0022-534 7/79/1215-0590$02.00/0 Vol. 121, May
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1979 by The Williams & Wilkins Co.
SURGICAL CORRECTION OF URETEROCELES W. HARDY HENDREN*
MICHAEL E. MITCHELLt
AND
From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
ABSTRACT
A personal experience in the management of 73 ureterocele cases during the last 17 years and points of technical importance in the correction of this sometimes complex problem are described. Small adult-type ureteroceles are best resected, reimplanting the ureter, since simple unroofing can cause reflux. The infant-type ureterocele with a duplex collecting system requires individualization according to the status of the patient. In ectopic ureterocele cases the bladder outlet often requires repair. The spectrum of management for this group of problems is shown by representative cases. involvement with a single system on one side and duplex system on the other. Vesicoureteral reflux was present in two-thirds of the cases, most frequently up the ipsilateral adjacent ureter in duplex systems (92 per cent) but also commonly up a contralateral ureter (table 3). An ectopic ureterocele was seen in 38 per cent of the cases (table 4). Ericsson defined ectopic ureterocele as one in which some portion reaches down into the bladder neck and posterior urethra, arising in a ureter with an ectopic lower end in the urethra. 3 Some large ureteroceles are confined entirely to the bladder, although the dome of the ureterocele may prolapse into the bladder neck during voiding if the ureterocele is not distended tensely. An ectopic ureterocele in this series refers to those in which the base of the ureterocele extended down through the bladder neck and urethra. This is of technical importance when a surgical repair is done.
Ureterocele is a descriptive term for obstruction of a ureteral orifice with cystic dilatation of the terminal ureter, which balloons into the bladder and sometimes extends down into the urethra. Some ureteroceles are small (adult-type), causing only mild obstruction and hydronephrosis; they generally present clinically in older children or adults and the associated ureter usually is single. However, much more common in childhood are large ureteroceles (infant-type), in which the renal collecting system on the side of the ureterocele usually is duplicated; the ureterocele is associated with the ureter that drains the upper pole of the duplex kidney. Large ureteroceles commonly cause bladder outlet obstruction. Rarely, they prolapse through the urethra and cause acute retention. In many cases there is not only obstruction but also massive vesicoureteral reflux up other ureters. The term ectopic ureterocele has been used by some authors to describe that variety in which the orifice of the ureterocele is located in the bladder neck or urethra. Herein is reported an experience with the management of 73 ureterocele cases (89 ureteroceles) seen from 1960 through 1977.
SURGICAL PROCEDURES
CLINICAL MATERIAL
In this series, as in all large series, Hi female patients outnumbered male patients 3.6 to 1 (fig. 1). Almost a quarter of the patients presented in the first year of life. As in most uropathies the worst cases usually presented during infancy and most (83 per cent) presented with urinary infection. The remainder had difficulty voiding, hematuria, a flank mass, incontinence or asymptomatic bacteriuria. Those without infection were usually male patients. Of the 73 cases there were 51 primary cases, while 22 cases were operated upon previously elsewhere and referred for an additional operation (table 1). About a third of the cases had a single collecting system with a ureterocele, which is much more common in male patients, and the remainder had a duplex collecting system, which is usually seen in female patients but seldomly observed in male patients. Bilateral involvement was seen in 22 per cent of the cases (table 2). In the pediatric age group bilateral single small ureteroceles are more likely to be seen in boys than in girls. On the other hand, the difficult problem of bilateral ureteroceles with bilateral duplex collecting systems is more often seen in girls than in boys. In no case was there bilateral
Accepted for publication July 28, 1978. Read at annual meeting of American Urological Association, Washington, D. C., May 21-25, 1978. * Requests for reprints: Massachusetts General Hospital, Boston, Massachusetts 02114. t Current address: Riley Children's Hospital, 1100 Michigan St., Indianapolis, Indiana 46207.
The operative repair in these cases should be individualized according to 1) age of patient, 2) whether the pathology is unilateral or bilateral, 3) whether the collecting system is single or duplex, 4) the degree of illness of the patient and 5) the experience of the surgeon. In general, we prefer a 1-stage total correction. However, that is not feasible in a sick little baby with severe bilateral involvement and acute infection. Incidentally, the diagnosis of a ureterocele should be suspected immediately in any female infant with severe hydronephrosis and infection, just as urethral valves are likely in a male infant with that clinical picture. The surgical treatment in the 51 primary cases is noted in table 5. In the 18 ureteroceles with a single collecting system 1 was small and without significant ureteral obstruction and, therefore, an operation was not done. This patient will be followed. In 5 cases the kidney was destroyed and, therefore, removed. In all other cases the ureterocele was excised and the ureter was reimplanted; in half of the cases it was tapered. Unroofing of a ureterocele endoscopically or by open operation should never be done since this results in reflux. Although this approach was advocated many years ago 1• 2 it should be remembered that the recommendation was made before the advent of ureteral reimplantation. To be sure there may be a place for minimal endoscopic incision of a small adult ureterocele in an older patient with a stone. 10 However, that approach is not applicable to cases seen in childhood or larger ureteroceles in adults. The 33 duplex collecting systems usually were managed by a 1-stage complete repair, including the lower and upper tracts. In 4 cases upper pole partial nephroureterectomy was
590
591
SURGICAL CORRECTION OF URETEROCELES
73 URETEROCELE CASES
8
LLJ
MALES - 16
~ FEMALES - 5 7
0 · 126712
I
months L_. years
2 3
4 5 6
7 8
9 10 11 12 13 14 15 16 17 18
AGE
t
Adult
FIG. 1. Age and sex of patients TABLE
1. 89 ureteroceles in 73 patients No. Cases
Primary Secondary (for reoperation)
Single system Duplex system
TABLE
51 22
MalePts.
FemalePts.
Totals(%)
11 5
14 43
25 (34) ~ (66)
16
57
73
done, the lower tract reconstruction being performed later. Although this has been described as a semi-definitive approach in some cases 11 we are inclined to proceed with the lower repair later in cases of this type because the defunctioned ureterocele can cause continued trouble (infection, reflux in adjacent segments, diverticulum formation and partial bladder outlet obstruction) and this fact has been noted by others 3• 4• 6 as well as ourselves. In 1 case with massive bilateral lower pole hydronephrosis and septic upper poles the lower tract reconstruction was done first but the upper repair was delayed until 1 week later. In 1 early case ileal loop urinary diversion was done but the patient subsequently underwent undiversion. 12 Management of a small ureterocele is resection and ureteral reimplantation (fig. 2). When a ureterocele is resected it is helpful to inject saline into the mucosa at its base. It is circumscribed and dissected out intact from its bed, preserving the mucosa of the adjacent trigone. Sometimes the ureteral hiatus is quite large. It should be closed, making a new one and reimplanting the ureter by a combined intravesical and extravesical technique. 13 If the ureter is grossly dilated lower tapering should be performed. There are various possible courses to manage a duplex system with a ureterocele (fig. 3). In a small infant with serious urinary infection it may be best to perform expeditiously upper pole partial nephrectomy, removing as much TABLE
No. Cases(%) Total Malepts.: Single Duplex Female pts.: Single Duplex Single systems Duplex systems
2. Bilateral ureteroceles (16 of73 patients) No. Cases Bilat. single Male pts., 5 Female pts., 4 Bilat. duplex Male pt., 1 Female pts., 6
9 7
TABLE
No. Cases (%)
A
2 4
2
20 4 (16)
24 (51)
5. Surgical treatment in 51 primary cases
Single: Excision and reimplant Taper ureter also N ephrectomy No operation Duplex: 1-stage repair Upper then lower Lower then upper Resection and diversion (later undiverted)
3. Refiux in ureterocele cases
Reflux present No reflux Location of reflux: lpsilat. Contralat. also Contralat. only
28 (38)
No. Cases
No admixture of types. TABLE
4. Ectopic ureteroceles (ureter or its orifice in urethra)
48 (66) 25 (34) 44 (92) 14 (29) 4
(8)
6 6
5
1 27 4
1 1
B
"Adult" ureterocele single ureter
Fm. 2. Management of small ureterocele with single collecting system. In case with larger ureterocele and great dilatation of ureter megaureter repair should be performed.
592
HENDREN AND MITCHELL
ureter as can be accomplished easily through the flank. We usually have managed the lower ureteral stump by aspirating the urine from it, rinsing it with neomycin solution and ligating it rather than leaving it open. This approach is especially appealing in a sick neonate with bilateral involvement. The timing for doing the second stage, that is the lower tract reconstruction, can depend on whether the infant is having trouble with urinary infection, how much reflux is present and the appearance of the upper tracts on a subsequent excretory urogram (IVP). In most cases in which a complete repair is done in 1 stage the lower end is done first. The ureterocele is removed, separating the 2 lower ureters and reimplanting the lower pole ureter. The large upper pole ureter to be removed is pushed upward into the posterior gutter to be dealt with later. After the lower tract reconstruction is completed the child is turned for exploration of the kidney through a separate flank incision. Although in many patients with complex urinary tract reconstructions we routinely use an extensive transabdominal exposure (from xyphoid to pubis) to be able to deal with all levels of the urinary tract in a single operative field, 14 in new ureterocele cases there is no disadvantage to using separate incisions for the lower and upper reconstruction. This approach avoids entering the peritoneal cavity, which gives a
smoother postoperative course and avoids causing intestinal adhesions that can result in intestinal obstruction. In the case of an ectopic ureterocele the base of the ureterocele extends down through the bladder neck and into the urethra (fig. 4). Therefore, there is an extensive defect in the muscle of the trigone and bladder neck and it is important to repair this defect carefully. If a cleft remains in the muscle it can result in stress urinary incontinence. Furthermore, this can result in a diverticulum during voiding and the lower rim of such a diverticulum can act in an obstructive manner, causing incomplete emptying of the bladder. Another point of importance is removal of the distal rim of the ureterocele, since it can act like a urethral valve if not removed. Careless dissection of an ectopic ureterocele can result in the distressing complication of vesicovaginal fistula. Therefore, in the removal of the distal extension of an ectopic ureterocele the dissection must be done close to the wall of the ureterocele to prevent this complication. In a patient who has had a ureterocele operation and bladder emptying is incomplete a careful search should be done for a residual obstructive rim of ureterocele and/or a diverticulum-like defect in the distal trigone or bladder neck. In the majority of the ureterocele reconstructions it is necessary to separate 2 ureters (fig. 5). Separation can be done
Upper pole nephrectomy
Portiol
s ,,,
,l/
Upper pole oflendyspfostic
c/,/ / I
Upper pole removed
\ Collapsed ureterocele
Occasionally pyeJopyeloslomJ
USUALLY complete repair in one stage through 2 incisions
'\t; 11
Infant 1 ureterocele Duplex system
Excise ureterocele and its ureter. Repair muscle defect. Reimplont 1-, pole ureter. Reimplant OjlpOSite ureter ~r.n
With temporary
exterio~izotion of upper pole ureter
Lower repair first
Fm. 3. Methods to manage case with duplex collecting system Both ureters mobilized. Excise larger, leoving all odventitio and blood supply for smaller ureter.
Ectopic ureterocele
Extensive defect in muscle of trigone and bladder neck ofter removing ureterocele and mobilizing ureters.
G Lower pole ureter reimplonted
Defect repaired, a. muscle closure b. mucosa - leave oo obstructive rim of ureterocele at lower end
Fm. 4. Technical aspects of repair in ectopic ureterocele
593
SURGICAL CORRECTION OF URETEROCELES
.i SEPARATING 2
URETERS,
PRESERVING BLOOD SUPPLY TO BETTER ONE
Distend ureters
with saline; dissect close 'ro wall of ureter to be removed; leave all periureterol tissue with smaller ureter
Transect
jusi above
"common ,;,,all" Usuall1• odherent for 1- 2 cm
C Open adventitio; ovoid
D
cutting vessels
Pass ureter upward
£
to next opening in periureteraJ
adventitia
Dissecting ureter
out of its sheath
Reimplont bet-rer ureter
on ureter
Trigone defect closed
Fm. 5. Technique for separating 2 ureters while preserving blood supply of lower pole ureter to be spared and reimplanted
without losing the blood supply of the better ureter to be preserved but requires great care. First the ureterocele is circumscribed, preserving as much mucosa as possible at its base to later resurface the trigone. The 2 ureters are then mobilized intravesically and extravesically. Usually they are too adherent distally to be separated one from the other. Therefore, the distal segment of both ureters is resected (fig. 5, B). A catheter is tied into both ureters and the ureters are distended with saline. This distension facilitates dissection, which is done close to the wall of the ureter to be removed, leaving all of the periureteral adventitia with the smaller ureter. The common periureteral adventitia should not be incised upward, since that will destroy collateral vessels. The ureter can be passed upward through the adventitia to a higher stair-step incision, continuing its mobilization upward to a rather high level (fig. 5, C and D). The ureter is ligated, leaving a segment of rubber catheter in it to facilitate its easy palpation later in the flank. The lower reconstruction is vu.u, 1,,t,sc·u with the well vascularized lower ureter. Great care be exercised to maintain accurate identification of the 2 U;'.'ete:rs, (Vole have seen a cs.st in vvhieh the vvrong
TABLE
6. The upper pole in duplex systems-51 primary cases No. Cases Removed Preserved by pyelopyelostomy (2 later removed) Double reimplant
19
Total
33
8 6
lower ureteral segment was removed.) The flank 1s then explored. In 19 of the 33 primary duplex systems the upper pole was removed (table 6). In 8 cases the upper pole was thought worthy of salvage but it was removed later in 2 cases. In 6 cases a double ureteral reimplantation was performed. In each case there was a small ureterocele and little dilatation of the involved upper pole ureter. It is tempting to a dilated upper pole ureter and ~n,c-,n~= a double -·····,,.-··" but we believe that a large upper removed. An 1S
594
HENDREN AND MITCHELL
managed by either removal or pyelopyelostomy. We tapered the upper pole ureter minimally and performed a double reimplant in only 1 case. In 1 case in this series the upper pole ureter was the better one and, therefore, the lower pole ureter was removed. This upper pole ureter was tapered and reimplanted and the lower pole was joined to it at the level of the kidney. The indications for an operation in the 22 secondary cases are noted in table 7. In 4 cases endoscopic incision of the ureterocele had been performed previously, resulting in massive reflux. The reflux was corrected by ureteral reimplantation. In 12 cases ureteral reimplantation had been performed previously: reflux persisted in 10 cases and obstruction was present in 2. There was contralateral reflux in 2. The cases were all corrected by reoperation. Four cases were referred after previous urinary diversions: all were undiverted. In 2 cases the primary problem was a persisting ureterocele remnant, which acted like an obstructive valve. To demonstrate such a valve a maleable probe is bent into the shape of a shepherd's crook, passed into the bladder next to the panendoscope and pulled gently along the floor of the distal trigone and urethra. With use of the panendoscope the valve-like rim can be pulled out through the urethral meatus and snipped away under direct vision. Alternatively, it can be incised with a cutting electrode.
6). The left ureter with reflux was tapered and reimplanted, a tiny right ureterocele was removed and reimplantation was done. The patient has done well subsequently. Case 2. A 10-year-old boy with recurrent pyuria was referred to us for surgical correction of right hydronephrosis. An IVP showed a large filling defect in the bladder consistent with a ureterocele (fig. 7, A). A cystogram showed no reflux
RESULTS
Of the 51 primary cases there was no postoperative problem in 42 (table 8). Late nephrectomy was done in 5 cases: 2 involved upper poles that had been spared originally and 3 involved lower poles that on long-term observation were atrophic segments, which contributed little function and were a source of infection. In 4 cases reflux necessitated repeat reimplantation despite previous reimplantation. A higher failure rate of ureteral reimplantation is to be expected in these cases, compared to children without ureteroceles, because the trigone and often the lower ureters are abnormal. In 1 case reflux persisted despite 3 cm. ureteral tunnels after 2 reimplantations. This case was managed by a third reimplantation of the better ureter to get a super-long tunnel and transureteroureterostomy of the other ureter. 14 An obstructive distal ureterocele remnant in 1 case was managed by endoscopic resection. There has been no death in this series. One of the undiversion patients who presented with marginal renal function underwent renal transplantation 6 years after a reconstructive operation.
Fm. 6. Case 1. A, preoperative !VP reveals small ureterocele on right side and moderate-sized ureterocele on left side, outlined by dye in bladder. B, later preoperative !VP shows left ureterocele now filled with dye. C, reflux on left side resulted from unroofing ureterocele, which is not satisfactory definitive procedure but merely exchanges reflux for obstruction. Secondary operation corrected reflux.
CASE REPORTS
Case 1 . A 23-year-old woman was referred to us after endoscopic unroofing of a left ureterocele with a single collecting system, which resulted in reflux and pyelonephritis (fig. 7. Secondary ureterocele in 22 cases No. Cases Unroofed resulting in reflux 4 Reimplant failures: Reflux 10 Obstruction 2 Contralat. reflux 2 Diversion (suprapubic tube, ileal loop, 4 nephrostomy, ureterostomies) Other (valve effect, reflux) 2 TABLE
TABLE
8. Results in 51 primary ureterocele cases No. Cases
No problem Problems: Late nephrectomy Reflux needing re-operation Obstructive ureterocele remnant (transurethral resection)
42 5 4
1
Fm. 7. Case 2. A, preoperative !VP reveals filling defect in bladder (arrow) and right hydronephrosis. B, percutaneous anterograde pyelogram outlines dilated right collecting system. C, anterograde perfusion study 5 months postoperatively reveals normal lower ureter. D, !VP 5 months postoperatively reveals less hydronephrosis. Perfusion pressures were normal. Cystogram showed no reflux.
SURGICAL CORRECTION OF URETEROCELES
and the urethra was normal. Right anterograde pyelography revealed the dilated system and high perfusion pressures (fig. 7, B). A large ureterocele was excised and the distal ureter was tapered and reimplanted. The bladder was trabeculated, indicating that the ureterocele had caused outlet obstruction. Postoperatively, the patient has been well. Anterograde perfusion showed normal pressures and a normal lower ureter (fig. 7, C). An IVP demonstrated less hydronephrosis and cystography showed no reflux (fig. 7, D). Case 3. A 7-year-old boy was referred to us with intermittent urinary retention and right flank pain. An IVP showed bilateral single collecting systems, a moderate-sized ureterocele on the right side and a small one on the left side (fig. 8, }.i.). Cystography showed the unusual dynamics of the larger ureterocele; on some films it prolapsed like a bladder diverticulum, while on other views it projected into the bladder and downward into the prostatic uretl1ra and blocked the stream (fig. 8, B and C). Whether a ureterocele projects outward or inward into the bladder depends on the in the ureterocele .,,..m,,m·nr1 to the pressure in the at any it is common to see a initially but it flattens as the bladder pressure is increased finally, everts like a diverticulum when intravesical pressure is high. Both ureteroceles were resected, a simple reimplantation was done on the
left side and the ureter was tapered and reimplanted on the right side. Interestingly, there were small but definite type I urethral valves, which were resected endoscopically. Postoperative films were satisfactory, with improved upper tracts and no reflux. The patient has had no further difficulty. Case 4. A 3-month-old female infant was referred to us with urinary infection. An IVP showed a normal right kidney. On the left side the findings were typical for a duplex collecting system with a large ureterocele (fig. 9, A). A cystogram revealed reflux. A 1-stage complete repair was done, the ureterocele and its upper pole ureter being removed. The defect in the trigone and bladder neck was repaired, the left lower pole ureter being reimplanted as was the contralateral single ureter by simple advancement. The left kidney was explored through a flank incision. The upper pole appeared to have reasonable ,-,~.,vi,v,.,, and so it was spared, draining it pyelopyelostomy into the lower pole (fig. 9, B). During the next 7 years there were several episodes of urinary infection, although cystogTaphy showed no reflux. The upper was removed when the child was 7112 years old because it was judged likely to be the source of the intermittent infection. The specimen showed chronic focal pyelonephritis. In retrospect it would have been best removed at the original procedure. Case 5. A 10-year-old girl was referred to us with urinary
FIG. 8. Case 3. A, preoperative IVP reveals large right ureterocele and tiny left ureterocele (arrows). B and C, spot films during cystogram show protrusion outward of right ureterocele, giving appearance of bladder diverticulum. On some voiding films ureterocele prolapsed into prostatic urethra, causing retention.
Fra. 9, Case 4. by hydronephrotic
IVP reveals large ureterocele (arrow) and displacement of lower pole of left kidney infe:ciorly and laterally uppe:t poie associated 1Nith ureterocele. B I\TP 2 years late:.r.
m·,,,,c,py,it_iivP
1
596
HENDREN AND MITCHELL
infection. An IVP showed bilateral filling defects in the was completed when the child was 21 months old. The patient bladder and bilaterally scarred lower poles (fig. 10, A). A has had no further difficulty (fig. 12, C andD). cystogram showed ureteroceles and bilateral lower pole reflux. DISCUSSION Intraoperative retrograde pyelography showed dysplastic upper poles (fig. 10, B). A lengthy (10-hour) complete reconstrucUnroofing ureteroceles, as often done 30 years ago, has no tion was done. Both ureteroceles and upper pole ureters were place in contemporary pediatric urologic surgery. Small ureresected through a transverse lower abdominal incision and teroceles are best resected together with reimplantation of the the lower pole ureters were reimplanted. Each upper pole was ureters to prevent reflux. When the ureter is dilated tapering resected through separate flank incisions. The patient had an should be performed also. Large ureteroceles with duplex uneventful postoperative convalescence. During the 6-year collecting systems can be managed in a variety of ways, followup she has had no further infection. Retrograde pyelog- depending on the circumstances of the individual case. A raphy (the patient being allergic to IVP dye) showed satisfac- small infant, such as in case 6, may be best managed by tory lower pole ureters and a cystogram showed no reflux (fig. simple upper pole partial nephroureterectomy and later elec10, C). tive lower tract reconstruction when the child is older. This is Case 6. A 6-week-old female newborn was referred to us true especially in bilateral cases. A more robust infant may with urinary infection (fig. 11). An IVP showed satisfactory well be managed by a 1-stage complete reconstruction, espefunction of both lower poles but no visualization of the upper cially if pathology is unilateral, as shown in case 4. An older poles (fig. 12, A). A cystogram showed reflux into the upper patient who can tolerate many hours of anesthesia can be pole on the right side and the lower pole on the left side. managed, on the other hand, by complete reconstruction in a Cystoscopy showed an ectopic ureterocele on the right side, single long operation, as illustrated by case 5. There is an which had unroofed itself spontaneously (probably by intra- occasional case in which still another approach may be feasiuterine prolapse with infarction), accounting for reflux to the ble, that is repairing the lower tract and temporarily exteriorupper pole. On the left side the upper pole orifice was ectopic izing the upper pole ureters to deal with them somewhat later; in the urethral meatus (fig. 12, B). By retrograde study all 4 we did this on only 1 occasion. Careful attention should be paid to the site of the ureterocollecting systems were visualized. Bilateral upper pole nephroureterectomy was performed. The patient was then well cele. If the bladder wall is not repaired after the ureterocele is clinically on suppressive antimicrobial therapy except for 1 resected, especially an ectopic one, this can result in stress urinary infection. Repeat cystography showed persisting re- incontinence and/or a partially obstructive diverticulum. We flux into the stump of the right upper pole ureter and into the have seen examples of both of these complications. Furtherlower pole ureter on the left side. Lower tract reconstruction more, care should be taken to remove the distal lip of the
Fm. 10. Case 5. A, preoperative IVP shows confluent filling defects in bladder (arrows), visualization of both lower poles but not upper poles. B, intraoperative retrograde study filling all 4 segments. C, retrograde pyelogram 1 year later. Cystogram showed no reflux. OP. f
PRE-OP
Age 6 wks.
OP. 2
Age 2
f
mos.
Upper pale nephraureterectamies
I
'
rlfrt--+-- 2 normotopic
orifices with reflux
Veil of mucosa over orifice
"-. Orifice distal to e1d. sphincter
of stumpi ...,..--1 cm remained
Fm. 11. Case 6. Anatomy before and after staged reconstructive operations
597
SURGICAL CORRECTION OF URETEROCELES REFERENCES
1. Gross, R. E. and Clatworthy, H. W., Jr.: Ureterocele in infancy and childhood. Pediatrics, 5: 68, 1950. 2. Campbell, M. F.: Ureterocele. A study of 94 instances in 80 infants and children. Surg., Gynec. & Obst., 93: 705, 1951. 3. Ericsson, N. 0.: Ectopic ureterocele in infants and children. Acta Chir. Scand., suppl. 197, 1954. 4. Williams, D. I. and Woodard, J. R.: Problems in the management of ectopic ureteroceles. J. Urol., 92: 635, 1964. 5. Mogg, R. A.: Some observations on the ectopic ureter and ureterocele. J. Urol., 97: 1003, 1967. 6. Johnston, J. H. and Johnson, L. M.: Experiences with ectopic ureteroceles. Brit. J. Urol. 41: 61, 1969. 7. Hendren, W. H. and G. J.: Surgical correction of ureteroceles in childhood. J. Ped. Surg., 6: 235, 1971. 8. Stephens, D.: Caecoureterocele and concepts on the embryology and aetiology of meteroceles. Aust. New Zeal. J. Surg., 40: 1971. 9. R. S,, Kelalis, P. P., Burke, E. C. and Stickler, G. B.: Simple and ectopic ureterocele in infancy and childhood. Gynec. & Obst., 134: 511, 1972. 10. P. and Ylonen, N.: Vesico-ureteral xeflux follo,ving transurethral slitting of the ureteral orifice. Ann. Chir. Gynaec. Fenn., 64: 220, 1975. 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. Hendren, W, H.: Urinary tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974. 13. Hendren, W. H.: Reoperntion for the failed ureteral reimplantation. J. Urol., 111: 403, 1974. 14. Hendren, W. H.: Reconstructive surgery of the urinary tract in children. In: Current Problems in Surgery. Edited by M. M. Ravitch and F. M. Steichen. Chicago: Year Book Medical Publisher, Inc., vol. 14, 1977. EDITORIAL COMMENT Fm. 12. Case 6. A, preoperative IVP reveals satisfactory function of both lower poles but absence of visualization of upper poles. Note marked displacement of right lower pole, axis of which is tipped outward by hydronephrotic upper pole system. This is seen also on left side but not so clearly. B, preoperative retrograde study shows right upper pole system to be removed and both systems on left side. C, postoperative IVP reveals satisfactory upper tract. Cystogram showed persisting reflux. Lower tract reconstruction performed later. D, IVP 3 months after lower tract reconstruction.
ureterocele since it can act as a valve. Separation of the 2 ureters, carefully preserving all of the periureteral adventitia for the one to be spared, is possible in these ureterocele reconstructions. If both ureters are of almost normal size a double reimplantation can be performed if the upper pole has good parenchyma. If one ureter is much larger than the other it is best removed rather than tapered. A reconstructive operation of the urinary tract in complex ureterocele cases can be as demanding as any of the technical problems in reconstructive pediatric urologic surgery.
This review of Hendren's experience with ureteroceles covers nearly all problems encountered in reconstruction of these complex cases. To handle them properly they must be recognized, which requires good radiograms, a sensitivity to the possibility of nonfunctioning duplication and bladder base filling defects, careful endoscopy and a high index of suspicion. The distinction between simple and ectopic ureteroceles is important because bladder and urethral repair may be much more complex in the latter type. The alternatives in surgical approach are well described and displayed. As advice to others, I would strongly favor performing heminephrectomy first in the 2-incision approach since it easily allows one to stage the procedure if conditions require it. The 2-stage procedure in which the bladder is reconstructed first and an upper pole ureterostomy is left seems to invite unnecessary complications between stages. I strongly agree that the transperitoneal approach should be avoided when the new case can be approached easily from 2 intraperitoneal incisions. Robert D. Jeffs Brady Urological Institute The Johns Hopkins Hospital Baltimore, Maryland
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1979 by The Williams & Wilkins Co.
SURGICAL CORRECTION OF URETEROCELES W. HARDY HENDREN*
MICHAEL E. MITCHELLt
AND
From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
ABSTRACT
A personal experience in the management of 73 ureterocele cases during the last 17 years and points of technical importance in the correction of this sometimes complex problem are described. Small adult-type ureteroceles are best resected, reimplanting the ureter, since simple unroofing can cause reflux. The infant-type ureterocele with a duplex collecting system requires individualization according to the status of the patient. In ectopic ureterocele cases the bladder outlet often requires repair. The spectrum of management for this group of problems is shown by representative cases. involvement with a single system on one side and duplex system on the other. Vesicoureteral reflux was present in two-thirds of the cases, most frequently up the ipsilateral adjacent ureter in duplex systems (92 per cent) but also commonly up a contralateral ureter (table 3). An ectopic ureterocele was seen in 38 per cent of the cases (table 4). Ericsson defined ectopic ureterocele as one in which some portion reaches down into the bladder neck and posterior urethra, arising in a ureter with an ectopic lower end in the urethra. 3 Some large ureteroceles are confined entirely to the bladder, although the dome of the ureterocele may prolapse into the bladder neck during voiding if the ureterocele is not distended tensely. An ectopic ureterocele in this series refers to those in which the base of the ureterocele extended down through the bladder neck and urethra. This is of technical importance when a surgical repair is done.
Ureterocele is a descriptive term for obstruction of a ureteral orifice with cystic dilatation of the terminal ureter, which balloons into the bladder and sometimes extends down into the urethra. Some ureteroceles are small (adult-type), causing only mild obstruction and hydronephrosis; they generally present clinically in older children or adults and the associated ureter usually is single. However, much more common in childhood are large ureteroceles (infant-type), in which the renal collecting system on the side of the ureterocele usually is duplicated; the ureterocele is associated with the ureter that drains the upper pole of the duplex kidney. Large ureteroceles commonly cause bladder outlet obstruction. Rarely, they prolapse through the urethra and cause acute retention. In many cases there is not only obstruction but also massive vesicoureteral reflux up other ureters. The term ectopic ureterocele has been used by some authors to describe that variety in which the orifice of the ureterocele is located in the bladder neck or urethra. Herein is reported an experience with the management of 73 ureterocele cases (89 ureteroceles) seen from 1960 through 1977.
SURGICAL PROCEDURES
CLINICAL MATERIAL
In this series, as in all large series, Hi female patients outnumbered male patients 3.6 to 1 (fig. 1). Almost a quarter of the patients presented in the first year of life. As in most uropathies the worst cases usually presented during infancy and most (83 per cent) presented with urinary infection. The remainder had difficulty voiding, hematuria, a flank mass, incontinence or asymptomatic bacteriuria. Those without infection were usually male patients. Of the 73 cases there were 51 primary cases, while 22 cases were operated upon previously elsewhere and referred for an additional operation (table 1). About a third of the cases had a single collecting system with a ureterocele, which is much more common in male patients, and the remainder had a duplex collecting system, which is usually seen in female patients but seldomly observed in male patients. Bilateral involvement was seen in 22 per cent of the cases (table 2). In the pediatric age group bilateral single small ureteroceles are more likely to be seen in boys than in girls. On the other hand, the difficult problem of bilateral ureteroceles with bilateral duplex collecting systems is more often seen in girls than in boys. In no case was there bilateral
Accepted for publication July 28, 1978. Read at annual meeting of American Urological Association, Washington, D. C., May 21-25, 1978. * Requests for reprints: Massachusetts General Hospital, Boston, Massachusetts 02114. t Current address: Riley Children's Hospital, 1100 Michigan St., Indianapolis, Indiana 46207.
The operative repair in these cases should be individualized according to 1) age of patient, 2) whether the pathology is unilateral or bilateral, 3) whether the collecting system is single or duplex, 4) the degree of illness of the patient and 5) the experience of the surgeon. In general, we prefer a 1-stage total correction. However, that is not feasible in a sick little baby with severe bilateral involvement and acute infection. Incidentally, the diagnosis of a ureterocele should be suspected immediately in any female infant with severe hydronephrosis and infection, just as urethral valves are likely in a male infant with that clinical picture. The surgical treatment in the 51 primary cases is noted in table 5. In the 18 ureteroceles with a single collecting system 1 was small and without significant ureteral obstruction and, therefore, an operation was not done. This patient will be followed. In 5 cases the kidney was destroyed and, therefore, removed. In all other cases the ureterocele was excised and the ureter was reimplanted; in half of the cases it was tapered. Unroofing of a ureterocele endoscopically or by open operation should never be done since this results in reflux. Although this approach was advocated many years ago 1• 2 it should be remembered that the recommendation was made before the advent of ureteral reimplantation. To be sure there may be a place for minimal endoscopic incision of a small adult ureterocele in an older patient with a stone. 10 However, that approach is not applicable to cases seen in childhood or larger ureteroceles in adults. The 33 duplex collecting systems usually were managed by a 1-stage complete repair, including the lower and upper tracts. In 4 cases upper pole partial nephroureterectomy was
590
591
SURGICAL CORRECTION OF URETEROCELES
73 URETEROCELE CASES
8
LLJ
MALES - 16
~ FEMALES - 5 7
0 · 126712
I
months L_. years
2 3
4 5 6
7 8
9 10 11 12 13 14 15 16 17 18
AGE
t
Adult
FIG. 1. Age and sex of patients TABLE
1. 89 ureteroceles in 73 patients No. Cases
Primary Secondary (for reoperation)
Single system Duplex system
TABLE
51 22
MalePts.
FemalePts.
Totals(%)
11 5
14 43
25 (34) ~ (66)
16
57
73
done, the lower tract reconstruction being performed later. Although this has been described as a semi-definitive approach in some cases 11 we are inclined to proceed with the lower repair later in cases of this type because the defunctioned ureterocele can cause continued trouble (infection, reflux in adjacent segments, diverticulum formation and partial bladder outlet obstruction) and this fact has been noted by others 3• 4• 6 as well as ourselves. In 1 case with massive bilateral lower pole hydronephrosis and septic upper poles the lower tract reconstruction was done first but the upper repair was delayed until 1 week later. In 1 early case ileal loop urinary diversion was done but the patient subsequently underwent undiversion. 12 Management of a small ureterocele is resection and ureteral reimplantation (fig. 2). When a ureterocele is resected it is helpful to inject saline into the mucosa at its base. It is circumscribed and dissected out intact from its bed, preserving the mucosa of the adjacent trigone. Sometimes the ureteral hiatus is quite large. It should be closed, making a new one and reimplanting the ureter by a combined intravesical and extravesical technique. 13 If the ureter is grossly dilated lower tapering should be performed. There are various possible courses to manage a duplex system with a ureterocele (fig. 3). In a small infant with serious urinary infection it may be best to perform expeditiously upper pole partial nephrectomy, removing as much TABLE
No. Cases(%) Total Malepts.: Single Duplex Female pts.: Single Duplex Single systems Duplex systems
2. Bilateral ureteroceles (16 of73 patients) No. Cases Bilat. single Male pts., 5 Female pts., 4 Bilat. duplex Male pt., 1 Female pts., 6
9 7
TABLE
No. Cases (%)
A
2 4
2
20 4 (16)
24 (51)
5. Surgical treatment in 51 primary cases
Single: Excision and reimplant Taper ureter also N ephrectomy No operation Duplex: 1-stage repair Upper then lower Lower then upper Resection and diversion (later undiverted)
3. Refiux in ureterocele cases
Reflux present No reflux Location of reflux: lpsilat. Contralat. also Contralat. only
28 (38)
No. Cases
No admixture of types. TABLE
4. Ectopic ureteroceles (ureter or its orifice in urethra)
48 (66) 25 (34) 44 (92) 14 (29) 4
(8)
6 6
5
1 27 4
1 1
B
"Adult" ureterocele single ureter
Fm. 2. Management of small ureterocele with single collecting system. In case with larger ureterocele and great dilatation of ureter megaureter repair should be performed.
592
HENDREN AND MITCHELL
ureter as can be accomplished easily through the flank. We usually have managed the lower ureteral stump by aspirating the urine from it, rinsing it with neomycin solution and ligating it rather than leaving it open. This approach is especially appealing in a sick neonate with bilateral involvement. The timing for doing the second stage, that is the lower tract reconstruction, can depend on whether the infant is having trouble with urinary infection, how much reflux is present and the appearance of the upper tracts on a subsequent excretory urogram (IVP). In most cases in which a complete repair is done in 1 stage the lower end is done first. The ureterocele is removed, separating the 2 lower ureters and reimplanting the lower pole ureter. The large upper pole ureter to be removed is pushed upward into the posterior gutter to be dealt with later. After the lower tract reconstruction is completed the child is turned for exploration of the kidney through a separate flank incision. Although in many patients with complex urinary tract reconstructions we routinely use an extensive transabdominal exposure (from xyphoid to pubis) to be able to deal with all levels of the urinary tract in a single operative field, 14 in new ureterocele cases there is no disadvantage to using separate incisions for the lower and upper reconstruction. This approach avoids entering the peritoneal cavity, which gives a
smoother postoperative course and avoids causing intestinal adhesions that can result in intestinal obstruction. In the case of an ectopic ureterocele the base of the ureterocele extends down through the bladder neck and into the urethra (fig. 4). Therefore, there is an extensive defect in the muscle of the trigone and bladder neck and it is important to repair this defect carefully. If a cleft remains in the muscle it can result in stress urinary incontinence. Furthermore, this can result in a diverticulum during voiding and the lower rim of such a diverticulum can act in an obstructive manner, causing incomplete emptying of the bladder. Another point of importance is removal of the distal rim of the ureterocele, since it can act like a urethral valve if not removed. Careless dissection of an ectopic ureterocele can result in the distressing complication of vesicovaginal fistula. Therefore, in the removal of the distal extension of an ectopic ureterocele the dissection must be done close to the wall of the ureterocele to prevent this complication. In a patient who has had a ureterocele operation and bladder emptying is incomplete a careful search should be done for a residual obstructive rim of ureterocele and/or a diverticulum-like defect in the distal trigone or bladder neck. In the majority of the ureterocele reconstructions it is necessary to separate 2 ureters (fig. 5). Separation can be done
Upper pole nephrectomy
Portiol
s ,,,
,l/
Upper pole oflendyspfostic
c/,/ / I
Upper pole removed
\ Collapsed ureterocele
Occasionally pyeJopyeloslomJ
USUALLY complete repair in one stage through 2 incisions
'\t; 11
Infant 1 ureterocele Duplex system
Excise ureterocele and its ureter. Repair muscle defect. Reimplont 1-, pole ureter. Reimplant OjlpOSite ureter ~r.n
With temporary
exterio~izotion of upper pole ureter
Lower repair first
Fm. 3. Methods to manage case with duplex collecting system Both ureters mobilized. Excise larger, leoving all odventitio and blood supply for smaller ureter.
Ectopic ureterocele
Extensive defect in muscle of trigone and bladder neck ofter removing ureterocele and mobilizing ureters.
G Lower pole ureter reimplonted
Defect repaired, a. muscle closure b. mucosa - leave oo obstructive rim of ureterocele at lower end
Fm. 4. Technical aspects of repair in ectopic ureterocele
593
SURGICAL CORRECTION OF URETEROCELES
.i SEPARATING 2
URETERS,
PRESERVING BLOOD SUPPLY TO BETTER ONE
Distend ureters
with saline; dissect close 'ro wall of ureter to be removed; leave all periureterol tissue with smaller ureter
Transect
jusi above
"common ,;,,all" Usuall1• odherent for 1- 2 cm
C Open adventitio; ovoid
D
cutting vessels
Pass ureter upward
£
to next opening in periureteraJ
adventitia
Dissecting ureter
out of its sheath
Reimplont bet-rer ureter
on ureter
Trigone defect closed
Fm. 5. Technique for separating 2 ureters while preserving blood supply of lower pole ureter to be spared and reimplanted
without losing the blood supply of the better ureter to be preserved but requires great care. First the ureterocele is circumscribed, preserving as much mucosa as possible at its base to later resurface the trigone. The 2 ureters are then mobilized intravesically and extravesically. Usually they are too adherent distally to be separated one from the other. Therefore, the distal segment of both ureters is resected (fig. 5, B). A catheter is tied into both ureters and the ureters are distended with saline. This distension facilitates dissection, which is done close to the wall of the ureter to be removed, leaving all of the periureteral adventitia with the smaller ureter. The common periureteral adventitia should not be incised upward, since that will destroy collateral vessels. The ureter can be passed upward through the adventitia to a higher stair-step incision, continuing its mobilization upward to a rather high level (fig. 5, C and D). The ureter is ligated, leaving a segment of rubber catheter in it to facilitate its easy palpation later in the flank. The lower reconstruction is vu.u, 1,,t,sc·u with the well vascularized lower ureter. Great care be exercised to maintain accurate identification of the 2 U;'.'ete:rs, (Vole have seen a cs.st in vvhieh the vvrong
TABLE
6. The upper pole in duplex systems-51 primary cases No. Cases Removed Preserved by pyelopyelostomy (2 later removed) Double reimplant
19
Total
33
8 6
lower ureteral segment was removed.) The flank 1s then explored. In 19 of the 33 primary duplex systems the upper pole was removed (table 6). In 8 cases the upper pole was thought worthy of salvage but it was removed later in 2 cases. In 6 cases a double ureteral reimplantation was performed. In each case there was a small ureterocele and little dilatation of the involved upper pole ureter. It is tempting to a dilated upper pole ureter and ~n,c-,n~= a double -·····,,.-··" but we believe that a large upper removed. An 1S
594
HENDREN AND MITCHELL
managed by either removal or pyelopyelostomy. We tapered the upper pole ureter minimally and performed a double reimplant in only 1 case. In 1 case in this series the upper pole ureter was the better one and, therefore, the lower pole ureter was removed. This upper pole ureter was tapered and reimplanted and the lower pole was joined to it at the level of the kidney. The indications for an operation in the 22 secondary cases are noted in table 7. In 4 cases endoscopic incision of the ureterocele had been performed previously, resulting in massive reflux. The reflux was corrected by ureteral reimplantation. In 12 cases ureteral reimplantation had been performed previously: reflux persisted in 10 cases and obstruction was present in 2. There was contralateral reflux in 2. The cases were all corrected by reoperation. Four cases were referred after previous urinary diversions: all were undiverted. In 2 cases the primary problem was a persisting ureterocele remnant, which acted like an obstructive valve. To demonstrate such a valve a maleable probe is bent into the shape of a shepherd's crook, passed into the bladder next to the panendoscope and pulled gently along the floor of the distal trigone and urethra. With use of the panendoscope the valve-like rim can be pulled out through the urethral meatus and snipped away under direct vision. Alternatively, it can be incised with a cutting electrode.
6). The left ureter with reflux was tapered and reimplanted, a tiny right ureterocele was removed and reimplantation was done. The patient has done well subsequently. Case 2. A 10-year-old boy with recurrent pyuria was referred to us for surgical correction of right hydronephrosis. An IVP showed a large filling defect in the bladder consistent with a ureterocele (fig. 7, A). A cystogram showed no reflux
RESULTS
Of the 51 primary cases there was no postoperative problem in 42 (table 8). Late nephrectomy was done in 5 cases: 2 involved upper poles that had been spared originally and 3 involved lower poles that on long-term observation were atrophic segments, which contributed little function and were a source of infection. In 4 cases reflux necessitated repeat reimplantation despite previous reimplantation. A higher failure rate of ureteral reimplantation is to be expected in these cases, compared to children without ureteroceles, because the trigone and often the lower ureters are abnormal. In 1 case reflux persisted despite 3 cm. ureteral tunnels after 2 reimplantations. This case was managed by a third reimplantation of the better ureter to get a super-long tunnel and transureteroureterostomy of the other ureter. 14 An obstructive distal ureterocele remnant in 1 case was managed by endoscopic resection. There has been no death in this series. One of the undiversion patients who presented with marginal renal function underwent renal transplantation 6 years after a reconstructive operation.
Fm. 6. Case 1. A, preoperative !VP reveals small ureterocele on right side and moderate-sized ureterocele on left side, outlined by dye in bladder. B, later preoperative !VP shows left ureterocele now filled with dye. C, reflux on left side resulted from unroofing ureterocele, which is not satisfactory definitive procedure but merely exchanges reflux for obstruction. Secondary operation corrected reflux.
CASE REPORTS
Case 1 . A 23-year-old woman was referred to us after endoscopic unroofing of a left ureterocele with a single collecting system, which resulted in reflux and pyelonephritis (fig. 7. Secondary ureterocele in 22 cases No. Cases Unroofed resulting in reflux 4 Reimplant failures: Reflux 10 Obstruction 2 Contralat. reflux 2 Diversion (suprapubic tube, ileal loop, 4 nephrostomy, ureterostomies) Other (valve effect, reflux) 2 TABLE
TABLE
8. Results in 51 primary ureterocele cases No. Cases
No problem Problems: Late nephrectomy Reflux needing re-operation Obstructive ureterocele remnant (transurethral resection)
42 5 4
1
Fm. 7. Case 2. A, preoperative !VP reveals filling defect in bladder (arrow) and right hydronephrosis. B, percutaneous anterograde pyelogram outlines dilated right collecting system. C, anterograde perfusion study 5 months postoperatively reveals normal lower ureter. D, !VP 5 months postoperatively reveals less hydronephrosis. Perfusion pressures were normal. Cystogram showed no reflux.
SURGICAL CORRECTION OF URETEROCELES
and the urethra was normal. Right anterograde pyelography revealed the dilated system and high perfusion pressures (fig. 7, B). A large ureterocele was excised and the distal ureter was tapered and reimplanted. The bladder was trabeculated, indicating that the ureterocele had caused outlet obstruction. Postoperatively, the patient has been well. Anterograde perfusion showed normal pressures and a normal lower ureter (fig. 7, C). An IVP demonstrated less hydronephrosis and cystography showed no reflux (fig. 7, D). Case 3. A 7-year-old boy was referred to us with intermittent urinary retention and right flank pain. An IVP showed bilateral single collecting systems, a moderate-sized ureterocele on the right side and a small one on the left side (fig. 8, }.i.). Cystography showed the unusual dynamics of the larger ureterocele; on some films it prolapsed like a bladder diverticulum, while on other views it projected into the bladder and downward into the prostatic uretl1ra and blocked the stream (fig. 8, B and C). Whether a ureterocele projects outward or inward into the bladder depends on the in the ureterocele .,,..m,,m·nr1 to the pressure in the at any it is common to see a initially but it flattens as the bladder pressure is increased finally, everts like a diverticulum when intravesical pressure is high. Both ureteroceles were resected, a simple reimplantation was done on the
left side and the ureter was tapered and reimplanted on the right side. Interestingly, there were small but definite type I urethral valves, which were resected endoscopically. Postoperative films were satisfactory, with improved upper tracts and no reflux. The patient has had no further difficulty. Case 4. A 3-month-old female infant was referred to us with urinary infection. An IVP showed a normal right kidney. On the left side the findings were typical for a duplex collecting system with a large ureterocele (fig. 9, A). A cystogram revealed reflux. A 1-stage complete repair was done, the ureterocele and its upper pole ureter being removed. The defect in the trigone and bladder neck was repaired, the left lower pole ureter being reimplanted as was the contralateral single ureter by simple advancement. The left kidney was explored through a flank incision. The upper pole appeared to have reasonable ,-,~.,vi,v,.,, and so it was spared, draining it pyelopyelostomy into the lower pole (fig. 9, B). During the next 7 years there were several episodes of urinary infection, although cystogTaphy showed no reflux. The upper was removed when the child was 7112 years old because it was judged likely to be the source of the intermittent infection. The specimen showed chronic focal pyelonephritis. In retrospect it would have been best removed at the original procedure. Case 5. A 10-year-old girl was referred to us with urinary
FIG. 8. Case 3. A, preoperative IVP reveals large right ureterocele and tiny left ureterocele (arrows). B and C, spot films during cystogram show protrusion outward of right ureterocele, giving appearance of bladder diverticulum. On some voiding films ureterocele prolapsed into prostatic urethra, causing retention.
Fra. 9, Case 4. by hydronephrotic
IVP reveals large ureterocele (arrow) and displacement of lower pole of left kidney infe:ciorly and laterally uppe:t poie associated 1Nith ureterocele. B I\TP 2 years late:.r.
m·,,,,c,py,it_iivP
1
596
HENDREN AND MITCHELL
infection. An IVP showed bilateral filling defects in the was completed when the child was 21 months old. The patient bladder and bilaterally scarred lower poles (fig. 10, A). A has had no further difficulty (fig. 12, C andD). cystogram showed ureteroceles and bilateral lower pole reflux. DISCUSSION Intraoperative retrograde pyelography showed dysplastic upper poles (fig. 10, B). A lengthy (10-hour) complete reconstrucUnroofing ureteroceles, as often done 30 years ago, has no tion was done. Both ureteroceles and upper pole ureters were place in contemporary pediatric urologic surgery. Small ureresected through a transverse lower abdominal incision and teroceles are best resected together with reimplantation of the the lower pole ureters were reimplanted. Each upper pole was ureters to prevent reflux. When the ureter is dilated tapering resected through separate flank incisions. The patient had an should be performed also. Large ureteroceles with duplex uneventful postoperative convalescence. During the 6-year collecting systems can be managed in a variety of ways, followup she has had no further infection. Retrograde pyelog- depending on the circumstances of the individual case. A raphy (the patient being allergic to IVP dye) showed satisfac- small infant, such as in case 6, may be best managed by tory lower pole ureters and a cystogram showed no reflux (fig. simple upper pole partial nephroureterectomy and later elec10, C). tive lower tract reconstruction when the child is older. This is Case 6. A 6-week-old female newborn was referred to us true especially in bilateral cases. A more robust infant may with urinary infection (fig. 11). An IVP showed satisfactory well be managed by a 1-stage complete reconstruction, espefunction of both lower poles but no visualization of the upper cially if pathology is unilateral, as shown in case 4. An older poles (fig. 12, A). A cystogram showed reflux into the upper patient who can tolerate many hours of anesthesia can be pole on the right side and the lower pole on the left side. managed, on the other hand, by complete reconstruction in a Cystoscopy showed an ectopic ureterocele on the right side, single long operation, as illustrated by case 5. There is an which had unroofed itself spontaneously (probably by intra- occasional case in which still another approach may be feasiuterine prolapse with infarction), accounting for reflux to the ble, that is repairing the lower tract and temporarily exteriorupper pole. On the left side the upper pole orifice was ectopic izing the upper pole ureters to deal with them somewhat later; in the urethral meatus (fig. 12, B). By retrograde study all 4 we did this on only 1 occasion. Careful attention should be paid to the site of the ureterocollecting systems were visualized. Bilateral upper pole nephroureterectomy was performed. The patient was then well cele. If the bladder wall is not repaired after the ureterocele is clinically on suppressive antimicrobial therapy except for 1 resected, especially an ectopic one, this can result in stress urinary infection. Repeat cystography showed persisting re- incontinence and/or a partially obstructive diverticulum. We flux into the stump of the right upper pole ureter and into the have seen examples of both of these complications. Furtherlower pole ureter on the left side. Lower tract reconstruction more, care should be taken to remove the distal lip of the
Fm. 10. Case 5. A, preoperative IVP shows confluent filling defects in bladder (arrows), visualization of both lower poles but not upper poles. B, intraoperative retrograde study filling all 4 segments. C, retrograde pyelogram 1 year later. Cystogram showed no reflux. OP. f
PRE-OP
Age 6 wks.
OP. 2
Age 2
f
mos.
Upper pale nephraureterectamies
I
'
rlfrt--+-- 2 normotopic
orifices with reflux
Veil of mucosa over orifice
"-. Orifice distal to e1d. sphincter
of stumpi ...,..--1 cm remained
Fm. 11. Case 6. Anatomy before and after staged reconstructive operations
597
SURGICAL CORRECTION OF URETEROCELES REFERENCES
1. Gross, R. E. and Clatworthy, H. W., Jr.: Ureterocele in infancy and childhood. Pediatrics, 5: 68, 1950. 2. Campbell, M. F.: Ureterocele. A study of 94 instances in 80 infants and children. Surg., Gynec. & Obst., 93: 705, 1951. 3. Ericsson, N. 0.: Ectopic ureterocele in infants and children. Acta Chir. Scand., suppl. 197, 1954. 4. Williams, D. I. and Woodard, J. R.: Problems in the management of ectopic ureteroceles. J. Urol., 92: 635, 1964. 5. Mogg, R. A.: Some observations on the ectopic ureter and ureterocele. J. Urol., 97: 1003, 1967. 6. Johnston, J. H. and Johnson, L. M.: Experiences with ectopic ureteroceles. Brit. J. Urol. 41: 61, 1969. 7. Hendren, W. H. and G. J.: Surgical correction of ureteroceles in childhood. J. Ped. Surg., 6: 235, 1971. 8. Stephens, D.: Caecoureterocele and concepts on the embryology and aetiology of meteroceles. Aust. New Zeal. J. Surg., 40: 1971. 9. R. S,, Kelalis, P. P., Burke, E. C. and Stickler, G. B.: Simple and ectopic ureterocele in infancy and childhood. Gynec. & Obst., 134: 511, 1972. 10. P. and Ylonen, N.: Vesico-ureteral xeflux follo,ving transurethral slitting of the ureteral orifice. Ann. Chir. Gynaec. Fenn., 64: 220, 1975. 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. Hendren, W, H.: Urinary tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974. 13. Hendren, W. H.: Reoperntion for the failed ureteral reimplantation. J. Urol., 111: 403, 1974. 14. Hendren, W. H.: Reconstructive surgery of the urinary tract in children. In: Current Problems in Surgery. Edited by M. M. Ravitch and F. M. Steichen. Chicago: Year Book Medical Publisher, Inc., vol. 14, 1977. EDITORIAL COMMENT Fm. 12. Case 6. A, preoperative IVP reveals satisfactory function of both lower poles but absence of visualization of upper poles. Note marked displacement of right lower pole, axis of which is tipped outward by hydronephrotic upper pole system. This is seen also on left side but not so clearly. B, preoperative retrograde study shows right upper pole system to be removed and both systems on left side. C, postoperative IVP reveals satisfactory upper tract. Cystogram showed persisting reflux. Lower tract reconstruction performed later. D, IVP 3 months after lower tract reconstruction.
ureterocele since it can act as a valve. Separation of the 2 ureters, carefully preserving all of the periureteral adventitia for the one to be spared, is possible in these ureterocele reconstructions. If both ureters are of almost normal size a double reimplantation can be performed if the upper pole has good parenchyma. If one ureter is much larger than the other it is best removed rather than tapered. A reconstructive operation of the urinary tract in complex ureterocele cases can be as demanding as any of the technical problems in reconstructive pediatric urologic surgery.
This review of Hendren's experience with ureteroceles covers nearly all problems encountered in reconstruction of these complex cases. To handle them properly they must be recognized, which requires good radiograms, a sensitivity to the possibility of nonfunctioning duplication and bladder base filling defects, careful endoscopy and a high index of suspicion. The distinction between simple and ectopic ureteroceles is important because bladder and urethral repair may be much more complex in the latter type. The alternatives in surgical approach are well described and displayed. As advice to others, I would strongly favor performing heminephrectomy first in the 2-incision approach since it easily allows one to stage the procedure if conditions require it. The 2-stage procedure in which the bladder is reconstructed first and an upper pole ureterostomy is left seems to invite unnecessary complications between stages. I strongly agree that the transperitoneal approach should be avoided when the new case can be approached easily from 2 intraperitoneal incisions. Robert D. Jeffs Brady Urological Institute The Johns Hopkins Hospital Baltimore, Maryland
