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Everting Ureteroceles: Radiographic and Endoscopic Observation, and Surgical Management
0022-5347 /80/1234-0538$02.00/0 Vol. 123, April
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1980 by The Williams & Wilkins Co.
EVERTING URETEROCELES: RADIOGRAPHIC AND ENDOSCOPIC OBSERVATION, AND SURGICAL MANAGEMENT TOMOHIKO KOYANAGI,* SADAKAZU HISAJIMA, TOSHIAKI GOTO, SOUHEI TOKUNAKA ICHIRO TSUJI
AND
From the Department of Urology, Hokkaido University School of Medicine, Sapporo, Hokkaido, Japan
ABSTRACT
We describe our experience with 8 cases of an everting ureterocele. The diagnostic features of uroradiography and endoscopy, surgical management and clinical implication are discussed. Several illustrative cases are reported. adult case, which was of the simple type, and the remaining cases were in children from 1½ to 5 years old. A simple ureterocele in children was more likely to be of the everting type (3 of 5, 60 per cent) than in adults (1 of 4, 25 per cent). An everting ectopic ureterocele was seen only in girls, while non-everting ectopic ureteroceles were seen in boys (single ectopic) (see table). Clinical features. There were no distinguishing clinical features between everting and non-everting ureteroceles. All patients presented with urinary tract infection and fever. Ectopic ureteroceles of both types often were associated with voiding disorders, probably from transient incarceration of the ureterocele wall in the bladder neck and urethra. Diagnosis. Excretory urography (IVP) alone did not distinguish between the 2 types. However, in a simple ureterocele ureterorenal dilatation appeared to be associated more frequently with the everting than the non-everting type. In 4 cases of simple everting ureteroceles 4 of 6 ureterorenal units were dilated, while only 1 of 5 ureterorenal units in 5 cases of simple non-everting ureteroceles showed dilatation. Radiographic confirmation of eversion must rely on voiding cystourethrography. A steep oblique projection is desirable. An everting type of ureterocele is characterized by a diverticularlike protrusion outside the general contour of the bladder or urethra, which tends to be progressively enlarged during voiding. An inconspicuously small ureterocele can be overlooked easily unless the exaininer suspects its presence. There were 5 cases of everting ureteroceles diagnosed on the basis of cystourethrographic findings alone. 5• 6 In 1 case an ectopic everting ureterocele was misinterpreted as a paraureteral diverticulum. 8 The other 2 cases, both of the simple type, were diagnosed as everting only after endoscopic examination. The final diagnosis of an everting ureterocele is based on endoscopy with the use of a foroblique lens. A redundant large ureterocele, which may sometimes be large enough to obscure the visual field, starts to shrink, collapses and, finally, protrudes externally through the ureteral hiatus either in the lateral cornu of the trigone in cases of simple orthotopic everting ureteroceles or in the ectopic hiatus9 corresponding to the ectopic pathway3
Despite numerous evolving concepts regarding ureteroceles they still remain a challenging issue to urologists relative to diagnosis and management. The embryologic saccular dilatation, 1 occurring in the caudal end of the ureter, affects not only its own accompanying ureter but also its ipsilateral mate in a duplex system, contralateral ureterorenal unit, bladder outlet and/ or even the urethra by virtue of its intravesical growth along the plane of the superficial trigone. 2 The multifarious behavior of a ureterocele is complicated further by its occasional extroversion through the enlarged ureteral hiatus. This was recognized first in 1963 by Stephens3 and was again indicated briefly by Ambrose and Nicolson,4 who described this as an "everting ureterocele". Subsequently, its diagnostic features, particularly urographic appearance, have been discussed by Friedland and Cunningham,5 Williams and associates,6 and Weiss and Spackman. 7 During the last 7 years we have seen a total of 24 cases of ureterocele, of which 8 were documented as everting ureteroceles. Herein we describe the radiographic and endoscopic features of our cases as well as the surgical management. MATERIAL AND METHOD
We reviewed all cases of simple and ectopic ureteroceles seen at our institution since 1971. Ureteroceles in the single orthotopic ureter in both sexes are referred to as simple. The term ectopic is used to describe ureteroceles involving the caudal end of an ectopic ureter that belongs to either the cranial accessory renal unit of the complete duplex system in girls or the single renal unit in boys and issues along the ectopic pathway3 of the bladder and urethra. The everting ureteroceles were analyzed according to their type (simple versus ectopic), site of involvement, age and sex distribution, and clinical features. A ureterocele was defined as everting when it was seen to be protruding outside the vesical or urethral wall on voiding cystourethrography. This was documented further by detailed endoscopic examination, which must show first the collapse of the ureterocele and subsequent outpouching through the enlarged ureteral hiatus. Urographic and endoscopic data of the everting and non-everting types were compared. RESULTS
Incidence of everting ureterocele (24 cases)
Incidence. The 24 cases of ureteroceles were divided further
Latera:lity
into 9 simple and 15 ectopic types. Of the 8 cases of everting ureteroceles 4 were simple and 4 were ectopic. Involvement was on the left side in 4 cases, the right side in 2 and bilateral in 2. The sex ratio was 3 male versus 5 female patients. All 3 male patients had the simple type of ureterocele. There was only 1 Accepted for publication June 8, 1979. . * Requests for reprints: Department of Urology, Hokkaido University School of Medicine, Sapporo Kita 15jo, Nishi 7chome, Hokkaido, Japan 060. 538
Simple ureterocele: No. cases everted No. cases not everted Totals Ectopic ureterocele: No. cases everted No. cases not everted Totals
Sex
Rt.
Lt.
Bilat.
Male
1 2 3
1 3 4
2 0 2
3 3
4
3 5
0 2
5
8
0 2 2
6
2
Age
Female Adult Child 1 2 3
1 3 4
3 2 5
4 9 13
0 4
4
4
11
7
EVERTING URETEROCELES
of the bladder or urethra in cases of ectopic everting ureteroceles. A small everting ureterocele, which is not uncommon in the simple type, may not be distinguishable from heaped-up mucosa of a paraureteral diverticulum8 but endoscopy is most helpful in the differentiation. In a paraureteral diverticulum the ureteral orifice usually stays at the verge of the diverticulum when the bladder is distended, while in the everting ureterocele the entire structure of the ureterocele, at least its orifice, protrudes outside the bladder wall through the enlarged ureteral hiatus. Management. All patients with simple everting ureteroceles underwent successful ureterocelectomy and ureterovesiconeostomy using a modified Paquin method. In 3 cases of ectopic everting ureteroceles ureterocelectomy and heminephroureterectomy were done, since the involved renal units were nonfunctioning. In the other case ureterovesiconeostomy with tapering was done. Our operative technique in renal conserving and ablative procedures has been reported previously. 9 CASE REPORTS
Case 1. S. Y., 54-21, a 3-year-old boy, had bilateral simple ureteroceles associated with marked left hydroureterone-
539
phrosis, which was demonstrated on an IVP (fig. 1, A). Right and left projections of a voiding cystourethrogram showed partial (fig. 1, B) and complete eversion (fig. 1, D and E), respectively. Endoscopy confirmed these radiographic differences by demonstrating incomplete eversion (fig. 1, C) and complete eversion (fig. 1, F) of the right and left simple ureteroceles, respectively. Subsequently, the patient underwent successful bilateral ureterocelectomy and ureterovesiconeostomy. The difference in the size of the ureteral hiatus, that is the left side was larger than the right side, was confirmed during reconstruction in preparation for ureterovesiconeostomy. Comment: Elasticity of the ureterocele wall and its resulting compressibility may be prerequisites for eversion. However, the degree of eversion appeared to be influenced by the size of the ureteral hiatus, the larger one being associated with complete eversion and accompanying the larger ureter, while the smaller one was associated with partial eversion and lesser ureteral dilatation. Case 2. S. S., 53-64, a 7-year-old boy, had a right simple ureterocele. An IVP showed a cobra head deformity of the right ureter pathognomonic of a ureterocele (fig. 2, A). Voiding cystourethrography revealed not only ipsilateral ureteral reflux
Fm. 1. Case 1. A, IVP. Band C, partial eversion of right ureterocele as seen on voiding cystourethrogram and endoscopy. D to F, complete eversion of left ureterocele as seen on voiding cystourethrogram and endoscopy.
540
KOYANAGI AND ASSOCIATES
urethrography demonstrated reflux into the right lower unit and superimposed scaphoid pooling of contrast material in the ureteral end, which mimicked a paraureteral diverticulum (fig. 4, B). Subsequent endoscopy showed a right ectopic ureterocele. Eversion of this ureterocele into the hiatus of the right lateral
Type
1
2
FIG. 2. Case 2. A, IVP. B, voiding cystourethrogram shows everting ureterocele superimposed over ureteral end with reflux.
but also an inconspicuously small accompanying semilunar density that was superimposed on the ureteral end but distinctively outside the general contour of either the bladder or ureter (fig. 2, B). Subsequent endoscopy confirmed this to be a result from the single ureterocele that everted. Whether this ureterocele everted into the ureteral lumen proper as in case 1 (fig. 3, type 1) or into the loose space between the ureteral wall and hiatus (fig. 3, type 2) could not be differentiated, even with careful endoscopic examination. Type 2 eversion was verified by careful operative dissection of the ureteral hiatus at the time of right ureterovesiconeostomy. Comment: We believe that there are 2 types in the mode of eversion. The type of eversion typically seen in the left ureter of case 1, when the ureterocele everted into the large ureteral lumen proper surrounded by the loose and enlarged ureteral hiatus, may be arbitrarily designated as type 1. Eversion into the loose hiatal space externally along the craniolateral aspect of the ureteral wall may be arbitrarily called type 2. Endoscopy cannot foretell if an everting ureterocele is type 1 or 2, the differentiation of which is facilitated better by cystourethrographic findings and further confirmed at operation. Case 3. N. M., 53-261, a 5-year-old girl, had a right completely duplex system with an ectopic everting ureterocele and orthotopic ureteral reflux. An IVP showed marked right hydroureteronephrosis, possible duplication of the ureter and a cobra head deformity in the bladder (fig. 4, A). Voiding cysto-
FIG. 3. Two types of eversion in ureterocele. Type 1 is designated when ureterocele is everting intraluminally into dilated ureter, which is issuing in bladder or urethral wall through enlarged hiatus. Type 2 is designated when ureterocele is everting exteriorly along craniolateral aspect of ureteral wall through loose hiatus.
FIG. 4. Case 3. A, IVP. B, voiding cystourethrogram. C, diagrammatic sketch of everting ectopic ureterocele, which could be misinterpreted as paraureteral diverticulum of right lower ureter with reflux.
541
EVERTING URETEROCELES
comu of the trigone was demonstrated easily by suprapubic manual compression. An orthotopic ureteral orifice that was slightly gapped was seen at the superior verge of the hiatus (fig. 5, D). An ectopic ureteral orifice was located at the bladder neck (fig. 5, A), through which an endoscope was inserted to visualize the interior of the ectopic ureterocele (fig. 5, B). The roof of the ureterocele was descended by manual compression and pulled into the large ureteral hiatus in the lateral comu of the trigone (fig. 5, C). The right hemitrigone was normal except for this enlarged ectopic hiatus. The ureterocele was excised without leaving an excess valvular flap and both ureters were tapered and reanastomosed to the bladder base by a modified Paquin method after firm closure of the ureteral hiatus. Comment: The cystourethrographic finding of this case closely simulated a paraureteral diverticulum with reflux. 8 However, endoscopy revealed that this diverticular-like density was found to be a result of an everting ectopic ureterocele of the upper renal unit. An embryologic study of paraureteral
A
B
C
D
Fm. 5. Case 3. A, orifice of right ectopic ureterocele at bladder neck. B, interior of ureterocele with enlarged ureteral hiatus. C, descension of roof of ureterocele and prolapse toward hiatus by light manual compression. D, right orthotopic ureteral orifice at superior verge of ureterocele.
diverticula did not reveal a case of a diverticulum caudiomedial to an orthotopic ureter. 10 These cystourethrographic findings, namely ureteral reflux into an orthotopic lower ureter and eversion of a ureterocele into an ectopic upper ureter, may be referred to as a case of a pseudoparaureteral diverticulum. Without endoscopy differentiation between a paraureteral and pseudoparaureteral diverticulum would have been impossible (fig. 4, C). This was an interesting case of a sphincteric ureterocele1 that also showed eversion. Case 4. K. Y., 52-317, a I-year-old girl, had marked right hydroureteronephrosis, drooping lily sign of the left kidney with fewer number of calices than usual and a large radiolucent filling defect in the base of the bladder compatible with an ectopic ureterocele, which were demonstrated on an IVP (fig. 6, A). Cystourethrography showed bladder outlet obstruction by the ureterocele, which was seen initially only as a thin linear extravesical density, but it progressively enlarged and eventually protruded completely out of the contour of the bladder and urethra (fig. 7). Endoscopy revealed a large redundant ureterocele occupying the entire trigone from just below the normal-appearing orthotopic ureteral orifices and extending caudally beyond the bladder neck into the urethra. With gradual distension of the bladder the ureterocele began to collapse and finally protruded exteriorly through the ectopically situated large hiatus in the urethra just below the bladder neck. Diagnosis was a left completely duplex system with an everting ectopic ureterocele and right megaloureter, probably secondary to bladder outlet obstruction by the ureterocele. After a heminephrectomy through a left flank incision the massively dilated ectopic ureter was explored through a separate lower abdominal incision. This was purchased and sepa-
b
Fm. 6. Case 4. A, preoperative IVP. B, postoperative IVP. C, diagrammatic sketch of operative steps in total (distal) ureterectomy and closure of (ectopic ureteral) hiatus.
542
KOYANAGI AND ASSOCIATES
Fm. 7. Case 4. A and B, voiding cystourethrogram. C, diagrammatic depiction of posteriorly everted ureterocele (b) superimposed over bladder and urethra (b') on anteroposterior view. I
'
rated easily from the twin mate orthotopic ureter down to the juxtavesical portion. Since both ureters were inseparable from that portion below without jeopardizing the blood supply to the ureters, an ectopic ureter that ran extravesically and was issuing ectopically in the urethra was opened longitudinally along the antimesenteric border of the ureters well down to its hiatus in the urethra (fig. 6, Ca and Cb). After the redundant ureteral wall was excised from both sides of the exposed ureter a small Foley catheter was inserted into the urethra through this hiatus (fig. 6, Cc and Cd). With slight traction on the Foley catheter the ectopic ureteral hiatus was brought up and easily identified. Several horizontal mattress sutures were placed around it (fig. 6, Ce) and securely tied after removal of the catheter, thus firmly closing the enlarged ectopic ureteral hiatus (fig. 6, Cf and Cg). The bladder was then opened, the ureterocele was simply excised as far caudally as possible so that no valvular flap remained and a simple running hemostatic suture was placed along the edge of the excised ureterocele wall (fig. 6, Ch). Convalescence was uneventful. There was no disturbance in urinary continence or voiding function. An IVP 2 years later revealed resolution of the right hydroureteronephrosis to an almost normal level (fig. 6, B). Comment: Surgical management of an ectopic ureterocele often is fraught with difficulties. Based on our experience we cannot endorse the idea of Belman and associates who advocate simple heminephrectomy with an optimistic expectation of subsequent spontaneous collapse of the ureterocele. 11 We believe that when heminephrectomy is indicated the dilated distal ureteral stump and the ectopic ureterocele should be excised in toto, since the ureterocele does not collapse and the excess ureteral stump may become a cause of persistent difficulties. Our technique in the management of a ureteral stump in a duplex anomaly of the ureters has been described previously. 9 Preoperative evaluation of the hiatal condition regarding its size and position, which can be conceived easily by careful cystourethrographic and endoscopic examination, proved to be most helpful in the surgical management of duplex anomalies. DISCUSSION
An everting ureterocele is a relatively new concept, although it eventually will be reported more often with the advent of uroradiographic evaluation of ureteroceles. 5 • 6 Our study disclosed that this is not uncommon conduct of a ureterocele as indicated by its incidence in a third of our cases. However, for the proper diagnosis one must be suspicious of its presence on detailed uroradiography and endoscopic confirmation is man-
datory. Regardless of the difference in the mode of eversion, that is whether type 1 or 2, this phenomenon indicates that the ureteral hiatus is enlarged, which must be a similar developmental phenomenon to other associated anomalies of the ureterocele, such as megaloureter and renal dysplasia. 12 Clinically, this implies that in a simple orthotopic ureterocele the ureterorenal anomalies of an everting ureterocele are more pronounced than a non-everting ureterocele, while in an ectopic ureterocele position of the ureteral orifice 12 and/or the ureteral hiatus9 may be another influencing factor for the associated anomalies. Regarding surgical management, simple excision is contraindicated, since this is merely converting an obstructing ureterocele to one with reflux. Reconstruction of the posterior bladder wall with firm closure of the enlarged ureteral hiatus and antirefluxing ureterovesiconeostomy definitely are indicated. REFERENCES
1. Stephens, F. C.: Caecoureterocele and concepts on the embryology and aetiology of ureteroceles. Aust. New Zeal. J. Surg., 40: 239, 1971. 2. Tanagho, E. A.: Anatomy and management ofureteroceles. J. Urol., 107: 729, 1972. 3. Stephens, F. D.: The ABC of megaureters. In: Congenital Malformations of the Rectum, Anus and Genitourinary Tract. London: E. S. Livingstone, pp. 178-195, 1963. 4. Ambrose, S. S. and Nicolson, W. P.: Ureteral reflux in duplicated ureters. J. Urol., 92: 439, 1964. 5. Friedland, G. W. and Cunningham, J.: The elusive ectopic ureteroceles. Amer. J. Roentgen., 116: 792, 1972. 6. Williams, D. I., Fay, R. and Lillie, J. G.: The functional radiology of ectopic ureterocele. Brit. J. Urol., 44: 417, 1972. 7. Weiss, R. M. and Spackman, T. J.: Everting ectopic ureteroceles. J. Urol., 111: 538, 1974. 8. Kelalis, P. P.: Bladder. In: Clinical Pediatric Urology, 1st ed. Edited by P. P. Kelalis and L. R. King. Philadelphia: W. B. Saunders Co., chapt. lOE, p. 282, 1976. 9. Koyanagi, T. and Tsuiji, I.: Experience of complete duplication of the collecting system: retrospective analysis of 23 cases with particular emphasis on the type of ureteral hiatus and its implication in the management of duplex. Int. J. Urol. Nephrol., 11: 27, 1979. 10. Wickramasinghe, S. F. and Stephens, F. D.: Paraureteral diverticula. Associated renal morphology and embryogenesis. Invest. Urol., 14: 381, 1977. 11. Belman, A. B., Filmer, R. B. and King, L. R.: Surgical management of duplication of the collecting system. J. Urol., 112: 316, 1974. 12. Mackie, G. G. and Stephens, F. D.: Duplex kidneys: a correlation of renal dyspl~sia with position of the ureteral orifice. J. Urol., 114: 274, 1975.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1980 by The Williams & Wilkins Co.
EVERTING URETEROCELES: RADIOGRAPHIC AND ENDOSCOPIC OBSERVATION, AND SURGICAL MANAGEMENT TOMOHIKO KOYANAGI,* SADAKAZU HISAJIMA, TOSHIAKI GOTO, SOUHEI TOKUNAKA ICHIRO TSUJI
AND
From the Department of Urology, Hokkaido University School of Medicine, Sapporo, Hokkaido, Japan
ABSTRACT
We describe our experience with 8 cases of an everting ureterocele. The diagnostic features of uroradiography and endoscopy, surgical management and clinical implication are discussed. Several illustrative cases are reported. adult case, which was of the simple type, and the remaining cases were in children from 1½ to 5 years old. A simple ureterocele in children was more likely to be of the everting type (3 of 5, 60 per cent) than in adults (1 of 4, 25 per cent). An everting ectopic ureterocele was seen only in girls, while non-everting ectopic ureteroceles were seen in boys (single ectopic) (see table). Clinical features. There were no distinguishing clinical features between everting and non-everting ureteroceles. All patients presented with urinary tract infection and fever. Ectopic ureteroceles of both types often were associated with voiding disorders, probably from transient incarceration of the ureterocele wall in the bladder neck and urethra. Diagnosis. Excretory urography (IVP) alone did not distinguish between the 2 types. However, in a simple ureterocele ureterorenal dilatation appeared to be associated more frequently with the everting than the non-everting type. In 4 cases of simple everting ureteroceles 4 of 6 ureterorenal units were dilated, while only 1 of 5 ureterorenal units in 5 cases of simple non-everting ureteroceles showed dilatation. Radiographic confirmation of eversion must rely on voiding cystourethrography. A steep oblique projection is desirable. An everting type of ureterocele is characterized by a diverticularlike protrusion outside the general contour of the bladder or urethra, which tends to be progressively enlarged during voiding. An inconspicuously small ureterocele can be overlooked easily unless the exaininer suspects its presence. There were 5 cases of everting ureteroceles diagnosed on the basis of cystourethrographic findings alone. 5• 6 In 1 case an ectopic everting ureterocele was misinterpreted as a paraureteral diverticulum. 8 The other 2 cases, both of the simple type, were diagnosed as everting only after endoscopic examination. The final diagnosis of an everting ureterocele is based on endoscopy with the use of a foroblique lens. A redundant large ureterocele, which may sometimes be large enough to obscure the visual field, starts to shrink, collapses and, finally, protrudes externally through the ureteral hiatus either in the lateral cornu of the trigone in cases of simple orthotopic everting ureteroceles or in the ectopic hiatus9 corresponding to the ectopic pathway3
Despite numerous evolving concepts regarding ureteroceles they still remain a challenging issue to urologists relative to diagnosis and management. The embryologic saccular dilatation, 1 occurring in the caudal end of the ureter, affects not only its own accompanying ureter but also its ipsilateral mate in a duplex system, contralateral ureterorenal unit, bladder outlet and/ or even the urethra by virtue of its intravesical growth along the plane of the superficial trigone. 2 The multifarious behavior of a ureterocele is complicated further by its occasional extroversion through the enlarged ureteral hiatus. This was recognized first in 1963 by Stephens3 and was again indicated briefly by Ambrose and Nicolson,4 who described this as an "everting ureterocele". Subsequently, its diagnostic features, particularly urographic appearance, have been discussed by Friedland and Cunningham,5 Williams and associates,6 and Weiss and Spackman. 7 During the last 7 years we have seen a total of 24 cases of ureterocele, of which 8 were documented as everting ureteroceles. Herein we describe the radiographic and endoscopic features of our cases as well as the surgical management. MATERIAL AND METHOD
We reviewed all cases of simple and ectopic ureteroceles seen at our institution since 1971. Ureteroceles in the single orthotopic ureter in both sexes are referred to as simple. The term ectopic is used to describe ureteroceles involving the caudal end of an ectopic ureter that belongs to either the cranial accessory renal unit of the complete duplex system in girls or the single renal unit in boys and issues along the ectopic pathway3 of the bladder and urethra. The everting ureteroceles were analyzed according to their type (simple versus ectopic), site of involvement, age and sex distribution, and clinical features. A ureterocele was defined as everting when it was seen to be protruding outside the vesical or urethral wall on voiding cystourethrography. This was documented further by detailed endoscopic examination, which must show first the collapse of the ureterocele and subsequent outpouching through the enlarged ureteral hiatus. Urographic and endoscopic data of the everting and non-everting types were compared. RESULTS
Incidence of everting ureterocele (24 cases)
Incidence. The 24 cases of ureteroceles were divided further
Latera:lity
into 9 simple and 15 ectopic types. Of the 8 cases of everting ureteroceles 4 were simple and 4 were ectopic. Involvement was on the left side in 4 cases, the right side in 2 and bilateral in 2. The sex ratio was 3 male versus 5 female patients. All 3 male patients had the simple type of ureterocele. There was only 1 Accepted for publication June 8, 1979. . * Requests for reprints: Department of Urology, Hokkaido University School of Medicine, Sapporo Kita 15jo, Nishi 7chome, Hokkaido, Japan 060. 538
Simple ureterocele: No. cases everted No. cases not everted Totals Ectopic ureterocele: No. cases everted No. cases not everted Totals
Sex
Rt.
Lt.
Bilat.
Male
1 2 3
1 3 4
2 0 2
3 3
4
3 5
0 2
5
8
0 2 2
6
2
Age
Female Adult Child 1 2 3
1 3 4
3 2 5
4 9 13
0 4
4
4
11
7
EVERTING URETEROCELES
of the bladder or urethra in cases of ectopic everting ureteroceles. A small everting ureterocele, which is not uncommon in the simple type, may not be distinguishable from heaped-up mucosa of a paraureteral diverticulum8 but endoscopy is most helpful in the differentiation. In a paraureteral diverticulum the ureteral orifice usually stays at the verge of the diverticulum when the bladder is distended, while in the everting ureterocele the entire structure of the ureterocele, at least its orifice, protrudes outside the bladder wall through the enlarged ureteral hiatus. Management. All patients with simple everting ureteroceles underwent successful ureterocelectomy and ureterovesiconeostomy using a modified Paquin method. In 3 cases of ectopic everting ureteroceles ureterocelectomy and heminephroureterectomy were done, since the involved renal units were nonfunctioning. In the other case ureterovesiconeostomy with tapering was done. Our operative technique in renal conserving and ablative procedures has been reported previously. 9 CASE REPORTS
Case 1. S. Y., 54-21, a 3-year-old boy, had bilateral simple ureteroceles associated with marked left hydroureterone-
539
phrosis, which was demonstrated on an IVP (fig. 1, A). Right and left projections of a voiding cystourethrogram showed partial (fig. 1, B) and complete eversion (fig. 1, D and E), respectively. Endoscopy confirmed these radiographic differences by demonstrating incomplete eversion (fig. 1, C) and complete eversion (fig. 1, F) of the right and left simple ureteroceles, respectively. Subsequently, the patient underwent successful bilateral ureterocelectomy and ureterovesiconeostomy. The difference in the size of the ureteral hiatus, that is the left side was larger than the right side, was confirmed during reconstruction in preparation for ureterovesiconeostomy. Comment: Elasticity of the ureterocele wall and its resulting compressibility may be prerequisites for eversion. However, the degree of eversion appeared to be influenced by the size of the ureteral hiatus, the larger one being associated with complete eversion and accompanying the larger ureter, while the smaller one was associated with partial eversion and lesser ureteral dilatation. Case 2. S. S., 53-64, a 7-year-old boy, had a right simple ureterocele. An IVP showed a cobra head deformity of the right ureter pathognomonic of a ureterocele (fig. 2, A). Voiding cystourethrography revealed not only ipsilateral ureteral reflux
Fm. 1. Case 1. A, IVP. Band C, partial eversion of right ureterocele as seen on voiding cystourethrogram and endoscopy. D to F, complete eversion of left ureterocele as seen on voiding cystourethrogram and endoscopy.
540
KOYANAGI AND ASSOCIATES
urethrography demonstrated reflux into the right lower unit and superimposed scaphoid pooling of contrast material in the ureteral end, which mimicked a paraureteral diverticulum (fig. 4, B). Subsequent endoscopy showed a right ectopic ureterocele. Eversion of this ureterocele into the hiatus of the right lateral
Type
1
2
FIG. 2. Case 2. A, IVP. B, voiding cystourethrogram shows everting ureterocele superimposed over ureteral end with reflux.
but also an inconspicuously small accompanying semilunar density that was superimposed on the ureteral end but distinctively outside the general contour of either the bladder or ureter (fig. 2, B). Subsequent endoscopy confirmed this to be a result from the single ureterocele that everted. Whether this ureterocele everted into the ureteral lumen proper as in case 1 (fig. 3, type 1) or into the loose space between the ureteral wall and hiatus (fig. 3, type 2) could not be differentiated, even with careful endoscopic examination. Type 2 eversion was verified by careful operative dissection of the ureteral hiatus at the time of right ureterovesiconeostomy. Comment: We believe that there are 2 types in the mode of eversion. The type of eversion typically seen in the left ureter of case 1, when the ureterocele everted into the large ureteral lumen proper surrounded by the loose and enlarged ureteral hiatus, may be arbitrarily designated as type 1. Eversion into the loose hiatal space externally along the craniolateral aspect of the ureteral wall may be arbitrarily called type 2. Endoscopy cannot foretell if an everting ureterocele is type 1 or 2, the differentiation of which is facilitated better by cystourethrographic findings and further confirmed at operation. Case 3. N. M., 53-261, a 5-year-old girl, had a right completely duplex system with an ectopic everting ureterocele and orthotopic ureteral reflux. An IVP showed marked right hydroureteronephrosis, possible duplication of the ureter and a cobra head deformity in the bladder (fig. 4, A). Voiding cysto-
FIG. 3. Two types of eversion in ureterocele. Type 1 is designated when ureterocele is everting intraluminally into dilated ureter, which is issuing in bladder or urethral wall through enlarged hiatus. Type 2 is designated when ureterocele is everting exteriorly along craniolateral aspect of ureteral wall through loose hiatus.
FIG. 4. Case 3. A, IVP. B, voiding cystourethrogram. C, diagrammatic sketch of everting ectopic ureterocele, which could be misinterpreted as paraureteral diverticulum of right lower ureter with reflux.
541
EVERTING URETEROCELES
comu of the trigone was demonstrated easily by suprapubic manual compression. An orthotopic ureteral orifice that was slightly gapped was seen at the superior verge of the hiatus (fig. 5, D). An ectopic ureteral orifice was located at the bladder neck (fig. 5, A), through which an endoscope was inserted to visualize the interior of the ectopic ureterocele (fig. 5, B). The roof of the ureterocele was descended by manual compression and pulled into the large ureteral hiatus in the lateral comu of the trigone (fig. 5, C). The right hemitrigone was normal except for this enlarged ectopic hiatus. The ureterocele was excised without leaving an excess valvular flap and both ureters were tapered and reanastomosed to the bladder base by a modified Paquin method after firm closure of the ureteral hiatus. Comment: The cystourethrographic finding of this case closely simulated a paraureteral diverticulum with reflux. 8 However, endoscopy revealed that this diverticular-like density was found to be a result of an everting ectopic ureterocele of the upper renal unit. An embryologic study of paraureteral
A
B
C
D
Fm. 5. Case 3. A, orifice of right ectopic ureterocele at bladder neck. B, interior of ureterocele with enlarged ureteral hiatus. C, descension of roof of ureterocele and prolapse toward hiatus by light manual compression. D, right orthotopic ureteral orifice at superior verge of ureterocele.
diverticula did not reveal a case of a diverticulum caudiomedial to an orthotopic ureter. 10 These cystourethrographic findings, namely ureteral reflux into an orthotopic lower ureter and eversion of a ureterocele into an ectopic upper ureter, may be referred to as a case of a pseudoparaureteral diverticulum. Without endoscopy differentiation between a paraureteral and pseudoparaureteral diverticulum would have been impossible (fig. 4, C). This was an interesting case of a sphincteric ureterocele1 that also showed eversion. Case 4. K. Y., 52-317, a I-year-old girl, had marked right hydroureteronephrosis, drooping lily sign of the left kidney with fewer number of calices than usual and a large radiolucent filling defect in the base of the bladder compatible with an ectopic ureterocele, which were demonstrated on an IVP (fig. 6, A). Cystourethrography showed bladder outlet obstruction by the ureterocele, which was seen initially only as a thin linear extravesical density, but it progressively enlarged and eventually protruded completely out of the contour of the bladder and urethra (fig. 7). Endoscopy revealed a large redundant ureterocele occupying the entire trigone from just below the normal-appearing orthotopic ureteral orifices and extending caudally beyond the bladder neck into the urethra. With gradual distension of the bladder the ureterocele began to collapse and finally protruded exteriorly through the ectopically situated large hiatus in the urethra just below the bladder neck. Diagnosis was a left completely duplex system with an everting ectopic ureterocele and right megaloureter, probably secondary to bladder outlet obstruction by the ureterocele. After a heminephrectomy through a left flank incision the massively dilated ectopic ureter was explored through a separate lower abdominal incision. This was purchased and sepa-
b
Fm. 6. Case 4. A, preoperative IVP. B, postoperative IVP. C, diagrammatic sketch of operative steps in total (distal) ureterectomy and closure of (ectopic ureteral) hiatus.
542
KOYANAGI AND ASSOCIATES
Fm. 7. Case 4. A and B, voiding cystourethrogram. C, diagrammatic depiction of posteriorly everted ureterocele (b) superimposed over bladder and urethra (b') on anteroposterior view. I
'
rated easily from the twin mate orthotopic ureter down to the juxtavesical portion. Since both ureters were inseparable from that portion below without jeopardizing the blood supply to the ureters, an ectopic ureter that ran extravesically and was issuing ectopically in the urethra was opened longitudinally along the antimesenteric border of the ureters well down to its hiatus in the urethra (fig. 6, Ca and Cb). After the redundant ureteral wall was excised from both sides of the exposed ureter a small Foley catheter was inserted into the urethra through this hiatus (fig. 6, Cc and Cd). With slight traction on the Foley catheter the ectopic ureteral hiatus was brought up and easily identified. Several horizontal mattress sutures were placed around it (fig. 6, Ce) and securely tied after removal of the catheter, thus firmly closing the enlarged ectopic ureteral hiatus (fig. 6, Cf and Cg). The bladder was then opened, the ureterocele was simply excised as far caudally as possible so that no valvular flap remained and a simple running hemostatic suture was placed along the edge of the excised ureterocele wall (fig. 6, Ch). Convalescence was uneventful. There was no disturbance in urinary continence or voiding function. An IVP 2 years later revealed resolution of the right hydroureteronephrosis to an almost normal level (fig. 6, B). Comment: Surgical management of an ectopic ureterocele often is fraught with difficulties. Based on our experience we cannot endorse the idea of Belman and associates who advocate simple heminephrectomy with an optimistic expectation of subsequent spontaneous collapse of the ureterocele. 11 We believe that when heminephrectomy is indicated the dilated distal ureteral stump and the ectopic ureterocele should be excised in toto, since the ureterocele does not collapse and the excess ureteral stump may become a cause of persistent difficulties. Our technique in the management of a ureteral stump in a duplex anomaly of the ureters has been described previously. 9 Preoperative evaluation of the hiatal condition regarding its size and position, which can be conceived easily by careful cystourethrographic and endoscopic examination, proved to be most helpful in the surgical management of duplex anomalies. DISCUSSION
An everting ureterocele is a relatively new concept, although it eventually will be reported more often with the advent of uroradiographic evaluation of ureteroceles. 5 • 6 Our study disclosed that this is not uncommon conduct of a ureterocele as indicated by its incidence in a third of our cases. However, for the proper diagnosis one must be suspicious of its presence on detailed uroradiography and endoscopic confirmation is man-
datory. Regardless of the difference in the mode of eversion, that is whether type 1 or 2, this phenomenon indicates that the ureteral hiatus is enlarged, which must be a similar developmental phenomenon to other associated anomalies of the ureterocele, such as megaloureter and renal dysplasia. 12 Clinically, this implies that in a simple orthotopic ureterocele the ureterorenal anomalies of an everting ureterocele are more pronounced than a non-everting ureterocele, while in an ectopic ureterocele position of the ureteral orifice 12 and/or the ureteral hiatus9 may be another influencing factor for the associated anomalies. Regarding surgical management, simple excision is contraindicated, since this is merely converting an obstructing ureterocele to one with reflux. Reconstruction of the posterior bladder wall with firm closure of the enlarged ureteral hiatus and antirefluxing ureterovesiconeostomy definitely are indicated. REFERENCES
1. Stephens, F. C.: Caecoureterocele and concepts on the embryology and aetiology of ureteroceles. Aust. New Zeal. J. Surg., 40: 239, 1971. 2. Tanagho, E. A.: Anatomy and management ofureteroceles. J. Urol., 107: 729, 1972. 3. Stephens, F. D.: The ABC of megaureters. In: Congenital Malformations of the Rectum, Anus and Genitourinary Tract. London: E. S. Livingstone, pp. 178-195, 1963. 4. Ambrose, S. S. and Nicolson, W. P.: Ureteral reflux in duplicated ureters. J. Urol., 92: 439, 1964. 5. Friedland, G. W. and Cunningham, J.: The elusive ectopic ureteroceles. Amer. J. Roentgen., 116: 792, 1972. 6. Williams, D. I., Fay, R. and Lillie, J. G.: The functional radiology of ectopic ureterocele. Brit. J. Urol., 44: 417, 1972. 7. Weiss, R. M. and Spackman, T. J.: Everting ectopic ureteroceles. J. Urol., 111: 538, 1974. 8. Kelalis, P. P.: Bladder. In: Clinical Pediatric Urology, 1st ed. Edited by P. P. Kelalis and L. R. King. Philadelphia: W. B. Saunders Co., chapt. lOE, p. 282, 1976. 9. Koyanagi, T. and Tsuiji, I.: Experience of complete duplication of the collecting system: retrospective analysis of 23 cases with particular emphasis on the type of ureteral hiatus and its implication in the management of duplex. Int. J. Urol. Nephrol., 11: 27, 1979. 10. Wickramasinghe, S. F. and Stephens, F. D.: Paraureteral diverticula. Associated renal morphology and embryogenesis. Invest. Urol., 14: 381, 1977. 11. Belman, A. B., Filmer, R. B. and King, L. R.: Surgical management of duplication of the collecting system. J. Urol., 112: 316, 1974. 12. Mackie, G. G. and Stephens, F. D.: Duplex kidneys: a correlation of renal dyspl~sia with position of the ureteral orifice. J. Urol., 114: 274, 1975.