Urinary Tract Obstruction in Polycystic Renal Disease 1
Diagnostic Radiology
Zoran L. Barbaric, M.D., Robert F. Spataro, M.D., and Arthur J. Segal, M.D.
TheIncidence and cause of urinary tractobstruction In 100consecutive patients withpolycystic renaldisease arereported. Because of nonspecific symptoms, poorrenal function. and calyceal distortion. obstruction may be difficult to detect. A number of patients had Infundibular obstruction caused bya calculus, clot, or Inflammation and edema of the pelvocalyceal wall. INDEX TERMS: Kidneys, cysts. 8 [1].3121 • Ureters, obstruction • Urinary tract, obstruction, 8[0).840 Radiology 125:627-629, December 1977
RINARY tract obstruction represents a serious complication in patients with polycystic renal disease. This is particularly true when renal function is already severely compromised. so that even partial obstruction can further contribute to its deterioration. Enlarged polycystic kidneys are difficult to examine by excretory urography. Slight changes in nephrographic density or functional delay are difficult to perceive and contribute to misdiagnosis. Delay in diagnosis can be critical for these patients and further decrease the number of functioning nephrons.
U
CLINICAL MATERIAL
Charts and radiographs of 100 consecutive patients seen in the University of Rochester Medical Center and Rochester General Hospital with proved polycystic renal disease were analyzed. Twenty per cent had renal or ureteral calculi. In the majority of patients. hematuria was documented at least once during the course of their illness. Ten patients had ureteral obstruction (TABLE I). Eight demonstrated the usual radiographic picture of ureteral obstruction (delayed and persistent nephrogram, pelvocalyceal dilatation. and ureteral dilatation to the point of the obstruction). In 2 patients the diagnosis was missed because of poor function, large kidneys. and pelvocalyceal distortion. Radiopaque calculi were responsible for ureteral obstruction in 6 patients (Fig. 1). In 3 the obstruction was caused by a nonopaque calculus or blood clot; in 1 it was carcinoma projecting into the renal pelvis (Fig. 2). Seven other patients demonstrated varying degrees of TABLE I:
INCIDENCE AND CAUSE OF URINARY TRACT OBSTRUCTION IN 100 PATIENTS WITH POLYCYSTIC RENAL D ISEASE CAUSE
Ureteral obstruction Calculus Neoplasm Unknown Infundibular obstruction Calculus Unknown Total
No. OF PATIENTS 10
6 1
3
Fig. 1. Excretory urogram shows a radiopaque calculus obstructing the distal left ureter. The calculus was also seen on a preliminary radiograph.
7 1
6
-17--
1 From the Department of Diagnostic Radiology, University of Rochester School of Medicine and Dentistry (Z.L.B., Associate Professor; R.F.S., Assistant Professor), and the Department of Radiology, Rochester General Hospital (A.J.S.),Rochester, N.Y. Accepted for publication In August 1977. slh
627
ZORAN L. BARBARIC AND OTHERS
628
December 1977
Fig. 2. Antegrade percutaneous pyelogram shows a large filling defect in the renal pelvis , causing obstruction and hydronephrosis. Surgery showed cystic nephroblastoma.
infundibular obstruction. A radiopaque calculus was the cause in 1 (Fig. 3); in the others no calculi were seen and it is only conjecture whether the obstruction was caused by a nonopaque calculus, inflammatory edema, compression by a cyst, or a combination of these factors (Figs. 4 and 5). DISCUSSION
Our study confirms the high incidence of urinary calculi in patients with polycystic kidney disease (1-3). The true incidence of urinary tract obstruction, however, is difficult to trace from the literature. Judging from our material, such obstruction is a common complication in these patients, and failure to recognize it may lead to inappropriate management and further deterioration of renal function. The symptoms of obstruction are difficult to separate from other findings commonly seen in patients with nonobstructed polycystic kidneys, including capsular distension, hemorrhage into the cyst, infection, and displacement and/or compression of the visceral organs (3). All of these can be associated with abdominal or flank pain and tenderness (4). Hematuria is a common complaint and is seen with both obstructed and nonobstructed polycystic kidneys. It is easy to see how all these symptoms can mask or be confused with those caused by surgically correctable obstruction. Indeed, a number of patients in our group of 100 did not have excretory urography during episodes of abdominal or flank pain and hematuria.
Fig. 3. Excretory urogram shows infundibular obstruction due to a 2-mm radiopaque calculus, which was also seen on a preliminary radiograph.
The unusually high number of patients with infundibular obstruction is interesting, at least radiographically. It is likely that because of cyst compression the infundibulaare narrowed due to stretching and elongation, trapping small calculi or blood clots. Swelling of the pelvocalyceal wall during frequent infectious episodes is likely to be another contributing factor. Relief of ureteral obstruction remains the primary indication for surgery (5); on the other hand, patients with infundibular obstruction are not likely to benefit from surgical correction. Other common reasons for surgery include nephrectomy prior to transplantation, hemorrhage, uncontrollable infection, neoplasm, or gastrointestinal dysfunction (3). Considering the frequency of ureteral obstruction and calculi in these patients and the ill-defined presenting symptoms, excretory urography should be performed whenever there is any possibility of obstruc tion. Department of Radiology University of Rochester School of Medicine and Dentistry Rochester, N. Y. 14642
REFERENCES 1.
Rail JE, Odel HM: Congenital polycystic disease of the kidney:
Vol . 125
URINARY TRACT OBSTRUCTION IN POLYCYSTIC RENAL DISEASE
Fig. 4. Excretory urogram shows infundibular obstruct ion (cause unknown) in a patient with polycystic disease. Presenting symptoms included pyuria and hematuria. review of the literature, and data on 207 cases. Am J Med Sci 218: 399-407, Oct 1949 2. Simon HB, Thompson GJ: Congenital renal polycystic disease. A clinical and therapeutic study of three hundredsixty-six cases. JAMA 159:657-662, 15 Oct 1955
629
Diagnostic Radiology
Fig. 5. Excretory urogram shows infundibular obstruction (cause unknown) in a patient with hematuria. Blood clots were seen in the renal pelvis.
3. Wallack HI, Kandel G, Presman DC: Polycystic kidneys. Indications for surgical intervention. Urology 3:552-556, May 1974 4. Dalgaard 02: Polycystic disease of the kidney. [In] Strauss MB, Welt LG, ed: Diseases of the Kidney. Boston, Little, Brown, 2d Ed, 1971, Vol 2, Chapt 35, pp 1223-1258 5. Ward IN, Draper JW, Lavengood RW Jr: A clinical review of polycystic kidney disease in 53 patients . J Urol 98:48-53, Jul 1967
Diagnostic Radiology
Zoran L. Barbaric, M.D., Robert F. Spataro, M.D., and Arthur J. Segal, M.D.
TheIncidence and cause of urinary tractobstruction In 100consecutive patients withpolycystic renaldisease arereported. Because of nonspecific symptoms, poorrenal function. and calyceal distortion. obstruction may be difficult to detect. A number of patients had Infundibular obstruction caused bya calculus, clot, or Inflammation and edema of the pelvocalyceal wall. INDEX TERMS: Kidneys, cysts. 8 [1].3121 • Ureters, obstruction • Urinary tract, obstruction, 8[0).840 Radiology 125:627-629, December 1977
RINARY tract obstruction represents a serious complication in patients with polycystic renal disease. This is particularly true when renal function is already severely compromised. so that even partial obstruction can further contribute to its deterioration. Enlarged polycystic kidneys are difficult to examine by excretory urography. Slight changes in nephrographic density or functional delay are difficult to perceive and contribute to misdiagnosis. Delay in diagnosis can be critical for these patients and further decrease the number of functioning nephrons.
U
CLINICAL MATERIAL
Charts and radiographs of 100 consecutive patients seen in the University of Rochester Medical Center and Rochester General Hospital with proved polycystic renal disease were analyzed. Twenty per cent had renal or ureteral calculi. In the majority of patients. hematuria was documented at least once during the course of their illness. Ten patients had ureteral obstruction (TABLE I). Eight demonstrated the usual radiographic picture of ureteral obstruction (delayed and persistent nephrogram, pelvocalyceal dilatation. and ureteral dilatation to the point of the obstruction). In 2 patients the diagnosis was missed because of poor function, large kidneys. and pelvocalyceal distortion. Radiopaque calculi were responsible for ureteral obstruction in 6 patients (Fig. 1). In 3 the obstruction was caused by a nonopaque calculus or blood clot; in 1 it was carcinoma projecting into the renal pelvis (Fig. 2). Seven other patients demonstrated varying degrees of TABLE I:
INCIDENCE AND CAUSE OF URINARY TRACT OBSTRUCTION IN 100 PATIENTS WITH POLYCYSTIC RENAL D ISEASE CAUSE
Ureteral obstruction Calculus Neoplasm Unknown Infundibular obstruction Calculus Unknown Total
No. OF PATIENTS 10
6 1
3
Fig. 1. Excretory urogram shows a radiopaque calculus obstructing the distal left ureter. The calculus was also seen on a preliminary radiograph.
7 1
6
-17--
1 From the Department of Diagnostic Radiology, University of Rochester School of Medicine and Dentistry (Z.L.B., Associate Professor; R.F.S., Assistant Professor), and the Department of Radiology, Rochester General Hospital (A.J.S.),Rochester, N.Y. Accepted for publication In August 1977. slh
627
ZORAN L. BARBARIC AND OTHERS
628
December 1977
Fig. 2. Antegrade percutaneous pyelogram shows a large filling defect in the renal pelvis , causing obstruction and hydronephrosis. Surgery showed cystic nephroblastoma.
infundibular obstruction. A radiopaque calculus was the cause in 1 (Fig. 3); in the others no calculi were seen and it is only conjecture whether the obstruction was caused by a nonopaque calculus, inflammatory edema, compression by a cyst, or a combination of these factors (Figs. 4 and 5). DISCUSSION
Our study confirms the high incidence of urinary calculi in patients with polycystic kidney disease (1-3). The true incidence of urinary tract obstruction, however, is difficult to trace from the literature. Judging from our material, such obstruction is a common complication in these patients, and failure to recognize it may lead to inappropriate management and further deterioration of renal function. The symptoms of obstruction are difficult to separate from other findings commonly seen in patients with nonobstructed polycystic kidneys, including capsular distension, hemorrhage into the cyst, infection, and displacement and/or compression of the visceral organs (3). All of these can be associated with abdominal or flank pain and tenderness (4). Hematuria is a common complaint and is seen with both obstructed and nonobstructed polycystic kidneys. It is easy to see how all these symptoms can mask or be confused with those caused by surgically correctable obstruction. Indeed, a number of patients in our group of 100 did not have excretory urography during episodes of abdominal or flank pain and hematuria.
Fig. 3. Excretory urogram shows infundibular obstruction due to a 2-mm radiopaque calculus, which was also seen on a preliminary radiograph.
The unusually high number of patients with infundibular obstruction is interesting, at least radiographically. It is likely that because of cyst compression the infundibulaare narrowed due to stretching and elongation, trapping small calculi or blood clots. Swelling of the pelvocalyceal wall during frequent infectious episodes is likely to be another contributing factor. Relief of ureteral obstruction remains the primary indication for surgery (5); on the other hand, patients with infundibular obstruction are not likely to benefit from surgical correction. Other common reasons for surgery include nephrectomy prior to transplantation, hemorrhage, uncontrollable infection, neoplasm, or gastrointestinal dysfunction (3). Considering the frequency of ureteral obstruction and calculi in these patients and the ill-defined presenting symptoms, excretory urography should be performed whenever there is any possibility of obstruc tion. Department of Radiology University of Rochester School of Medicine and Dentistry Rochester, N. Y. 14642
REFERENCES 1.
Rail JE, Odel HM: Congenital polycystic disease of the kidney:
Vol . 125
URINARY TRACT OBSTRUCTION IN POLYCYSTIC RENAL DISEASE
Fig. 4. Excretory urogram shows infundibular obstruct ion (cause unknown) in a patient with polycystic disease. Presenting symptoms included pyuria and hematuria. review of the literature, and data on 207 cases. Am J Med Sci 218: 399-407, Oct 1949 2. Simon HB, Thompson GJ: Congenital renal polycystic disease. A clinical and therapeutic study of three hundredsixty-six cases. JAMA 159:657-662, 15 Oct 1955
629
Diagnostic Radiology
Fig. 5. Excretory urogram shows infundibular obstruction (cause unknown) in a patient with hematuria. Blood clots were seen in the renal pelvis.
3. Wallack HI, Kandel G, Presman DC: Polycystic kidneys. Indications for surgical intervention. Urology 3:552-556, May 1974 4. Dalgaard 02: Polycystic disease of the kidney. [In] Strauss MB, Welt LG, ed: Diseases of the Kidney. Boston, Little, Brown, 2d Ed, 1971, Vol 2, Chapt 35, pp 1223-1258 5. Ward IN, Draper JW, Lavengood RW Jr: A clinical review of polycystic kidney disease in 53 patients . J Urol 98:48-53, Jul 1967
