was abruptly seized with severe left flank pain while sitting quietly at home. He was immediately admitted to another hospital wheie examination revealed mild hypertension, obesity, and a tender mass in the left flank. The presence of a mass in the left flank was confirmed by a plain x-ray film of the abdomen. Additional diagnostic work-up included retrograde pyelography, intravenous pyelogram, and repeated abdominal films, which revealed no abnormality except for the continued suspicion of a retroperitoneal mass. The pL?ient had a past history of a cerebrovascular accident twelve years prior to admission from which he made a complete recoveiy, returning to active practice. He had also been treated for marked diastolic and systolic hypertension attributed in large measure to obesity. Additional complaints include one episode of right ienal colic, diverticulosis coli, and hyperuricemia. After a course of observation, the patient was transferred to the New England Medical Center for further study. From the New England Medical Center and the Boston Veterans Administration Hospital, Boston, Massachusetts. Reprint requests should be addressed to Dr Squires, Boston Veterans Administration Hospital, Surgical Service, 150 South Huntington Avenue, Boston, Massachusetts 02130. Presented at the Fifty-Second Annual Meeting of the New England Surgical Society, Portsmouth, New Hampshire, September 30. October 1 and2, 1971.
484
On admission, the only complaint was vague discomfort in the left flank. Physical examination revealed marked obesity. The blood pressure was 180/110 mm Hg and the pulse was 120 per minute and regular. There was distinct tenderness in the left flank with involuntary guarding lateral to the rectus sheath on the left side. Needle aspiration revealed a small amount of old, unclotted blood. Pertinent laboratory studies revealed hemoglobin of 12.6 gm per 100 ml, white blood cell count of 18,300 per mm3, and a normal urine sediment. Mild azotemia was present with a blood urea nitrogen level of 54 mg per 100 ml; and a creatinine value of 2.4 mg per 100 ml. Abdominal aortography with a selective left renal arteriogram was immediately obtained. (Figure 1.) The left renal artery appeared to be displaced downward and distorted by a l&ge perinephric shadow. No intrinsic lesion of the kidney was seen. The aorta and its principal branches were considered to be normal. During the next forty-eight hours the patient continued to exhibit tenderness over the left flank associated with a falling hemoglobin and hematocrit as well as persistent tachycardia. On the second hospital day, emergency abdominal exploration was performed. A large retroperitoneal hematoma was approached by reflection of the descending colon and splenic flexure towards the midline. The source of bleeding appeared to be the lower pole of the left kidney which contained a small area of clot resting in a depression in the cortex measuring approximately 2.5 by 2.5 cm. The remainder of the kidney appeared grossly normal. Because of the pre-existing renal disease and the absence of significant pathology in the remaining kidney parenchyma, the small lower pole clot was excised and oversewn. Postoperatively a transient period of oliguria developed which was corrected by the administration of fluids. He was discharged with no further evidence of bleeding. Microscopic examination of the specimen obtained from the lower pole revealed ischemic necrosis of the cortex with hemorrhage. Marked changes of arterial and arteriolar nephrosclerosis were present. Case II. The patient (SA, NECH 76-60-83), a seventyfive year old man, was admitted five days after the abrupt
The American Journal of Surgery
Spontaneous
onset of severe right flank pain, which had prompted admission to another hospital. For many years the patient had been treated for gout, prostatic hypertrophy, chronic obstructive pulmonary disease, and hypertension. Physical examination initially was unrevealing except for the presence of severe pain. Laboratory studies revealed the following: hemoglobin, 11.7 gm per 100 ml; white blood cell count 15,000 per mm3; and normal urine sediment. Intravenous pyelogram showed nonfunction of the right kidney. A tentative diagnosis of right ureteral calculi was made. On the third hospital day the hemoglobin was 8.6 gm per 100 ml. The following day the patient continued to exhibit signs of anemia as well as a bilateral bronchopneumonia which precluded further diagnostic studies. Because of difficulties in cross matching blood, he was transferred to the New England Medical Center Hospitals for further diagnosis and therapy. Upon admission to this hospital, the blood pressure was 250/100 mm Hg, and the pulse 68 per minute and regular. The patient was complaining of severe right flank and subscapular pain. Abdominal examination was again unremarkable. Results of laboratory studies were unchanged from those of the preceding day with continued evidence of anemia. On the day after admission, aortography and inferior vena cava studies were carried out. The right kidney was displaced laterally with stretching of the right renal artery. The inferior vena cava was displaced toward the left by a mass adjacent to the right kidney. Abdominal aspiration revealed nonclotting blood, and prompted emergency laparotomy after the contrast studies. A large hematoma was present in the retroperitoneal space, surrounding the right kidney. There was a tear in the superior pole of the right kidney. No other source of bleeding could be identified. Biopsy of the kidney parenchyma in the area of the superior pole revealed fresh focal hemorrhagic necrosis with patchy tubular ischemic necrosis and severe occlusive vascular disease. The hematoma was evacuated and the area oversewn. No active bleeding was observed at the time of surgery. Immediately after surgery, the patient appeared to be doing well with adequate urine output and stable vital signs. Six hours postoperatively, however, respiratory and cardiac arrest abruptly developed and he could not be resuscitated. An autopsy was not performed. Case III. The patient (PW, NECH 79-97-29), a fifty-two year old white woman, was admitted for the first time to the New England Medical Center Hospitals for evaluation of relapsing polychondritis of eight years’ duration, for which steroids and salicylates had been intermittently administered. At the time of admission she was receiving prednisone, 30 mg per day. During the weeks immediately preceding hospitalization, the patient complained of laryngitis,’ myalgias, and vague arthralgias. Physical examination was unrevealing except for a blood pressure of 180/109 mm Hg, an apical systolic murmur and a healed incision from a prior hysterectomy. Laboratory work-up revealed, the following: hematocrit, 36 per cent; white blood cell count 16,900 per mms; many red blood cells and white cells as well as some large granular cells in the urine sediment;
Volume
123,
April 1972
Rupture of Kidney
Flgure 1. Case I. Left renal arteriogram showing artery displaced downward and distorted by large perinephric shadow.
blood urea nitrogen 9 mg per 100 ml; and creatinine, 0.8 mg per 100 ml. A clean catch urine culture revealed coliform organisms in a concentration of 10,000 to 30,000 colonies per ml; and Diptheroids, Staphylococcus albus, and Enterococcus in a concentration greater than 100,000 colonies per ml. The antinuclear antibody was positive to a dilution of 1.64. The remainder of results of the initial laboratory work-up were within normal limits. On the first hospital day, the patient suddenly noted the onset of severe pain in the left flank which radiated to the anterior abdominal wall and which was associated with a fall in blood pressure to 70/50 mm Hg. Intensive monitoring and resuscitative measures were promptly instituted followed by an emergency intravenous pyelogram. This study showed no visualization of the left kidney until seven hours after the injection, when it was seen to be displaced laterally by a soft tissue mass. The calyces appeared normal. Later that same day emergency left renal and adrenal arteriography was performed. This revealed stretching and displacement of the renal and adrenal arteries by a large left paravertebral mass. No definite intrinsic abnormalities were noted within the left kidney. The patient was carefully observed during the next several days with a diagnosis of spontaneous hemorrhage from the left kidney. During this time the urine sediment gradually reverted to normal, the blood urea nitrogen and creatinine values remained unchanged. The hematocrit fell to 23.1 per cent on the day of the bleeding, but gradually returned to 35.4 per cent by the fourth hospital day with appropriate replacement therapy. A repeat intravenous pyelogram on the tenth hospital day showed prompt excretion of the dye by both kidneys but with continued displacement by the mass on the left side. The patient was discharged home on
485
Howalt and Squires
Figure 2.
Diagnostic outline of retroperitoneal Meedlng.
the fourteenth day, without complaint and on a regimen of steroids, antihypertensives, and antimetabolites. Comments A painful tender flank mass associated with blood loss is the common clinical feature of retroperitoneal bleeding. Immediate consideration should be devoted to deciding whether or not a history of trauma is present or, in fact, the bleeding has arisen atraumatically. The proper management of traumatic retroperitoneal bleeding remains a matter of debate and will not be further discussed. Atraumatic retroperitoneal collections most commonly, in older patients, arise from rupture of abdominal aortic aneurysms or related vascular pathology. A second general group includes bleeding secondary to the administration of drugs or resulting from hemorrhagic diathiasis. A final category includes spontaneous bleeding from viscera, which for all practical purposes means the kidney or adrenal gland. In either instance there may be preexisting disease or the hemorrhage may arise de novo from previously normal parenchyma. Bleeding from the adrenal gland has recently been reviewed by Lawson et al [l]. Spontaneous disruption of the kidney has been described for many years. Such patients usually present the evidence of bleeding although rarely urine is sequestered in the retroperitoneum [2]. Very infrequently perirenal collections of air are generated [3] by the presence of gas-producing organisms in the upper urinary tract which escape to the adjacent tissue planes through disruptions of the collecting system. It is not always easy to document a history of trauma. Mechanical injury to the bony pelvis and/or damage to the great vessels and viscera constitute the common sources of bleeding in these circumstances. None of our patients could recall any traumatic episodes, although emphasis was placed on the
486
significance of this information. In two instances, aspiration of the flank confirmed the presence of blood. In the two cases in which tissue specimens were obtained, evidence of occlusive vascular disease was obtained. The only preoperative clue to the correct tissue diagnosis was the long-standing history of hypertension supported by blood pressure measurements subsequent to admission. A summary of these considerations is outlined in Figure 2. Spontaneous rupture of the kidney with perirenal hematoma was originally described by Wunderlich [4] in 1856. For a period of time the condition was associated with his name, a custom which has been abandoned in modern times. It is not known if this original case was truly idiopathic or secondary. An exhaustive review of the problem covering the preceding seventy-six years was presented by Polkey and Vynalek [5] in 1933. This report is difficult to interpret, however, because the authors include examples of subscapular hematomas as well as renal and perirenal cysts. In more recent years a number of reports described spontaneous bleeding from the kidney. Unfortunately many of them are not sufficiently detailed to accurately determine predisposing causes. In the majority, however, as in the patients described here, renal pathology is present. A frequently cited example is the patient presented by Irwin [6] in 1943. Retroperitoneal and abdominal bleeding was encountered in a young male patient undergoing exploration for suspected perforation of a duodenal ulcer. The source of bleeding proved to be the right kidney upon which appeared a break in the cortex. There was no history of trauma. However, microscopic examination revealed “chronic pyelonephritis,” placing this case in the category of spontaneous secondary hemorrhage. A large variety of pathologic processes have been implicated in spontaneous secondary bleeding from the kidney. These include two reports associated with pregnancy. One of these involved rupture of a hydronephrotic kidney in the immediate postpartum period along with thrombosis of the ovarian vein [7]. Cohen and Pearlman [8] described a woman in the sixth month of pregnancy who underwent emergency nephrectomy for bleeding from an angiomyolipoma (renal hamartoma). There was no history of trauma. In 1946 Ekman [9] reviewed seventeen cases of “spontaneous perirenal hematoma” in the European literature. Thirteen patients presented with documented renal disease including glomerulonephritis, hydronephrosis, periarteritis nodosa, neoplasms, hemophilia, and arteriosclerosis. In three patients no tissue was obtained, the area being drained. In one patient the kidney appeared normal, and in one final
The American Journal of Surgery
Spontaneous
patient the site of bleeding was thought to have originated in the adrenal gland. Isolated case reports have appeared over the last twenty-five years describing patients with specific underlying causes. These have included hydronephrosis [lo], aneurysms of the interlobular arteries and arteritis [II], periarteritis nodosa [12], hypertension [13], congenital hydronephrosis [14], and in a series by Uson et al [15] anaplastic carcinoma, polycythemia secondary to the tetralogy of Fallot, liposarcoma, and pyelonephritis. There are scattered reports that appear to document true atraumatic primary renal bleeding. These include two patients described by Uson et al [15] and one patient by Martin [14]. Levy and Hudson [16] noted the problems of a forty-four year old woman who presented with sudden flank pain and a mass; the abdomen was explored, showing the kidney to be surrounded by blood. No definite lesion could be identified by the pathologist although the authors note the “suspicion” of a cyst wall in one of the microscopic sections. Murphy and Harney [17] reported a twenty-four year old man with a similar clinical picture and no history of trauma. A 5 cm long fissure was present on the posterior surface of the right kidney which was removed. A massive retroperitoneal hemorrhage appeared to have arisen from this source. Microscopic examination revealed a normal renal parenchyma except for the cortical disruption. Despite these isolated reports, primary bleeding from a normal kidney must be exceedingly rare. Awareness of these facts is essential for proper evaluation of a patient presenting with a retroperitoneal collection of blood in the absence of trauma. Clinical examination will reveal hypertension or other types of primary vascular disease. Routine roentgenographic diagnostic procedures will help to establish the presence of known predisposing causes including hydronephrosis, tumors, cysts, and congenital abnormalities. Hematologic study will aid in uncovering a primary bleeding disorder as well as the effects of anticoagulant therapy. The management of these problems will obviously depend upon the nature of the suspected pathology and the urgency of the bleeding. An unanswered question in the current literature is the proper course to follow when presented with the clinical picture of retroperitoneal bleeding with no history of trauma and no demonstrable disease on roentgenographic examination except for nonvisualization or displacement of the kidney shadow. The most difficult differential problem is to exclude the possibility of a bleeding aortic aneurysm. In each of our patients the bleeding was occurring at a rate slow enough to provide time for observation as well as diagnostic maneuvers. More urgent problems would
Volume 123, April 1972
Rupture of Kidney
clearly require operative intervention at an early time. The normal appearance of the aorta on aortography, although not completely excluding the possibility of an aneurysm, was also helpful in this clinical setting of slow-hemorrhage. The presence of hypertension wab the only clue to underlying renal disease except in the first patient who exhibited mild azotemia. Surgical intervention was carried out because of evidence of continued bleeding at a slow rate. The case reports reviewed herein indicate that such patients, in general, have been managed by nephrectomy. It is our opinion that a conservative approach is indicated for spontaneous retroperitoneal bleeding if the appropriate diagnostic studies fail to demonstrate vascular or renal disease. In the patients who underwent operation, the bleeding had ceased by the time surgery was performed. The third patient benefited from the experience of the other two and conservative management was fully justified. It must be emphasized, however, that this nonoperative approach is indicated only when radiologic studies have failed to demonstrate such known predisposing renal causes of bleeding such as tumors, cysts, and hydronephrosis as well as extrarenal pathology. Inability to demonstrate the renal shadow probably reflects a more generalized process within the renal parenchyma as demonstrated in our patients. The fact that this is a reversible phenomenon can be seen in the patient who did not undergo surgery. The basic pathology in all these patients appears to be hypertensive vascular disease involving the smaller vessels of the kidney. This is evident in both specimens obtained for microscopic examination. The bilateral nature of these changes makes nephrectomy unappealing and further justifies a conservative approach or the minimal procedure necessary to control bleeding if surgery becomes necessary. Summary Retroperitoneal bleeding presents a familiar clinical picture to the practicing surgeon. The majority of such cases arise from traumatic injury to retroperitoneal structures or to complications associated with abdominal aneurysms. This report describes three patients -who spontaneously presented the symptoms and signs of retroperitoneal bleeding without any antecedent cause. In two instances exploratory laparotomy demonstrated a bleeding point in the kidney which appeared to be secondary to spontaneous disruption of the renal cortex. Microscopic findings revealed only nephrosclerosis. Both operative cases were managed by evacuation of the clot and oversewing of the bleeding point. One patient survived and one died. The third patient was treated
487
Howalt and Squires
with volume replacement and made an uneventful recovery. The management of such cases and a review of the earlier literature are presented. References 1. Lawson DW et al: Massive retroperitoneal adrenal hemorrhage. Surg Gynec Obstet 129: 989, 1969. 2. Valtonen EJ: Spontaneous rupture of an apparently normal kidney; some criticism concerning this diagnosis. Brit J Ural 38: 484, 1966. 3. Yu SF: Spontaneous renal and perirenal emphysema. Brit J Radio/ 39: 466, 1966. 4. Wunderlich CRA: Hundbuch der Pathologie und Therapie, ed 2. Stuttgart, Ebner & Seubert, 1856. 5. Polkey HS, Vynalek WJ: Spontaneous non-traumatic perirenal and renal hematomas. Arch Surg 26: 196, 1933. 6. Irwin RG: Spontaneous rupture of kidney: case report. US Naval Med Bull 41: 818, 1943. 7. Bridge RAC: Spontaneous rupture of the kidney secondary to ovarian vein obstruction. Amer Surg 35: 67, 1989. 8. Cohen SG, Pearlman CK: Spontaneous rupture of the kidney in pregnancy. J Ural 100: 365, 1968. 9. Ekman H: Displacement of the kidney consequent to spontaneous perirenal hematoma. Acta Chir Stand 93: 531, 1946. 10. Shaw RE: Spontaneous rupture of kidney. Brit J Surg 45: 68, 1957. 11. Joachim GR, Becker EL: Spontaneous rupture of the kidney. Arch Int Med 115: 176,1965. 12. Horner BA, et al: Perirenal hematoma secondary to intrarenal microaneurysms of periarteritis nodosa demonstrated radiographically. Proc Mayo C/in 41: 169, 1966. 13. Link GS: Spontaneous bilateral perirenal hematoma. J Ural 69: 13, 1953. 14. Martin KW: Spontaneous circumrenal hematoma. Brit Med Jl: 1118,1949. 15. Uson AC, et al: Nontraumatic perirenal hematomas: a report based on 7 cases. J Ural 81: 388.1959. 16. Levy SI, Hudson N: A case of spontaneous perirenal hematoma. Brit J Ural 23: 168, 1951. 17. Murphy GE, Harney C: Spontaneous rupture of the normal kidney parenchyma. Ann Surg 134: 127, 1951.
Discussion Ernest K. Landsteiner (Providence, RI): The term spontaneous conjures up the notion that traumatic rupture can occur in a completely healthy kidney. I believe that, although this may occur, it is indeed very rare. On the other hand, rupture of a previously diseased kidney is not so uncommon, although it is still not an everyday occurrence. This paper serves to remind us of this possibility and this entity should be thought of when one is confronted with a patient with flank pain, a flank mass, and signs of internal bleeding. Predisposing causes, as reviewed by Drs Howalt and Squires, include occlusive vascular disease, hypertension and vascular anomalies and aneurysms, periarteritis calculus disease and hydronodosa, pyelonephritis, nephrosis, renal cyst, tumor (particularly hamartoma), nephritis, and hydronephrosis associated with pregnancy. Renal hamartomas are more prone to spontaneous rupture, as are renal artery aneurysms associated with pregnancy. The triad of x-ray findings in spontaneous rupture are:
488
large renal mass, nonvisualization on intravenous pyelography, and an essentially normal retrograde pyelogram with displacement. Awareness of this entity and its particular association with vascular disease will help to assure correct diagnosis and treatment. Frank C. Wheelock, Jr (Boston, Mass): We have encountered one episode of this entity which we did not handle quite as well as did Drs Howalt and Squires. This patient presented with a large hematoma, having had bleeding for four or five days. We operated, thinking it was a ruptured aneurysm, and found a large hematoma around the kidney which felt as though it contained a tumor. We therefore removed the entire mass and, on opening it, were quite surprised that there was just a hematoma formed by a small spontaneous rupture of the kidney. I would like to ask the authors if they agree that sometimes it is hard to rule out the presence of a tumor in the midst of a large hematoma. If one is faced with such a problem, I presume that one should not dissect into the hematoma to look for a tumor because, if one were found, a chance of cure would be obviated by the spilling of the cancer cells. Richard E. Wilson (Boston, Mass): Just for completeness, I want to point out that in our transplant series at the Peter Bent Brigham Hospital we have had six patients with spontaneous rupture of the transplant and massive bleeding. In all cases but one, we have been able to save the kidney. Usually, there is a laceration on the convex surface of the kidney, but sometimes the laceration starts at the hilum and extends toward one of the poles. Suture of the laceration with Gelfoams packing and replacement of blood lost has made preservation of kidneys possible. Gerald 0. Strauch (Stamford, Conn): It is interesting to hear this report at a time when one hears of an increasing number of cases of hemorrhage from apparent spontaneous rupture of small branches of the celiac and mesenteric vascular beds. It is fascinating to speculate as to the cause. James W. Squires (closing): From the left side, we approach the lesion by reflection of the spleen, swinging the tail of the pancreas toward the midline, in the manner in which one might approach pheochromocytoma on that side. On the right side, we excise the peritoneal reflection along the colon, swinging that structure away from the duodenum, towards the midline. I think there are, undoubtedly, a number of ways to approach the problem, depending upon the patient. Obviously, I think the transabdominal approach is indicated. In regard to Dr Wheelock’s comment, if one had the opportunity, as we did, to obtain an arteriogram and selective renal studies, the possibility of ‘stumbling unbeknownst on a tumor would be diminished. I think that this fear is very real, but we did not have the problem because of the arteriographic studies. Doctor Wilson’s comment is interesting. In the transplant situation, I would think that in addition to the diagnoses presented here, thrombosis of the renal vein and acute graft rejection would have to be included in the differential diagnosis.
The American Journal of Surgery