Vol. 114,
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
Printed
SPONTANEOUS RUPTURE OF THE KIDNEY WITH PERIRENAL HEMATOJVIA W. SCOTT MCDOUGAL,* ELROY D. KURSH
AND
LESTER PERSKY
From the Department ol Surgery, Division of Urology, University Hospitals of Cleveland, Case Western University Medical School, Cleveland, Ohio
ABSTRACT
kidney involves either the parenchyma, the latter associated with a subcapsular or nAr,r,A,~ A review was made of 78 cases of spontaneous hematoma, 4 of our own. One of cases is the spontaneous of the kidney secondary to renal vein thrombosis. The are usually in the fourth decade of life and present with an acute onset of flank a tender mass and symptoms and signs of shock. Hematuria is often ~"·"N"~,- and generally reveals a mass, distorted non-visualization of the
vascular diseases, should be unsuccessful.
Tumors are the most common cause fol.lowed
and blood
HU.~vc,vu
rw,"'"'"''=
0
when
"
rupture of the renal parenchyma with perirenal hematoma occurs rarely, is often undiagnosed preoperatively and results in a high mortality if operative intervention is delayed. Originally reported by Bonet' and later described by Wunderlich 2 it has been called spontaneous apoplexy of the renal or Wunderlich's disease. More than 300 cases have been reported if rupture of the renal extraparenchymal vessels and perirenal structures are included. 3 only 74 documented cases of parenchymal rupture with perirenal have been reported in the English literature. The first case of spontaneous rupture owing to renal. vein thrombosis is reported, 3 other cases of spontaneous rupture are included and the literature is reviewed. CASE REPORTS
Case 1. 8. white women, had dysuria, frequency, gross hematuria and bilateral angle tenderness 2 months before admission to the hospital. A Escherichia coli urine culture at that time and the patient was symptoms abated and she did well until 2 before hospitalization when she noted the sudden onset of severe left flank radiating anteriorly to the suprapubic area, chills and fever. There was no history of trauma. The was a thin, acutely ill appearing woman a blood pressure Accepted for publication November 8, 1974. Read at annual meeting of North Central Section, American Urological Association, Columbus, Ohio, September 18-21, 1974. * Requests for reprints: of Urology, 2065 Adelbert Rd., Cleveland, Ohio
of 28, pulse 100 and temperature 37.9C. The abdomen was slightly distended and a tender mass was in the left lower quadrant. Urinalysis 1.016 gravity, pH 6, 1 plus many white blood cells per high power field and red blood cells (RBCs) and glucose. Hematocit was 25.8, WBC 9.4, 6.9, carbon sodium 136, chlorine 100, blood urea (BUN) 113 and creatinine 7.7. Abdominal roent genograms demonstrated a mass in the left rant. The nous potassium necessitated The urine culture yielded E. coli and antibiotics were started. Retrograde ,w-1rnr1nv revealed calicectasis distortion of the collecting system 1, A) During the ensuing 2 the hematocrit de creased to 18 and tenderness over the abd.omina 1 mass increased. revealed large n>trn,n,01,,t,,n,,,, hematoma with 3 separate fractures of the renal th us necessi tating a nephrectomy 1, B). E. coli was cultured from the parenchyma. n~,wnnn sections showed acute papillary necrosis, and acute m,d chronic pyelonephritis with no evidence of abscess formation. Convalescence was uneventful and though the BUN has remained around 80 tne patient is doing well 8 months ~ncs,,n,a,ec,,-, Case 2. 0. T., 255732, a black woman, was known to have lupus nephritis which controlled with prednisone. The suffered dull pain in the right flank 2 weeks before fadmission to the hospital. The pain 2 days prior to when she was awakuuun,-•uuy
181
182
MCDOUGAL, KURSH AND PERSKY
ened by severe right flank pain. The following morning she had gross hematuria. There was no history of trauma. The patient was an obese woman in moderate distress with a blood pressure of 150/90, pulse 96, respiration 24 and temperature 37.5C. Examination revealed an obese abdomen with tenderness in the right upper quadrant
and flank. There were no palpable masses. Urinalysis revealed 1.016 specific gravity, pH 7, 4 pius protein, occasional WBCs, many RBCs per high power field, and negative glucose and ketones. Hematocrit was 30, WBC 10, BUN 20 and creatinine 1.2. The right kidney was not visualized on excretory urography (IVP). Cystoscopy demon-
FIG. 1. Case 1. A, left retrograde pyelogram shows calicectasis and distortion of collecting system. B, kidney illustrates parenchymal fractures.
FIG. 2. Case 2. A, right retrograde pyelogram demonstrates medial displacement of proximal ureter and distortion of pelvis. B, venogram shows thrombus from right renal vein extending into vena cava.
18'3.
SPONTANEOUS RUPTURE OF KIDNEY WITH PERIRENAL HEMATOMA
strated blood issuing from the right ureteral orifice and retrograde pyelography showed a distorted and displaced pelvis and ureter (fig. 2, A). Renal arteriography revealed delayed filling of the terminal vessels. A venogram demonstrated a thrombus in the right renal vein extending into the vena cava 2, B). Dyspnea and chest pain subsequently developed. A pulmonary embolus was suspected and at operation a large retroperitoneal hematoma was found. Nephrectomy and thrombectomy of the vena cava were performed. Convalescence was uneventful. Pathology showed renal vein thrombosis, renal infarct with fracture of the lower pole and glomerular changes consistent with lupus nephritis. Case 3. L. D., 355888, an 8-year-old white boy, was in good health until 6 days before admission to the hospital when an upper respiratory infection developed. A rash was discovered 3 days later. The diagnosis was varicella. The patient suffered the sudden onset of right upper quadrant pain 1 day before hospitalization. Admission vital signs included blood pressure of 120/70, pulse 100, respiration 24 and temperature 39C. Vesicular lesions in crops with umbilicated centers were noted over the extremities, face and trunk. The abdomen was , tender in the right upper quadrant with spasm of the right rectus muscle. No masses were palpable. Urinalysis revealed 1.026 specific gravity, pH 6, 2 plus protein and negative ketones, RBC, WBC and glucose. IVP revealed an enlarged right kidney with blunting of the superior calix. Liver scan, chest x-ray and bone survey were negative. Severe tenderness in the right upper quadrant, persistent fever and rectus spasm prompted an exploratory operation. A large retroperitoneal hemorrhagic tumor of the right kidney was found and a nephrectomy was performed. Pathology revealed a hemorrhagic and necrotic Wilms tumor. Radiation and actinomycin D were given. The patient is well with no evidence of recurrence 2 years postoperatively. Case 4. B. F., 936502, a 51-year-old white woman with no significant medical had the sudden onset of right flank pain 2 days before admission to the hospital. She denied any history of trauma or urinary tract symptoms. Admission blood pressure was 140/80, pulse 80, respiration 18 and temperature 37C. A tender right lower quadrant mass was palpable. Urinalysis revealed 1.017 specific gravity, pH 7.5, trace of protein, many RBCs per high power field, occasional WBCs, and negative ketones and glucose. Hematocrit was 33.5, WBC 9.8, BUN 12 and creatinine 0.9. An IVP showed a mass in the mid portion of the right kidney and renal angiograms were suggestive of a tumor. The patient was explored through the flank and a renal tumor surrounded by a large amount of recently clotted blood was found. Nephrectomy was performed. Microscopic sections demonstrated a renal cell carcinoma, a renal infarct and perirenal hemorrhage. The patient is well 4 years postoperatively without evidence of recurrent disease.
DISCUSSION
Spontaneous rupture of the classified as involving either the the collecting system. The former is further subdivided with respect to the location of the hematoma-subcapsular or perirenal. A review of literature reveals 78 cases (including our 4) of spontaneous rupture of the renal parenchyma with perirenal hematoma. Cases not documented roentgenography or by visualization of the at operation or autopsy, renal transplants cases involving extraparenchymal tissues wen" excluded. The average age of the 78 patients was 44.5 yearn with a range of 6 to 83 years. There were as many male as female subjects and the rupture occuned with equal frequency on both sides. Of these patients 61 per cent presented with the acute onset of flank or abdominal pain, 43 per cent had either gross or microscopic hematuria and 32.5 per cent presented with symptoms and signs of shock All patients were either tender to palpation or had a mass on the affected side. Of the 78 patients 3 were either pregnant or immediately post parturn_ Of the 40 patients who had an IVP or pyelogram performed, 17 per cent) demonUnderlying diseases and incidence of these diseases in spontaneous rupture of the renal parenchy ma with perirenal hematoma No. Pts. Tumor (57.7%): Benign: Angiomyolipoma Lipoma Adenoma Fibromyoma Hamartoma Papilloma Mesenchymoma Malignant: Clear cell Ca Papillary Ca Sarcoma Renal cell Ca in cyst Wilms Granular cell Ca Vascular (17.9%): Periarteritis nodosa Atheromatous arterial disease Arteriolar nephrosclerosis Renal vein thrombosis with lupus nephritis Infection (10.3%): With abscess Without abscess Nephritis (5.1%): Chronic Acute Focal embolic Blood dyscrasias (5 .1 %) : Coumadin anticoagulation Polycythemia Miscellaneous (3.9%): No pathology Calculus
9 3 3
1 1 1 1 13 4 4
2
2 1 10
2 1
4
4
2 1 1 3
2 1 Total
78
184
MCDOUGAL, KURSH AND PERSKY
strated a mass, 8 (20 per cent) showed non-visuali- literature describes renal vein occlusion. Indeed, zation of the affected kidney and 11 (27 .5 per cent) case 2 is the first report of spontaneous rupture in a revealed a distorted collecting system. Three pa- kidney with an acutely occluded vein. tients had normal IVPs and 1 had a normal Nephrectomy should be performed expeditiously retrograde pyelogram. if mortality and morbidity are to be kept to a The etiology of spontaneous parenchymal rup- minimum. All 12 of the 78 individuals who did not ture with perirenal hematoma is illustrated in the undergo a nephrectomy died. However, of the 61 table. Tumors account for the majority of cases patients who underwent a nephrectomy, 48 re(57.7 per cent) with clear cell carcinoma being covered postoperatively, 10 died and the outcome predominant, followed closely by benign angi- of 3 is unknown. Four patients had suture of the omyolipomas. Vascular disease was the next most parenchymal rupture, 2 of whom died. It appears common offender (17.9 per cent). In this group of from these data that nephrectomy when possible patients periarteritis nodosa occurred most fre- provides the best chance for recovery. quently. Infection, nephritis and blood dyscrasias occurred less commonly. REFERENCES An acute increase in renal vein pressure has been 1. Polkey, H. J. and Vynalek, W. J.: Spontaneous proposed by Polkey and Vynalek as the underlying nontraumatic perirenal and renal hematomas. An mechanism by which parenchymal rupture occurs. experimental and clinical study. Arch. Surg., 26: These investigators ligated the renal vein in 22 196, 1933. dogs and found that 35 per cent had subcapsular 2. Wunderlich, C.R. A.: Handbuch der Pathologie und hematomas, 42.8 per cent had extracapsular hemTherapie, 2nd ed. Stuttgart: Ebner & Seubert, :l856. orrhage and 100 per cent had evidence of a paren- 3. Uson, A. C., Knappenberger, S. T. and Melicow, M. 1 chymal bleed. Unfortunately, this hypothesis has M.: Nontraumatic perirenal hematomas: a report little clinical support since none of the cases in the based on 7 cases. J. Urol., 81: 388, 1959.
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
Printed
SPONTANEOUS RUPTURE OF THE KIDNEY WITH PERIRENAL HEMATOJVIA W. SCOTT MCDOUGAL,* ELROY D. KURSH
AND
LESTER PERSKY
From the Department ol Surgery, Division of Urology, University Hospitals of Cleveland, Case Western University Medical School, Cleveland, Ohio
ABSTRACT
kidney involves either the parenchyma, the latter associated with a subcapsular or nAr,r,A,~ A review was made of 78 cases of spontaneous hematoma, 4 of our own. One of cases is the spontaneous of the kidney secondary to renal vein thrombosis. The are usually in the fourth decade of life and present with an acute onset of flank a tender mass and symptoms and signs of shock. Hematuria is often ~"·"N"~,- and generally reveals a mass, distorted non-visualization of the
vascular diseases, should be unsuccessful.
Tumors are the most common cause fol.lowed
and blood
HU.~vc,vu
rw,"'"'"''=
0
when
"
rupture of the renal parenchyma with perirenal hematoma occurs rarely, is often undiagnosed preoperatively and results in a high mortality if operative intervention is delayed. Originally reported by Bonet' and later described by Wunderlich 2 it has been called spontaneous apoplexy of the renal or Wunderlich's disease. More than 300 cases have been reported if rupture of the renal extraparenchymal vessels and perirenal structures are included. 3 only 74 documented cases of parenchymal rupture with perirenal have been reported in the English literature. The first case of spontaneous rupture owing to renal. vein thrombosis is reported, 3 other cases of spontaneous rupture are included and the literature is reviewed. CASE REPORTS
Case 1. 8. white women, had dysuria, frequency, gross hematuria and bilateral angle tenderness 2 months before admission to the hospital. A Escherichia coli urine culture at that time and the patient was symptoms abated and she did well until 2 before hospitalization when she noted the sudden onset of severe left flank radiating anteriorly to the suprapubic area, chills and fever. There was no history of trauma. The was a thin, acutely ill appearing woman a blood pressure Accepted for publication November 8, 1974. Read at annual meeting of North Central Section, American Urological Association, Columbus, Ohio, September 18-21, 1974. * Requests for reprints: of Urology, 2065 Adelbert Rd., Cleveland, Ohio
of 28, pulse 100 and temperature 37.9C. The abdomen was slightly distended and a tender mass was in the left lower quadrant. Urinalysis 1.016 gravity, pH 6, 1 plus many white blood cells per high power field and red blood cells (RBCs) and glucose. Hematocit was 25.8, WBC 9.4, 6.9, carbon sodium 136, chlorine 100, blood urea (BUN) 113 and creatinine 7.7. Abdominal roent genograms demonstrated a mass in the left rant. The nous potassium necessitated The urine culture yielded E. coli and antibiotics were started. Retrograde ,w-1rnr1nv revealed calicectasis distortion of the collecting system 1, A) During the ensuing 2 the hematocrit de creased to 18 and tenderness over the abd.omina 1 mass increased. revealed large n>trn,n,01,,t,,n,,,, hematoma with 3 separate fractures of the renal th us necessi tating a nephrectomy 1, B). E. coli was cultured from the parenchyma. n~,wnnn sections showed acute papillary necrosis, and acute m,d chronic pyelonephritis with no evidence of abscess formation. Convalescence was uneventful and though the BUN has remained around 80 tne patient is doing well 8 months ~ncs,,n,a,ec,,-, Case 2. 0. T., 255732, a black woman, was known to have lupus nephritis which controlled with prednisone. The suffered dull pain in the right flank 2 weeks before fadmission to the hospital. The pain 2 days prior to when she was awakuuun,-•uuy
181
182
MCDOUGAL, KURSH AND PERSKY
ened by severe right flank pain. The following morning she had gross hematuria. There was no history of trauma. The patient was an obese woman in moderate distress with a blood pressure of 150/90, pulse 96, respiration 24 and temperature 37.5C. Examination revealed an obese abdomen with tenderness in the right upper quadrant
and flank. There were no palpable masses. Urinalysis revealed 1.016 specific gravity, pH 7, 4 pius protein, occasional WBCs, many RBCs per high power field, and negative glucose and ketones. Hematocrit was 30, WBC 10, BUN 20 and creatinine 1.2. The right kidney was not visualized on excretory urography (IVP). Cystoscopy demon-
FIG. 1. Case 1. A, left retrograde pyelogram shows calicectasis and distortion of collecting system. B, kidney illustrates parenchymal fractures.
FIG. 2. Case 2. A, right retrograde pyelogram demonstrates medial displacement of proximal ureter and distortion of pelvis. B, venogram shows thrombus from right renal vein extending into vena cava.
18'3.
SPONTANEOUS RUPTURE OF KIDNEY WITH PERIRENAL HEMATOMA
strated blood issuing from the right ureteral orifice and retrograde pyelography showed a distorted and displaced pelvis and ureter (fig. 2, A). Renal arteriography revealed delayed filling of the terminal vessels. A venogram demonstrated a thrombus in the right renal vein extending into the vena cava 2, B). Dyspnea and chest pain subsequently developed. A pulmonary embolus was suspected and at operation a large retroperitoneal hematoma was found. Nephrectomy and thrombectomy of the vena cava were performed. Convalescence was uneventful. Pathology showed renal vein thrombosis, renal infarct with fracture of the lower pole and glomerular changes consistent with lupus nephritis. Case 3. L. D., 355888, an 8-year-old white boy, was in good health until 6 days before admission to the hospital when an upper respiratory infection developed. A rash was discovered 3 days later. The diagnosis was varicella. The patient suffered the sudden onset of right upper quadrant pain 1 day before hospitalization. Admission vital signs included blood pressure of 120/70, pulse 100, respiration 24 and temperature 39C. Vesicular lesions in crops with umbilicated centers were noted over the extremities, face and trunk. The abdomen was , tender in the right upper quadrant with spasm of the right rectus muscle. No masses were palpable. Urinalysis revealed 1.026 specific gravity, pH 6, 2 plus protein and negative ketones, RBC, WBC and glucose. IVP revealed an enlarged right kidney with blunting of the superior calix. Liver scan, chest x-ray and bone survey were negative. Severe tenderness in the right upper quadrant, persistent fever and rectus spasm prompted an exploratory operation. A large retroperitoneal hemorrhagic tumor of the right kidney was found and a nephrectomy was performed. Pathology revealed a hemorrhagic and necrotic Wilms tumor. Radiation and actinomycin D were given. The patient is well with no evidence of recurrence 2 years postoperatively. Case 4. B. F., 936502, a 51-year-old white woman with no significant medical had the sudden onset of right flank pain 2 days before admission to the hospital. She denied any history of trauma or urinary tract symptoms. Admission blood pressure was 140/80, pulse 80, respiration 18 and temperature 37C. A tender right lower quadrant mass was palpable. Urinalysis revealed 1.017 specific gravity, pH 7.5, trace of protein, many RBCs per high power field, occasional WBCs, and negative ketones and glucose. Hematocrit was 33.5, WBC 9.8, BUN 12 and creatinine 0.9. An IVP showed a mass in the mid portion of the right kidney and renal angiograms were suggestive of a tumor. The patient was explored through the flank and a renal tumor surrounded by a large amount of recently clotted blood was found. Nephrectomy was performed. Microscopic sections demonstrated a renal cell carcinoma, a renal infarct and perirenal hemorrhage. The patient is well 4 years postoperatively without evidence of recurrent disease.
DISCUSSION
Spontaneous rupture of the classified as involving either the the collecting system. The former is further subdivided with respect to the location of the hematoma-subcapsular or perirenal. A review of literature reveals 78 cases (including our 4) of spontaneous rupture of the renal parenchyma with perirenal hematoma. Cases not documented roentgenography or by visualization of the at operation or autopsy, renal transplants cases involving extraparenchymal tissues wen" excluded. The average age of the 78 patients was 44.5 yearn with a range of 6 to 83 years. There were as many male as female subjects and the rupture occuned with equal frequency on both sides. Of these patients 61 per cent presented with the acute onset of flank or abdominal pain, 43 per cent had either gross or microscopic hematuria and 32.5 per cent presented with symptoms and signs of shock All patients were either tender to palpation or had a mass on the affected side. Of the 78 patients 3 were either pregnant or immediately post parturn_ Of the 40 patients who had an IVP or pyelogram performed, 17 per cent) demonUnderlying diseases and incidence of these diseases in spontaneous rupture of the renal parenchy ma with perirenal hematoma No. Pts. Tumor (57.7%): Benign: Angiomyolipoma Lipoma Adenoma Fibromyoma Hamartoma Papilloma Mesenchymoma Malignant: Clear cell Ca Papillary Ca Sarcoma Renal cell Ca in cyst Wilms Granular cell Ca Vascular (17.9%): Periarteritis nodosa Atheromatous arterial disease Arteriolar nephrosclerosis Renal vein thrombosis with lupus nephritis Infection (10.3%): With abscess Without abscess Nephritis (5.1%): Chronic Acute Focal embolic Blood dyscrasias (5 .1 %) : Coumadin anticoagulation Polycythemia Miscellaneous (3.9%): No pathology Calculus
9 3 3
1 1 1 1 13 4 4
2
2 1 10
2 1
4
4
2 1 1 3
2 1 Total
78
184
MCDOUGAL, KURSH AND PERSKY
strated a mass, 8 (20 per cent) showed non-visuali- literature describes renal vein occlusion. Indeed, zation of the affected kidney and 11 (27 .5 per cent) case 2 is the first report of spontaneous rupture in a revealed a distorted collecting system. Three pa- kidney with an acutely occluded vein. tients had normal IVPs and 1 had a normal Nephrectomy should be performed expeditiously retrograde pyelogram. if mortality and morbidity are to be kept to a The etiology of spontaneous parenchymal rup- minimum. All 12 of the 78 individuals who did not ture with perirenal hematoma is illustrated in the undergo a nephrectomy died. However, of the 61 table. Tumors account for the majority of cases patients who underwent a nephrectomy, 48 re(57.7 per cent) with clear cell carcinoma being covered postoperatively, 10 died and the outcome predominant, followed closely by benign angi- of 3 is unknown. Four patients had suture of the omyolipomas. Vascular disease was the next most parenchymal rupture, 2 of whom died. It appears common offender (17.9 per cent). In this group of from these data that nephrectomy when possible patients periarteritis nodosa occurred most fre- provides the best chance for recovery. quently. Infection, nephritis and blood dyscrasias occurred less commonly. REFERENCES An acute increase in renal vein pressure has been 1. Polkey, H. J. and Vynalek, W. J.: Spontaneous proposed by Polkey and Vynalek as the underlying nontraumatic perirenal and renal hematomas. An mechanism by which parenchymal rupture occurs. experimental and clinical study. Arch. Surg., 26: These investigators ligated the renal vein in 22 196, 1933. dogs and found that 35 per cent had subcapsular 2. Wunderlich, C.R. A.: Handbuch der Pathologie und hematomas, 42.8 per cent had extracapsular hemTherapie, 2nd ed. Stuttgart: Ebner & Seubert, :l856. orrhage and 100 per cent had evidence of a paren- 3. Uson, A. C., Knappenberger, S. T. and Melicow, M. 1 chymal bleed. Unfortunately, this hypothesis has M.: Nontraumatic perirenal hematomas: a report little clinical support since none of the cases in the based on 7 cases. J. Urol., 81: 388, 1959.
