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POLYCYSTIC HORSESHOE KIDNEY
0022-5347/97/1586-2229$03 0010 JIIUKRAI. OF UKOLOCY
Copynght 0 1997 by
h E K l C Ah' UKOLlMI('AI.
Vol. 158,2229,December 1997 Printed i n U.S.A.
ASSOCIATION, INC
POLYCYSTIC HORSESHOE KIDNEY F U V I O ANTONIO BRUM, MONICA BECKER, ANGELA UGLIONE CARLOS TEODOSIO DA ROS*
AND
From the Department of Urology, Charity Hospital, Santa Maria, Brazil
KEY W o r m : kidney, polycystic, autosomal dominant; kidney; abnormalities Horseshoe kidney is a renal anomaly in which there is fusion of the 2 renal units across the midline. The incidence of this anomaly ranges from 1 in 400 to 1 in 1,800 Bilateral polycystic disease of the kidney that occurs in adults shows a n autosomal dominant hereditary.] The association of horseshoe kidney with polycystic renal disease in adults is a n extremely rare condition. We report a case of this rare renal abnormality. CASE REPORT
A 27-year-old white man presented with episodic macroscopic hematuria 6 years in duration. H e had no other symptoms. During the physical examination we perceived 2 large Accepted for publication June 20, 1997. * Requests for reprints: Av Carlos Gomes 403, Room 604, Port0 Alcgre. Brazil 90480-003.
FIG.2. Abdominal computerized tomography reveals multiple cysts and fusion on lower pole of kidneys.
nontender masses in t h e abdomen. There were no signs of hepatosplenomegaly. Laboratory data revealed blood urea nitrogen 32 mg./100 ml. (normal less than 40) and creatinine 0.7 mg.llOO ml. (normal less than 1.2). Electrolytes were normal. Urine was grossly bloody and urine culture was negative. Excretory urography revealed polycystic kidney disease within a horseshoe kidney (fig. 1). Abdominal computerized tomography showed both kidneys to be massively enlarged with numerous cysts. The lower pole of t h e kidneys was fused (fig. 2). Our suggestion to t h e patient was to control only t h e blood pressure. At 7-year followup renal function was normal.
DISCUSSION
A horseshoe kidney develops when the medial fusion of the 2 mesonephric blastemas does not occur during embryogenesis. The reported incidence ranges from 1i n 500 t o 1in 1,800 cases.'-3 Adult polycystic disease i s a n autosomal dominant disorder whose incidence is reported in 1i n 250 to 1 i n 5,000 c a ~ e s .The ~ . ~association of these 2 abnormalities, adult polycystic disease and horseshoe kidney, is rare with incidence ranges of 1 in 134,000 to 1 in 8 million cases.2 Less t h a n 20 cases have been reported i n t h e literature.'-3 The approach for these cases must be based on the clinical manifestations of the disease.
REFERENCES
h;. 1. Excretory urography shows horseshoe aspect of kidney, suggesting presence of cysts 2229
1. Trapp, J. D.. Zukoski, C. F., Shelley, H. S. and Goldner, F.: Nephrectomy for polycystic horseshoe kidney with successful cadaver renal allografl. South Med. J., 5 8 1370, 1965. 2. Correa, R. J. and Paton, R. R.: Polycystic horseshoe kidney. J . Urol., 11: 802. 1976. 3. Dobranowski, J.. Somers, S. and Pirani, M.: Adult polycystic horseshoe kidney. J . Cancer Assn. Rad., 37: 208, 1986.
Copynght 0 1997 by
h E K l C Ah' UKOLlMI('AI.
Vol. 158,2229,December 1997 Printed i n U.S.A.
ASSOCIATION, INC
POLYCYSTIC HORSESHOE KIDNEY F U V I O ANTONIO BRUM, MONICA BECKER, ANGELA UGLIONE CARLOS TEODOSIO DA ROS*
AND
From the Department of Urology, Charity Hospital, Santa Maria, Brazil
KEY W o r m : kidney, polycystic, autosomal dominant; kidney; abnormalities Horseshoe kidney is a renal anomaly in which there is fusion of the 2 renal units across the midline. The incidence of this anomaly ranges from 1 in 400 to 1 in 1,800 Bilateral polycystic disease of the kidney that occurs in adults shows a n autosomal dominant hereditary.] The association of horseshoe kidney with polycystic renal disease in adults is a n extremely rare condition. We report a case of this rare renal abnormality. CASE REPORT
A 27-year-old white man presented with episodic macroscopic hematuria 6 years in duration. H e had no other symptoms. During the physical examination we perceived 2 large Accepted for publication June 20, 1997. * Requests for reprints: Av Carlos Gomes 403, Room 604, Port0 Alcgre. Brazil 90480-003.
FIG.2. Abdominal computerized tomography reveals multiple cysts and fusion on lower pole of kidneys.
nontender masses in t h e abdomen. There were no signs of hepatosplenomegaly. Laboratory data revealed blood urea nitrogen 32 mg./100 ml. (normal less than 40) and creatinine 0.7 mg.llOO ml. (normal less than 1.2). Electrolytes were normal. Urine was grossly bloody and urine culture was negative. Excretory urography revealed polycystic kidney disease within a horseshoe kidney (fig. 1). Abdominal computerized tomography showed both kidneys to be massively enlarged with numerous cysts. The lower pole of t h e kidneys was fused (fig. 2). Our suggestion to t h e patient was to control only t h e blood pressure. At 7-year followup renal function was normal.
DISCUSSION
A horseshoe kidney develops when the medial fusion of the 2 mesonephric blastemas does not occur during embryogenesis. The reported incidence ranges from 1i n 500 t o 1in 1,800 cases.'-3 Adult polycystic disease i s a n autosomal dominant disorder whose incidence is reported in 1i n 250 to 1 i n 5,000 c a ~ e s .The ~ . ~association of these 2 abnormalities, adult polycystic disease and horseshoe kidney, is rare with incidence ranges of 1 in 134,000 to 1 in 8 million cases.2 Less t h a n 20 cases have been reported i n t h e literature.'-3 The approach for these cases must be based on the clinical manifestations of the disease.
REFERENCES
h;. 1. Excretory urography shows horseshoe aspect of kidney, suggesting presence of cysts 2229
1. Trapp, J. D.. Zukoski, C. F., Shelley, H. S. and Goldner, F.: Nephrectomy for polycystic horseshoe kidney with successful cadaver renal allografl. South Med. J., 5 8 1370, 1965. 2. Correa, R. J. and Paton, R. R.: Polycystic horseshoe kidney. J . Urol., 11: 802. 1976. 3. Dobranowski, J.. Somers, S. and Pirani, M.: Adult polycystic horseshoe kidney. J . Cancer Assn. Rad., 37: 208, 1986.