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Unilateral ureteral obstruction secondary to sarcoidosis
CASE REPORTS
UNILATERAL URETERAL OBSTRUCTION SECONDARY TO SARCOIDOSIS* MAJ . LTC. COL . COL . LTC. LTC.
ROGER H . SCHOENFELD, M .C . (USA) WILLIAM D . BELVILLE, M .C. (USA) ALFRED S . BUCK, M .C. (USA) MARTIN L . DRESNER, M .C . (USA) SAMUEL J INSALACO, M .C . (USA) TIMOTHY E . McNAMARA, M .C . (USA)
From the Departments of Surgery (Urology), Pathology, and Medicine, Madigan Army Medical Center, Tacoma, Washington
ABSTRACT-An episode of unilateral ureteral obstruction secondary to sarcoidosis is described .
Corticosteroid treatment resulted in prompt and complete resolution of the obstruction and associated lymphadenopathy. We believe this represents the first reported case of ureteral obstruction secondary to sarcoidosis.
Sarcoidosis is a multisystem granulomatous disease usually involving the lungs and lymphatics . The urinary tract is infrequently involved; however, granulomatous involvement of the renal parenchyma, nephrocalcinosis, and nephrolithiasis have been reported . 1-5 McCoy and Tischer' described the association of membranous and proliferative glomerulonephritis with sarcoidosis . A patient with systemic sarcoidosis was recently evaluated for symptoms of unilateral ureteral obstruction . Urography demonstrated extrinsic ureteral compression . This case is unique in that the obstruction was eliminated by corticosteroid therapy rather than further operative intervention . Case Report A twenty-seven-year-old black man presented to our medical center with fever (102°F), chest pain, and hemoptysis . A pleural "The opinions or assertions contained herein are the private views of the authors and are not be be construed as official or as reflecting the views of the Department of the Army or the Department of Defense .
UROLOGY
/
JANUARY 1985
/ VOLUME XXV, NUMBER I
effusion was seen on chest x-ray film . Shortly thereafter deep vein thrombophlebitis developed requiring anticoagulation . This was followed by recurrent fever, nocturnal diaphoresis, hepatosplenomegaly, and diffuse lymphadenopathy. Sputum smears and cultures for tuberculosis, fungi, and bacteria were all negative . A pleural biopsy revealed a nonspecific fibrinous pleuritis . Inguinal lymph node biopsy demonstrated noncaseating granulomas . An infiltrating process was found by lymphangiogram (Fig . 1A) while computerized axial tomography showed the periaortic nodes to be grossly enlarged . At exploration, a biopsy of the liver and retroperitoneal nodes was done . A splenectomy was performed . All the tissues demonstrated noncaseating epithelial granulomas . Again, special stains and cultures were all negative . By exclusion, therefore, the diagnosis of systemic sarcoidosis was made (Fig . 1B) . Despite no further therapy after laparotomy, all signs and symptoms abated . He was discharged on a coumadin only regimen . One month later colicky left flank pain developed . Physical examination demonstrated left flank
57
(A) Lymphangiogram demonstrating infiltrating process consistent with sarcoidosis . (B) Lowpower magnification of lymph node showing noncaseating granulomas . (C) Excretory urogram (2-hour delay) demonstrating distal left ureteral obstruction . (D) Left retrograde pyeloureterogram showing extrinsic left ureteral compromise . (E) Excretory urogram three weeks after steroid therapy demonstrating resolution of ureteropyelocalycectasis . FIGURE 1 .
tenderness and marked diffuse bilateral adenopathy. Chest x-ray film now showed resolution of the effusion . Complete blood cell count was normal . Prothrombin time was prolonged to forty seconds (control 12 seconds) . Microhematuria was seen on urinalysis . Excretory urography showed a normal right renal unit . Delayed films demonstrated left distal ureteral obstruction (Fig . 1C) . An extrinsic obstruction was later shown by retrograde pyelography (Fig . 1D) . There was no evidence of any intrinsic ureteral lesion . Given the diagnosis of systemic sarcoidosis in this setting, it was presumed that retroperitoneal lymphadenopathy was the cause of the ureteral obstruction . Accordingly, he was be58
gun on 60 mg of prednisone daily. Within the first week of therapy both the lymphadenopathy and the flank pain responded dramatically. Repeat excretory urography three weeks later showed complete resolution of the obstruction (Fig . 1E) . Comment The differential diagnosis primarily entertained in this setting included obstruction secondary to lymphadenopathy or periureteral fibrosis . Alternatively, an anticoagulation complication such as retroperitoneal hematoma or intraureteral clot was considered . We believed the most likely diagnosis was sarcoidUROLOGY
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JANUARY 1985
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VOLUME XXV, NUMBER 1
induced lymphadenopathy especially since classically involved nodes were seen in the area on lymphography. Obstruction here therefore could be direct nodal compression or secondary to periureteral fibrosis .' Fibrosis was considered less likely due to the short duration and ease of reversal of this process . The usual manifestation of urinary obstruction associated with anticoagulation is intraureteral clot . This process was effectively ruled out by retrograde pyelography. Similarly, a retroperitoneal hemorrhage was considered given the delayed clotting parameters . Although unlikely, it is possible that such a hematoma might have resolved within three weeks ; however, there was no change in hematocrit or bilirubin, no flank ecchymosis, nor any evidence of ureteral deviation . Such a prompt response to the steroid therapy associated with the total resolution of palpable lymphadenopathy renders sarcoid nodal compression the most likely diagno-
UROLOGY
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JANUARY 1985 / VOLUME XXV, NUMBER 1
sis. This rapid improvement spared unnecessary surgical intervention . 1031 McIntosh Circle Drive
Joplin, Missouri 64801 (DR . SCHOENFELD) References 1 . Longcope WT, and Freiman DG : A study of sarcoidosis based on combined investigation of 160 cases including 30 autopsies from John S . Hopkins Hospital and Massachusetts General Hospital, Medicine 31 : 1 (1952) . 2. Klatskin G, and Cordon M : Renal complications of sarcoidosis and their relationship to h}percalcemia, Am J Med 15 : 484 (1953) . 3. Lebacq E, Verhaegen H, and Desmet V: Renal involvement in sarcoidosis, Post Grad Med 146 : 526 (1970) . 4 . Bolton WK, et al : Reversible renal failure from isolated granulomatous renal sarcoidosis, Clin Nephrol 5 : 91 (1976) . 5 . Falls WF, et a! : Nonhypercalcemic sarcoid nephropathy, Arch Intern Med 130 : 285 (1972) . 6. McCoy RC, and Tisher CC : Clomerulonephritis associated with sarcoidosis, Am J Pathol 68 : 339 (1972) . 7 . Persky L, Kuish E, and Feldman S : Extrinsic obstruction of the ureter, in Harrison JH, et al (Eds) : Campbell's Urology, Philadelphia, WB Saunders, 1978, vol 1, pp 415-445 .
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UNILATERAL URETERAL OBSTRUCTION SECONDARY TO SARCOIDOSIS* MAJ . LTC. COL . COL . LTC. LTC.
ROGER H . SCHOENFELD, M .C . (USA) WILLIAM D . BELVILLE, M .C. (USA) ALFRED S . BUCK, M .C. (USA) MARTIN L . DRESNER, M .C . (USA) SAMUEL J INSALACO, M .C . (USA) TIMOTHY E . McNAMARA, M .C . (USA)
From the Departments of Surgery (Urology), Pathology, and Medicine, Madigan Army Medical Center, Tacoma, Washington
ABSTRACT-An episode of unilateral ureteral obstruction secondary to sarcoidosis is described .
Corticosteroid treatment resulted in prompt and complete resolution of the obstruction and associated lymphadenopathy. We believe this represents the first reported case of ureteral obstruction secondary to sarcoidosis.
Sarcoidosis is a multisystem granulomatous disease usually involving the lungs and lymphatics . The urinary tract is infrequently involved; however, granulomatous involvement of the renal parenchyma, nephrocalcinosis, and nephrolithiasis have been reported . 1-5 McCoy and Tischer' described the association of membranous and proliferative glomerulonephritis with sarcoidosis . A patient with systemic sarcoidosis was recently evaluated for symptoms of unilateral ureteral obstruction . Urography demonstrated extrinsic ureteral compression . This case is unique in that the obstruction was eliminated by corticosteroid therapy rather than further operative intervention . Case Report A twenty-seven-year-old black man presented to our medical center with fever (102°F), chest pain, and hemoptysis . A pleural "The opinions or assertions contained herein are the private views of the authors and are not be be construed as official or as reflecting the views of the Department of the Army or the Department of Defense .
UROLOGY
/
JANUARY 1985
/ VOLUME XXV, NUMBER I
effusion was seen on chest x-ray film . Shortly thereafter deep vein thrombophlebitis developed requiring anticoagulation . This was followed by recurrent fever, nocturnal diaphoresis, hepatosplenomegaly, and diffuse lymphadenopathy. Sputum smears and cultures for tuberculosis, fungi, and bacteria were all negative . A pleural biopsy revealed a nonspecific fibrinous pleuritis . Inguinal lymph node biopsy demonstrated noncaseating granulomas . An infiltrating process was found by lymphangiogram (Fig . 1A) while computerized axial tomography showed the periaortic nodes to be grossly enlarged . At exploration, a biopsy of the liver and retroperitoneal nodes was done . A splenectomy was performed . All the tissues demonstrated noncaseating epithelial granulomas . Again, special stains and cultures were all negative . By exclusion, therefore, the diagnosis of systemic sarcoidosis was made (Fig . 1B) . Despite no further therapy after laparotomy, all signs and symptoms abated . He was discharged on a coumadin only regimen . One month later colicky left flank pain developed . Physical examination demonstrated left flank
57
(A) Lymphangiogram demonstrating infiltrating process consistent with sarcoidosis . (B) Lowpower magnification of lymph node showing noncaseating granulomas . (C) Excretory urogram (2-hour delay) demonstrating distal left ureteral obstruction . (D) Left retrograde pyeloureterogram showing extrinsic left ureteral compromise . (E) Excretory urogram three weeks after steroid therapy demonstrating resolution of ureteropyelocalycectasis . FIGURE 1 .
tenderness and marked diffuse bilateral adenopathy. Chest x-ray film now showed resolution of the effusion . Complete blood cell count was normal . Prothrombin time was prolonged to forty seconds (control 12 seconds) . Microhematuria was seen on urinalysis . Excretory urography showed a normal right renal unit . Delayed films demonstrated left distal ureteral obstruction (Fig . 1C) . An extrinsic obstruction was later shown by retrograde pyelography (Fig . 1D) . There was no evidence of any intrinsic ureteral lesion . Given the diagnosis of systemic sarcoidosis in this setting, it was presumed that retroperitoneal lymphadenopathy was the cause of the ureteral obstruction . Accordingly, he was be58
gun on 60 mg of prednisone daily. Within the first week of therapy both the lymphadenopathy and the flank pain responded dramatically. Repeat excretory urography three weeks later showed complete resolution of the obstruction (Fig . 1E) . Comment The differential diagnosis primarily entertained in this setting included obstruction secondary to lymphadenopathy or periureteral fibrosis . Alternatively, an anticoagulation complication such as retroperitoneal hematoma or intraureteral clot was considered . We believed the most likely diagnosis was sarcoidUROLOGY
/
JANUARY 1985
1
VOLUME XXV, NUMBER 1
induced lymphadenopathy especially since classically involved nodes were seen in the area on lymphography. Obstruction here therefore could be direct nodal compression or secondary to periureteral fibrosis .' Fibrosis was considered less likely due to the short duration and ease of reversal of this process . The usual manifestation of urinary obstruction associated with anticoagulation is intraureteral clot . This process was effectively ruled out by retrograde pyelography. Similarly, a retroperitoneal hemorrhage was considered given the delayed clotting parameters . Although unlikely, it is possible that such a hematoma might have resolved within three weeks ; however, there was no change in hematocrit or bilirubin, no flank ecchymosis, nor any evidence of ureteral deviation . Such a prompt response to the steroid therapy associated with the total resolution of palpable lymphadenopathy renders sarcoid nodal compression the most likely diagno-
UROLOGY
/
JANUARY 1985 / VOLUME XXV, NUMBER 1
sis. This rapid improvement spared unnecessary surgical intervention . 1031 McIntosh Circle Drive
Joplin, Missouri 64801 (DR . SCHOENFELD) References 1 . Longcope WT, and Freiman DG : A study of sarcoidosis based on combined investigation of 160 cases including 30 autopsies from John S . Hopkins Hospital and Massachusetts General Hospital, Medicine 31 : 1 (1952) . 2. Klatskin G, and Cordon M : Renal complications of sarcoidosis and their relationship to h}percalcemia, Am J Med 15 : 484 (1953) . 3. Lebacq E, Verhaegen H, and Desmet V: Renal involvement in sarcoidosis, Post Grad Med 146 : 526 (1970) . 4 . Bolton WK, et al : Reversible renal failure from isolated granulomatous renal sarcoidosis, Clin Nephrol 5 : 91 (1976) . 5 . Falls WF, et a! : Nonhypercalcemic sarcoid nephropathy, Arch Intern Med 130 : 285 (1972) . 6. McCoy RC, and Tisher CC : Clomerulonephritis associated with sarcoidosis, Am J Pathol 68 : 339 (1972) . 7 . Persky L, Kuish E, and Feldman S : Extrinsic obstruction of the ureter, in Harrison JH, et al (Eds) : Campbell's Urology, Philadelphia, WB Saunders, 1978, vol 1, pp 415-445 .
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