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The Management of Ureteroceles
Vol. 106, July Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
THE MANAGEMENT OF URETEROCELES MICHAEL G. ROYLE*
AND
WILLARD E. GOODWIN
From the Department of Surgery/Urology, University of California School of Medicine and Wadsworth Hospital, Veterans Administration Center, Los Angeles, and the Harbor General Hospital, Torrance, California
neck or into the urethra (fig. 1, B) .3 The distinction is important, for not only are ectopic ureteroceles part of a more widespread urinary tract abnormality but they tend to be larger and more likely to obstruct the ureters and urethra.
Ureterocele is a common congenital anomaly which may become manifest in patients at any age. Severity varies greatly, ranging from the ureterocele found by chance during a urologic investigation to that producing a debilitating, sometimes fatal, disease in the neonatal period or in early childhood. Rarely it may cause severe sepsis and death in late life. 1 The 52 cases of ureterocele seen at the UCLA and affiliated hospitals during the past 14 years are reviewed herein. Emphasis is placed on the complicated cases and the lessons learned in their management. A ureterocele is a cystic dilatation of the lower end of the ureter which may remain intravesical or extend to the bladder neck and urethra. Occasionally a ureterocele will protrude from the female external urethral meatus. The ureterocele has vesical epithelium externally, ureteral epithelium internally and various amounts of connective tissue and smooth muscle between (fig. 1). At the 15 mm. stage of fetal development the end of the ureter is in contact with the urogenital sinus but separated from it by a membrane which later disappears. Chwalla suggested that this membrane may persist in certain instances as a ureterocele. 2 Ericsson was the first to make a distinction between ureteroceles confined to the bladder and ectopic ureteroceles that extend to the bladder
ECTOPIC URETEROCELE
Nineteen cases of ectopic ureterocele were reviewed, 15 in female and 4 in male patients. The ureterocele was on the right side in 10 cases, on the left in 7 and bilateral in 2. The ureteroceles in this series were usually large (fig. 2). Their orifices varied in size from a pinpoint (or invisible) to quite wide and capable of causing reflux. Despite this variation, dilatation of the upper tract in continuity with the ureterocele was a consistent finding. The renal elements were hydronephrotic, pyelonephritic, and, frequently, dysplastic. Primitive tubules, preglomerular cysts, smooth muscle and cartilage were evident on microscopy. This dysplasia was extreme in 2 cases in which the renal elements were almost non-functioning. As in previous series, 4 duplicated upper tracts were often present on the side of the ureterocele (15 of 19 cases). During its formation an ectopic ureterocele tends to weaken the surrounding detrusor muscle of the trigone. This defect becomes evident later as a herniated bulge or saccule and may account for the large atonic bladders that can follow removal of a ureterocele without subsequent plication of the underlying muscle (fig. 3). Depending on the size of the ureterocele, the ipsilateral and later the contralateral ureteral orifices may become obstructed. The ipsilateral and sometimes the contralateral ureter may allow reflux. Ureteroceles causing acute retention are surprisingly uncommon (only 1 occurred in our series, in a female neonate). Usually there are minor degrees of urethral obstruction due to 'Williams, D. I. and Woodard, J. R.: Problems in the management of ectopic ureteroceles. J. Ural., 92: 635, 1964.
Accepted for publication October 1, 1970. Read at annual meeting of Western Section ~merican Urological Association, Seattle, Wash~ mgton, July 27-August 1, 1969. * Requests for reprints: Royal Sussex County Hospital, Brighton, Sussex, England. 1 Goodwin, W. E., Fonkalsrud, E.W., Goldman R., Kaufman, J. !-, Martin, D. C., Riley, J. M.'. R?e, C. W., Shapiro, A. E. and Wilkerson, J. A.: Diagnostic problems m retroperitoneal disease. Ann. Intern. Med., 65: 160, 1966 . . ' Chwalla, R.: Progress of formation of cystic d1latat10ns of ves1cal end of ureter and of diverticula at ureteral ostium. Ural. & Cutan. Rev., 31: 499, 1927. 3 Ericsson, N. 0.: Ectopic ureterocele in infants and children. Acta Chir. Scand., suppl. 197, 1954. 42
43
MANAGEMENT OF URETEROCELES
B A
INTRAVESICAL
ECTOPIC
URETEROCELE
UREHROCELE
POSTER1on DETRUSOR WEAKNESS
FIG. l
FIG. 2. A, IVP with filling defect in bladder caused by ectopic ureterocele. B, retrograde pyelogram of left ectopic ureterocele. C, specimen.
plugging by the ureterocele, and this accounts for the bladder trabeculation. Clinical and diagnostic features. An awareness of the condition and a full systematic urologic investigation are essential. All our patients were seen before the age of 5 years and 14 were seen in the first year of life. The presenting features were non-specific (table 1). Excretory urography (IVP) is the single most useful investigative technique. Typically the
upper pole of the affected side has poor or absent function and the ureterocele stands out as a negative shadow on the bladder films (fig. 4). Delayed films of up to 24 hours frequently are helpful and in the neonate may be essentiaL When contrast medium has been injected into the femoral vein the inferior vena cava may be seen to be indented or displaced by any dilated renal elements. The cystogram is important for demonstrating
I
'I
44
ROYLE AND GOODWIN
Ureterocele in a Female Infant from upper segment of upper right tract duplication
duplicate ureter bound in fibers of { ·
view of opened bladder FIG. 3. Autopsy specimen of premature female neonate, death due to renal failure, shows ectopic ureterocele with herniation posteriorly.
the presence or absence of reflux. Opaque contrast medium is usually used but occasionally an air cystogram is equally helpful. A ureterocele may show only on an oblique cystogram film. In the male patient, when the ureterocele is completely obscured by opaque contrast medium, dilatation of the posterior urethra may suggest the anomaly. 5 Retrograde pyelography demonstrates the extent of the upper tract disease. If the ureterocele cannot be catheterized, contrast medium can be injected into it after puncture with a needle. When the IVP is poor and the kidney is dilated, an anterograde pyelogram will delineate the upper tract. 6 Aortography is generally not indicated, although in the first 2 weeks of life it can be done with simplicity by catheterizing the umbilical artery. The pyelographic results are excellent. Cystourethoscopy confirms the diagnosis. However, the extent of the ureterocele is more accurately visualized at open operation. Williams, D. I. and Royle, M.: Ectopic ureter in the male child. Brit. J. Urol., 41: 421, 1969. 6 Casey, W. C. and Goodwin, W. E.: Percutaneous antegrade pyelography and hydronephrosis. J. Urol., 74: 164, 1955. 5
Treatment. The basic definitive treatments are diverse (table 2). A number of cases of ureterocele treated elsewhere and referred to UCLA owing to complications required a further operation. The upper tract of an ectopic ureterocele is usually so dysplastic and pyelonephritic that it is worth saving only if the over-all renal function is depressed. The ipsilateral lower pole also may be irreparably damaged and require simultaneous removal. The operation is best begun by opening the bladder to determine the extent of the abnormality. The ureterocele is excised and the defect and weakened detrusor are repaired and plicated. Reimplantation of the normal ureter may sometimes be necessary. The kidney is exposed through a flank incision and the destroyed renal element and dilated ureter are removed. In the neonate or seriously ill child the operation should be done in 2 stages, with ureterocele excision and cutaneous diversion of the affected ureter as a first stage. Anesthesia in the neonate is hazardous. Two of our 3-monthold patients died of postoperative atelectasis following a 1-stage bladder and kidney operation. When the upper tract of a patient with ureterocele is to be preserved, excision of the ureterocele and a ureteroureterostomy between lower and upper pole ureters are the best therapy. Case summary. J. S., UCLA014-47-93, was a 14month-old girl with recurrent urinary tract infection. Diagnosis was left ectopic ureterocele with reflux into the ipsilateral lower pole ureter (fig. 5). At the first operation the ureterocele was excised and a cutaneous ureterostomy was fashioned. Urinary drainage from the ureterostomy was good, and 1 month later a ureteroureterostomy was performed. Eight years later the child was clinically well and had sterile urine, an excellent pyelogram and no reflux. This case shows that mild reflux into the ipsilateral ureter may disappear following removal of the ureterocele. For ureteroureterostomy TABLE
1. Presenting features in 19 cases of ectopic ureterocele
Urinary tract infection Abdominal mass Retention of urine Failure to thrive Incontinence of urine Incidental finding with heart anomaly
11
4
45
MANAGEMENT OF URETEROCELES
Fm. 4. A, large left ureterocele, anteroposterior view. B, oblique film to show posterior view TABLE
2. Basic definitive treatments in 19 cases of ectopic ureterocele
Excision of ureterocele with nephrectomy or hemi-
ECTOPIC
UllETEROCELE
11
nephrectomy Excision of ureterocele with ligation of upper pole ureter Excision of ureterocele with ipsilateral ureteroureterostomy
Uncapping of ureterocele Transurethral resection of ureterocele Reimplant of ureterocele, ureterocele untouched Bilateral cutaneous ureterostomy Died of cardiac anomaly before treatment
1 2
to function satisfactorily the recipient ureter should be as normal as possible; there should be no reflux. Ligation of the upper pole ureter in the pelvis as definitive treatment was successful in 1 patient. However, it is not recommended because of the possibility of subsequent pyoriephrosis. Resection of the ureterocele. Resection alone, either transurethrally or suprapubically, is not an adequate or desirable treatment for ectopic ureteroceles. This treatment invariably leaves an infected refluxing system. Inadequate transurethral resection which leaves remnants of ureterocele further encourages infection. The remnants also may interfere with the bladder neck to cause obstruction or incontinence. Leaving the ureterocele. Two cases in this group were referred to UCLA after a preliminary
IEFORE
TREATMENT
EXCISED
URETEROCELE CUTANEOUS URETEROSTOMY
URETEIIOUltETEROSTOMY
Fm. 5 operation had been done elsewhere. These cases illustrate that not only may the ureterocele obstruct the bladder outlet and progressively
46
ROYLE AND GOODWIN
increase the upper tract dilatation but that it may form an abscess. Bladder neck problems. Care must be taken when operating on a bladder neck already damaged by a ureterocele. It may be wise to accept some incontinence in young children rather than to risk the development of bladder neck stenosis. Marshall-Marchetti incontinence procedures were performed on 2 occasions and each time severe bladder neck stenosis developed. Eventually both children required Bricker cutaneous urinary diversion. Reflux problems. Although reflux into the ipsilateral and occasionally into the contralateral ureter is well recognized, it caused difficulties only in patients who had bladder neck stenosis. In other cases reflux disappeared after excision of the ureterocele. Temporary diversion. If the upper tracts are severely dilated and infected, immediate urinary diversion is required. When the child's condition improves further investigation and definitive treatment can be carried out. Bilateral nephrostomy done in one of our cases was successful. Loop cutaneous ureterostomy was less effective in a premature girl with bilateral abdominal masses and a creatinine of 6.6 mg. per 100 ml. Unfortunately, as a result of kinking, the dilated tortuous ureter drained poorly and the infant died of infection and renal failure. Pathologically the kidneys were pyelonephritic, dysplastic and hydronephrotic. INTRA VESICAL URETEROCELES
Intravesical ureteroceles are a less severe abnormality than the ectopic type and, consequently, their management is generally easier. Intravesical ureteroceles vary in size but usually are quite small and sometimes only just visible. The degree of damage sustained by the associated upper tract depends on infection and the amount of obstruction caused by the ureterocele. Dysplasia of the renal elements is not characteristic TABLE
3. Treatment in 13 cases of intravesical ureterocele in children
No treatment required Dilatation of ureterocele stoma Transurethral resection of ureterocele Suprapubic excision of ureterocele and ureteral reimplant Excision of ureterocele, ipsilateral ureteroureterostomy Ligation of ureterocele ureter extravesically and upper pole partial nephrectomy
2 1 4 4
of this group. A large ureterocele will have a plugging, ball-valve type of action at the bladder neck during voiding. This group will be divided into adult and pediatric cases. I ntravesical ureteroceles in children. These are less common than ectopic ureteroceles. Thirteen cases are reviewed here: 12 girls and 1 boy. All patients were less than 5 years old and 2 were in their first year of life. The ureteroceles were on the right side in 3 cases, on the left side in 5 and bilateral in 5. Upper tract duplication was not a prominent part of the abnormality and occurred only 5 times. Presenting complaints were non-specific but all patients had urinary tract infection, and in 3 there was associated back or abdominal pain. Damage to the upper tract was uncommon and varied with the degree of stenosis at the ureterocele orifice. Vesicoureteral reflux may occur. Management. Diagnosis is made by techniques similar to those used with ectopic ureteroceles. Because the upper tract above an intravesical ureterocele usually has good function, the ureterocele may appear in the bladder as a radio-dense shadow. Treatment is summarized in table 3. Small ureteroceles are often incidental findings and require, at the most, ureteral dilatation and antibacterial drugs. Transurethral resection will relieve the obstruction caused by large ureteroceles but frequently results in vesicoureteral reflux. This occurred in 2 of our 4 cases. Inferior meatotomy as described by Hutch lessens the chance of reflux but requires an open operation. 7 Suprapubic excision of ureteroceles always results in reflux, and a reflux-preventing operation should accompany the initial operation. Two of our 3 patients had complications. One had severe ureterovesical stenosis and pyelonephritis eventually requiring nephrectomy. The other, a girl with a solitary kidney, had persistent gross reflux. She has remained in excellent health, however, and 11 years later is free of infection and the pyelogram is normal. The rapid flow rate down the solitary ureter may account for the absence of infection. When there are duplex upper tracts an alternative to reimplantation is ureteroureterostomy or pyelopyelostomy between the ipsilateral systems. Even in cases ,vith single upper tracts a transureteroureterostomy is an acceptable procedure. Care must be taken when excising the ureterocele 7
Hutch, J. A. and Chisholm, E. R.: Surgical
repair of ureterocele. J. Urol., 96: 445, 1966.
MANAGEMENT OF URETEROCELES
and distal 1 cm. of ureter in a system for at this level both ureters have the same adventitial sheath and the normal ureter is vVilliams advocated uncapping the ureterocele and leaving the attached distal 1 cm. advocated ec1)rr,pr·es:31on of of ureter. 8 u stenotic ureterocele from outside the bladder and then ligation of the terminal ureter, the cv1m1.meu ureterocele. 9 I ntravesical ureteroceles adults. All ureteroceles in adults were intravesical and the "vvere small. No ureterocele interfered with the bladder neck. were reviewed, ranging in age from 24 to 64 years. Fourteen ,J~u,vc,uo were women and 6 were men. Nine had bilateral ureteroceles. Six had upper tracts on the side of the lesion. Stones and infection were the cause of symptoms in most cases. All cases were the same general lines as the previous groups. Stones were removed when present and were mvP•,n-o·q,·"" for stone-forming diseases. D. I.: Paediatric Urology. London: & Co., Ltd., 1968. 9 Stephens, F. D.: Rectum, Anus and more: The Williams & Wilkins Co.,
when there was severe calculous disea.se with infection all the patients made excellent recoveries. SUMMARY
cases of ureterocele were reviewed: and 33 intravesical ureteroceles. ureteroceles are and cause more urinary tract obstruction. Associated upper tracts are also abnormal. Diagnosis depends on investigation. Treatment is directed to infection and restoring normal anatomy. ureterosurgical removal of ureteroceles are treated dilated ureter and """n·m,·<>n renal elements. Less radical operations for intermediate groups are described. Small, uncomplicated, intravesical treated ureteroceles may be dilation of the orifice. Ectopic ureteroceles have a good when treated early if the upper tracts have not been severely damaged. Intravesical ureteroceles have an excellent prognosis. when there is gross associated calculous disease is there nificant morbidity or mortality.
19
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
THE MANAGEMENT OF URETEROCELES MICHAEL G. ROYLE*
AND
WILLARD E. GOODWIN
From the Department of Surgery/Urology, University of California School of Medicine and Wadsworth Hospital, Veterans Administration Center, Los Angeles, and the Harbor General Hospital, Torrance, California
neck or into the urethra (fig. 1, B) .3 The distinction is important, for not only are ectopic ureteroceles part of a more widespread urinary tract abnormality but they tend to be larger and more likely to obstruct the ureters and urethra.
Ureterocele is a common congenital anomaly which may become manifest in patients at any age. Severity varies greatly, ranging from the ureterocele found by chance during a urologic investigation to that producing a debilitating, sometimes fatal, disease in the neonatal period or in early childhood. Rarely it may cause severe sepsis and death in late life. 1 The 52 cases of ureterocele seen at the UCLA and affiliated hospitals during the past 14 years are reviewed herein. Emphasis is placed on the complicated cases and the lessons learned in their management. A ureterocele is a cystic dilatation of the lower end of the ureter which may remain intravesical or extend to the bladder neck and urethra. Occasionally a ureterocele will protrude from the female external urethral meatus. The ureterocele has vesical epithelium externally, ureteral epithelium internally and various amounts of connective tissue and smooth muscle between (fig. 1). At the 15 mm. stage of fetal development the end of the ureter is in contact with the urogenital sinus but separated from it by a membrane which later disappears. Chwalla suggested that this membrane may persist in certain instances as a ureterocele. 2 Ericsson was the first to make a distinction between ureteroceles confined to the bladder and ectopic ureteroceles that extend to the bladder
ECTOPIC URETEROCELE
Nineteen cases of ectopic ureterocele were reviewed, 15 in female and 4 in male patients. The ureterocele was on the right side in 10 cases, on the left in 7 and bilateral in 2. The ureteroceles in this series were usually large (fig. 2). Their orifices varied in size from a pinpoint (or invisible) to quite wide and capable of causing reflux. Despite this variation, dilatation of the upper tract in continuity with the ureterocele was a consistent finding. The renal elements were hydronephrotic, pyelonephritic, and, frequently, dysplastic. Primitive tubules, preglomerular cysts, smooth muscle and cartilage were evident on microscopy. This dysplasia was extreme in 2 cases in which the renal elements were almost non-functioning. As in previous series, 4 duplicated upper tracts were often present on the side of the ureterocele (15 of 19 cases). During its formation an ectopic ureterocele tends to weaken the surrounding detrusor muscle of the trigone. This defect becomes evident later as a herniated bulge or saccule and may account for the large atonic bladders that can follow removal of a ureterocele without subsequent plication of the underlying muscle (fig. 3). Depending on the size of the ureterocele, the ipsilateral and later the contralateral ureteral orifices may become obstructed. The ipsilateral and sometimes the contralateral ureter may allow reflux. Ureteroceles causing acute retention are surprisingly uncommon (only 1 occurred in our series, in a female neonate). Usually there are minor degrees of urethral obstruction due to 'Williams, D. I. and Woodard, J. R.: Problems in the management of ectopic ureteroceles. J. Ural., 92: 635, 1964.
Accepted for publication October 1, 1970. Read at annual meeting of Western Section ~merican Urological Association, Seattle, Wash~ mgton, July 27-August 1, 1969. * Requests for reprints: Royal Sussex County Hospital, Brighton, Sussex, England. 1 Goodwin, W. E., Fonkalsrud, E.W., Goldman R., Kaufman, J. !-, Martin, D. C., Riley, J. M.'. R?e, C. W., Shapiro, A. E. and Wilkerson, J. A.: Diagnostic problems m retroperitoneal disease. Ann. Intern. Med., 65: 160, 1966 . . ' Chwalla, R.: Progress of formation of cystic d1latat10ns of ves1cal end of ureter and of diverticula at ureteral ostium. Ural. & Cutan. Rev., 31: 499, 1927. 3 Ericsson, N. 0.: Ectopic ureterocele in infants and children. Acta Chir. Scand., suppl. 197, 1954. 42
43
MANAGEMENT OF URETEROCELES
B A
INTRAVESICAL
ECTOPIC
URETEROCELE
UREHROCELE
POSTER1on DETRUSOR WEAKNESS
FIG. l
FIG. 2. A, IVP with filling defect in bladder caused by ectopic ureterocele. B, retrograde pyelogram of left ectopic ureterocele. C, specimen.
plugging by the ureterocele, and this accounts for the bladder trabeculation. Clinical and diagnostic features. An awareness of the condition and a full systematic urologic investigation are essential. All our patients were seen before the age of 5 years and 14 were seen in the first year of life. The presenting features were non-specific (table 1). Excretory urography (IVP) is the single most useful investigative technique. Typically the
upper pole of the affected side has poor or absent function and the ureterocele stands out as a negative shadow on the bladder films (fig. 4). Delayed films of up to 24 hours frequently are helpful and in the neonate may be essentiaL When contrast medium has been injected into the femoral vein the inferior vena cava may be seen to be indented or displaced by any dilated renal elements. The cystogram is important for demonstrating
I
'I
44
ROYLE AND GOODWIN
Ureterocele in a Female Infant from upper segment of upper right tract duplication
duplicate ureter bound in fibers of { ·
view of opened bladder FIG. 3. Autopsy specimen of premature female neonate, death due to renal failure, shows ectopic ureterocele with herniation posteriorly.
the presence or absence of reflux. Opaque contrast medium is usually used but occasionally an air cystogram is equally helpful. A ureterocele may show only on an oblique cystogram film. In the male patient, when the ureterocele is completely obscured by opaque contrast medium, dilatation of the posterior urethra may suggest the anomaly. 5 Retrograde pyelography demonstrates the extent of the upper tract disease. If the ureterocele cannot be catheterized, contrast medium can be injected into it after puncture with a needle. When the IVP is poor and the kidney is dilated, an anterograde pyelogram will delineate the upper tract. 6 Aortography is generally not indicated, although in the first 2 weeks of life it can be done with simplicity by catheterizing the umbilical artery. The pyelographic results are excellent. Cystourethoscopy confirms the diagnosis. However, the extent of the ureterocele is more accurately visualized at open operation. Williams, D. I. and Royle, M.: Ectopic ureter in the male child. Brit. J. Urol., 41: 421, 1969. 6 Casey, W. C. and Goodwin, W. E.: Percutaneous antegrade pyelography and hydronephrosis. J. Urol., 74: 164, 1955. 5
Treatment. The basic definitive treatments are diverse (table 2). A number of cases of ureterocele treated elsewhere and referred to UCLA owing to complications required a further operation. The upper tract of an ectopic ureterocele is usually so dysplastic and pyelonephritic that it is worth saving only if the over-all renal function is depressed. The ipsilateral lower pole also may be irreparably damaged and require simultaneous removal. The operation is best begun by opening the bladder to determine the extent of the abnormality. The ureterocele is excised and the defect and weakened detrusor are repaired and plicated. Reimplantation of the normal ureter may sometimes be necessary. The kidney is exposed through a flank incision and the destroyed renal element and dilated ureter are removed. In the neonate or seriously ill child the operation should be done in 2 stages, with ureterocele excision and cutaneous diversion of the affected ureter as a first stage. Anesthesia in the neonate is hazardous. Two of our 3-monthold patients died of postoperative atelectasis following a 1-stage bladder and kidney operation. When the upper tract of a patient with ureterocele is to be preserved, excision of the ureterocele and a ureteroureterostomy between lower and upper pole ureters are the best therapy. Case summary. J. S., UCLA014-47-93, was a 14month-old girl with recurrent urinary tract infection. Diagnosis was left ectopic ureterocele with reflux into the ipsilateral lower pole ureter (fig. 5). At the first operation the ureterocele was excised and a cutaneous ureterostomy was fashioned. Urinary drainage from the ureterostomy was good, and 1 month later a ureteroureterostomy was performed. Eight years later the child was clinically well and had sterile urine, an excellent pyelogram and no reflux. This case shows that mild reflux into the ipsilateral ureter may disappear following removal of the ureterocele. For ureteroureterostomy TABLE
1. Presenting features in 19 cases of ectopic ureterocele
Urinary tract infection Abdominal mass Retention of urine Failure to thrive Incontinence of urine Incidental finding with heart anomaly
11
4
45
MANAGEMENT OF URETEROCELES
Fm. 4. A, large left ureterocele, anteroposterior view. B, oblique film to show posterior view TABLE
2. Basic definitive treatments in 19 cases of ectopic ureterocele
Excision of ureterocele with nephrectomy or hemi-
ECTOPIC
UllETEROCELE
11
nephrectomy Excision of ureterocele with ligation of upper pole ureter Excision of ureterocele with ipsilateral ureteroureterostomy
Uncapping of ureterocele Transurethral resection of ureterocele Reimplant of ureterocele, ureterocele untouched Bilateral cutaneous ureterostomy Died of cardiac anomaly before treatment
1 2
to function satisfactorily the recipient ureter should be as normal as possible; there should be no reflux. Ligation of the upper pole ureter in the pelvis as definitive treatment was successful in 1 patient. However, it is not recommended because of the possibility of subsequent pyoriephrosis. Resection of the ureterocele. Resection alone, either transurethrally or suprapubically, is not an adequate or desirable treatment for ectopic ureteroceles. This treatment invariably leaves an infected refluxing system. Inadequate transurethral resection which leaves remnants of ureterocele further encourages infection. The remnants also may interfere with the bladder neck to cause obstruction or incontinence. Leaving the ureterocele. Two cases in this group were referred to UCLA after a preliminary
IEFORE
TREATMENT
EXCISED
URETEROCELE CUTANEOUS URETEROSTOMY
URETEIIOUltETEROSTOMY
Fm. 5 operation had been done elsewhere. These cases illustrate that not only may the ureterocele obstruct the bladder outlet and progressively
46
ROYLE AND GOODWIN
increase the upper tract dilatation but that it may form an abscess. Bladder neck problems. Care must be taken when operating on a bladder neck already damaged by a ureterocele. It may be wise to accept some incontinence in young children rather than to risk the development of bladder neck stenosis. Marshall-Marchetti incontinence procedures were performed on 2 occasions and each time severe bladder neck stenosis developed. Eventually both children required Bricker cutaneous urinary diversion. Reflux problems. Although reflux into the ipsilateral and occasionally into the contralateral ureter is well recognized, it caused difficulties only in patients who had bladder neck stenosis. In other cases reflux disappeared after excision of the ureterocele. Temporary diversion. If the upper tracts are severely dilated and infected, immediate urinary diversion is required. When the child's condition improves further investigation and definitive treatment can be carried out. Bilateral nephrostomy done in one of our cases was successful. Loop cutaneous ureterostomy was less effective in a premature girl with bilateral abdominal masses and a creatinine of 6.6 mg. per 100 ml. Unfortunately, as a result of kinking, the dilated tortuous ureter drained poorly and the infant died of infection and renal failure. Pathologically the kidneys were pyelonephritic, dysplastic and hydronephrotic. INTRA VESICAL URETEROCELES
Intravesical ureteroceles are a less severe abnormality than the ectopic type and, consequently, their management is generally easier. Intravesical ureteroceles vary in size but usually are quite small and sometimes only just visible. The degree of damage sustained by the associated upper tract depends on infection and the amount of obstruction caused by the ureterocele. Dysplasia of the renal elements is not characteristic TABLE
3. Treatment in 13 cases of intravesical ureterocele in children
No treatment required Dilatation of ureterocele stoma Transurethral resection of ureterocele Suprapubic excision of ureterocele and ureteral reimplant Excision of ureterocele, ipsilateral ureteroureterostomy Ligation of ureterocele ureter extravesically and upper pole partial nephrectomy
2 1 4 4
of this group. A large ureterocele will have a plugging, ball-valve type of action at the bladder neck during voiding. This group will be divided into adult and pediatric cases. I ntravesical ureteroceles in children. These are less common than ectopic ureteroceles. Thirteen cases are reviewed here: 12 girls and 1 boy. All patients were less than 5 years old and 2 were in their first year of life. The ureteroceles were on the right side in 3 cases, on the left side in 5 and bilateral in 5. Upper tract duplication was not a prominent part of the abnormality and occurred only 5 times. Presenting complaints were non-specific but all patients had urinary tract infection, and in 3 there was associated back or abdominal pain. Damage to the upper tract was uncommon and varied with the degree of stenosis at the ureterocele orifice. Vesicoureteral reflux may occur. Management. Diagnosis is made by techniques similar to those used with ectopic ureteroceles. Because the upper tract above an intravesical ureterocele usually has good function, the ureterocele may appear in the bladder as a radio-dense shadow. Treatment is summarized in table 3. Small ureteroceles are often incidental findings and require, at the most, ureteral dilatation and antibacterial drugs. Transurethral resection will relieve the obstruction caused by large ureteroceles but frequently results in vesicoureteral reflux. This occurred in 2 of our 4 cases. Inferior meatotomy as described by Hutch lessens the chance of reflux but requires an open operation. 7 Suprapubic excision of ureteroceles always results in reflux, and a reflux-preventing operation should accompany the initial operation. Two of our 3 patients had complications. One had severe ureterovesical stenosis and pyelonephritis eventually requiring nephrectomy. The other, a girl with a solitary kidney, had persistent gross reflux. She has remained in excellent health, however, and 11 years later is free of infection and the pyelogram is normal. The rapid flow rate down the solitary ureter may account for the absence of infection. When there are duplex upper tracts an alternative to reimplantation is ureteroureterostomy or pyelopyelostomy between the ipsilateral systems. Even in cases ,vith single upper tracts a transureteroureterostomy is an acceptable procedure. Care must be taken when excising the ureterocele 7
Hutch, J. A. and Chisholm, E. R.: Surgical
repair of ureterocele. J. Urol., 96: 445, 1966.
MANAGEMENT OF URETEROCELES
and distal 1 cm. of ureter in a system for at this level both ureters have the same adventitial sheath and the normal ureter is vVilliams advocated uncapping the ureterocele and leaving the attached distal 1 cm. advocated ec1)rr,pr·es:31on of of ureter. 8 u stenotic ureterocele from outside the bladder and then ligation of the terminal ureter, the cv1m1.meu ureterocele. 9 I ntravesical ureteroceles adults. All ureteroceles in adults were intravesical and the "vvere small. No ureterocele interfered with the bladder neck. were reviewed, ranging in age from 24 to 64 years. Fourteen ,J~u,vc,uo were women and 6 were men. Nine had bilateral ureteroceles. Six had upper tracts on the side of the lesion. Stones and infection were the cause of symptoms in most cases. All cases were the same general lines as the previous groups. Stones were removed when present and were mvP•,n-o·q,·"" for stone-forming diseases. D. I.: Paediatric Urology. London: & Co., Ltd., 1968. 9 Stephens, F. D.: Rectum, Anus and more: The Williams & Wilkins Co.,
when there was severe calculous disea.se with infection all the patients made excellent recoveries. SUMMARY
cases of ureterocele were reviewed: and 33 intravesical ureteroceles. ureteroceles are and cause more urinary tract obstruction. Associated upper tracts are also abnormal. Diagnosis depends on investigation. Treatment is directed to infection and restoring normal anatomy. ureterosurgical removal of ureteroceles are treated dilated ureter and """n·m,·<>n renal elements. Less radical operations for intermediate groups are described. Small, uncomplicated, intravesical treated ureteroceles may be dilation of the orifice. Ectopic ureteroceles have a good when treated early if the upper tracts have not been severely damaged. Intravesical ureteroceles have an excellent prognosis. when there is gross associated calculous disease is there nificant morbidity or mortality.
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