Vol. 118, Novernbe,
THE JOURNAL OF UROLOGY
Copyright © 1977 by The Williams & Wilkins Co.
Printed in
POLYCYSTIC KIDNEY DISEASE: A REVIEW OF A. J. SEGAL,* R F. SPATARO
AND
Z. L. BARBARIC
From the vena.nnum.i:s of Radiology, Rochester General Hospital and University of Rochester School of Medicine and Dentistry, Rochester, New York
ABSTRACT
Herein we review 100 cases of adult kidney disease. Flank pain was the most cm,u.c,ac1,uu in these cases. In 20 per cent of the patients calculi were noted and 10 per obstruction. The average age at death was 57 years. The of is emphasized with key radiographic features, such as bilateral large inhomogeneous nephrogram, caliceal distortion and poorly defined renal margins. ment of the liver and spleen may help to confirm the diagnosis. Differential diagnosis bilateral simple cysts, ~"''h'~H•., ~'" .... ~,..~ and bilateral renal carcinomas. Adult disease is inherited as an autosomal dominant at autopsy has been reported from 1 of 222 cases to 1 of 1,019 cases. 1 Inheritance, relative and clinical were incentives for reviewing 100 cases. attention was directed to radioMETHODS
All charts, available radiograms and scans of 100 consecutive ,,-,,,.v,c.o~ with adult polycystic kidney disease at our Memorial Hospital were reviewed. Radiofindings, associated conditions and presenting symptoms or findings were ~H~,.,,~~u
arachnoid hemorrhage or because performed on an asymptomatic patient a history only. Patients ranged from 16 to 90 years old. were present in 20 per cent of the cases, 10 pelviocaliceal system obstruction and 7 per cent obstruction. At least 13 per cent had smaller percentages of polycystic pancreas, and cystic ovary. One cystadenocarcinoma pancreas was diag-nosed and 2 per cent of patients had renal tumors. Most patients ultimately died of renal failure or '"v,u,.,w.,u .. tions of cardiovascular disease. Eight per cent had subarachnoid hemorrhage, 2 per cent had thrombosed renal arteries and l per cent had aortic dissection. The average age at death was approximately 57 years. DISCUSSION
Tomography is invaluable in all cases and, for the young asymptomatic person with a history. There are usually no abnormal radiographic until young adulthood. 1- 3 The only early findings multiple, round, relative lucencies causing an ,,~,c-,o•,c•b nephrogram. 6 The lucencies, which represent
to multiple cysts of
Fm. 1.
polycystic
contours. DESCRIPTION
In this series the diagnosis of polycystic disease was made most because patients presented v«u."·~··.1 with fiank usually was secondary to a calculus or a blood clot with partial c.hd·r·n"t' hemorrhage into a or infection. Other presented with uremia, abdominal mass or masses, subMarch 18, 1977. reprints: Rochester General Hospital, 1425 Port' Rochester, New York 14621. 711
Fm. 2. Venous phase of celiac angiogram shows multiple hepatic and splenic cysts.
712
SEGAL, SPATARO AND BARBARIC
Fm. 3. Multiple simple cysts cause bilateral renal enlargement Demonstration of cystic involvement in liver, spleen or pancreas may help confirm the diagnosis of polycystic kidney disease, which can be proved at the time of operation or with the aid of radionuclides, ultrasound, angiography (fig. 2) or total body opacification. Differential diagnosis is limited. Bilateral multiple simple cysts may be confusing but usually are countable and without as much disturbance of parenchyma or renal contours (fig. 31. 1- 5 Intervening parenchyma is normal. There is no supportive clinical or family history and there are no additional diagnostic examinations to indicate cystic involvement of other organs. Angiography has been helpful in distinguishing diffuse, multiple simple cysts (bilaterally) from polycystic kidney disease. 16 Angiomyolipomatosis, often associated with tuberous sclerosis, may be diagnosed by clinical findings and typical hypervascularity at angiography (fig. 4). It may simulate polycystic kidney disease urographically if both kidneys are involved and there is renal enlargement with deformed calices. 2• 4 • 17• 18 Rarely, bilateral carcinomas may present with bilateral renal enlargement and uremia. 1• 2 REFERENCES
Fm. 4. Angiomyolipomatosis causes bilateral renal enlargement and caliceal distortion. numerous to count. As the disease progresses the cysts enlarge with associated increase in renal size. 7 The latter is almost always bilateral but may be asymmetric or unilateral. 8 • 9 The renal margin becomes less well defined owing to a multitude of cysts deforming it and the pelviocaliceal system becomes stretched and distorted (fig. 1). Nephrolithiasis may be associated and segmental obstruction may occur, usually owing to a calculus, cyst or blood clot. 1- 5, 7• 9--1 4 Occasionally, cyst walls may calcify. 15 There may be associated parenchymal or urothelial tumors. 1• 2 • 4 Although 20 per cent of our patients had 1 or more calculi, this is slightly higher than has been reported in other series. 1• 11 • 13 Dalgaard indicated a sex predilection for calculous formation in polycystic patients. 1 This series does not support that observation. Calculi were present equally in both sexes (10 men and 10 women). Some authors have indicated that the mean survival is approximately 50 years. 1• 2 • 12• 13 Increased longevity (mean age 57 years) in this series is attributed to the increased use of hemodialysis and renal transplantation in the last decade.
1. Dalgaard, 0. Z.: Polycystic disease of the kidneys. In: Diseases
2. 3. 4. 5. 6. 7. 8. 9. 10.
of the Kidney. Edited by M. B. Strauss and L. G. Welt. Boston: Little, Brown & Co., 1963. Bosniak, M. A. and Ambos, M. A.: Polycystic kidney disease. Semin. Roentgen., 10: 133, 1975. Elkin, M. and Bernstein, J.: Cystic diseases of the kidneyradiological and pathological considerations. Clin. Radiol., 20: 65, 1969. Emmett, J. L. and Witten, D. M.: Clinical Urography, 3rd ed. Philadelphia: W. B. Saunders Co., 1971. Hatfield, P. M. and Pfister, R. C.: Adult polycystic disease of the kidneys (Potter type 3). J.A.M.A., 222: 1527, 1972. Halpern, M., Dalrymple, G. and Young, J.: The nephrogram in polycystic disease: an important radiographic sign. J. Urol., 103: 21, 1970. Lalli, A. F. and Poirier, V. C.: Urographic analysis of the development ofpolycystic kidney disease. Amer. J. Roentgen., 119: 705, 1973. Sellers, A. L., Winfield, A. and Rosen, V.: Unilateral polycystic kidney disease. J. Urol., 107: 527, 1972. Simon, H.B. and Thompson, G. J.: Congenital renal polycystic disease: clinical and therapeutic study of 366 cases. J.A.M.A., 159: 657, 1955. Billing, L.: The roentgen diagnosis of polycystic kidneys. Acta Radiol., 41: 305, 1954.
ADULT POLYCYSTIC KIDNEY DISEASE
0. Z.: Bilateral polycystic disease of the kidneys. A of two hundred and eighty-four patients and their families. Acta Med. Scand., suppl. 328, 1957. 12. C. C.: Bilateral polycystic kidney disease: review of 94 Surg., 65: 318, 1952. cases. 13. Rall, J.E. and Odel, H. M.: Congenital polycystic disease of the review of the literature and data on 207 cases. Amer. Sci., 218: 399, 1949. 14. VVard, J. N., Draper, J. W. and Lavengoocl, R. W., Jr.: A clinical review of polycystic kidney disease in 53 patients. J. Urol., 98: 48, 1967. 11.
15. Kutcher, R., Schneider, M. and Gordon, D. H .. Calcification in polycystic disease. Radiolog-y, 122: 77, 1977. 16. Ettinger, A., Kahn, P. C. and Wise, H. 1\/L, Jr.: The of selective renal angiography in the diagnosis of disease. J. Urol., 102: 156, 1969. 17. Anderson, D. and Tannen, R. L.: Tuberous sclerosis and chrnnk renal failure. Potential confusion with polycystic kidney ease. Amer. J. Med., 47: 163, 1969. 18. Wright, F. W., Ledingham, J. G. G., Dunnill, M. S. and Grieve, N. W. T.: Polycystic kidneys, renal hamartomas, their v;iriants and complications. Clin. R.adiol., 25: 27, 1974.
THE JOURNAL OF UROLOGY
Copyright © 1977 by The Williams & Wilkins Co.
Printed in
POLYCYSTIC KIDNEY DISEASE: A REVIEW OF A. J. SEGAL,* R F. SPATARO
AND
Z. L. BARBARIC
From the vena.nnum.i:s of Radiology, Rochester General Hospital and University of Rochester School of Medicine and Dentistry, Rochester, New York
ABSTRACT
Herein we review 100 cases of adult kidney disease. Flank pain was the most cm,u.c,ac1,uu in these cases. In 20 per cent of the patients calculi were noted and 10 per obstruction. The average age at death was 57 years. The of is emphasized with key radiographic features, such as bilateral large inhomogeneous nephrogram, caliceal distortion and poorly defined renal margins. ment of the liver and spleen may help to confirm the diagnosis. Differential diagnosis bilateral simple cysts, ~"''h'~H•., ~'" .... ~,..~ and bilateral renal carcinomas. Adult disease is inherited as an autosomal dominant at autopsy has been reported from 1 of 222 cases to 1 of 1,019 cases. 1 Inheritance, relative and clinical were incentives for reviewing 100 cases. attention was directed to radioMETHODS
All charts, available radiograms and scans of 100 consecutive ,,-,,,.v,c.o~ with adult polycystic kidney disease at our Memorial Hospital were reviewed. Radiofindings, associated conditions and presenting symptoms or findings were ~H~,.,,~~u
arachnoid hemorrhage or because performed on an asymptomatic patient a history only. Patients ranged from 16 to 90 years old. were present in 20 per cent of the cases, 10 pelviocaliceal system obstruction and 7 per cent obstruction. At least 13 per cent had smaller percentages of polycystic pancreas, and cystic ovary. One cystadenocarcinoma pancreas was diag-nosed and 2 per cent of patients had renal tumors. Most patients ultimately died of renal failure or '"v,u,.,w.,u .. tions of cardiovascular disease. Eight per cent had subarachnoid hemorrhage, 2 per cent had thrombosed renal arteries and l per cent had aortic dissection. The average age at death was approximately 57 years. DISCUSSION
Tomography is invaluable in all cases and, for the young asymptomatic person with a history. There are usually no abnormal radiographic until young adulthood. 1- 3 The only early findings multiple, round, relative lucencies causing an ,,~,c-,o•,c•b nephrogram. 6 The lucencies, which represent
to multiple cysts of
Fm. 1.
polycystic
contours. DESCRIPTION
In this series the diagnosis of polycystic disease was made most because patients presented v«u."·~··.1 with fiank usually was secondary to a calculus or a blood clot with partial c.hd·r·n"t' hemorrhage into a or infection. Other presented with uremia, abdominal mass or masses, subMarch 18, 1977. reprints: Rochester General Hospital, 1425 Port' Rochester, New York 14621. 711
Fm. 2. Venous phase of celiac angiogram shows multiple hepatic and splenic cysts.
712
SEGAL, SPATARO AND BARBARIC
Fm. 3. Multiple simple cysts cause bilateral renal enlargement Demonstration of cystic involvement in liver, spleen or pancreas may help confirm the diagnosis of polycystic kidney disease, which can be proved at the time of operation or with the aid of radionuclides, ultrasound, angiography (fig. 2) or total body opacification. Differential diagnosis is limited. Bilateral multiple simple cysts may be confusing but usually are countable and without as much disturbance of parenchyma or renal contours (fig. 31. 1- 5 Intervening parenchyma is normal. There is no supportive clinical or family history and there are no additional diagnostic examinations to indicate cystic involvement of other organs. Angiography has been helpful in distinguishing diffuse, multiple simple cysts (bilaterally) from polycystic kidney disease. 16 Angiomyolipomatosis, often associated with tuberous sclerosis, may be diagnosed by clinical findings and typical hypervascularity at angiography (fig. 4). It may simulate polycystic kidney disease urographically if both kidneys are involved and there is renal enlargement with deformed calices. 2• 4 • 17• 18 Rarely, bilateral carcinomas may present with bilateral renal enlargement and uremia. 1• 2 REFERENCES
Fm. 4. Angiomyolipomatosis causes bilateral renal enlargement and caliceal distortion. numerous to count. As the disease progresses the cysts enlarge with associated increase in renal size. 7 The latter is almost always bilateral but may be asymmetric or unilateral. 8 • 9 The renal margin becomes less well defined owing to a multitude of cysts deforming it and the pelviocaliceal system becomes stretched and distorted (fig. 1). Nephrolithiasis may be associated and segmental obstruction may occur, usually owing to a calculus, cyst or blood clot. 1- 5, 7• 9--1 4 Occasionally, cyst walls may calcify. 15 There may be associated parenchymal or urothelial tumors. 1• 2 • 4 Although 20 per cent of our patients had 1 or more calculi, this is slightly higher than has been reported in other series. 1• 11 • 13 Dalgaard indicated a sex predilection for calculous formation in polycystic patients. 1 This series does not support that observation. Calculi were present equally in both sexes (10 men and 10 women). Some authors have indicated that the mean survival is approximately 50 years. 1• 2 • 12• 13 Increased longevity (mean age 57 years) in this series is attributed to the increased use of hemodialysis and renal transplantation in the last decade.
1. Dalgaard, 0. Z.: Polycystic disease of the kidneys. In: Diseases
2. 3. 4. 5. 6. 7. 8. 9. 10.
of the Kidney. Edited by M. B. Strauss and L. G. Welt. Boston: Little, Brown & Co., 1963. Bosniak, M. A. and Ambos, M. A.: Polycystic kidney disease. Semin. Roentgen., 10: 133, 1975. Elkin, M. and Bernstein, J.: Cystic diseases of the kidneyradiological and pathological considerations. Clin. Radiol., 20: 65, 1969. Emmett, J. L. and Witten, D. M.: Clinical Urography, 3rd ed. Philadelphia: W. B. Saunders Co., 1971. Hatfield, P. M. and Pfister, R. C.: Adult polycystic disease of the kidneys (Potter type 3). J.A.M.A., 222: 1527, 1972. Halpern, M., Dalrymple, G. and Young, J.: The nephrogram in polycystic disease: an important radiographic sign. J. Urol., 103: 21, 1970. Lalli, A. F. and Poirier, V. C.: Urographic analysis of the development ofpolycystic kidney disease. Amer. J. Roentgen., 119: 705, 1973. Sellers, A. L., Winfield, A. and Rosen, V.: Unilateral polycystic kidney disease. J. Urol., 107: 527, 1972. Simon, H.B. and Thompson, G. J.: Congenital renal polycystic disease: clinical and therapeutic study of 366 cases. J.A.M.A., 159: 657, 1955. Billing, L.: The roentgen diagnosis of polycystic kidneys. Acta Radiol., 41: 305, 1954.
ADULT POLYCYSTIC KIDNEY DISEASE
0. Z.: Bilateral polycystic disease of the kidneys. A of two hundred and eighty-four patients and their families. Acta Med. Scand., suppl. 328, 1957. 12. C. C.: Bilateral polycystic kidney disease: review of 94 Surg., 65: 318, 1952. cases. 13. Rall, J.E. and Odel, H. M.: Congenital polycystic disease of the review of the literature and data on 207 cases. Amer. Sci., 218: 399, 1949. 14. VVard, J. N., Draper, J. W. and Lavengoocl, R. W., Jr.: A clinical review of polycystic kidney disease in 53 patients. J. Urol., 98: 48, 1967. 11.
15. Kutcher, R., Schneider, M. and Gordon, D. H .. Calcification in polycystic disease. Radiolog-y, 122: 77, 1977. 16. Ettinger, A., Kahn, P. C. and Wise, H. 1\/L, Jr.: The of selective renal angiography in the diagnosis of disease. J. Urol., 102: 156, 1969. 17. Anderson, D. and Tannen, R. L.: Tuberous sclerosis and chrnnk renal failure. Potential confusion with polycystic kidney ease. Amer. J. Med., 47: 163, 1969. 18. Wright, F. W., Ledingham, J. G. G., Dunnill, M. S. and Grieve, N. W. T.: Polycystic kidneys, renal hamartomas, their v;iriants and complications. Clin. R.adiol., 25: 27, 1974.
