Single-System ByM.
Rohatgi,
Cecoureterocele
D.K. Gupta, New
l A rare case of single system cecoureterocele associated with ipsilateral dysplastic kidney in a 4-year-old girl is reported. The preoperative radiological and endoscopic investigations identified ureterocele but not its cecal extension. The latter was diagnosed only during the transvesical surgery. Complete cure of her symptoms was achieved following staged procedures comprising of right ipsilateral nephroureterectomy followed by combined transvesical and transurethral deroofing of the cecoureterocele, excision of ureteric stump and its extension in the bladder and repair of the detrusor. This is the second case report in English language literature of single-system cecoureterocele. Copyright Q 1992 by W.B. Saunders Company INDEX WORDS:
Ureterocele;
cecoureterocele.
S
ectopic ureterocele with or INGLE-SYSTEM without prolapse in female children has been sporadically reported. 1-3Review of the English language literature has shown only one case report of single-system cecoureterocele.4 We herein report the second case of single-system cecoureterocele in a girl who presented with dribbling of urine and recurrent urinary tract infection since birth. CASE
REPORT
A /l-year-old girl was admitted in the Department of Paediatric Surgery, AIIMS, for incontinence of urine since birth and recurrent urinary tract infection since early infancy. On clinical examination she was found to have no abnormality except for dribbling of urine from the normally located urethral orifice with wellpreserved urinary stream at volition. Prior to admission the patient had been investigated outside by intravenous pyelogram and right retrograde pyelogram, which showed normal left kidney and poorly defined right pelvicalyceal system with a dilated tortuous ureter terminating near bladder neck (Fig 1). Ultrasonogram showed two dilated fluid-filled sacs in place of the right kidney (Fig 2A). Bladder area showed dilated right distal ureter, which was entering the bladder at a lower level (Fig 2B). Technetium 99m DTPA renogram showed nonfunctioning right kidney and normal left kidney. Cystogram showed a filling defect in the base of the bladder suggestive of ureterocele (Fig 3). Also, there was some resistance while introducing the cystoscope during cystourethroscopy, the latter showed a ureterocele occupying entire trigonal region extending into bladder neck and the right ureteric orifice could not be identified. A provisional diagnosis of right ureterocele with ipsilateral cystic dysplastic kidney was made. On day 6, right nephroureterectomy was performed; however, postoperatively the dribbling of urine persisted. A bladder ultrasonogram 3 months later showed the persistence of ureterocele (Fig 4). The child was reoperated 19 months later. On opening the bladder, ureterocele was found to occupy whole of the trigone and extended submucosally into the urethra just proximal to the external urinary meatus. The right ureteric orifice was small in size and located high up in its vesical portion and the diagnosis of cecoureterocele was made. Deroofing of the vesical portion of cecoureterocele. excision of right ureteric stump along with its
JournalofPediatr~
and P. Subbarao
Delhi, India
Surgery, Vol 27, No 9 (September), 1992: pp 1241-1243
extension in bladder, repair of detrusor, and transurethral deroofing of caecal extension were performed. In the early postoperative period the patient had intermittent episodes of wetting despite normal micturition. However, after 3 months the child became totally continent. At follow-up after 1 year, the child was found to be asymptomatic except for occasional nocturnal enuresis. DISCUSSION
Cecoureterocele is an extremely rare type of ureterocele seen with nonduplicated system. Stephen reported three such cases, two were associated with upper pole ureter of complete duplex system and the other with the unilateral single system.j Pathologically, cecoureterocele is a congenital deformity of the intravesical segment of ureter which expands beneath the mucosal layer of the trigone and urethra. It characteristically has a large floating vesical component with a wide incompetent ureteric orifice on its dome and a long submucosal tongue or cecum extending into the urethra. Rarely, the distended cecal part may present as a cystic swelling at the anterior vaginal wall or may protude out of the external urethral meatus during micturition or screaming. This cystic protusion must be differentiated from the prolapsed ectopic ureterocele.6 The latter is a vesical ureterocele that has been discharged through the urethra together with bladder trigone and very often it may get strangulated.’ In contrast, in cecoureterocele prolapse of the trigone and the strangulation is uncommon. Single-system ectopic ureterocele is more often associated with cardiac and genital anomalies,8 whereas cecoureterocele has been reported only in females irrespective of its being associated with single- or double-ureter system. This is because the Mullerian duct in females is responsible for the urethral extension of cecoureterocele as the latter is tied to the Mullerian ducts in their descent to the exterior. The two major symptoms in these patients are dribbling of urine since birth in addition to wellmaintained normal micturition pattern and persistent urinary infection. It is known that the ectopic ureter (with or without ureterocele) causes wetting only From the Department of Paediatric Surgety All India Institute of Medical Sciences, New Delhi. India. Address reprint requests to Professor M Rohatgi, FRCS. FIGS, Head, Department of Paediatric Surgery. All India Institute of Medical Sciences, New Delhi, India. Copyright Q I992 by W.B. Saunders Company 0022-346819212709-0021$03.OOJO 1241
1242
ROHATGI, GUPTA, AND SUBBARAO
However, the cecal component of ureterocele was not visualized on any of the radiological studies as demonstrated by Stephen4 nor was it identified during cystoscopy, although there was some resistance while introducing the scope. Lack of entry of dye into ureterocele during micturiting cystouretherogram (MCXJ) may be due to its narrow ureteral orifice. Such an orifice will allow urine to enter the ureterocele during micturition but will not permit free drainage causing persistence of urterocele. The diagnosis of cecal extension was made only during transvesical deroofing of the ureterocele. In the early stage of management only right nephrouretrectomy upto the level of muscular hiatus of the bladder, as suggested by Kroovand and Perlmutter,” was performed with the hope that the ureterocele
Fig 1. Retrograde pyelogram (right) showing poorly defined renal pelvis with grossly dilated and tortuous ureter terminating near the bladder neck.
when the ureteric orifice opens at a point distal to the zone of the internal sphincter into the bladder neck, urethra, or vulva. The intrinsic peristalsis in the ureter overcomes the tone of the internal sphincter and leakage of urine occurs, which cannot be prevented by the external urethral sphincter. In cecoureterocele, true urinary incontinence is observed due to ineffecient external urethral sphincter, which may be congenitally lax and deficient or rendered dilated and deficient by a large suburethrally traversing cecal extension. 9 Due to such anatomical deficiency, many of these patients fail to become continent even after corrective surgery.‘O The distended cecoureterocele may also cause problems in initiation and progression of urinary stream during micturition. The recurrent urinary tract infection occurs due to urinary stasis resulting from (1) intermittent bladder neck obstruction caused by large floating vesical component of ureterocele acting as a ball valve, and (2) entry of large amount of urine into the cecoureterocele through its “fish mouth” ureteric orifice during micturition. The patient reported herein passed urine in a stream in addition to dribbling of urine since birth. There was history of recurrent urinary tract infection. A retrograde pyelogram and ultrasound scan had confirmed the presence of right single dysplastic kidney with tortous dilated ureter and ureterocele.
Fig 2. Ultrasound scan showing (A) two fluid-filled dilated sacs in place of right kidney with no cortex, and (6) dilated distal part of the right ureter entering at a lower level into the bladder.
SINGLE-SYSTEM
1243
CECCkJRETEROCELE
Fig 4.
Fig 3. Cystogram showing elevated base of the bladder suggestive of ureterocele.
would collapse and the dribbling would stop. However, this procedure did not give any symptomatic relief and necessitated excision of ureteric stump and combined transvesical and transurethral deroofing of the ureterocele, after which the child regained total continence within a period of few months. Batter and Retik” reported that when nonobstructive ectopic ureterocele is associated with cystic dysplastic kidney, the ureterocele alone may be dealt with and upper tract can be ignored. However, in the present case
Ultrasound scan showing persistence of ureterocele.
dysplastic multicystic kidney had no function and its dilated tortuous ureter was a chronic source of infection. Therefore, its total excision was justified. Also, simple excision of ureterocele would have resulted in the ipsilateral reflux and persistance of infection. Hence, when cecoureterocele is associated with dysplastic kidney, in addition to ipsilateral nephroureterectomy, the cecoureterocele should be completely deroofed because its wide incompetent ureteric orifice would not allow it to collapse. Excision of ureteral stump and repair of bladder wall is essential to prevent diverticulum formation. Mild delay in recovery of continence in this patient was due to prolonged overstretching and dilatation of urethra by its cecal component. Although in some cases persistence of incontinence has been reported, the present patient became fully continent after 3 months.
REFERENCES 1. Mandell J. Colodny AH, Lebowitz RL, et al: Ureteroceles in infants and children. J Urol 123:921-926, 1980 2. Snyder HM, Johnston JH: Orthotopic ureteroceles in children. J Ural 119:543-546. 1978 3. Ahmed S: Prolapsed single system ureterocele in a girl. J Urol 132:1180,1984 4. Stephen FD: Caecoureterocele and concepts of the embryology and etiology of ureteroceles. Aust N Z J Surg 40:239-248. 1971 5. Stephen FD: Ureteroceles on duplex ureters, in Stephen FD (ed): Congenital Malformations of the Urinary Tract. New York. NY, Praeger, 1983, pp 320-351 6. Catarci A, Mazzoni G, Morocco G, et al: Strangulated prolapsed ureterocele: A case report, It J Pediatr Surg Sci 1:49-51, 1987
7. Klauber GT. Crawford DB: Prolapse of ectopic ureterocele and bladder trigone. Urology 15:164-167, 1980 8. Johnson DK, Perlmutter AD: Single system ectopic ureterocele with anomalies of the heart. testis and vas deference. J Urol 123:81-83, 1980 9. Malck RS, Kelalis PP, Burke EC, et al: Simple and ectopic ureterocele in infancy and childhood. Surg Gynecol Obstet 134:61 l616,1972 10. Leadbetter GW JR: Ectopic ureterocele as a cause of urinary incontinence. J Urol 103:222-226, 1970 Il. Kroovand LR, Perlmutter AD: A one stage surgical approach to ectopic ureterocele. J Urol 122:367-369, 1979 12. Bauer SB, Retik AB: The nonobstructive ureterocele. J Ural 119:804-807. 1978
Rohatgi,
Cecoureterocele
D.K. Gupta, New
l A rare case of single system cecoureterocele associated with ipsilateral dysplastic kidney in a 4-year-old girl is reported. The preoperative radiological and endoscopic investigations identified ureterocele but not its cecal extension. The latter was diagnosed only during the transvesical surgery. Complete cure of her symptoms was achieved following staged procedures comprising of right ipsilateral nephroureterectomy followed by combined transvesical and transurethral deroofing of the cecoureterocele, excision of ureteric stump and its extension in the bladder and repair of the detrusor. This is the second case report in English language literature of single-system cecoureterocele. Copyright Q 1992 by W.B. Saunders Company INDEX WORDS:
Ureterocele;
cecoureterocele.
S
ectopic ureterocele with or INGLE-SYSTEM without prolapse in female children has been sporadically reported. 1-3Review of the English language literature has shown only one case report of single-system cecoureterocele.4 We herein report the second case of single-system cecoureterocele in a girl who presented with dribbling of urine and recurrent urinary tract infection since birth. CASE
REPORT
A /l-year-old girl was admitted in the Department of Paediatric Surgery, AIIMS, for incontinence of urine since birth and recurrent urinary tract infection since early infancy. On clinical examination she was found to have no abnormality except for dribbling of urine from the normally located urethral orifice with wellpreserved urinary stream at volition. Prior to admission the patient had been investigated outside by intravenous pyelogram and right retrograde pyelogram, which showed normal left kidney and poorly defined right pelvicalyceal system with a dilated tortuous ureter terminating near bladder neck (Fig 1). Ultrasonogram showed two dilated fluid-filled sacs in place of the right kidney (Fig 2A). Bladder area showed dilated right distal ureter, which was entering the bladder at a lower level (Fig 2B). Technetium 99m DTPA renogram showed nonfunctioning right kidney and normal left kidney. Cystogram showed a filling defect in the base of the bladder suggestive of ureterocele (Fig 3). Also, there was some resistance while introducing the cystoscope during cystourethroscopy, the latter showed a ureterocele occupying entire trigonal region extending into bladder neck and the right ureteric orifice could not be identified. A provisional diagnosis of right ureterocele with ipsilateral cystic dysplastic kidney was made. On day 6, right nephroureterectomy was performed; however, postoperatively the dribbling of urine persisted. A bladder ultrasonogram 3 months later showed the persistence of ureterocele (Fig 4). The child was reoperated 19 months later. On opening the bladder, ureterocele was found to occupy whole of the trigone and extended submucosally into the urethra just proximal to the external urinary meatus. The right ureteric orifice was small in size and located high up in its vesical portion and the diagnosis of cecoureterocele was made. Deroofing of the vesical portion of cecoureterocele. excision of right ureteric stump along with its
JournalofPediatr~
and P. Subbarao
Delhi, India
Surgery, Vol 27, No 9 (September), 1992: pp 1241-1243
extension in bladder, repair of detrusor, and transurethral deroofing of caecal extension were performed. In the early postoperative period the patient had intermittent episodes of wetting despite normal micturition. However, after 3 months the child became totally continent. At follow-up after 1 year, the child was found to be asymptomatic except for occasional nocturnal enuresis. DISCUSSION
Cecoureterocele is an extremely rare type of ureterocele seen with nonduplicated system. Stephen reported three such cases, two were associated with upper pole ureter of complete duplex system and the other with the unilateral single system.j Pathologically, cecoureterocele is a congenital deformity of the intravesical segment of ureter which expands beneath the mucosal layer of the trigone and urethra. It characteristically has a large floating vesical component with a wide incompetent ureteric orifice on its dome and a long submucosal tongue or cecum extending into the urethra. Rarely, the distended cecal part may present as a cystic swelling at the anterior vaginal wall or may protude out of the external urethral meatus during micturition or screaming. This cystic protusion must be differentiated from the prolapsed ectopic ureterocele.6 The latter is a vesical ureterocele that has been discharged through the urethra together with bladder trigone and very often it may get strangulated.’ In contrast, in cecoureterocele prolapse of the trigone and the strangulation is uncommon. Single-system ectopic ureterocele is more often associated with cardiac and genital anomalies,8 whereas cecoureterocele has been reported only in females irrespective of its being associated with single- or double-ureter system. This is because the Mullerian duct in females is responsible for the urethral extension of cecoureterocele as the latter is tied to the Mullerian ducts in their descent to the exterior. The two major symptoms in these patients are dribbling of urine since birth in addition to wellmaintained normal micturition pattern and persistent urinary infection. It is known that the ectopic ureter (with or without ureterocele) causes wetting only From the Department of Paediatric Surgety All India Institute of Medical Sciences, New Delhi. India. Address reprint requests to Professor M Rohatgi, FRCS. FIGS, Head, Department of Paediatric Surgery. All India Institute of Medical Sciences, New Delhi, India. Copyright Q I992 by W.B. Saunders Company 0022-346819212709-0021$03.OOJO 1241
1242
ROHATGI, GUPTA, AND SUBBARAO
However, the cecal component of ureterocele was not visualized on any of the radiological studies as demonstrated by Stephen4 nor was it identified during cystoscopy, although there was some resistance while introducing the scope. Lack of entry of dye into ureterocele during micturiting cystouretherogram (MCXJ) may be due to its narrow ureteral orifice. Such an orifice will allow urine to enter the ureterocele during micturition but will not permit free drainage causing persistence of urterocele. The diagnosis of cecal extension was made only during transvesical deroofing of the ureterocele. In the early stage of management only right nephrouretrectomy upto the level of muscular hiatus of the bladder, as suggested by Kroovand and Perlmutter,” was performed with the hope that the ureterocele
Fig 1. Retrograde pyelogram (right) showing poorly defined renal pelvis with grossly dilated and tortuous ureter terminating near the bladder neck.
when the ureteric orifice opens at a point distal to the zone of the internal sphincter into the bladder neck, urethra, or vulva. The intrinsic peristalsis in the ureter overcomes the tone of the internal sphincter and leakage of urine occurs, which cannot be prevented by the external urethral sphincter. In cecoureterocele, true urinary incontinence is observed due to ineffecient external urethral sphincter, which may be congenitally lax and deficient or rendered dilated and deficient by a large suburethrally traversing cecal extension. 9 Due to such anatomical deficiency, many of these patients fail to become continent even after corrective surgery.‘O The distended cecoureterocele may also cause problems in initiation and progression of urinary stream during micturition. The recurrent urinary tract infection occurs due to urinary stasis resulting from (1) intermittent bladder neck obstruction caused by large floating vesical component of ureterocele acting as a ball valve, and (2) entry of large amount of urine into the cecoureterocele through its “fish mouth” ureteric orifice during micturition. The patient reported herein passed urine in a stream in addition to dribbling of urine since birth. There was history of recurrent urinary tract infection. A retrograde pyelogram and ultrasound scan had confirmed the presence of right single dysplastic kidney with tortous dilated ureter and ureterocele.
Fig 2. Ultrasound scan showing (A) two fluid-filled dilated sacs in place of right kidney with no cortex, and (6) dilated distal part of the right ureter entering at a lower level into the bladder.
SINGLE-SYSTEM
1243
CECCkJRETEROCELE
Fig 4.
Fig 3. Cystogram showing elevated base of the bladder suggestive of ureterocele.
would collapse and the dribbling would stop. However, this procedure did not give any symptomatic relief and necessitated excision of ureteric stump and combined transvesical and transurethral deroofing of the ureterocele, after which the child regained total continence within a period of few months. Batter and Retik” reported that when nonobstructive ectopic ureterocele is associated with cystic dysplastic kidney, the ureterocele alone may be dealt with and upper tract can be ignored. However, in the present case
Ultrasound scan showing persistence of ureterocele.
dysplastic multicystic kidney had no function and its dilated tortuous ureter was a chronic source of infection. Therefore, its total excision was justified. Also, simple excision of ureterocele would have resulted in the ipsilateral reflux and persistance of infection. Hence, when cecoureterocele is associated with dysplastic kidney, in addition to ipsilateral nephroureterectomy, the cecoureterocele should be completely deroofed because its wide incompetent ureteric orifice would not allow it to collapse. Excision of ureteral stump and repair of bladder wall is essential to prevent diverticulum formation. Mild delay in recovery of continence in this patient was due to prolonged overstretching and dilatation of urethra by its cecal component. Although in some cases persistence of incontinence has been reported, the present patient became fully continent after 3 months.
REFERENCES 1. Mandell J. Colodny AH, Lebowitz RL, et al: Ureteroceles in infants and children. J Urol 123:921-926, 1980 2. Snyder HM, Johnston JH: Orthotopic ureteroceles in children. J Ural 119:543-546. 1978 3. Ahmed S: Prolapsed single system ureterocele in a girl. J Urol 132:1180,1984 4. Stephen FD: Caecoureterocele and concepts of the embryology and etiology of ureteroceles. Aust N Z J Surg 40:239-248. 1971 5. Stephen FD: Ureteroceles on duplex ureters, in Stephen FD (ed): Congenital Malformations of the Urinary Tract. New York. NY, Praeger, 1983, pp 320-351 6. Catarci A, Mazzoni G, Morocco G, et al: Strangulated prolapsed ureterocele: A case report, It J Pediatr Surg Sci 1:49-51, 1987
7. Klauber GT. Crawford DB: Prolapse of ectopic ureterocele and bladder trigone. Urology 15:164-167, 1980 8. Johnson DK, Perlmutter AD: Single system ectopic ureterocele with anomalies of the heart. testis and vas deference. J Urol 123:81-83, 1980 9. Malck RS, Kelalis PP, Burke EC, et al: Simple and ectopic ureterocele in infancy and childhood. Surg Gynecol Obstet 134:61 l616,1972 10. Leadbetter GW JR: Ectopic ureterocele as a cause of urinary incontinence. J Urol 103:222-226, 1970 Il. Kroovand LR, Perlmutter AD: A one stage surgical approach to ectopic ureterocele. J Urol 122:367-369, 1979 12. Bauer SB, Retik AB: The nonobstructive ureterocele. J Ural 119:804-807. 1978
