CONGENITAL URETHRAL FISTULA (Case Report) Lt Col MM HARJAI ., Lt Col RK BAL +, Brig KK MAUDAR # MJAFI 1998; 54 : 369-370 KEY WORDS: Anorectal malformations; Urethrocutaneous fistula; Hypospadias.
Introduction
M
ale congenital urethral fistula is most commonly seen in association with anorectal malformations [I]. Congenital posterior urethro-perineal fistula as a variant of urethral duplication is a relatively more common type of fistula. Isolated congenital urethral fistula is very rare with less than 20 cases reported in the available literature. We report here such a case and discuss its management. Case report
A three year old boy was admitted with complaints of passage of urine from the undersurface of the penis as well as from the tip since birth. He had no history of an injury or operations in the past. Physically. the patient had a patent distal urethra and a normal prepuce. There was no chordee. The urethra distal to the fistula was surrounded by corpus spongiosum. An opening was present on the ventral aspect of the midshall of the penis. A cathter could be passed through the fistula and out of the normally situated meatus at glans. Both testicles were in the scrotum. There were no other associated anomalies (Fig I). Abdominal ultrasonography and micturating cystourethrogram were normal.
The fistula was closed with a preputial onla)' flap. There was abundant tissue present to form an additional layer before skin closure. A small silicon catheter was lell in the bladder for drainage. The catheter was removed on the fillh postoperative day. The patient is doing well without recurrence.
Discussion Isolated congenital fistula of the penile urethra without hypospadias or chordee is very rare and hence even senior residents may misdiagnose it as hypospadias without chordee. The urethra develops from the caudal end of the urogenital sinus. Olbourne suggests that the formation of congenital urethral fistula is due to a focal or temporary defect in the urethral plate, which prevents fusion of the urethral folds [2]. The distal glandular penile urethra is normal as it develops from surface ectodermal invagination [3]. Karnak et al regarded congenital urethrocutaneous fistula (excluding that associated with anorectal malformation and true duplication) as one set of anomalies [4]. Before planning the surgical approach, it is important to exclude urethral duplication, which produces the Y type fistula with different management implications. Treatment of congenital urethral fistula depends on the associated penile abnormality and fistula location. If the urethra beyond the fistula is congenitally defective, then simple closure is usually unsuccessful and the fistula is likely to recur [5]. If adequate lateral skin is present, repair can be carried out as one would perform a second stage Johanson urethroplasty. Otherwise an onlay flap can be used.
Fig. I: Congenital uretheral fistula located on the ventral mid penile shall with catheter exiting through fistula and normal meatus at glans
Thus, the congenital urethral fistula is an extremely rare but easily manageable anomaly which might readily be confused with hypopospadias. An awareness of the entity will avoid the complicated management.
• Rcadcr and Pcdiatric surgcon. + Classificd spccialist Surgcr)' and Pediatric surgery. II Consultant. Profcssor and Head & Pediatric surgeon. Department of Surgery. Armed Forces Medical College and Command Hospital (Southern Command). Pune-40.
370 REFERENCES I. Stephens FD. Congenital Malfonnations of the Urinary Tract. New York: Praeger Publishers. 1983.
2. Olbourne NA. Congenital urethral fistula. Plast Reconstr Surg 1976;57:237-8.
Harjai. Bal and Maudar 4. Karnak I. Tanyel FC. Hicsonmez A. Congenital urethraeutaneous fistula: a case report and literature review. with a nomenclature proposal. J Pediatr Surg 1995;30: 1504-5. 5. Maarafie A. Azmy AF. Congenital fistula of the penile urethra. Dr J Uroll997;79:814.
3. Ritchy ML. Sinha A. Argueso L. Congenital fistula of the penile urethra. J Urol 1994;151 :1061-2.
MJAH Vol. 5-1. N,:-. -I. /99H
Introduction
M
ale congenital urethral fistula is most commonly seen in association with anorectal malformations [I]. Congenital posterior urethro-perineal fistula as a variant of urethral duplication is a relatively more common type of fistula. Isolated congenital urethral fistula is very rare with less than 20 cases reported in the available literature. We report here such a case and discuss its management. Case report
A three year old boy was admitted with complaints of passage of urine from the undersurface of the penis as well as from the tip since birth. He had no history of an injury or operations in the past. Physically. the patient had a patent distal urethra and a normal prepuce. There was no chordee. The urethra distal to the fistula was surrounded by corpus spongiosum. An opening was present on the ventral aspect of the midshall of the penis. A cathter could be passed through the fistula and out of the normally situated meatus at glans. Both testicles were in the scrotum. There were no other associated anomalies (Fig I). Abdominal ultrasonography and micturating cystourethrogram were normal.
The fistula was closed with a preputial onla)' flap. There was abundant tissue present to form an additional layer before skin closure. A small silicon catheter was lell in the bladder for drainage. The catheter was removed on the fillh postoperative day. The patient is doing well without recurrence.
Discussion Isolated congenital fistula of the penile urethra without hypospadias or chordee is very rare and hence even senior residents may misdiagnose it as hypospadias without chordee. The urethra develops from the caudal end of the urogenital sinus. Olbourne suggests that the formation of congenital urethral fistula is due to a focal or temporary defect in the urethral plate, which prevents fusion of the urethral folds [2]. The distal glandular penile urethra is normal as it develops from surface ectodermal invagination [3]. Karnak et al regarded congenital urethrocutaneous fistula (excluding that associated with anorectal malformation and true duplication) as one set of anomalies [4]. Before planning the surgical approach, it is important to exclude urethral duplication, which produces the Y type fistula with different management implications. Treatment of congenital urethral fistula depends on the associated penile abnormality and fistula location. If the urethra beyond the fistula is congenitally defective, then simple closure is usually unsuccessful and the fistula is likely to recur [5]. If adequate lateral skin is present, repair can be carried out as one would perform a second stage Johanson urethroplasty. Otherwise an onlay flap can be used.
Fig. I: Congenital uretheral fistula located on the ventral mid penile shall with catheter exiting through fistula and normal meatus at glans
Thus, the congenital urethral fistula is an extremely rare but easily manageable anomaly which might readily be confused with hypopospadias. An awareness of the entity will avoid the complicated management.
• Rcadcr and Pcdiatric surgcon. + Classificd spccialist Surgcr)' and Pediatric surgery. II Consultant. Profcssor and Head & Pediatric surgeon. Department of Surgery. Armed Forces Medical College and Command Hospital (Southern Command). Pune-40.
370 REFERENCES I. Stephens FD. Congenital Malfonnations of the Urinary Tract. New York: Praeger Publishers. 1983.
2. Olbourne NA. Congenital urethral fistula. Plast Reconstr Surg 1976;57:237-8.
Harjai. Bal and Maudar 4. Karnak I. Tanyel FC. Hicsonmez A. Congenital urethraeutaneous fistula: a case report and literature review. with a nomenclature proposal. J Pediatr Surg 1995;30: 1504-5. 5. Maarafie A. Azmy AF. Congenital fistula of the penile urethra. Dr J Uroll997;79:814.
3. Ritchy ML. Sinha A. Argueso L. Congenital fistula of the penile urethra. J Urol 1994;151 :1061-2.
MJAH Vol. 5-1. N,:-. -I. /99H
