0022-5347/80/1241-0136$02.00/0 Vol. 124, July
THE JOURNAL OF UROLOGY
Copyright© 1980 by The Williams & Wilkins Co.
Printed in U.S.A.
REFLUXING URETEROCELES J. LEONG, B. MIKHAEL AND J. F. SCHILLINGER From the Division of Urology, Children's Hospital of Eastern Ontario, Ottawa, Canada
ABSTRACT
We report 4 cases of 3 different forms of ureteroceles to illustrate spontaneous reflux without any evidence of obstruction. The presence of reflux tends to support those concepts that ureterocele formation is not on an obstructive basis. The obstruction that is often associated with ureteroceles is probably caused by the same stimulus as that which created the ureterocele. Obstruction does not necessarily accompany every ureterocele and is not causative of its formation. Campbell encountered ureteroceles in 4.0 per cent of children examined for pyrexia and also in 1 of about 3,500 autopsies. 1 Gummess and associates found this lesion in 2. 7 per cent of all children subjected to urologic studies. 2 Ureteroceles may be simple or ectopic (with or without a duplex collecting system), depending on the site of opening of the ureteral meatus. 3 Furthermore, Stephens has described a cecoureterocele with a large, purely intravesical meatus but with intraurethral extension of the ureterocele. 4 A number of theories have been advanced to explain the development of ureteroceles. 4 - 6 Classically, the cause of the condition is thought to be owing to the minute size of the ureteral ostium. The persistence of Chwalle's membrane leads to obstruction at the junction of the mesonephric membrane and the primitive urogenital sinus. However, Williams and Woodard questioned this theory, since they found that not every ureterocele had a stenotic orifice. 7 Stephens theorized that the development of ureteroceles is not determined by obstruction but embryologically. 4 He believes that in some cases, at least, by virtue of mishaps in either embryologic timing or positioning, a segment of the ureter lying in the trigonal zone may be caught in the expansion stimulus to bladder growth and undergo focal enlargement, becoming a ureterocele. The size, shape and orifice site of the ureterocele thus developed are then determined by the nature of the expansion zone, regardless of the size of the ureteral orifice. Tanagho explains that the occurrence of ureteroceles is owing to expansion of the caudal end plate prior to tubularization of its connection with the wolffian duct. 6 It generally is conceded that free reflux may occur into ureteroceles that have been unroofed or incised, 8- 10 or spontaneously ruptured. 7 Reflux into intact ureteroceles presents some controversy in the literature. Borden and Martinez found difficulty in documenting reflux into any intact orthotopic or ectopic variety other than cecoureteroceles. 11 They then presented 2 cases of reflux into orthotopic simple ureteroceles. One highly regarded text states that other than reflux into the cecoureterocele, reflux into ectopic ureteroceles (intact) never occurs. 12 However, another reputable text states that 10 to 15 per cent of ectopic ureteroceles (with 2 illustrations) are associated with reflux into the ureterocele-bearing segment. 13 This latter, however, does not indicate whether the ureterocele is intact. Admittedly, the concept of an ectopic intact ureterocele and reflux may purely be one of semantics, defining the site of the ureteral opening within or at the bladder neck. Herein we present 4 cases of reflux in a variety of apparently intact ureteroceles. CASE REPORTS
Case i. K. E., a 4-year-old girl with cerebral palsy, had acute left pyelonephritis immediately after an excretory urogram Accepted for publication September 21, 1979.
(IVP) was done as part of the investigation of urinary incontinence. The IVP showed 2 kidneys with a normal right side but a ureterocele-like structure on the mildly dilated single left side (fig. 1, A). A catheter cine-cystogram defined a ureterocele with grade 3 reflux into a solitary collecting system on the left side. There was also grade 1 reflux to the contralateral right single system (fig. 1, B). At subsequent cystoscopy a normal ureteral orifice was situated normally on the right side. On the left side a ureterocele-like structure was seen extending over the left trigone, to and over the bladder neck, with a large, gaping orifice within the bladder. At operation the ureterocele (cecoureterocele), with a patulous intravesical orifice, was seen to collapse and distend with urine and undermined the mucosa of the trigone beyond the bladder neck into the urethra. The ureterocele (cecoureterocele) was excised, the ureter was reimplanted and the bladder neck was reconstructed. Case 2. C. C., an 8-year-old girl, was hospitalized for investigation ofrecurrent urinary tract infections during the preceding 18 months characterized by frequency and dysuria. She was otherwise developing normally and physical examination was non-contributory. An IVP showed a slightly enlarged left kidney with a complete duplex collecting system. The upper and lower moiety ureters were dilated moderately, with the former ending as a ureterocele. The right kidney and collecting system were normal (fig. 2, A). On a catheter cine-cystogram the ureterocele was hidden on initial bladder filling. Grade 2 reflux was seen in the left lower pole ureter during voiding. In the late voiding film grade 1 reflux into the ureterocele was evident (fig. 2, B), although the ureterocele was again seen as a filling defect in the bladder, empty of contrast material. At cystoscopy and again at open operation the ectopic orifice was not enlarged and was at the bladder neck. Subsequently, the lesion was deroofed and excised along with the ends of both ureters of the duplex system, and reimplantation was done. No communication between the ureters above the ureterocele was identified. Case 3. B. L., a 10-year-old boy, was seen with meatal stenosis associated with a urinary tract infection. The right kidney was not visualized on an IVP (fig. 3). A catheter cine-cystogram revealed reflux into a blind-ending ureter on bladder filling (fig. 4). At meatotomy and cystoscopy the left ureteral orifice was of normal configuration and was mildly displaced laterally on the trigone. In place of the right hemitrigone a ureterocele-like structure was noted, represented by a floppy area of mucosa with 2 openings situated just left of the midline. This structure appeared to collapse and distend rhythmically, although urine efflux was not seen. Retrograde pyelography of the right ureter was performed by inserting a ureteral catheter into one of the openings of the ureterocele-like structure. This outlined the ureterocele and ureter filling only to the pelvic brim. Before exploration was
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FIG. 1. Case 1. A, 5-minute film of IVP shows left collecting system with ureterocele indicated by arrow. B, catheter cine-cystogram reveals ureterocele with grade 2 reflux to left side and grade 1 reflux to right side.
FIG. 2. Case 2. A, 5-minute film of IVP shows normal right kidney and duplex left kidney with mild calicectasis. Suggestion of ureterocele is seen in bladder. B, late voiding catheter cine-cystogram reveals filling defect of ureterocele (dark broad arrow), complete reflux into lower pole (sharp arrowhead) and grade 1 reflux into upper pole ureter (broad arrow).
done renal angiography failed to show any renal artery on the right side. The ureterocele was excised, along with the atretic ureter, which was followed to its termination at the pelvic brim in a band of fibrous tissue. Histology of the tissue at the end of the ureter showed some glomeruli, compatible with marked renal dysplasia. Case 4. J. R., a 2½-year-old boy, was born with an imperforate anus, left inguinal hernia and mid penile hypospadias. Subsequently, a colostomy was performed, which was followed by an anal pull-through and takedown of the colostomy. At the time of repair of the left inguinal hernia a normal testis was noted in the scrotum with a normal epididymis, although atresia of the vas deferens was present from the level of the internal ring to the epididymis. There was no evidence of a neurogenic bladder. An IVP showed a solitary right kidney (fig. 5, A). A catheter cine-cystogram revealed a normal-appearing bladder that emptied completely but showed free reflux to a wide ureter ending below the pelvic brim (fig. 5, B). Cystoscopy through the hypospadiac opening revealed a normal right trigone and ure-
teral orifice. On the left side a ureterocele-like structure covered the entire left trigone and contained 2 openings. Retrograde injection of dye showed this to be consistent with the apparent atretic ureter described on the catheter cine-cystogram (fig. 5, C). The remainder of the bladder was unremarkable. At open operation the ureterocele-like structure with the 2 openings was confirmed and it was noted that both openings belonged to the same cavity. The ureterocele extended to but not over the bladder neck. Proximal dissection was done to almost the pelvic brim, where the ureter became atretic. No renal tissue was found. Approximately 2 cm. proximal to the bladder a coiled mass of tubing 2 cm. in diameter was found, 1 end proceeding inferiorly in the course expected of the vas deferens and another end crossing the ureter again, consistent with the vas deferens and ending atretically 1 cm. past the ureter. This was confirmed on microscopic examination to be the vas deferens. The structures noted were removed, the vas deferens inferiorly to the level of the bladder neck. The posterior wall of the bladder was reconstructed. Hypospadias repair is planned in the near future.
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LEONG, MIKHAEL AND SCHILLINGER DISCUSSION
We have described 3 different forms of ureteroceles with reflux. Case 1 was the classical cecoureterocele, which differs from the more common forms of ureterocele in that its lumen extends as a long tongue in the urethral submucosal layer and the large incompetent orifice is intravesical. Reflux classically occurs into this form of ureterocele. Case 2 was the ectopic variety, associated with a duplex system. Reflux was present before surgical manipulation and there was no apparent ragged, necrotic edge to the ureterocele to suggest spontaneous rupture as the cause of reflux. Case 3 resembles those described by Amar, 14 and Snyder and Johnston, 15 with the added feature of reflux with bladder distension. We believe that it is comparable to a simple orthotopic ureterocele. Although 2 orifices were noted both were smooth, symmetrical and with firm, rounded edges, not suggestive of spontaneous rupture. Case 4 is similar to case 3 in the placement of the ureterocele-like termination of the ureter, although there is no functioning renal tissue at all and the abnormal wolffian segment is remarkable.
3. IVP in case 3 shows hypertrophied left kidney and nonvisualization of right kidney. FIG.
FIG. 4. Case 3. A, cystogram shows reflux (arrow) into non-dilated ureter on non-functioning right side. B, x-ray indicates same structure on retrograde injection of ureterocele.
FIG. 5. Case 4. A, IVP shows non-function on left side. Arrow indicates filling of ureterocele and ureter above it. B, catheter cine-cystogram reveals reflux into ureterocele and stump of left ureter. C, retrograde injection of ureterocele demonstrates ureteral stump.
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That ureteroceles are primarily associated with obstruction of their supra-tending renal moiety is an undisputed fact. Furthermore, the presence of reflux does not necessarily mean that an element of obstruction is not present. Reflux will occur when the intravesical pressure exceeds the intraureterocele pressure if the intrinsic valve mechanism of the ureteral ending is destroyed. However, free reflux, as in cases 1 (cecoureterocele), 3 and 4, would indicate that no element of obstruction was present. Moreover, the lack of significant functioning renal tissue, likely in case 3 and certainly in case 4, would deny a "supraureterocele" hydrostatic force to create the ureterocele ending. One might argue that the kidney was destroyed or dysplastic because of the obstruction and reflux occurred subsequent to renal destruction because of altered intraureteral to intravesical pressures. However, this seems unlikely because of the ease of reflux and normal ureteral caliber in case 3. These cases support the thesis that ureteral meatal obstruction commonly seen with ureteroceles is the result and not the cause of the ureterocele. Thus, the classic concept of ureterocele formation is questioned and theories, such as those of Stephens or Tanagho, are supported. REFERENCES
1. Campbell, M. F.: Ureterocele: a study of 94 instances in 80 infants and children. Surg., Gynec. & Obst., 93: 705, 1951. 2. Gummess, G. H., Charnock, D. A., Riddell, H. I. and Stewart, C. M.: Ureteroceles in children. J. Urol., 74: 331, 1955. 3. Malek, R. S., Kelalis, P. P., Burke, E. C. and Stickler, G. B.: Simple and ectopic ureterocele in infancy and childhood. Surg., Gynec. & Obst., 134: 611, 1972. 4. Stephens, D.: Caecoureterocele and concepts on the embryology and aetiology of ureteroceles. Aust. New Zeal. J. Surg., 40: 239, 1971. 5. Williams, D. I.: Urology in Childhood, 2nd ed. New York: Springer Publishing Co., Inc., p. 148, 1974. 6. Tanagho, E. A.: Embryologic basis for lower ureteral anomalies: a hypothesis. Urology, 7: 451, 1976. 7. Williams, D. I. and Woodard, J. R.: Problems in the management of ectopic ureteroceles. J. Urol., 92: 635, 1964. 8. Thompson, G. J. and Kelalis, P. P.: Ureterocele: clinical appraisal of 176 cases. J. Urol., 91: 488, 1964.
9. Wines, R. D. and O'Flynn, J. D.: Transurethral treatment of ureteroceles: a report of 45 cases mostly treated by transurethral resection. Brit. J. Urol., 44: 207, 1972. 10. Hutch, J. A. and Chisholm, E. R.: Surgical repair of ureterocele. J. Urol., 96: 445, 1966. 11. Borden, T. A. and Martinez, A.: Vesicoureteral reflux associated with intact orthotopic ureterocele. Urology, 9: 182, 1977. 12. Williams, D. I.: Ureteroceles. In: Urology in Childhood, 2nd ed. New York: Springer Publishing Co., Inc., p. 151, 1974. 13. Kelalis, P. P. and King, L. R.: Clinical Pediatric Urology. Philadelphia: W. B. Saunders Co., p. 521, 1976. 14. Amar, A. D.: Simple ureterocele at the distal end of a blind-ending ureter. J. Urol., 106: 423, 1971. 15. Snyder, H. M. and Johnston, J. H.: Orthotopic ureteroceles in children. J. Urol., 119: 543, 1978. EDITORIAL COMMENT A paraureteral diverticulum may be mistaken cystoscopically as a simple ureterocele. Correct diagnosis can only be made when the bladder is slightly overdistended and the diverticulum is fully developed. I have seen this cystoscopic misdiagnosis several times. Reflux in the ectopic ureterocele does occur but rarely. If the mode of treatment is excision of the ureterocele the presence of reflux is merely of academic interest. Nonetheless, if a more conservative approach is used, such as heminephrectomy with subtotal ureterectomy, it is important to take into consideration the presence or absence of reflux into the ureterocele. With subsequent distension of the bladder the ureterocele remains collapsed and the obstructive features do not occur. However, if reflux into the ureterocele is taking place recurrence of the ureterocele and associated symptoms will reappear and a second operation at the bladder level is necessary. Panayotis P. Kelalis Mayo Clinic Rochester, Minnesota REPLY BY AUTHORS The authors readily acknowledge that ureteroceles may evert through the hiatus, mimicking periureteral diverticulum. This phenomenon was not observed on cystoscopy in any of these cases. We are not aware of the converse phenomenon.