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Anterior Urethral Valves in Children
Vol. 108, December Printed in U.S·.A.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
ANTERIOR URETHRAL VALVES IN CHILDREN CASIMIR F. FIRLIT
AND
LOWELL R. KING
From the General Medical and Surgical Research Service, Veterans Administration Hospital, Hines, the Department of Urology, Northwestern University and the Division of Urology, Children's Memorial Hospital, Chicago, Illinois
Since the introduction of the voiding cystourethrogram, posterior urethral valves have been recognized as a relatively common cause of severe bladder outlet obstruction. Anterior urethral valves have received much less attention and even today are not always included in the differential diagnosis of boys with symptoms suggesting bladder outlet obstruction. Occasional reports have appeared defining anterior valves as a clinical entity. 1 • 8 A review of the literature discloses 14 reported cases, in most of which the lesion is well defined. Owing to the relative rarity of this urethral anomaly, our purpose herein is to emphasize that severe degrees of obstruction may be caused by anterior valves and to discuss their identification and treatment. The lesion may be noted in infancy or childhood because of the obvious presence of a proximal diverticulum of the urethra. If diverticula are absent the child presents because of urinary tract infection or a weak, dribbling stream. In these instances, the anterior urethral valve may easily be overlooked at initial evaluation unless the entity is kept in mind. To avoid this a lateral or oblique view of the entire urethra must be obtained on voiding cystourethrography. Similarly, the anterior urethra must be carefully examined at cystoscopy. A Vest (straight ahead view) lens system is best in this respect. An anterior valve can usually be seen with a foroblique panendoscope but the appearance is more subtle and the valve is Accepted for publication May 19, 1972. 1 Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., vol. 2, p. 1621, 1970. 2 Daniel, J., Stewart, A. M. and Blair, D. W.: Congenital anterior urethral valve-diagnosis and treatment. Brit. J. Urol., 40: 589, 1968. 3 Gross, R. E. and Bill, A. H., Jr.: Concealed diverticulum of the male urethra as a cause of urinary obstruction. Pediatrics, 1: 44, 1948. 4 Hope, J. W., Jameson, P. J. and Michie, A. J.: Diagnosis of anterior urethral valves by voiding urethrography: report of two cases. Radiology, 74: 798, 1960. 5 Nesbit, R. M. and Labardini, M. M.: Urethral valves in the male child. J. Urol., 96: 218, 1966. 6 Waterhouse, K. and Scord'amaglia, L. J.: Anterior urethral valve: a rare cause of bilateral hydronephrosis. J. Urol., 87: 556, 1962. 7 Williams D. I.: Urology in childhood. In: Encyclopedia of Urology. Edited by C. E. Alken, V. W. Dix, H. M. Weyrauch and E. Wildbolz. Berlin: Springer-Verlag, vol. 15, p. 81, 1958. 8 Texter, J. H. and Engel, R. M. E.: Anterior urethral valve as cause for urinary obstruction: a case report. J. Urol., 107: 316, 1972.
Fig. 1. B. R. Five-minute delayed retrograde pyelogram demonstrates bilateral hydroureteronephrosis, marked ureterectasis and ureteral atonia secondary to mid-pendulous urethral valve and diverticulum. more likely to appear as an unobstructive mucosal fold. MATERIALS AND METHODS
All cases of urethral valves diagnosed and treated at Children's Memorial Hospital between 1952 and 1971 were reviewed. During this period 67 children were seen with posterior urethral valves, while 7 boys had anterior urethral valves. Medical records were evaluated with respect to initial complaint, presenting signs, age, diagnostic regimen, surgical treatment and complications (see table). RESULTS AND DISCUSSION
Most patients with anterior urethral valves presented for urological evaluation because of impeded urethral urinary flow, although one presented with urinary tract infection. Age at diagnosis ranged from 2 days to 11 years. In the last 18 months there were 2 patients less than 1 week old when found to have anterior urethral valves. Anterior urethral valves were identified in the bulbous urethra in 2 patients and in the mid-pendulous urethra in the remaining 5 patients. Of the 7 patients, 2 presented with large urethral diverticula, which, of course, suggested the proper diagnosis.
972
973
ANTERIOR URETHRAL VALVES IN CHILDREN
Anterior urethral values p atient
Location of Valve
Age
Presenting Signs
Diagnostic Evaluation
Treatment
Complication
---
1 yr.
Mid-pendulous urethra
Inability to void
11 yrs.
Mid-pendulous urethra
Narrow urinary
(1965)
Mid-pendulous urethra and
N a:now l01v-pressure stream and postvoiding dribbling
JH
9½ yrs.
ticulum Bulbous urethra
3 yrs.
Bulbous urethra
6 days
Mid-pendulous urethra and proximal diverticulum
2 days
Mid-pendulous urethra
l\lIH (1958)
JB (1962)
KM
2 yrs.
proximal diver-
(1965)
JH (1967)
BR (1971)
JD (1971)
IVP and cystogram. Open excision of valve Vesicoureteral reflux, and urethroplasty It. hydronephrosis (Johanson) Voiding cystourethro- TUR gram and urethras-
strea:i.11
Urinary tract infection and unexplained lt. vesicoureteral reflux and hydronephrosis Difficulty in urination. Narrow low pressure urinary stream S-welling of penis and low pressure, narrow urinary stream
Urinary dribbling, enlarged bladder
copy Voiding cystourethrogram
Urinary extravasation; second urethral stric-
ture None
Open resection of valve and diverticulectomy
Extravasation
IVP, cystoscopy and urethroscopy
TUR
None
IVP and urethroscopy. Hydronephrosis
TUR
None
Voiding cystourethro- Open excision and digram and cystoverticulectomy and gram. Dilatation of loop ureterostomies ant. urethra. Azotemia and bilat. vesicoureteral reflux, tortuous ureters Voiding cystourethro- Loop cutaneous uregram and IVP. Diterostomies latation of ant. urethra. Bilat. dilated tortuous ureters
Penile fistula
None
Fm. 2. K. M. Retrograde urethrogram demonstrates anterior urethral valve (arrow) and urethral diverticulum.
All patients with urethral flow problems were carefully evaluated for abdominal masses and for adequacy of the distal urethral meatus. Subsequently, voiding cystourethrography and excretory urography (IVP) were performed. In this series 5 patients had bilateral hydroureteronephrosis (fig. 1) and one also had vesicoureteral reflux. The voiding demonstrated a mid-
.
-.-·. - --
".
--·' _-
-
---
><~--··-c~-------
patients (fig. 2). The proximal urethral dilatation and the site of the urethral valve were clearly demonstrated roentgenographically (fig. 3). In 3 cases voiding films were not obtained with the initial cystogram because these children had difficulty in initiating and maintaining the urinary stream. In such patients, the dysuria following catheterization makes voiding even more difficult. In 2 of these 3 in whom a urethrog;ram was not ob-
-- --~
,--·~·- -·'--•
~---------
.
- --~ ~-----
974
FIRLIT AND KING
Fm. 3. J. D. Voiding cystourethrogram illustrates proximal urethral dilatation and anterior urethral valve (arrows).
Fig. 4. B. R. Voiding cystourethrogram. Note markedly dilated proximal urethra secondary to anterior urethral valve.
tained, the diagnosis was also missed at initial cystoscopy. Bougie calibration of the urethra did not detect the valve. These 2 patients had objective signs of bladder outlet obstruction, that is marked trabeculation in 1 and trabeculation with reflux and residual urine in the other. Both patients underwent Y-V plasty of the bladder neck. Both proved to have secondary bladder neck hypertrophy occurring behind the more distal obstruction and, of course, neither patient was improved by the Y-V plasty. Voiding urethrograms could never be obtained in either boy. Consequently, the anterior urethral valve was not discovered in one until the
fourth cystoscopy and in the other until the third systematic evaluation. These experiences emphasize the need to keep anterior urethral valves in mind when evaluating boys with obstructive signs and symptoms and the necessity for voiding films during cystourethrography. Careful urethroscopy and examination of the anterior urethra should be performed, particularly when obstructive symptoms are present. In 2 patients anterior urethral valves were diagnosed during the first week of life. These infants were evaluated because of urinary dribbling and a narrow urinary stream. Bilateral hydroureteronephrosis and proximal urethral dilatation secondary to an anterior valve were found on IVP, retrograde pyelography and voiding cystourethrography (fig. 4). Both patients were treated initially by high loop cutaneous ureterostomy because of coexistent azotemia and dilated tortuous ureters. In such circumstances high diversion permits optimum renal growth and development and minimizes the risk of infection. The loop ureterostomies are closed after the valve has been removed and it is determined that the bladder can empty completely. The resectoscope is extremely valuable in precisely localizing the valve by the hanging-up effect of the extended loop on the urethral valve. Three patients were treated by transurethral resection of the valve alone, which is readily accomplished at the time of cystoscopy. Resection of the most distal portion of the flap is all that is necessary and a satisfactory result was obtained in each case. Two patients with anterior urethral valves and secondary urethral diverticula were treated by open diverticulectomy with excision of the valve. One additional patient had the valve excised by an open approach in combination with the first stage of a Johanson urethroplasty. In both patients in
ANTERIOR URETHRAL VALVES IN CHILDREN
whom primary excision of the anterior urethral valve and diverticula was done, urinary extravasation developed subsequently. In one the extravasation was due to a malfunctioning suprapubic tube that had to be replaced. In the other extravasation was due to intractable bladder spasms and resulted in a secondary urethral stricture.
'r
I
(
,
SUMMARY
Our primary purpose in describing these 7 patients with anterior urethral valves is to emphasize the presence of the entity, the extent of associated morbidity and the difficulties that may be encountered in arriving at the proper diagnosis. It is noteworthy that several patients underwent re-
I,-
975
peated hospitalizations owing to symptoms suggesting intravesical obstruction before the proper diagnosis was reached. In patients with obstructive symptoms careful attention should be paid to the anterior urethra on endoscopy and at voiding cystourethrography. Early diagnosis should minimize the morbidity associated with unnecessary or inappropriate surgical procedures and should considerably reduce the incidence of urinary tract infection. The specific treatment of the anterior urethral valve is transurethral resection and/or fulguration. When a urethral valve is associated with a diverticulum, open resection of the valve together with excision of the diverticulum, with suprapubic cystostomy for temporary diversion, 1s the recommended approach.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
ANTERIOR URETHRAL VALVES IN CHILDREN CASIMIR F. FIRLIT
AND
LOWELL R. KING
From the General Medical and Surgical Research Service, Veterans Administration Hospital, Hines, the Department of Urology, Northwestern University and the Division of Urology, Children's Memorial Hospital, Chicago, Illinois
Since the introduction of the voiding cystourethrogram, posterior urethral valves have been recognized as a relatively common cause of severe bladder outlet obstruction. Anterior urethral valves have received much less attention and even today are not always included in the differential diagnosis of boys with symptoms suggesting bladder outlet obstruction. Occasional reports have appeared defining anterior valves as a clinical entity. 1 • 8 A review of the literature discloses 14 reported cases, in most of which the lesion is well defined. Owing to the relative rarity of this urethral anomaly, our purpose herein is to emphasize that severe degrees of obstruction may be caused by anterior valves and to discuss their identification and treatment. The lesion may be noted in infancy or childhood because of the obvious presence of a proximal diverticulum of the urethra. If diverticula are absent the child presents because of urinary tract infection or a weak, dribbling stream. In these instances, the anterior urethral valve may easily be overlooked at initial evaluation unless the entity is kept in mind. To avoid this a lateral or oblique view of the entire urethra must be obtained on voiding cystourethrography. Similarly, the anterior urethra must be carefully examined at cystoscopy. A Vest (straight ahead view) lens system is best in this respect. An anterior valve can usually be seen with a foroblique panendoscope but the appearance is more subtle and the valve is Accepted for publication May 19, 1972. 1 Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., vol. 2, p. 1621, 1970. 2 Daniel, J., Stewart, A. M. and Blair, D. W.: Congenital anterior urethral valve-diagnosis and treatment. Brit. J. Urol., 40: 589, 1968. 3 Gross, R. E. and Bill, A. H., Jr.: Concealed diverticulum of the male urethra as a cause of urinary obstruction. Pediatrics, 1: 44, 1948. 4 Hope, J. W., Jameson, P. J. and Michie, A. J.: Diagnosis of anterior urethral valves by voiding urethrography: report of two cases. Radiology, 74: 798, 1960. 5 Nesbit, R. M. and Labardini, M. M.: Urethral valves in the male child. J. Urol., 96: 218, 1966. 6 Waterhouse, K. and Scord'amaglia, L. J.: Anterior urethral valve: a rare cause of bilateral hydronephrosis. J. Urol., 87: 556, 1962. 7 Williams D. I.: Urology in childhood. In: Encyclopedia of Urology. Edited by C. E. Alken, V. W. Dix, H. M. Weyrauch and E. Wildbolz. Berlin: Springer-Verlag, vol. 15, p. 81, 1958. 8 Texter, J. H. and Engel, R. M. E.: Anterior urethral valve as cause for urinary obstruction: a case report. J. Urol., 107: 316, 1972.
Fig. 1. B. R. Five-minute delayed retrograde pyelogram demonstrates bilateral hydroureteronephrosis, marked ureterectasis and ureteral atonia secondary to mid-pendulous urethral valve and diverticulum. more likely to appear as an unobstructive mucosal fold. MATERIALS AND METHODS
All cases of urethral valves diagnosed and treated at Children's Memorial Hospital between 1952 and 1971 were reviewed. During this period 67 children were seen with posterior urethral valves, while 7 boys had anterior urethral valves. Medical records were evaluated with respect to initial complaint, presenting signs, age, diagnostic regimen, surgical treatment and complications (see table). RESULTS AND DISCUSSION
Most patients with anterior urethral valves presented for urological evaluation because of impeded urethral urinary flow, although one presented with urinary tract infection. Age at diagnosis ranged from 2 days to 11 years. In the last 18 months there were 2 patients less than 1 week old when found to have anterior urethral valves. Anterior urethral valves were identified in the bulbous urethra in 2 patients and in the mid-pendulous urethra in the remaining 5 patients. Of the 7 patients, 2 presented with large urethral diverticula, which, of course, suggested the proper diagnosis.
972
973
ANTERIOR URETHRAL VALVES IN CHILDREN
Anterior urethral values p atient
Location of Valve
Age
Presenting Signs
Diagnostic Evaluation
Treatment
Complication
---
1 yr.
Mid-pendulous urethra
Inability to void
11 yrs.
Mid-pendulous urethra
Narrow urinary
(1965)
Mid-pendulous urethra and
N a:now l01v-pressure stream and postvoiding dribbling
JH
9½ yrs.
ticulum Bulbous urethra
3 yrs.
Bulbous urethra
6 days
Mid-pendulous urethra and proximal diverticulum
2 days
Mid-pendulous urethra
l\lIH (1958)
JB (1962)
KM
2 yrs.
proximal diver-
(1965)
JH (1967)
BR (1971)
JD (1971)
IVP and cystogram. Open excision of valve Vesicoureteral reflux, and urethroplasty It. hydronephrosis (Johanson) Voiding cystourethro- TUR gram and urethras-
strea:i.11
Urinary tract infection and unexplained lt. vesicoureteral reflux and hydronephrosis Difficulty in urination. Narrow low pressure urinary stream S-welling of penis and low pressure, narrow urinary stream
Urinary dribbling, enlarged bladder
copy Voiding cystourethrogram
Urinary extravasation; second urethral stric-
ture None
Open resection of valve and diverticulectomy
Extravasation
IVP, cystoscopy and urethroscopy
TUR
None
IVP and urethroscopy. Hydronephrosis
TUR
None
Voiding cystourethro- Open excision and digram and cystoverticulectomy and gram. Dilatation of loop ureterostomies ant. urethra. Azotemia and bilat. vesicoureteral reflux, tortuous ureters Voiding cystourethro- Loop cutaneous uregram and IVP. Diterostomies latation of ant. urethra. Bilat. dilated tortuous ureters
Penile fistula
None
Fm. 2. K. M. Retrograde urethrogram demonstrates anterior urethral valve (arrow) and urethral diverticulum.
All patients with urethral flow problems were carefully evaluated for abdominal masses and for adequacy of the distal urethral meatus. Subsequently, voiding cystourethrography and excretory urography (IVP) were performed. In this series 5 patients had bilateral hydroureteronephrosis (fig. 1) and one also had vesicoureteral reflux. The voiding demonstrated a mid-
.
-.-·. - --
".
--·' _-
-
---
><~--··-c~-------
patients (fig. 2). The proximal urethral dilatation and the site of the urethral valve were clearly demonstrated roentgenographically (fig. 3). In 3 cases voiding films were not obtained with the initial cystogram because these children had difficulty in initiating and maintaining the urinary stream. In such patients, the dysuria following catheterization makes voiding even more difficult. In 2 of these 3 in whom a urethrog;ram was not ob-
-- --~
,--·~·- -·'--•
~---------
.
- --~ ~-----
974
FIRLIT AND KING
Fm. 3. J. D. Voiding cystourethrogram illustrates proximal urethral dilatation and anterior urethral valve (arrows).
Fig. 4. B. R. Voiding cystourethrogram. Note markedly dilated proximal urethra secondary to anterior urethral valve.
tained, the diagnosis was also missed at initial cystoscopy. Bougie calibration of the urethra did not detect the valve. These 2 patients had objective signs of bladder outlet obstruction, that is marked trabeculation in 1 and trabeculation with reflux and residual urine in the other. Both patients underwent Y-V plasty of the bladder neck. Both proved to have secondary bladder neck hypertrophy occurring behind the more distal obstruction and, of course, neither patient was improved by the Y-V plasty. Voiding urethrograms could never be obtained in either boy. Consequently, the anterior urethral valve was not discovered in one until the
fourth cystoscopy and in the other until the third systematic evaluation. These experiences emphasize the need to keep anterior urethral valves in mind when evaluating boys with obstructive signs and symptoms and the necessity for voiding films during cystourethrography. Careful urethroscopy and examination of the anterior urethra should be performed, particularly when obstructive symptoms are present. In 2 patients anterior urethral valves were diagnosed during the first week of life. These infants were evaluated because of urinary dribbling and a narrow urinary stream. Bilateral hydroureteronephrosis and proximal urethral dilatation secondary to an anterior valve were found on IVP, retrograde pyelography and voiding cystourethrography (fig. 4). Both patients were treated initially by high loop cutaneous ureterostomy because of coexistent azotemia and dilated tortuous ureters. In such circumstances high diversion permits optimum renal growth and development and minimizes the risk of infection. The loop ureterostomies are closed after the valve has been removed and it is determined that the bladder can empty completely. The resectoscope is extremely valuable in precisely localizing the valve by the hanging-up effect of the extended loop on the urethral valve. Three patients were treated by transurethral resection of the valve alone, which is readily accomplished at the time of cystoscopy. Resection of the most distal portion of the flap is all that is necessary and a satisfactory result was obtained in each case. Two patients with anterior urethral valves and secondary urethral diverticula were treated by open diverticulectomy with excision of the valve. One additional patient had the valve excised by an open approach in combination with the first stage of a Johanson urethroplasty. In both patients in
ANTERIOR URETHRAL VALVES IN CHILDREN
whom primary excision of the anterior urethral valve and diverticula was done, urinary extravasation developed subsequently. In one the extravasation was due to a malfunctioning suprapubic tube that had to be replaced. In the other extravasation was due to intractable bladder spasms and resulted in a secondary urethral stricture.
'r
I
(
,
SUMMARY
Our primary purpose in describing these 7 patients with anterior urethral valves is to emphasize the presence of the entity, the extent of associated morbidity and the difficulties that may be encountered in arriving at the proper diagnosis. It is noteworthy that several patients underwent re-
I,-
975
peated hospitalizations owing to symptoms suggesting intravesical obstruction before the proper diagnosis was reached. In patients with obstructive symptoms careful attention should be paid to the anterior urethra on endoscopy and at voiding cystourethrography. Early diagnosis should minimize the morbidity associated with unnecessary or inappropriate surgical procedures and should considerably reduce the incidence of urinary tract infection. The specific treatment of the anterior urethral valve is transurethral resection and/or fulguration. When a urethral valve is associated with a diverticulum, open resection of the valve together with excision of the diverticulum, with suprapubic cystostomy for temporary diversion, 1s the recommended approach.