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Congenital Urethral Perineal Fistulas
109. IVIa.rch
Printed in
ilkin~; Co.
.S.A
CONGENITAL URETHRAL PERINEAL FISTULAS G. GRANT GEHRING, JACK H. VITENSON*
AND
DAVID M. WOODHEADt
From the Urology Service, Department of Surgery, Wilford Hall, United States Air Force Medical Center, Lackktnd Air Force Base, San Antonio, Texas
More than 100 cases of anterior urethral duplications have been reported.,_, Posterior urethrorectal fistula is uncommon and, when it occurs, it is usually in association with other anomalies of the rectum or anus. 4 Only 2 cases of congenital posterior urethroperineal fistula unassociated with anorectal anomalies have been documented.'· 6 Two additional cases of this entity are described herein. CASE REPORTS
Case 1. R. L., a 21-year-old white man, was admitted to the medical center in February 1971 for evaluation of urinary leakage from the perineum. Perinea! dribbling occurred during micturition and required voiding in a sitting position. At adolescence the patient was able to void upright without leakage by flexing the gluteal muscles. He experienced nocturnal and dribbling incontinence per perineum when he did not void frequently. There was no of genitourinary tract infections, hematuria, dysuria or impairment of sexual function. On physical examination there was a small opening· just lateral to the mid line in front of the anal verge. There were no rectal or anal abnormalities. A retrograde cystourethrogram revealed a fistulous communication from the floor of the prostatic urethra just in front of the verumontanum to the perineum (fig. 1). Excretory urography (IVP) was normal. On cystourethroscopy a small opening was observed just proximal to the verumontanum and to the right of the midline. Catheterization with a No. 4 ureteral catheter established this to be the opening of the fistulous communication in the urethra. This ostium was fulgurated with initial resolution of the symptoms but the perinea! incontinence recurred after 7 vveeks. Accepted for publication July
Case 2. L. F., a newborn white boy, was seen in August 1971 after a 2 cm. cystic perinea! mass was found from which urine could be expressed from a perinea! midline foramen (fig. 2, A and B). There were no anorectal developmental defects. Blood urea nitrogen was 13 mg. per cent, IVP revealed no abnormalities and a retrograde cystourethrogram was normal. On a retrograde fistulogram a posterior urethroperineal fistula was visualized. The small perinea! opening was surgically enlarged to provide improved drainage and the patient was discharged from the hospital. In November 1971 a retrograde cystourethrogram demonstrated persistence of the urethroperineal fistula, originating to the left of the verumontanum coursing anteriorly and ending as a dilation at its cutaneous termination (fig. 2, C). During cystourethroscopy methylene blue dye was injected into the perinea! fistulous opening and 1~as see·n to emerge on the left side of the verumontanum. The patient is to be returned to the hospital when he is 18 months old for perinea! excision of the fistulous tract. D!SCFSSION
Anterior urethral duplication presenting typically with ectopic dorsal urethra is frequently affected by urethritis in the accessory urethra but occasionally is seen with urinary obstructive symptoms. 1- 3 Incontinence is not present because the duplication occurs distal to the external sphincter. However, there may be post-micturitional dribbling and spraying of the urinary stream. In complete urethral duplication there is usually no associated incontinence because the accessory urethra is either too small in caliber to permit egress of urine or the external sphincter surrounds both passages. 1 - 3 Anterior urethral duplication is presumably secondary to anomalous union of the urethral folds or, in the case of f'nrn,nrs,,,o duplication, a continued division the urorectal septum with prolongation into the anterior urethra. 2 Posterior urethral usually occurs as a rectourethral fistula in association with imperforate anus or other rectal anomalies. This is due to the abnormal fusion of the urorectal septum with the proctodeum. 7 In the 2 cases of posterior urethroperineal fistulas previously reported and in our 2 cases
* Current address: 445 Prospect Hackensack, l'·J ew 076()1. Died 1972. ""~'"·~»ua, J., Batalla, J. L. and Fernandez, A.: Uretra accesoria dermoide perineal. Arch. Espan. Urol., 23: 147, 2 R. A.: Congenital anomalies of the urethra. Brit. J. 40: 1968. 3 Gross, R. E. and T. C.: Duplication of the urethra; report of 2 cases summary of the literature. Arch. Surg., 60: 749, 1950. 4 Le Due, E.: Congenital rectourethral fistula: report of case without rectal anomaly. J. Urol., 93: 272, 1965. 'Mitchell-Heggs, F. S.: A case of anal micturition. Proc. Roy. Soc. Med., 57: 552, 1964. 'Harrow, B. R.: Peri-anal micturition due to congenital posterior urethral fistula. J. Ural., 96: 328, 1966.
'Pegum, J.M., Loly, P. C. M. and Falkiner, N. M.: Development and classification of anorectal anomalies. Arch. Surg., 89: 481, 1964.
419
420
GEHRING, VITENSON AND WOODHEAD
symptoms consisted of dribbling incontinence from the ectopic opening. In none of these cases were there manifestations of urinary obstruction, infection or anorectal anomalies. Diagnosis is suggested by the history of drib-
FIG. 1. Case 1. Retrograde cystourethrogram reveals posterior urethroperineal fistula.
bling incontinence from the ectopic opening and can be established by urethrography, retrograde fistulography and retrograde injection of dye with concurrent urethroscopy. Treatment is directed toward the elimination of incontinence from the fistula. Initial conservative treatment by urethral fulguration of the identified posterior urethral foramen and concurrent curettage of the fistulous tract may be attempted. In case 1 this treatment was instituted with initial success but within 6 weeks incontinence recurred. Le Due•· 5 and Harrow• treated their patients with complete perineal excision of the fistulous tract. Neither reported impotence or stricture and the tracts were excised only to the prostatic capsule. Embryogenetically, the cloaca is normally divided by the urorectal folds into an anterior urogenital sinus and a posterior rectal segment. It has been postulated that rather than a cephalocaudal migration of the urorectal folds there is a migration of lateral ridges (urorectal folds) with fusion near the midline. 6 • 7 A partial defect in fusion or possible overlapping of the encroaching lateral ridges could, therefore, conceivably create an accessory urogenital sinus. Congenital ure-
FIG. 2. Case 2. A, perinea! cyst-like mass with urine leaking from perinea! opening. B, urine expressed from cyst in perineum. C, retrograde cystourethrogram reveals posterior urethroperineal fistula.
fistulas ~2&y then urethral folds have coalesced over this accessory sinus. The extreme of this embryoiogiducal error wouid also explain the plicated urethra occurring with a single normal bladder. Sl:MV!ARY
The third and fourth documented cases of congenital urethroperineai fistulas presented as perinea! dribbling incontinence and were confirmed by cystourethrogram, retrograde fistulogram and
of Nitt concurrent uretreatment includes fulguration of the fistulous tract or fistulectomy. The occurrence of this anomaly suggests 1) the urorectal folds develop as lateral ridges with fusion near midline, 2) anomalies of complete urethral duplication and urethral perinea! fistula are effected by abnormal fusion at the midline of these lateral ridges which results in an accessory urogenital sinus and 3) the accessory urogenital sinus induces development of the completely duplicated urethra or the urethral-perinea! fistula. 1
Printed in
ilkin~; Co.
.S.A
CONGENITAL URETHRAL PERINEAL FISTULAS G. GRANT GEHRING, JACK H. VITENSON*
AND
DAVID M. WOODHEADt
From the Urology Service, Department of Surgery, Wilford Hall, United States Air Force Medical Center, Lackktnd Air Force Base, San Antonio, Texas
More than 100 cases of anterior urethral duplications have been reported.,_, Posterior urethrorectal fistula is uncommon and, when it occurs, it is usually in association with other anomalies of the rectum or anus. 4 Only 2 cases of congenital posterior urethroperineal fistula unassociated with anorectal anomalies have been documented.'· 6 Two additional cases of this entity are described herein. CASE REPORTS
Case 1. R. L., a 21-year-old white man, was admitted to the medical center in February 1971 for evaluation of urinary leakage from the perineum. Perinea! dribbling occurred during micturition and required voiding in a sitting position. At adolescence the patient was able to void upright without leakage by flexing the gluteal muscles. He experienced nocturnal and dribbling incontinence per perineum when he did not void frequently. There was no of genitourinary tract infections, hematuria, dysuria or impairment of sexual function. On physical examination there was a small opening· just lateral to the mid line in front of the anal verge. There were no rectal or anal abnormalities. A retrograde cystourethrogram revealed a fistulous communication from the floor of the prostatic urethra just in front of the verumontanum to the perineum (fig. 1). Excretory urography (IVP) was normal. On cystourethroscopy a small opening was observed just proximal to the verumontanum and to the right of the midline. Catheterization with a No. 4 ureteral catheter established this to be the opening of the fistulous communication in the urethra. This ostium was fulgurated with initial resolution of the symptoms but the perinea! incontinence recurred after 7 vveeks. Accepted for publication July
Case 2. L. F., a newborn white boy, was seen in August 1971 after a 2 cm. cystic perinea! mass was found from which urine could be expressed from a perinea! midline foramen (fig. 2, A and B). There were no anorectal developmental defects. Blood urea nitrogen was 13 mg. per cent, IVP revealed no abnormalities and a retrograde cystourethrogram was normal. On a retrograde fistulogram a posterior urethroperineal fistula was visualized. The small perinea! opening was surgically enlarged to provide improved drainage and the patient was discharged from the hospital. In November 1971 a retrograde cystourethrogram demonstrated persistence of the urethroperineal fistula, originating to the left of the verumontanum coursing anteriorly and ending as a dilation at its cutaneous termination (fig. 2, C). During cystourethroscopy methylene blue dye was injected into the perinea! fistulous opening and 1~as see·n to emerge on the left side of the verumontanum. The patient is to be returned to the hospital when he is 18 months old for perinea! excision of the fistulous tract. D!SCFSSION
Anterior urethral duplication presenting typically with ectopic dorsal urethra is frequently affected by urethritis in the accessory urethra but occasionally is seen with urinary obstructive symptoms. 1- 3 Incontinence is not present because the duplication occurs distal to the external sphincter. However, there may be post-micturitional dribbling and spraying of the urinary stream. In complete urethral duplication there is usually no associated incontinence because the accessory urethra is either too small in caliber to permit egress of urine or the external sphincter surrounds both passages. 1 - 3 Anterior urethral duplication is presumably secondary to anomalous union of the urethral folds or, in the case of f'nrn,nrs,,,o duplication, a continued division the urorectal septum with prolongation into the anterior urethra. 2 Posterior urethral usually occurs as a rectourethral fistula in association with imperforate anus or other rectal anomalies. This is due to the abnormal fusion of the urorectal septum with the proctodeum. 7 In the 2 cases of posterior urethroperineal fistulas previously reported and in our 2 cases
* Current address: 445 Prospect Hackensack, l'·J ew 076()1. Died 1972. ""~'"·~»ua, J., Batalla, J. L. and Fernandez, A.: Uretra accesoria dermoide perineal. Arch. Espan. Urol., 23: 147, 2 R. A.: Congenital anomalies of the urethra. Brit. J. 40: 1968. 3 Gross, R. E. and T. C.: Duplication of the urethra; report of 2 cases summary of the literature. Arch. Surg., 60: 749, 1950. 4 Le Due, E.: Congenital rectourethral fistula: report of case without rectal anomaly. J. Urol., 93: 272, 1965. 'Mitchell-Heggs, F. S.: A case of anal micturition. Proc. Roy. Soc. Med., 57: 552, 1964. 'Harrow, B. R.: Peri-anal micturition due to congenital posterior urethral fistula. J. Ural., 96: 328, 1966.
'Pegum, J.M., Loly, P. C. M. and Falkiner, N. M.: Development and classification of anorectal anomalies. Arch. Surg., 89: 481, 1964.
419
420
GEHRING, VITENSON AND WOODHEAD
symptoms consisted of dribbling incontinence from the ectopic opening. In none of these cases were there manifestations of urinary obstruction, infection or anorectal anomalies. Diagnosis is suggested by the history of drib-
FIG. 1. Case 1. Retrograde cystourethrogram reveals posterior urethroperineal fistula.
bling incontinence from the ectopic opening and can be established by urethrography, retrograde fistulography and retrograde injection of dye with concurrent urethroscopy. Treatment is directed toward the elimination of incontinence from the fistula. Initial conservative treatment by urethral fulguration of the identified posterior urethral foramen and concurrent curettage of the fistulous tract may be attempted. In case 1 this treatment was instituted with initial success but within 6 weeks incontinence recurred. Le Due•· 5 and Harrow• treated their patients with complete perineal excision of the fistulous tract. Neither reported impotence or stricture and the tracts were excised only to the prostatic capsule. Embryogenetically, the cloaca is normally divided by the urorectal folds into an anterior urogenital sinus and a posterior rectal segment. It has been postulated that rather than a cephalocaudal migration of the urorectal folds there is a migration of lateral ridges (urorectal folds) with fusion near the midline. 6 • 7 A partial defect in fusion or possible overlapping of the encroaching lateral ridges could, therefore, conceivably create an accessory urogenital sinus. Congenital ure-
FIG. 2. Case 2. A, perinea! cyst-like mass with urine leaking from perinea! opening. B, urine expressed from cyst in perineum. C, retrograde cystourethrogram reveals posterior urethroperineal fistula.
fistulas ~2&y then urethral folds have coalesced over this accessory sinus. The extreme of this embryoiogiducal error wouid also explain the plicated urethra occurring with a single normal bladder. Sl:MV!ARY
The third and fourth documented cases of congenital urethroperineai fistulas presented as perinea! dribbling incontinence and were confirmed by cystourethrogram, retrograde fistulogram and
of Nitt concurrent uretreatment includes fulguration of the fistulous tract or fistulectomy. The occurrence of this anomaly suggests 1) the urorectal folds develop as lateral ridges with fusion near midline, 2) anomalies of complete urethral duplication and urethral perinea! fistula are effected by abnormal fusion at the midline of these lateral ridges which results in an accessory urogenital sinus and 3) the accessory urogenital sinus induces development of the completely duplicated urethra or the urethral-perinea! fistula. 1