British Journal ofcirology (1992), 70, 196-200
01992 British Journal of Urology
PAEDIATRIC UROLOGY
Current Management of Duplex-System Ureteroceles: Experience with 4 1 Patients A. M. K. RICKWOOD, I. R E I N E R , M. JONES and C. POURNARAS Regional Department of Paediatric Urology, Royal Liverpool Children's Hospital (Alder Hey), Liverpool
Summary-Experience is described of 4 1 infants and children with duplex-system ureteroceles, 25 presenting clinically and 16 by prenatal ultrasonography. Bladder outflow obstruction was rare but lower polar vesicoureteric reflux (VUR), usually of lesser grades, was common. Upper polar function, as assessed by ""Tc-DMSA, was negligible in children with truly ectopic ureteroceles but well preserved in those where the lesion lay wholly intravesically. Lower polar function was good, even in the presence of secondary obstruction, except in 2 infants with major VUR. Twenty-three patients were treated by upper polar nephrectomy plus aspiration of the ureterocele; 2 subsequently required ureterocele excision. Histology of excised specimens indicated that a more conservative approach would not have been rewarded. Where upper polar function was good, conservation was maintained in 3 cases by pyelopyelostomy and in 5 more by excision of the ureterocele plus bipolar ureteric reimplantation. Other operative strategies were employed in 2 cases. Finally, a defined group of 8 children was managed expectantly without untoward results. It was concluded that the variable anatomy and function associated with duplex-system ureteraceles require a flexibile approach to treatment, including, possibly, no treatment at all.
The management of duplex-system ureteroceles continues to attract a measure of controversy that doubtless reflects the variations of anatomy and renal function with which they are associated. Although these lesions are usually synonymously termed ectopic ureteroceles, implying encroachment to or beyond the bladder neck, a proportion are situated wholly intravesically. Truly ectopic lesions may cause bladder outflow obstruction, especially if the ureterocele is tense. Function of the affected upper renal poles varies from little at all to perfect preservation. Vesicoureteric reflux to the lower renal pole is common, though seldom severe, and may be associated with congenital dysplasia or with acquired renal scarring. Less often, a tense ureterocele may cause secondary obstruction of the lower polar ureter, with or Accepted for publication 30 August 1991
without impairment of polar function. Lastly, bladder outflow obstruction may cause global dilatation of the upper renal tracts, leading in severe cases to permanently compromised overall renal function. Symptoms are similarly diverse and range from a life-threatening combination of septicaemia and azotaemia to none whatever. The latter has become common with the advent of prenatal ultrasonography . It would seem to be generally accepted that these lesions always merit surgical intervention, regardless of severity or mode of presentation, and until recently such treatment has concentrated upon the ureterocele itself, either in the form of endoscopic incision (Tank, 1980; Gerridzen and Schillinger, 1984) or operative excision combined with lower polar ureteric reimplantation and, usually, simultaneous (Kroovand and Perlmutter, 1979) or subsequent (Johnston and Johnson, 1969) upper polar nephrectomy. Because excision of truly ectopic
196
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MANAGEMENT OF DUPLEX-SYSTEM URETEROCELES
ureteroceles is not without difficulty or hazard (Williams and Woodward, 1965), especially where the bladder is thick-walled due to outflow obstruction, it has more recently been advocated that initial treatment be addressed to the upper renal pole, with excision where function is poor or pyelopyelostomy where useful function remains (King et al., 1983; Caldamone et al., 1984). In either case, the ureterocele itself is collapsed by aspiration via the distal ureteric remnant, so relieving any bladder outflow obstruction or secondary obstruction of the lower polar ureter. Also, it is claimed that this policy makes for easier excision of the ureterocele should this later prove necessary. It is our contention that the variable nature and presentation of duplex-system ureteroceles call for a flexible approach and that while most recommended strategies have merit in individual cases, none is universally applicable. In addition, our experience leads us to question whether some cases require surgical intervention at all.
polar function. Where initial treatment was directed to the upper renal pole, cystography was performed after, rather than before, such intervention. When other approaches were adopted, cystography formed part of the initial investigative regimen.
Results
The study group comprised 41 patients (29 females, 12 males) whose ages at presentation ranged from birth to 6 years (mean 1.1 year); 23 presented with urinary infection, 16 by prenatal ultrasonography and 1 each with urinary retention and with urinary ascites. Three had bilateral lesions and a further 3 had non-concordant contralateral duplex-system anomalies (1 each urethral ureteric ectopia, introital ureteric ectopia, lower polar VUR). Among the 44 lesions, 29 encroached to or beyond the bladder neck, including 1 prolapsing; 9 lay wholly intravesically and 6, where cystoscopy was not performed, were unknown. Thirty-five patients were assessed by 99mTc-DMSAscintigraPatients and Methods phy : upper polar function was negligible or absent All of the patients reviewed here presented with in 29 instances (Fig. 1) and normally preserved in 6 duplex-system ureteroceles from 1984 to 1990 (Fig. 2), all with intravesical lesions. Of the 23 inclusive. Ultrasonography was the usual initial patients who underwent cystography prior to any investigation, followed in most cases by IVU and surgical intervention, 15 had ipsilateral lower polar VUR (4 grade I, 7 Grade I I , 4 Grade 111) (Internacystoscopy. As a rule, 9 9 m Tdimercaptosuccinic ~ acid (DMSA) scintigraphy was undertaken to assess tional Reflux Study Committee, 1981); in 2 of these
Fig. 1 Female aged 3 months: right duplex-system ectopic ureterocele. (A) The right upper renal pole is non-functioning on IVU. (B) 99mTc-DMSAscintigraphy shows the right lower pole to be functionally and anatomically normal.
198
BRITISH JOURNAL OF UROLOGY
Fig. 2 Female aged 3 months: right intravesical duplex-system ureterocele. (A) IVU shows gross hydronephrosis of both renal poles. (B) g9"Tc-DMSA scintigraphy shows that the uptake of isotope is generally reduced (38% of total) but is equally distributed to both renal poles. Right renal function returned to normal after excision of the ureterocele with bipolar ureteric reimplantation.
there was also contralateral VUR. One patient had pelviureteric junction obstruction in the lower polar system and 3 had non-refluxing dilatation of the lower polar ureter due to secondary obstruction by the ureterocele (2 ectopic, 1 orthotopic). Function of the lower renal pole was normal except in 2 cases where negligible function was associated with Grade I11 VUR. Only 4 patients had clinical or radiological evidence of bladder outflow obstruction, of whom only 1 had global dilatation of the upper renal tracts. Lastly, one girl had calculi lying within the ureterocele. Management Generalpolicy. As a rule, where upper polar function was severely compromised, initial treatment comprised upper polar nephrectomy with aspiration of the ureterocele via the distal ureteric remnant, which was then left open with drainage postoperatively. Where there was useful function in the upper pole, but gross dilatation of its ureter, pyelopyelostomy was performed, again with aspiration of the ureterocele. In both groups antibiotic prophylaxis was given if VUR was not demonstrated post-operatively, Where good upper polar function was not associated with gross dilatation of the associated ureter, the ureterocele was excised and the ureters reimplanted within their common
sheath, if necessary with tailoring of the distal upper polar ureter. Some asymptomatic cases were managed expectantly when there was no evidence of bladder outflow obstruction and where the upper urinary tracts were otherwise normal, apart from the non-functioning upper renal pole and its associated ureter. Upper polar nephrectomy. Twenty-three patients ( 3 with bilateral lesions) were initially treated by this means at ages ranging from 10 days to 4.7 years; 21 had truly ectopic and 2 had intravesical orthotopic lesions. There was 1 complication related to the procedure; nephropexy had not been performed, as was normally routine, and downward displacement of the hemi-kidney led to secondary pelviureteric junction obstruction requiring pyelolysis. Histological examination of the excised upper renal poles showed the appearance of severe dysplasia sometimes compounded by secondary inflammatory changes. Post-operative cystography showed no VUR in 9 cases (3 GradeI, 4 Grade 11, 2 Grade 111) and contralateral VUR in 1. Two children, both with truly ectopic lesions, later required excision of the ureterocele plus lower polar ureteric reimplantation, because of symptomatic breakthrough urinary infections associated with persisting VUR but not secondary renal
199
MANAGEMENT OF DUPLEX-SYSTEM URETEROCELES
scarring. These procedures presented no difficulties and had a satisfactory outcome. Twenty-one children (9 1%) in this treatment group have thus far required no further surgery during follow-up ranging from 0.5 to 6.2 years (mean 2). None has evidence of bladder outflow obstruction or obstructive changes in the lower polar ureter and at their most recent assessment 17 have no VUR and 4 have minor degrees of ipsilateral VUR. Pyelopyelostomy. Three children, all with intravesical lesions, were treated by this means. One had ipsilateral lower polar pelviureteric junction obstruction and pyeloplasty was performed as part of the procedure. Two have good results, with diminution of upper polar dilatation and no VUR. In the other, dilatation remains unchanged, as does function. In this case the distal ureter had been ligated. Pyoureter developed some months later and was treated by excision of the ureterocele and its associated ureteric remnant plus lower polar ureteric reimplantation. Primary excision of ureterocele. Five patients with intravesical lesions underwent this procedure together with bipolar ureteric reimplantation. Tailoring of the distal upper polar ureter was necessary on 1 occasion. All results were satisfactory, with improvement or resolution of dilatation and elimination of VUR. Other surgical strategies. Two infants had virtual non-function of both renal poles. One, with unilateral disease, underwent nephroureterectomy plus excision of the (truly ectopic) ureterocele. The other had bilateral disease with, contralaterally, a nonfunctioning upper pole and a normally functioning lower pole with non-refluxing dilatation of its associated ureter. The non-functioning kidney with its associated ureters and ureterocele was excised together with contralateral upper polar nephrectomy. The lower polar ureteric dilatation resolved post-operatively. Nephrectomy specimens showed the appearance of severe congenital dysplasia in both renal poles. Another infant, diagnosed prenatally, had a contralateral duplex-system anomaly with a urethrally ectopic ureter and lower polar VUR with renal rupture and pseudocyst formation. The complications and management of this patient are described elsewhere (Williams et al., in preparation).
Expectant management. Eight patients satisfied our criteria for expectant management. Five had been diagnosed prenatally, while 3 girls presented with symptoms of cystitis which, it was felt, were unlikely to relate to the ureterocele. During followup, which ranged from 0.5 to 6 years (mean 2.3), none has had symptoms and serial ultrasound examinations remain unchanged from the presenting appearances.
Discussion Despite the variable nature of duplex-system ureteroceles-and their secondary features-certain themes emerge from this series. Bladder outflow obstruction is uncommon and consequent secondary changes in the upper renal tracts are rarer still. Function of the upper renal pole is either virtually normal or virtually absent and in this series the former was always associated with ureteroceles which lay wholly intravesically and the latter with truly ectopic lesions. This observation accords with the contention of Mackie and Stephens (1975) that the severity of congenital polar dysplasia relates to the degree of any associated ureteric ectopia; with ureteroceles situated wholly intravesically this is minimal, so that good upper polar function would be anticipated. The virtual non-function found with truly ectopic ureteroceles is associated with histological appearances of severe congenital dysplasia and hence any surgical strategy intended to achieve useful functional recovery is unlikely to be rewarded. By contrast, lower polar function, if not always appearance, is usually perfectly preserved and exceptions to this rule are associated not with obstruction but rather with relatively severe VUR where there is again good reason to believe that the process represents congenital dysplasia rather than acquired reflux nephropathy. Lastly, limited experience of expectant management suggests that the natural history of duplex-system ureteroceles is somewhat more benign than hitherto supposed. What are the implications for management? Clear indications for intervention are bladder outflow obstruction, with or without secondary changes in the upper renal tracts, obstruction of the lower polar ureter by the ureterocele and infection of the upper urinary tracts, especially if associated with VUR or with obstruction. Endoscopic ureterocele incision (Tank, 1980) has occasional value in an emergency but it is not usually regarded as definitive treatment. The difficulties of excising
200 ectopic ureteroceles are well recognised and where the upper renal pole is virtually non-functioning, as is usually the case, its excision plus ureterocele aspiration represents a logical alternative. However, in some series (Mandell et al., 1980; King et al., 1983; Caldamone et al., 1984), secondary excision of the ureterocele was necessary in 20 to 38% of patients, usually because of persisting VUR. In the present series the rate (9%) was much lower and VUR, if existing immediately post-operatively, tended to resolve in time. Although the collapsed ureterocele usually remained visible on ultrasonography, this appears to be inconsequential. In short, our experience thus far with this form of treatment has been satisfactory. Where there is useful upper polar function, pyelopyelostomy has its advocates (Mandell et al., 1980; King et al., 1983; Decter et al., 1989), yet this procedure is not without difficulty if it involves anastomosis of a massively dilated (and potentially infected) upper polar system to a lower polar renal pelvis of normal size. Good upper polar function is usually associated with wholly intravesical ureteroceles which do not present the difficulties posed by truly ectopic lesions. Here we consider that the better policy is excision of the ureterocele plus bipolar reimplantation, if necessary with distal ureteric tailoring. In retrospect, our patients treated by pyelopyelostomy might have been better managed this way. Lastly, what of expectant management? Our experience is too slight to advocate this policy and we merely draw attention to it as a possibility in appropriate circumstances. This might seem especially relevant in a proportion of asymptomatic infants diagnosed by prenatal ultrasonography.
BRITISH JOURNAL OF UROLOGY
References Caldamone, A. A., Snyder H. M. and Duckett, J. W. (1984). Ureteroceles in children : follow-up of management with upper tract approach. J. Urol., 131,1130-1 132. Decter, R. M., Roth, D. R. and Gonzales, E. T. (1989). Individualised treatment of ureteroceles. J. Urol., 142, 535537. Gerridzen, R. andSchillinger, J. F. (1984).Transurethral puncture in management of ectopic ureteroceles. Urology, 23,4347. International Reflux Study Committee (1981). Medical versus surgical management of primary vesicoureteric reflux : a prospective internaticnal reflux study in children. J. Urol., 125,277-283. Johnston, J. H. and Johnson, L. M. (1969). Experience with ectopic ureteroceles. Br. J. Urol., 41,61-70. King, L. R., Kozlowski, J. M. and Schacht, M. J. (1983). Ureteroceles in children-a simplified and successful approach to management. J.A.M.A.,249,1461-1465. Kroovand, L. R. and Perlmutter, A. D. (1979). A one-stage approach to ectopic ureterocele. J. Urol., 122,367-369. Mackie, C. G. and Stephens, F. D. (1975). Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice. J. Urol., 114,274277. Mandell, J., Colodny, A. H., Lebowitz, R. et rrl. (1980). Ureteroceles in infants and children. J. Urol., 123,921-926. Tank, E. S., Jr. (1980). Unroofing of ureteroceles. Society for Pediatric Urology Newsletter, 3&31. Williams, D. I. and Woodard, J. R. (1965). Problems in the management of ectopic ureteroceles. J. Urol., 92,653-662.
The Authors A. M. K. Rickwood, FRCS, Urological Surgeon. I. Reiner, MD, Herbert Johnston Research Fellow. M. Jones, FRCS, Surgical Registrar. C. Pournaras, MD, Senior House Officer. Requests for reprints to: A. M. K. Rickwood, Regional Department of Paediatric Urology, Royal Liverpool Children’s Hospital (Alder Hey), Eaton Road, Liverpool L12 2AP.
01992 British Journal of Urology
PAEDIATRIC UROLOGY
Current Management of Duplex-System Ureteroceles: Experience with 4 1 Patients A. M. K. RICKWOOD, I. R E I N E R , M. JONES and C. POURNARAS Regional Department of Paediatric Urology, Royal Liverpool Children's Hospital (Alder Hey), Liverpool
Summary-Experience is described of 4 1 infants and children with duplex-system ureteroceles, 25 presenting clinically and 16 by prenatal ultrasonography. Bladder outflow obstruction was rare but lower polar vesicoureteric reflux (VUR), usually of lesser grades, was common. Upper polar function, as assessed by ""Tc-DMSA, was negligible in children with truly ectopic ureteroceles but well preserved in those where the lesion lay wholly intravesically. Lower polar function was good, even in the presence of secondary obstruction, except in 2 infants with major VUR. Twenty-three patients were treated by upper polar nephrectomy plus aspiration of the ureterocele; 2 subsequently required ureterocele excision. Histology of excised specimens indicated that a more conservative approach would not have been rewarded. Where upper polar function was good, conservation was maintained in 3 cases by pyelopyelostomy and in 5 more by excision of the ureterocele plus bipolar ureteric reimplantation. Other operative strategies were employed in 2 cases. Finally, a defined group of 8 children was managed expectantly without untoward results. It was concluded that the variable anatomy and function associated with duplex-system ureteraceles require a flexibile approach to treatment, including, possibly, no treatment at all.
The management of duplex-system ureteroceles continues to attract a measure of controversy that doubtless reflects the variations of anatomy and renal function with which they are associated. Although these lesions are usually synonymously termed ectopic ureteroceles, implying encroachment to or beyond the bladder neck, a proportion are situated wholly intravesically. Truly ectopic lesions may cause bladder outflow obstruction, especially if the ureterocele is tense. Function of the affected upper renal poles varies from little at all to perfect preservation. Vesicoureteric reflux to the lower renal pole is common, though seldom severe, and may be associated with congenital dysplasia or with acquired renal scarring. Less often, a tense ureterocele may cause secondary obstruction of the lower polar ureter, with or Accepted for publication 30 August 1991
without impairment of polar function. Lastly, bladder outflow obstruction may cause global dilatation of the upper renal tracts, leading in severe cases to permanently compromised overall renal function. Symptoms are similarly diverse and range from a life-threatening combination of septicaemia and azotaemia to none whatever. The latter has become common with the advent of prenatal ultrasonography . It would seem to be generally accepted that these lesions always merit surgical intervention, regardless of severity or mode of presentation, and until recently such treatment has concentrated upon the ureterocele itself, either in the form of endoscopic incision (Tank, 1980; Gerridzen and Schillinger, 1984) or operative excision combined with lower polar ureteric reimplantation and, usually, simultaneous (Kroovand and Perlmutter, 1979) or subsequent (Johnston and Johnson, 1969) upper polar nephrectomy. Because excision of truly ectopic
196
197
MANAGEMENT OF DUPLEX-SYSTEM URETEROCELES
ureteroceles is not without difficulty or hazard (Williams and Woodward, 1965), especially where the bladder is thick-walled due to outflow obstruction, it has more recently been advocated that initial treatment be addressed to the upper renal pole, with excision where function is poor or pyelopyelostomy where useful function remains (King et al., 1983; Caldamone et al., 1984). In either case, the ureterocele itself is collapsed by aspiration via the distal ureteric remnant, so relieving any bladder outflow obstruction or secondary obstruction of the lower polar ureter. Also, it is claimed that this policy makes for easier excision of the ureterocele should this later prove necessary. It is our contention that the variable nature and presentation of duplex-system ureteroceles call for a flexible approach and that while most recommended strategies have merit in individual cases, none is universally applicable. In addition, our experience leads us to question whether some cases require surgical intervention at all.
polar function. Where initial treatment was directed to the upper renal pole, cystography was performed after, rather than before, such intervention. When other approaches were adopted, cystography formed part of the initial investigative regimen.
Results
The study group comprised 41 patients (29 females, 12 males) whose ages at presentation ranged from birth to 6 years (mean 1.1 year); 23 presented with urinary infection, 16 by prenatal ultrasonography and 1 each with urinary retention and with urinary ascites. Three had bilateral lesions and a further 3 had non-concordant contralateral duplex-system anomalies (1 each urethral ureteric ectopia, introital ureteric ectopia, lower polar VUR). Among the 44 lesions, 29 encroached to or beyond the bladder neck, including 1 prolapsing; 9 lay wholly intravesically and 6, where cystoscopy was not performed, were unknown. Thirty-five patients were assessed by 99mTc-DMSAscintigraPatients and Methods phy : upper polar function was negligible or absent All of the patients reviewed here presented with in 29 instances (Fig. 1) and normally preserved in 6 duplex-system ureteroceles from 1984 to 1990 (Fig. 2), all with intravesical lesions. Of the 23 inclusive. Ultrasonography was the usual initial patients who underwent cystography prior to any investigation, followed in most cases by IVU and surgical intervention, 15 had ipsilateral lower polar VUR (4 grade I, 7 Grade I I , 4 Grade 111) (Internacystoscopy. As a rule, 9 9 m Tdimercaptosuccinic ~ acid (DMSA) scintigraphy was undertaken to assess tional Reflux Study Committee, 1981); in 2 of these
Fig. 1 Female aged 3 months: right duplex-system ectopic ureterocele. (A) The right upper renal pole is non-functioning on IVU. (B) 99mTc-DMSAscintigraphy shows the right lower pole to be functionally and anatomically normal.
198
BRITISH JOURNAL OF UROLOGY
Fig. 2 Female aged 3 months: right intravesical duplex-system ureterocele. (A) IVU shows gross hydronephrosis of both renal poles. (B) g9"Tc-DMSA scintigraphy shows that the uptake of isotope is generally reduced (38% of total) but is equally distributed to both renal poles. Right renal function returned to normal after excision of the ureterocele with bipolar ureteric reimplantation.
there was also contralateral VUR. One patient had pelviureteric junction obstruction in the lower polar system and 3 had non-refluxing dilatation of the lower polar ureter due to secondary obstruction by the ureterocele (2 ectopic, 1 orthotopic). Function of the lower renal pole was normal except in 2 cases where negligible function was associated with Grade I11 VUR. Only 4 patients had clinical or radiological evidence of bladder outflow obstruction, of whom only 1 had global dilatation of the upper renal tracts. Lastly, one girl had calculi lying within the ureterocele. Management Generalpolicy. As a rule, where upper polar function was severely compromised, initial treatment comprised upper polar nephrectomy with aspiration of the ureterocele via the distal ureteric remnant, which was then left open with drainage postoperatively. Where there was useful function in the upper pole, but gross dilatation of its ureter, pyelopyelostomy was performed, again with aspiration of the ureterocele. In both groups antibiotic prophylaxis was given if VUR was not demonstrated post-operatively, Where good upper polar function was not associated with gross dilatation of the associated ureter, the ureterocele was excised and the ureters reimplanted within their common
sheath, if necessary with tailoring of the distal upper polar ureter. Some asymptomatic cases were managed expectantly when there was no evidence of bladder outflow obstruction and where the upper urinary tracts were otherwise normal, apart from the non-functioning upper renal pole and its associated ureter. Upper polar nephrectomy. Twenty-three patients ( 3 with bilateral lesions) were initially treated by this means at ages ranging from 10 days to 4.7 years; 21 had truly ectopic and 2 had intravesical orthotopic lesions. There was 1 complication related to the procedure; nephropexy had not been performed, as was normally routine, and downward displacement of the hemi-kidney led to secondary pelviureteric junction obstruction requiring pyelolysis. Histological examination of the excised upper renal poles showed the appearance of severe dysplasia sometimes compounded by secondary inflammatory changes. Post-operative cystography showed no VUR in 9 cases (3 GradeI, 4 Grade 11, 2 Grade 111) and contralateral VUR in 1. Two children, both with truly ectopic lesions, later required excision of the ureterocele plus lower polar ureteric reimplantation, because of symptomatic breakthrough urinary infections associated with persisting VUR but not secondary renal
199
MANAGEMENT OF DUPLEX-SYSTEM URETEROCELES
scarring. These procedures presented no difficulties and had a satisfactory outcome. Twenty-one children (9 1%) in this treatment group have thus far required no further surgery during follow-up ranging from 0.5 to 6.2 years (mean 2). None has evidence of bladder outflow obstruction or obstructive changes in the lower polar ureter and at their most recent assessment 17 have no VUR and 4 have minor degrees of ipsilateral VUR. Pyelopyelostomy. Three children, all with intravesical lesions, were treated by this means. One had ipsilateral lower polar pelviureteric junction obstruction and pyeloplasty was performed as part of the procedure. Two have good results, with diminution of upper polar dilatation and no VUR. In the other, dilatation remains unchanged, as does function. In this case the distal ureter had been ligated. Pyoureter developed some months later and was treated by excision of the ureterocele and its associated ureteric remnant plus lower polar ureteric reimplantation. Primary excision of ureterocele. Five patients with intravesical lesions underwent this procedure together with bipolar ureteric reimplantation. Tailoring of the distal upper polar ureter was necessary on 1 occasion. All results were satisfactory, with improvement or resolution of dilatation and elimination of VUR. Other surgical strategies. Two infants had virtual non-function of both renal poles. One, with unilateral disease, underwent nephroureterectomy plus excision of the (truly ectopic) ureterocele. The other had bilateral disease with, contralaterally, a nonfunctioning upper pole and a normally functioning lower pole with non-refluxing dilatation of its associated ureter. The non-functioning kidney with its associated ureters and ureterocele was excised together with contralateral upper polar nephrectomy. The lower polar ureteric dilatation resolved post-operatively. Nephrectomy specimens showed the appearance of severe congenital dysplasia in both renal poles. Another infant, diagnosed prenatally, had a contralateral duplex-system anomaly with a urethrally ectopic ureter and lower polar VUR with renal rupture and pseudocyst formation. The complications and management of this patient are described elsewhere (Williams et al., in preparation).
Expectant management. Eight patients satisfied our criteria for expectant management. Five had been diagnosed prenatally, while 3 girls presented with symptoms of cystitis which, it was felt, were unlikely to relate to the ureterocele. During followup, which ranged from 0.5 to 6 years (mean 2.3), none has had symptoms and serial ultrasound examinations remain unchanged from the presenting appearances.
Discussion Despite the variable nature of duplex-system ureteroceles-and their secondary features-certain themes emerge from this series. Bladder outflow obstruction is uncommon and consequent secondary changes in the upper renal tracts are rarer still. Function of the upper renal pole is either virtually normal or virtually absent and in this series the former was always associated with ureteroceles which lay wholly intravesically and the latter with truly ectopic lesions. This observation accords with the contention of Mackie and Stephens (1975) that the severity of congenital polar dysplasia relates to the degree of any associated ureteric ectopia; with ureteroceles situated wholly intravesically this is minimal, so that good upper polar function would be anticipated. The virtual non-function found with truly ectopic ureteroceles is associated with histological appearances of severe congenital dysplasia and hence any surgical strategy intended to achieve useful functional recovery is unlikely to be rewarded. By contrast, lower polar function, if not always appearance, is usually perfectly preserved and exceptions to this rule are associated not with obstruction but rather with relatively severe VUR where there is again good reason to believe that the process represents congenital dysplasia rather than acquired reflux nephropathy. Lastly, limited experience of expectant management suggests that the natural history of duplex-system ureteroceles is somewhat more benign than hitherto supposed. What are the implications for management? Clear indications for intervention are bladder outflow obstruction, with or without secondary changes in the upper renal tracts, obstruction of the lower polar ureter by the ureterocele and infection of the upper urinary tracts, especially if associated with VUR or with obstruction. Endoscopic ureterocele incision (Tank, 1980) has occasional value in an emergency but it is not usually regarded as definitive treatment. The difficulties of excising
200 ectopic ureteroceles are well recognised and where the upper renal pole is virtually non-functioning, as is usually the case, its excision plus ureterocele aspiration represents a logical alternative. However, in some series (Mandell et al., 1980; King et al., 1983; Caldamone et al., 1984), secondary excision of the ureterocele was necessary in 20 to 38% of patients, usually because of persisting VUR. In the present series the rate (9%) was much lower and VUR, if existing immediately post-operatively, tended to resolve in time. Although the collapsed ureterocele usually remained visible on ultrasonography, this appears to be inconsequential. In short, our experience thus far with this form of treatment has been satisfactory. Where there is useful upper polar function, pyelopyelostomy has its advocates (Mandell et al., 1980; King et al., 1983; Decter et al., 1989), yet this procedure is not without difficulty if it involves anastomosis of a massively dilated (and potentially infected) upper polar system to a lower polar renal pelvis of normal size. Good upper polar function is usually associated with wholly intravesical ureteroceles which do not present the difficulties posed by truly ectopic lesions. Here we consider that the better policy is excision of the ureterocele plus bipolar reimplantation, if necessary with distal ureteric tailoring. In retrospect, our patients treated by pyelopyelostomy might have been better managed this way. Lastly, what of expectant management? Our experience is too slight to advocate this policy and we merely draw attention to it as a possibility in appropriate circumstances. This might seem especially relevant in a proportion of asymptomatic infants diagnosed by prenatal ultrasonography.
BRITISH JOURNAL OF UROLOGY
References Caldamone, A. A., Snyder H. M. and Duckett, J. W. (1984). Ureteroceles in children : follow-up of management with upper tract approach. J. Urol., 131,1130-1 132. Decter, R. M., Roth, D. R. and Gonzales, E. T. (1989). Individualised treatment of ureteroceles. J. Urol., 142, 535537. Gerridzen, R. andSchillinger, J. F. (1984).Transurethral puncture in management of ectopic ureteroceles. Urology, 23,4347. International Reflux Study Committee (1981). Medical versus surgical management of primary vesicoureteric reflux : a prospective internaticnal reflux study in children. J. Urol., 125,277-283. Johnston, J. H. and Johnson, L. M. (1969). Experience with ectopic ureteroceles. Br. J. Urol., 41,61-70. King, L. R., Kozlowski, J. M. and Schacht, M. J. (1983). Ureteroceles in children-a simplified and successful approach to management. J.A.M.A.,249,1461-1465. Kroovand, L. R. and Perlmutter, A. D. (1979). A one-stage approach to ectopic ureterocele. J. Urol., 122,367-369. Mackie, C. G. and Stephens, F. D. (1975). Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice. J. Urol., 114,274277. Mandell, J., Colodny, A. H., Lebowitz, R. et rrl. (1980). Ureteroceles in infants and children. J. Urol., 123,921-926. Tank, E. S., Jr. (1980). Unroofing of ureteroceles. Society for Pediatric Urology Newsletter, 3&31. Williams, D. I. and Woodard, J. R. (1965). Problems in the management of ectopic ureteroceles. J. Urol., 92,653-662.
The Authors A. M. K. Rickwood, FRCS, Urological Surgeon. I. Reiner, MD, Herbert Johnston Research Fellow. M. Jones, FRCS, Surgical Registrar. C. Pournaras, MD, Senior House Officer. Requests for reprints to: A. M. K. Rickwood, Regional Department of Paediatric Urology, Royal Liverpool Children’s Hospital (Alder Hey), Eaton Road, Liverpool L12 2AP.
