UNUSUAL PRESENTATION NATHAN JAMES
P. SHAPPLEY,
E. KEETON,
OF CECOURETEROCELE
M.D.
M.D.
From the Department of Surgery, University Medical Center, Jackson, Mississippi
of Mississippi
ABSTRACT - A three-year-old female had a nephroureterectomy for a hydronephrotic left kidney with a total duplicated collecting system. Postoperatively urinary retention developed which was found to be secondary to a previously nonobstructive cecoureterocele. A brief review of cecoureteroceles is given along with the possible cause of this unusual presentation.
The cecoureterocele is a rare type of ectopic ureterocele which has only recently been recognized as a separate clinical entity in the literais used to describe ture. 1,2The term ureterocele a cystic ballooning of the lower end of the ureter in the bladder lumen3 There are two types of ureterocele: the simple ureterocele usually seen in adults and the ectopic, which is usually seen in children.4 An ectopic ureterocele is most commonly associated with an ectopic ureter from the upper pole segment of a duplicated collecting system. Generally, there is associated renal dysplasia of the upper pole.5 The cecoureterocele is a special type of ureterocele because the lumen extends beyond its orifice as a long tongue or cecum in the urethral submucosa, and the orifice communicates with the bladder lumen and is large and incompetent. ’ Case Report A three-year-old white female was seen by her family physician in September, 1973, with dysuria, frequency, and low-grade fever. She had two additional febrile episodes in February, 1974, and was treated for respiratory infections. Later that month, she was found to have a urinary tract infection and was admitted to the hospital for treatment. At that time a cystogram showed massive left ureteral reflux into the lower collecting system (Fig. 1A). Intravenous pyelogram showed prompt function in a hypertrophied right kidney and function in the left upper collection system (Fig. 1B). Cystoscopy showed a normal right ureteral orifice and a patulous left ureteral
UROLOGY
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NOVEMBER
1975
/
VOLUME VI, NUMBER 5
orifice. Because of the extremely poor function, a left nephrectomy was performed. At the time of nephrectomy the kidney was found to have a total duplicated collecting system. The distal ureter was removed through a second low abdominal incision, and the ureters were transected flush with the bladder. The patient had an uneventful postoperative course and was discharged on March 6, 1974, on cephalexin therapy. After discharge, she had dysuria, difficulty voiding, and marked constipation. She was treated with numerous antibiotics without relief of symptoms. Intravenous pyelogram in May, 1974, showed massive hydronephrosis and hydroureter on the right (Fig. 1C). After adequate treatment of her urinary tract infection, a cystogram showed massive right ureterovesical reflux and a distorted bladder neck (Fig. lD), both of which were not present on the previous radiologic studies. She was found to have a residual urine of 150 cc. ; cystoscopic examination, however, revealed no obstruction. She was placed on catheter drainage and referred to the University of Mississippi Medical Center in May, 1974, for further evaluation. Cystoscopy showed a markedly inflamed bladder. The right ureteral orifice was not seen; however, what was thought to be the stump of the left ureter was seen. This appeared as a small diverticulum. A bifid vagina was noted at vaginoscopy. A catheter was reinserted and removed one day later. The following day she was found to have 100 cc. residual urine, and the catheter was left indwelling. Bentamicin therapy was
605
FIGURE 1. (A) Preoperative cystogram showing massive left ureterovesical r-e&x. (9) Preoperative intravenous pyelogram showing left upper pole collecting system (arrow) and normal right kidney. (C) Postoperative intravenous pyelogram demonstrating moderate hydronephrosis and hydroureter. (D) Postoperative voiding cystourethrogram showing massive right ureteral reflux and distorted bladder neck. (E) Postoperative intravenous pyelogram after resection of ureterocele showing normal collecting system. (F) Postoperative cystogram after resection of ureterocele showing less distortion of bladder neck.
given, and the urine remained sterile. Blood urea nitrogen was 13 mg. and creatinine 0.1 mg. per 100 ml. Intermittent catheter drainage was tried without success. A repeat voiding cystogram again showed right massive ureterovesical reflux and an irregular bladder neck, suggesting an extrinsic obstruction. Intravenous pyelogram again demonstrated marked hydronephrosis. Because of the possibility of extrinsic obstruction, the suprapubic area was explored on May 27, 1974, and appeared to be within normal limits. The bladder was opened, and the right ureteral orifice was in a normal position but was
606
patulous. On the left, there was a small orifice in normal position. Probing of this orifice showed a large cecoureterocele. The distal portion of this cecoureterocele extended within a few millimeters of the external meatus and completely made up the floor of the posterior urethra. The anterior wall was excised, and the edges were sutured with 5-O chromic catgut for hemostasis. The posterior wall of the ureterocele was left intact. A suprapubic catheter was left indwelling. On the seventh postoperative day the catheter was clamped and only 30 cc. residium was present, and the catheter was removed. She voided
UROLOGY
/ NOVEMBER1975
I VOLUMEVI,
NUMBER5
well without frequency and only mild dysuria. Intravenous pyelogram showed improvement in the hydronephrosis and hydroureter (Fig. 1E). Voiding cystogram three months postoperatively showed slight reflux but a more normal appearing urethra and bladder neck (Fig. 1F).
Most ureteroceles can be diagnosed on the basis of the excretory urogram by the following findings: (1) drooping lily sign of the lower pole collecting system secondary to mass affect of the hydronephrotic upper pole; (2) the rim sign opacification of the thinned parenchyma of the upper pole by contrast media; (3) use of total body opacification with there being greater lucency of the fluid in the hydronephrotic upper pole and the dilated ectopic ureter compared with abdominal structures; (4) lateral deviation of lower pole ureter by the dilated ectopic ureter or possible scalloping of lower pole ureters secondary to kinking of the ectopic ureter; and (5) bladder may show a large lucent intravesical filling defect that may be globular or lobulated.‘,1° The lucent bladder defect often is absent in the cecoureterocele and in ureteroceles with large orifices because of possible filling of the ureterocele when voiding. There is reflux or filling of the cecum portion of the ureterocele posterior to the urethra. The voiding cystourethrogram is occasionally helpful, but often the dense contrast material will obscure the radiolucent defect. However, occasionally the sphincteric or the cecoureterocele will either fill completely at the end of voiding or vanish entirely if the cecoureterocele is emptied during the act of voiding. Again, reflux into the lower pole ureter of the duplicated system might be demonstrated. Cystoscopy can be helpful in this diagnostic dilemma if one is aware of the entity, and the cecoureterocele can be easily overlooked. g The most common treatment of this entity is excision of the ureterocele along with removal of the upper pole segment of the kidney and involved ureter. This may be done as a one- or two-stage procedure. Alternatives to this have been ureterectomy with ureteroureterostomy if the upper pole parenchyma has significant function. Partial upper pole nephrectomy and removal of the remaining involved ureter without removal of the ureterocele is also advocated. 5 The belief is that this can be done without difficulty if the ureterocele collapses and there is no reflux into the ipsilateral ectopic ureter. It must be mentioned that in dealing with cecoureteroceles it can conceivably warrant more surgical correction than simply excision of the ureterocele. If possible, the posterior portion of the ureterocele should be left in place since to remove this portion might cause injury to the bladder neck and posterior urethra. If removal is necessary, then plication of the bladder neck may be needed.
Comment The ureterocele is an uncommon congenital anomaly varying from 1 in 5,000 to 1 in 2,000 pediatric admissions. 6 When diagnosed in infancy, it is usually associated with severe impairment of the upper tracts more commonly involving the ipsilateral side, but actually can cause obstruction or massive reflux of the contralateral collecting system. Approximately three fourths of the cases occur in females. Bilateral ureteroceles occur in about 10 per cent of the cases and are more often associated with obstruction of the urethra. 7 Until recently the only theories to explain the occurrence of ureteroceles were those of Chawalle and Ericson.’ Both of these theories were associated with the persistence of a membrane causing obstruction or a stenotic orifice. This would lead to ballooning of the terminal portion of the ureter. However, until the advent of Stephens’ theory, there was no explanation for the cecoureterocele, sphincteric ectopic ureterocele, or the unobstructed ureterocele. His theory is that the development of ureteroceles is not determined by obstruction but on embryologic grounds. The Miillerian duct migration is responsible for the transposition of the orifices of some ectopic ureters from the trigone to various ectopic locations. It is responsible also for the urethral extension of the cecoureterocele which is “tongue tied” or actually attached to the Mullerian ducts in their descent to the exterior. All other ureteroceles with “giant” orifices are likewise developmental in origin; although in many, an obstructive component is present by virtue of the location of the orifice within the grasp of the internal sphincter of the urethra. l The presentation of a ureterocele is variable, but the most common is urinary infection, as in our case. Other indications are abdominal mass, mass on rectal examination, failure to thrive, urinary incontinence, urinary retention, gross hematuria, or ureterocele prolapse. Bladder neck obstruction has been reported in from I5 to 20 per cent of cases.8’9 Of the literature reviewed, there has not been a case of bladder neck obstruction after removal of the collecting system connected to the ureterocele.
UROLOGY
/ NOVEMBER
1975 / VOLUME
VI, NUMBER
5
607
Voiding cystourethrograms show that the ureterocele will fill with voiding and cause actual obstruction to the flow of urine in the urethra. If this finding can be demonstrated on ureteroceles with upper collecting systems intact, there is no reason to speculate this could not occur when the upper tracts have been surgically removed and the ureterocele left intact. Apparently this is what has occurred in our case. There is, however, no explanation as to why urinary retention occurred only after the removal of the upper collecting system. Possibly, fibrosis from
surgical alteration of the trigone region with different angulation of the wide-mouthed ureteral orifice caused filling of the ureterocele with voiding. Also, there was reflux into the left lower pole collecting system prior to the left nephrectomy which may have decreased filling of the cecoureterocele simply by reflux. This reflux decreased the pressure in the tongue of the ureterocele and alleviated the obstructing potential. However, after the nephrectomy the cecoureterocele could easily fill and obstruct (Fig. 2). 2500 North State Street Jackson, Mississippi 39216 (DR. KEETON) References
FIGURE 2. (A) Arrows show ureter-al reflux into left lOWc?r collecting system prior to left nephroureterectomy. (B) Arrows show filling of cecoureterocele after left nephroureterectomy with obstruction of bladder neck; also right ureterovesical rejlux.
608
1. FRIEDLAND, G. W., and CUNNINGHAM, J.: The elusive ectopic ureteroceles, Am. J. Roentgenol. 116: 792 (1972). 2. STEPHENS, D.: Caecoureterocele and concepts on the embryology and etiology of ureterocele, Aust. N. Z. J. Surg. 40: 239 (1971). 3. WILLIAMS, D. I, and WOODARD,J. R.: Problems in the management of ectopic ureteroceles, J. Ural. 92: 635 (1964). Surgical 4. HENDREN, W. H., and MONFORT, G. J.: correction of ureterocele in children, J. Pediatr. Surg. 6: 235 (1971). 5. JOHNSTON, J. H., and JOHNSON, L. M.: Experiences with ectopic ureteroceles, Br. J. UroI. 41: 61 (1969). 6. MALEK, R. S., KELALIS, P. P., BURKE, E. C., and STRICKLER, G. B.: Simple and ectopic ureterocele in infancy and childhood, Surg. Gynecol. Obstet. 134: 611 (1972). RERDON, W. E., BAKER, D. H., BECKER, J. A., and USON, A. C.: Ectopic ureterocele, Radiol. Chin. North Am. 6: 205 (1968). Everting 8 WEISS, R. M., and SPACKMAN, J. J.: ectopic ureteroceles, J. Urol. 111: 538 (1974). 9. CAMPBELL, M. : Ureterocele, Surg. Gynecol. Obstet. 93: 705 (1951). 10. WILLIAMS, D. I., FAY, R., and LILLIE, J. G.: The functional radiology of ectopic ureteroceles, Br. J. Ural. 44: 417 (1972).
UROLOGY / NOVEMBER 1975 / VOLUME VI, NUMBER 5
P. SHAPPLEY,
E. KEETON,
OF CECOURETEROCELE
M.D.
M.D.
From the Department of Surgery, University Medical Center, Jackson, Mississippi
of Mississippi
ABSTRACT - A three-year-old female had a nephroureterectomy for a hydronephrotic left kidney with a total duplicated collecting system. Postoperatively urinary retention developed which was found to be secondary to a previously nonobstructive cecoureterocele. A brief review of cecoureteroceles is given along with the possible cause of this unusual presentation.
The cecoureterocele is a rare type of ectopic ureterocele which has only recently been recognized as a separate clinical entity in the literais used to describe ture. 1,2The term ureterocele a cystic ballooning of the lower end of the ureter in the bladder lumen3 There are two types of ureterocele: the simple ureterocele usually seen in adults and the ectopic, which is usually seen in children.4 An ectopic ureterocele is most commonly associated with an ectopic ureter from the upper pole segment of a duplicated collecting system. Generally, there is associated renal dysplasia of the upper pole.5 The cecoureterocele is a special type of ureterocele because the lumen extends beyond its orifice as a long tongue or cecum in the urethral submucosa, and the orifice communicates with the bladder lumen and is large and incompetent. ’ Case Report A three-year-old white female was seen by her family physician in September, 1973, with dysuria, frequency, and low-grade fever. She had two additional febrile episodes in February, 1974, and was treated for respiratory infections. Later that month, she was found to have a urinary tract infection and was admitted to the hospital for treatment. At that time a cystogram showed massive left ureteral reflux into the lower collecting system (Fig. 1A). Intravenous pyelogram showed prompt function in a hypertrophied right kidney and function in the left upper collection system (Fig. 1B). Cystoscopy showed a normal right ureteral orifice and a patulous left ureteral
UROLOGY
/
NOVEMBER
1975
/
VOLUME VI, NUMBER 5
orifice. Because of the extremely poor function, a left nephrectomy was performed. At the time of nephrectomy the kidney was found to have a total duplicated collecting system. The distal ureter was removed through a second low abdominal incision, and the ureters were transected flush with the bladder. The patient had an uneventful postoperative course and was discharged on March 6, 1974, on cephalexin therapy. After discharge, she had dysuria, difficulty voiding, and marked constipation. She was treated with numerous antibiotics without relief of symptoms. Intravenous pyelogram in May, 1974, showed massive hydronephrosis and hydroureter on the right (Fig. 1C). After adequate treatment of her urinary tract infection, a cystogram showed massive right ureterovesical reflux and a distorted bladder neck (Fig. lD), both of which were not present on the previous radiologic studies. She was found to have a residual urine of 150 cc. ; cystoscopic examination, however, revealed no obstruction. She was placed on catheter drainage and referred to the University of Mississippi Medical Center in May, 1974, for further evaluation. Cystoscopy showed a markedly inflamed bladder. The right ureteral orifice was not seen; however, what was thought to be the stump of the left ureter was seen. This appeared as a small diverticulum. A bifid vagina was noted at vaginoscopy. A catheter was reinserted and removed one day later. The following day she was found to have 100 cc. residual urine, and the catheter was left indwelling. Bentamicin therapy was
605
FIGURE 1. (A) Preoperative cystogram showing massive left ureterovesical r-e&x. (9) Preoperative intravenous pyelogram showing left upper pole collecting system (arrow) and normal right kidney. (C) Postoperative intravenous pyelogram demonstrating moderate hydronephrosis and hydroureter. (D) Postoperative voiding cystourethrogram showing massive right ureteral reflux and distorted bladder neck. (E) Postoperative intravenous pyelogram after resection of ureterocele showing normal collecting system. (F) Postoperative cystogram after resection of ureterocele showing less distortion of bladder neck.
given, and the urine remained sterile. Blood urea nitrogen was 13 mg. and creatinine 0.1 mg. per 100 ml. Intermittent catheter drainage was tried without success. A repeat voiding cystogram again showed right massive ureterovesical reflux and an irregular bladder neck, suggesting an extrinsic obstruction. Intravenous pyelogram again demonstrated marked hydronephrosis. Because of the possibility of extrinsic obstruction, the suprapubic area was explored on May 27, 1974, and appeared to be within normal limits. The bladder was opened, and the right ureteral orifice was in a normal position but was
606
patulous. On the left, there was a small orifice in normal position. Probing of this orifice showed a large cecoureterocele. The distal portion of this cecoureterocele extended within a few millimeters of the external meatus and completely made up the floor of the posterior urethra. The anterior wall was excised, and the edges were sutured with 5-O chromic catgut for hemostasis. The posterior wall of the ureterocele was left intact. A suprapubic catheter was left indwelling. On the seventh postoperative day the catheter was clamped and only 30 cc. residium was present, and the catheter was removed. She voided
UROLOGY
/ NOVEMBER1975
I VOLUMEVI,
NUMBER5
well without frequency and only mild dysuria. Intravenous pyelogram showed improvement in the hydronephrosis and hydroureter (Fig. 1E). Voiding cystogram three months postoperatively showed slight reflux but a more normal appearing urethra and bladder neck (Fig. 1F).
Most ureteroceles can be diagnosed on the basis of the excretory urogram by the following findings: (1) drooping lily sign of the lower pole collecting system secondary to mass affect of the hydronephrotic upper pole; (2) the rim sign opacification of the thinned parenchyma of the upper pole by contrast media; (3) use of total body opacification with there being greater lucency of the fluid in the hydronephrotic upper pole and the dilated ectopic ureter compared with abdominal structures; (4) lateral deviation of lower pole ureter by the dilated ectopic ureter or possible scalloping of lower pole ureters secondary to kinking of the ectopic ureter; and (5) bladder may show a large lucent intravesical filling defect that may be globular or lobulated.‘,1° The lucent bladder defect often is absent in the cecoureterocele and in ureteroceles with large orifices because of possible filling of the ureterocele when voiding. There is reflux or filling of the cecum portion of the ureterocele posterior to the urethra. The voiding cystourethrogram is occasionally helpful, but often the dense contrast material will obscure the radiolucent defect. However, occasionally the sphincteric or the cecoureterocele will either fill completely at the end of voiding or vanish entirely if the cecoureterocele is emptied during the act of voiding. Again, reflux into the lower pole ureter of the duplicated system might be demonstrated. Cystoscopy can be helpful in this diagnostic dilemma if one is aware of the entity, and the cecoureterocele can be easily overlooked. g The most common treatment of this entity is excision of the ureterocele along with removal of the upper pole segment of the kidney and involved ureter. This may be done as a one- or two-stage procedure. Alternatives to this have been ureterectomy with ureteroureterostomy if the upper pole parenchyma has significant function. Partial upper pole nephrectomy and removal of the remaining involved ureter without removal of the ureterocele is also advocated. 5 The belief is that this can be done without difficulty if the ureterocele collapses and there is no reflux into the ipsilateral ectopic ureter. It must be mentioned that in dealing with cecoureteroceles it can conceivably warrant more surgical correction than simply excision of the ureterocele. If possible, the posterior portion of the ureterocele should be left in place since to remove this portion might cause injury to the bladder neck and posterior urethra. If removal is necessary, then plication of the bladder neck may be needed.
Comment The ureterocele is an uncommon congenital anomaly varying from 1 in 5,000 to 1 in 2,000 pediatric admissions. 6 When diagnosed in infancy, it is usually associated with severe impairment of the upper tracts more commonly involving the ipsilateral side, but actually can cause obstruction or massive reflux of the contralateral collecting system. Approximately three fourths of the cases occur in females. Bilateral ureteroceles occur in about 10 per cent of the cases and are more often associated with obstruction of the urethra. 7 Until recently the only theories to explain the occurrence of ureteroceles were those of Chawalle and Ericson.’ Both of these theories were associated with the persistence of a membrane causing obstruction or a stenotic orifice. This would lead to ballooning of the terminal portion of the ureter. However, until the advent of Stephens’ theory, there was no explanation for the cecoureterocele, sphincteric ectopic ureterocele, or the unobstructed ureterocele. His theory is that the development of ureteroceles is not determined by obstruction but on embryologic grounds. The Miillerian duct migration is responsible for the transposition of the orifices of some ectopic ureters from the trigone to various ectopic locations. It is responsible also for the urethral extension of the cecoureterocele which is “tongue tied” or actually attached to the Mullerian ducts in their descent to the exterior. All other ureteroceles with “giant” orifices are likewise developmental in origin; although in many, an obstructive component is present by virtue of the location of the orifice within the grasp of the internal sphincter of the urethra. l The presentation of a ureterocele is variable, but the most common is urinary infection, as in our case. Other indications are abdominal mass, mass on rectal examination, failure to thrive, urinary incontinence, urinary retention, gross hematuria, or ureterocele prolapse. Bladder neck obstruction has been reported in from I5 to 20 per cent of cases.8’9 Of the literature reviewed, there has not been a case of bladder neck obstruction after removal of the collecting system connected to the ureterocele.
UROLOGY
/ NOVEMBER
1975 / VOLUME
VI, NUMBER
5
607
Voiding cystourethrograms show that the ureterocele will fill with voiding and cause actual obstruction to the flow of urine in the urethra. If this finding can be demonstrated on ureteroceles with upper collecting systems intact, there is no reason to speculate this could not occur when the upper tracts have been surgically removed and the ureterocele left intact. Apparently this is what has occurred in our case. There is, however, no explanation as to why urinary retention occurred only after the removal of the upper collecting system. Possibly, fibrosis from
surgical alteration of the trigone region with different angulation of the wide-mouthed ureteral orifice caused filling of the ureterocele with voiding. Also, there was reflux into the left lower pole collecting system prior to the left nephrectomy which may have decreased filling of the cecoureterocele simply by reflux. This reflux decreased the pressure in the tongue of the ureterocele and alleviated the obstructing potential. However, after the nephrectomy the cecoureterocele could easily fill and obstruct (Fig. 2). 2500 North State Street Jackson, Mississippi 39216 (DR. KEETON) References
FIGURE 2. (A) Arrows show ureter-al reflux into left lOWc?r collecting system prior to left nephroureterectomy. (B) Arrows show filling of cecoureterocele after left nephroureterectomy with obstruction of bladder neck; also right ureterovesical rejlux.
608
1. FRIEDLAND, G. W., and CUNNINGHAM, J.: The elusive ectopic ureteroceles, Am. J. Roentgenol. 116: 792 (1972). 2. STEPHENS, D.: Caecoureterocele and concepts on the embryology and etiology of ureterocele, Aust. N. Z. J. Surg. 40: 239 (1971). 3. WILLIAMS, D. I, and WOODARD,J. R.: Problems in the management of ectopic ureteroceles, J. Ural. 92: 635 (1964). Surgical 4. HENDREN, W. H., and MONFORT, G. J.: correction of ureterocele in children, J. Pediatr. Surg. 6: 235 (1971). 5. JOHNSTON, J. H., and JOHNSON, L. M.: Experiences with ectopic ureteroceles, Br. J. UroI. 41: 61 (1969). 6. MALEK, R. S., KELALIS, P. P., BURKE, E. C., and STRICKLER, G. B.: Simple and ectopic ureterocele in infancy and childhood, Surg. Gynecol. Obstet. 134: 611 (1972). RERDON, W. E., BAKER, D. H., BECKER, J. A., and USON, A. C.: Ectopic ureterocele, Radiol. Chin. North Am. 6: 205 (1968). Everting 8 WEISS, R. M., and SPACKMAN, J. J.: ectopic ureteroceles, J. Urol. 111: 538 (1974). 9. CAMPBELL, M. : Ureterocele, Surg. Gynecol. Obstet. 93: 705 (1951). 10. WILLIAMS, D. I., FAY, R., and LILLIE, J. G.: The functional radiology of ectopic ureteroceles, Br. J. Ural. 44: 417 (1972).
UROLOGY / NOVEMBER 1975 / VOLUME VI, NUMBER 5
