SURGICAL MANAGEMENT
OF
ECTOPIC URETEROCELE STUART FELDMAN, LEON G. LOME,
M.D.
M.D.
From the Department of Urology, Michael Reese Hospital and Medical Center, Chicago, Illinois
ABSTRACT - Nine patients with ectopic ureterocele, complete duplication, and nonvisualization of the upper renal segment are presented. All were managed with heminephrectomy and partial ureterectomy only. The procedure immediately controlled urosepsis, and ultimately was successful in the management of associated reflux. Excision of ureterocele and reimplantation of the ipsilateral ureter was unnecessary.
Ectopic ureterocele is a cystic dilatation of the submucosal ureter from the ectopic or cephalad portion of a completely duplicated collecting system. Frequently the associated upper segment is dysplastic, poorly functioning, and fails to visualize on excretory urography. Herein we review our experience with 9 patients with ectopic ureterocele and nonvisualizing upper renal segments. Material and Methods Between 1973 and 1979, 9 patients with ectopic ureterocele and duplication were seen at the Michael Reese Hospital and Medical Center. Five were children under one year of age, and 4 were adults, seventeen through thirty-one years. Seven were female; 2 were male. Seven of 9 patients had positive urine cultures. Excretory urography was performed on all patients. An intravesical radiolucent filling defect was identified on the excretory urogram in all 5 children. In 3 adults, initial studies were read as normal. All ectopic ureteroceles were associated with a completely duplicated collecting system in which the upper renal segment failed to visualize on the urogram. Seven
occurred on the left, two on the right. Voiding cystourethrography was performed in all patients. Vesicoureteral reflux was present in 4 of 9 patients. Ipsilateral reflux occurred into the orthotopic ureter in 3 patients, and contralateral reflux occurred in 1 patient. Cystoscopy revealed a submucosal, subtrigonal mass in 7 patients. In 2 adults the trigone was flat and the ureterocele initially was not identified. The ureterocele orifice could be identified in 6 patients. It was located near the bladder neck in 4 and in the proximal urethra in 2. In 3, the orifice was patulous, readily accepting a pediatric endoscope. Case Reports Case 1
Presented at the Annual Meeting, North Central Section of the American Urological Association, Inc., October, 1979, Phoenix, Arizona.
A three-month-old girl was hospitalized with a history of fever, poor appetite, lethargy, and failure to thrive. Urine cultured Escherichia coli. An excretory urogram showed left duplication with hydronephrosis and a nonvisualizing upper pole. A radiolucent filling defect was seen in the bladder (Fig. 1A). The voiding cystourethrogram showed reflux into the left ipsilateral lower pole ureter (Fig. 1B). Cystoscopy demonstrated the ureterocele, however, the ectopic orifice could not be identified. The orthotopic ureteral orifice was lateralized and patulous. An upper segment heminephrectomy
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FIGURE 1. Case 1. (A) ZVP reveals ectopic ureterocele with left hydronephrosis and nonvisualizing upper pole; (B) cystogram shows rejlux into left lower pole; and (C) cystogram demonstrates bilateral refux.
and subtotal ureterectomy were performed. Pathology showed a dysplastic upper renal segment. Postoperatively the cystogram showed bilateral ureteral vesical reflux (Fig. 1C). The patient was maintained on prophylactic antibacterials. Over a period of a year the child remained asymptomatic and the bilateral reflux ultimately abated. Case 2 A one-month-old girl was hospitalized with a history of fever, foul-smelling urine, and sepsis. Urine cultured Klebsiella. The excretory urogram showed a left hydronephrotic, “drooping flower” lower pole, and nonvisualization of the upper renal segment. A radiolucent filling defect was seen in the bladder. The voiding cystourethrogram showed a reflux into the contralateral ureter (Fig. 2A). At cystoscopy the ectopic ureterocele was seen with a patulous orifice at the bladder neck. Retrograde ureterogram into the ectopic ureterocele demonstrated a dilated, tortuous ureter (Fig. 2B), draining a small hydronephrotic upper renal segment. The patient underwent an upper pole heminephrectomy and partial ureterectomy. Pathology showed acute and chronic pyelonephritis. One month postoperatively, the child was asymptomatic and the urine sterile. However, the cystogram showed reflux up both ureters as well as the ureteral stump (Fig. 2C). The patient was maintained on prophylactic antibacterials. Fifteen months postoperatively reflux occurred only into the ureteral stump (Fig. 2D), and two years postoperatively, all
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reflux had ceased. Antibacterials have been discontinued, and the patient remains asymptomatic with sterile urine. Case 3 A thirty-one-year-old woman presented with a fifteen-year history of left flank pain, dysuria, and dyspareunia. The urine cultured E. coli. Physical examination revealed a large cystic, fusiform mass in the anterior vaginal wall. An excretory urogram demonstrated a large left pole renal mass (Fig. 3A), and a filling defect in the bladder. The voiding cystourethrogram showed no reflux. Cystoscopy showed elevation of the trigone, but an ectopic orifice was not seen. A percutaneous needle puncture of the upper pole renal mass recovered 400 cc. of purulent urine which cultured E. coli. An antegrade pyelogram demonstrated a massively dilated upper pole with a hydroureter terminating in a ureterocele (Fig. 3B). A left heminephrectomy with a subtotal ureterectomy was performed. Pathology showed acute and chronic pyelonephritis. Postoperatively the patient has been asymptomatic, and the vaginal mass is no longer palpable. The urine is sterile. Comment Ectopic ureterocele is a rare anomaly occurring in 1 in 5,000 to 1 in 12,000 general pediatric admissions. l As defined by Ericsson,2 it arises from the upper renal segment of a completely duplicated system. The caudal or lower pole moiety is referred to as orthotopic. Ectopic ureterocele most likely results from a defect
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FIGURE 2. Case 2. (A) Cystogram reveals ectopic ureterocele with right reflux; (B) retrograde ureterogram into left ectopic ureterocele demonstrates its dilated, tortuous ureter; (C) cystogram shows bilateral reflux as well as reflux into ureteral stump; (D) cystogram reveals rejlux only into ureteral stump.
in the ureter-al bud, and its faulty incorporation into the urethra and bladder base. Its frequent association with a “cap” of dysplastic renal tissue suggests an intrinsic abnormality of the entire segment. 3 The diagnosis of ectopic ureterocele in children is indeed straightforward, if only considered. The most frequent mode of presentation is urosepsis and failure to thrive, as 4 of 5 infants in this series demonstrated. One newborn with phocomelia was found to have an ectopic ureterocele on routine urography. Excretory urograms showed the consortium of an intravesical radiolucent filling defect, duplication, and nonvisualization of the upper pole
renal segment. Its presence was inferred by the classic “drooping flower” configuration of the excreting lower pole segment and lateral displacement of the orthotopic ureter by the hydronephrotic ectopic ureter. In adults the diagnosis of ectopic ureterocele may be elusive. Mandell, Stevens, and Lucey4 describe 2 adults in whom the diagnosis was not made until the time of surgery. Three adults in this series had five- to fifteen-year histories, respectively, of recurring flank pain and urinary tract infections (Table I). All had undergone multiple urologic investigations before the diagnosis of ectopic ureterocele was made. Two adult females presented to the gynecologic
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TABLE I. Age/Sex
Nine patients managed by heminephrectomylpartial Urine Culture
Presentation
1 mo. F
Urosepsis
Klebsiella
3 mo. F
Urosepsis; failure to thrive
E. coli; Klebsiella
5 mo. F
Urosepsis; failure to thrive
E. coli
5 mo. M
Urosepsis; failure to thrive
E. coli
12 mo. F
Phocomelia
Sterile
17 yr. F
L flank pain, 5 yr.
E. coli
21 yr. M
“DitRculty voiding”
Sterile
19 yr. F
L flank pain, 8 yr; dyspareunia; suburethral diverticulum L flank pain, 15 yr.; dyspareunia
E. coli
31 yr. F
Side
E. coli
ureterectomy
Intravenous Pyelogram Nonvis. upper pole; “drooping flower”; filling defect bladder Nonvis. upper pole; L hydronephrosis; filling defect bladder Nonvis. upper pole; “drooping flower”; filling defect bladder Nonvis. upper pole; “drooping flower”; filling defect bladder Nonvis. upper pole; .“drooping flower”; filling defect bladder Nonvis. upper pole; “drooping flower,” L hydronephrosis; filling defect bladder Bilateral duplication; nonvis. upper pole; “drooping flower”; filling defect bladder R hydronephrosis; nonvis. upper pole; “drooping flower”; filling defect bladder Nonvis. upper pole; “drooping flower”
Voiding Cystourethrogram R reflux
L orthotopic reflux
Negative
Negative
R orthotopic reflux
Negative
Negative
L orthotopic reflux
Negative
FIGURE 3. Case 3. (A) ZVP shows nonvisualizing left upper pole with “drooping P ower” of lower pole; (B) antegrade p yelogram reveals left upper pole hydronephrosis terminating into ectopic ureterocele.
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service complaining of dyspareunia and flank pain after intercourse. Both were found to have an anterior vaginal mass diagnosed as a “suburethral diverticulum.” Their excretory urograms, interpreted by the urology department, showed the classic radiographic features as described previously. In only 1 adult was the diagnosis of ectopic ureterocele made on initial urologic evaluation. Ectopic ureterocele is associated with complete pelvic-ureter-al duplication and invariably arises from the upper renal segment. The ureter to this dysplastic segment is often hydronephrotic. In addition, the ipsilateral caudad ureter and contralateral ureter may be hydronephrotic secondary to being drawn onto the ureterocele. Another consequence of distortion of the ureterovesical junction is that it allows reflux to occur into the nonureterocele-bearing ureters. Voiding cystourethrography should, therefore, always be used in evaluation of the urinary tract. The ectopic ureterocele rarely refluxes, however, reflux into the orthotopic and contralateral ureters is not unusual, being reported in 50 per cent of cases by Brock and Kaplan.5 Four of our patients had ureterovesical reflux. No patient initially had reflux into the ectopic ureterocele. Management of ectopic ureterocele with duplication remains controversial. Any treatment regimen ideally should be aimed at eradication of infection, preservation or improvement of renal function, and alleviation of symptoms. Simple transurethral unroofing of the ureterocele as definitive therapy is mentioned only to condemn it. This merely trades obstruction for massive reflux. There is general agreement in the literature that heminephrectomy of the poorly functioning upper pole segment should be an integral part of the therapeutic plan. 1,6*7The issue to be resolved is the fate of the distal ectopic ureter and ureterocele itself. Some authors advocate total ureterectomy, excision of the ureterocele, and ipsilateral ureteral reimplantation. 5-7 Whether to do this as a staged procedure or at the time of heminephrectomy has been well aired in the literature. * Malek et al. l have indicated that heminephrectomy and partial ureterectomy alone, leaving the collapsed ureterocele intact, may indeed be curative for this disease. In this series all 9 patients were treated in this manner. Brock and Kaplan5 report on 4 patients managed by heminephrectomy and partial ureterectomy.
Three of the 4 subsequently required excision of the ureterocele and ureteral stump with ipsilateral ureteral reimplantation for control of recurring urinary tract infections. Our experience has been contrary to this. Heminephrectomy and partial ureterectomy successfully control1 e d urosepsis and urinary symptoms in all 9 patients. Ureterovesical reflux was present in 4 patients prior to surgery. In 2 patients, the reflux stopped immediately after collapse of the ureterocele secondary to heminephrectomy. In 2 other children reflux not only persisted after heminephrectomy and partial ureterectomy, but additional ureterovesical reflux also developed. In 1 patient it occurred into the ureterocele and ureteral stump, and in one into the contralateral ureter. However, these children remained free of sepsis, thrived, and maintained sterile urine on prophylactic antibacterials. Over a period of two years, reflux into the ureters, ureterocele, and ureteral stump ultimately abated requiring no additional surgery on the lower urinary tract in either child. From this review, heminephrectomy and partial ureterectomy should be considered the procedure of choice for management of ectopic ureterocele. The advantages of this procedure lie in avoiding compromise of the blood supply of the ipsilateral lower ureteral segment and damaging the external urinary sphincter by extensive dissection. Collapse of the ureterocele, immediately controlled urosepsis and ultimately was successful in the management of associated reflux. Complete symptomatic relief and objective cure were achieved eventually in all patients.
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Chicago, Illinois 60616 (DR. LOME) References 1. Malek RS, Kelahs PP, Burke EC, and Stickler GB: Simple and ectopic ureterocele in infancy and childhood, Surg. Gynecol. Obstet. 134: 611 (1972). 2. Ericsson NO: Ectopic ureterocele in infants and children; clinical study, Acta Clin. &and. (Suppl.) 197: 1 (1954). 3. Ma&e GG, and Stephens FD: Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice, I. Urol. 114: 274 (1975).4. Mandell J, Stevens PS, and Lucey DT: Management of the unsuspected ectopic ureterocele, ibid. 120: 496 (1978). 5. Brock WA, and Kaplan WG: Ectopic‘ureteroceles in children, ibid. 119: 899 (1978). 6. Johnston JH, and Johnson LM: Experiences with ectopic ureteroceles, Br. J. Ural. 41: 61 (1969). 7. Williams DI, and Woodard JR: Problems in the management of ectopic ureteroceles, J. Ural. 92: 6‘35 (1964). 8. Culp OS: Heminephro-ureterectomy: comparison of onestage and two-stage operations, ibid. 83: 369 (1960).
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