Congenital f3yA.R.
Urethral Fistula With Normal Anus: A Report of Two Cases
Hong, D.P. Croitoru,
L.T. Nguyen, J.-M. Laberge, Montreal, Quebec
l Congenital rectourethral or anourethral fistulae without imperforate anus in males are rare, representing less than 1% of anorectal malformations. We report our experience with two males with “N type” urethral fistulae. One, a B-year-old boy, presented with recurrent urinary tract infections (UTls) and passage of urine per anus. Investigations included a voiding cystourethrogram (VCUG), which demonstrated a fistula from the urethra to the anus. On physical examination, a small perianal opening was noted just outside the anus, which drained a small amount of urine after voiding. The fistula was excised via a perineal approach. The second patient is a 5-year-old boy with a long history of recurrent UTI requiring multiple hospitalizations since the newborn period. Chronic renal failure developed as a complication of repeated urinary tract infections. Investigations showed a single hydronephrotic pelvic kidney and a small bladder. He underwent numerous diagnostic and reconstructive procedures including cystoscopy and augmentation cystoplasty. Recurrent infections continued and an N type anourethral fistula was eventually diagnosed. The fistula was located between the anal canal and the membranous urethra. An anterior perineal approach was also used. Roth fistulae were easily located, and reconstructive surgery of the urethra was not required. Postoperative VCUGs in both patients were normal. They have been free of infection with normal urinary continence since resection of the fistula. Congenital N type anourethral fistulae are rare, but should be considered in cases of recurrent urinary tract infections. The diagnosis may be missed by endoscopic procedures, but VCUG should demonstrate the fistulous tract. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Fistula, anourethral, tal, N type, H-type.
rectourethral,
congeni-
A
Y. Homsy,
and G.H. Kiruluta
CASE
REPORTS
Case 1 A 5-year-old boy presented with a 2-year history of passing urine via the anus. He had had two urinary tract infections (UTIs) during that time. A voiding cystourethrogram (VCUG) showed a fistulous communication originating from the posterior urethra and going toward the anorectum (Fig 1). On careful examination of the perineum, a small opening in the anoderm was noted approximately 0.5 cm below the anocutaneous junction (Fig 2). A small amount of urine could be expressed from this opening after the patient had voided. A sinogram was performed, and showed the fistulous tract entering the membranous urethra. The patient’s past medical history was unremarkable except for the UTIs. There were no symptoms of constipation or diarrhea. The anus was normal in position, size, and sphincter tone. Examination of the genitalia was also normal. During urethrocystoscopy methylene blue was injected from the perianal opening and dye was observed coming out of the membranous urethra. The fistula was dissected and excised via an anterior perineal approach, with suture ligation of the tract adjacent to the urethra. The patient’s postoperative course was unremarkable and he is currently asymptomatic after 15 months. Bowel movements are normal, and there has been no recurrent infection or fistula. Histological examination of the specimen showed a tract lined with stratified squamous epithelium with chronic inflammation (Fig 3). Case
2
The second patient came to the attention of the pediatric surgery service at the age of 4 years. He had a history of recurrent UTIs since the newborn period that required multiple hospitalizations and numerous visits to the emergency department. During that time, an extensive workup of his urinary tract was obtained. Investigations showed a single pelvic kidney with short double ureters.
BNORMAL connections between the anorectum and the urethra in males and the anorectum and the vagina in females are usually associated with imperforate anus. Fistulae associated with normal anal anatomy have been reported almost exclusively in females.1*2 We describe two male patients with urethral fistulae without any other anorectal malformation. Diagnosis was delayed in both patients, with consequent disastrous results in the second.
From the Depattments of Pediatric General Surgery and Pediatric Urology, Montreal Children’s Hospital, McGill University, Montreal, Quebec. Presented at the 23rd Annual Meeting ofthe Canadian Association of Paediatric Surgeons, Quebec City, Quebec, September 19-22, 1991. Address reprint requests to L.T. Nguyen, MD, Department of Pediatric General Surgery The Montreal Children’s Hospital, 2300 Tupper St, Montreal, Quebec H3H IP3, Canada. Copyright o 1992 by W. B. Saunders Company 0022-3468192/2710-0005$03.00l0 1278
Fig 1. Patient 1. VCUG at time of diagnosis. Journal of Pediatric Surgery,
Vol 27,No
10 (October),
1992: up
1278-l 280
CONGENITAL
Fig 2.
URETHRAL FISTULA WITH NORMAL
ANUS
1279
Patient 1. Small perianal opening (arrow) of urethral fistula.
He underwent numerous diagnostic tests including several VCUGs and urethrocystoscopies. He developed chronic renal failure because of recurrent UTIs. A gastrocystoplasty was performed with the goal of improving bladder compliance. Postoperatively, the patient had fever and positive urine cultures. Findings on an abdominal computed tomography scan suggested an intraabdominal abscess and he underwent exploratory laparotomy. No abscess was found at the time of operation. Before surgery. a Foley catheter was inserted but the balloon could not be palpated in the bladder once the abdomen was opened. Further examination after closure showed the balloon to be in the rectum (Fig 4). Once the patient had recovered from his operation, a repeat VCUG was performed, and a fistula was demonstrated between the membranous urethra and the anus 1.5 cm above the dentate line (Fig 5). A review of the VCUG that had been obtained in the newborn period showed that contrast present in both the bladder and the rectum, but the significance of this finding had not been realized (Fig 6). At the time of repair, an 8F cystoscope was easily passed from the urethra through the short fistula into the rectum. An anterior perineal approach was used to excise the fistula and repair the urethra and the recta1 wall. The patient’s recovery was uneventful and he also remains without recurrent infection or fistula 9 months after surgery. Microscopy of the specimen revealed a fistulous tract lined with fibrinous material, with anal mucosa at one end and transitional epithelium at the other (Fig 7).
Fig 3.
Patient 1. Photomicrograph
of operative specimen
Fig 4. Patient 2. Foley catheter going through fistula and exiting from rectum.
DISCUSSION
Fistulae between the anus and the urethra in association with a normal anus are rare.133 In males, these malformations have been called N type or I-I type anourethral fistulae.4J Brem et al6 recently reviewed the literature and found a total of 21 cases of anourethral fistula in males. Of these, 14 had normal anal anatomy and 7 had mild anal anomalies. The diagnosis of an anourethral fistula can be difficult to make in these cases. Patients can present at birth with meconium in the urine, or later in life
Fig 5. Patient 2. VCUG at 5 years of age showing short urethroanal fistule (arrow).
HONG ET AL
1280
Fig 7. Patient 2. Photomicrograph of specimen showing part of fistulous tract and transitional epithelium.
Fig 6. Patient 2. VCUG from newborn period. Contrast can be seen in both the bladder (b) and the rectum (arrow).
with complaints of passage of urine per rectum or recurrent UTI. Careful physical examination is important. The perianal opening in our first patient was missed for severa months even after the VCUG had demonstrated the fistula. The most useful diagnostic test is a VCUG, which should demonstrate the fistula between the proximal urethra and the low anus. In most cases, if the fistula is not associated with other
anomalies of the urethra, excision of the fistula with primary repair of the urethra via an anterior perineal approach is possible.5 A temporary diverting colostomy might be necessary in those cases in which the intestinal opening is high in the rectum. In our review of the literature we have not found any cases similar to our patient with the urethralperianal fistula. The tract cannot be considered a urethral duplication because of the squamous epithelial lining. Stephens4 and more recently van der Putte,’ have offered embryological theories for these anomalies. Our two cases illustrate that N type anourethral fistulae can be easily missed, sometimes with disastrous consequences. A thorough investigation is indicated in any male with symptoms of recurrent UTIs or abnormal urination. ACKNOWLEDGMENT The authors thank Dr Ian Neilson, former surgical fellow, for providing the slide for Fig 2, and Dr Haj-Shahine, Department of Pathology, for reviewing the pathology and providing the photomicrographs.
REFERENCES 1. Chattejee SK: Double termination of the alimentary tract: A second look. J Pediatr Surg 15:623-627,198O 2. Tsuchida Y, Saito S, Honna T, et al: Double termination of the alimentary tract in females: A report of 12 cases and a literature review. J Pediatr Surg 19:292-296,1984 3. Smith ED: The bath water needs changing, but don’t throw out the baby: An overview of anorectal anomalies. J Pediatr Surg 22:335-348,1987
4. Stephens FD, Donnellan WL: “H-type” urethroanal fistula. J Pediatr Surg 12:95-102,1977 5. White JJ, Haller JA, Scott JR Jr, et al: N type anorectal malformations. J Pediatr Surg 13:631-637,1978 6. Brem H, Guttman FM, Laberge J-M, et al: Congenital anal fistula with normal anus. J Pediatr Surg 24~183-1851989 7. van der Putte SCT: Normal and abnormal development of the anorectum. J Pediatr Surg 21:434-440,1986
Urethral Fistula With Normal Anus: A Report of Two Cases
Hong, D.P. Croitoru,
L.T. Nguyen, J.-M. Laberge, Montreal, Quebec
l Congenital rectourethral or anourethral fistulae without imperforate anus in males are rare, representing less than 1% of anorectal malformations. We report our experience with two males with “N type” urethral fistulae. One, a B-year-old boy, presented with recurrent urinary tract infections (UTls) and passage of urine per anus. Investigations included a voiding cystourethrogram (VCUG), which demonstrated a fistula from the urethra to the anus. On physical examination, a small perianal opening was noted just outside the anus, which drained a small amount of urine after voiding. The fistula was excised via a perineal approach. The second patient is a 5-year-old boy with a long history of recurrent UTI requiring multiple hospitalizations since the newborn period. Chronic renal failure developed as a complication of repeated urinary tract infections. Investigations showed a single hydronephrotic pelvic kidney and a small bladder. He underwent numerous diagnostic and reconstructive procedures including cystoscopy and augmentation cystoplasty. Recurrent infections continued and an N type anourethral fistula was eventually diagnosed. The fistula was located between the anal canal and the membranous urethra. An anterior perineal approach was also used. Roth fistulae were easily located, and reconstructive surgery of the urethra was not required. Postoperative VCUGs in both patients were normal. They have been free of infection with normal urinary continence since resection of the fistula. Congenital N type anourethral fistulae are rare, but should be considered in cases of recurrent urinary tract infections. The diagnosis may be missed by endoscopic procedures, but VCUG should demonstrate the fistulous tract. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Fistula, anourethral, tal, N type, H-type.
rectourethral,
congeni-
A
Y. Homsy,
and G.H. Kiruluta
CASE
REPORTS
Case 1 A 5-year-old boy presented with a 2-year history of passing urine via the anus. He had had two urinary tract infections (UTIs) during that time. A voiding cystourethrogram (VCUG) showed a fistulous communication originating from the posterior urethra and going toward the anorectum (Fig 1). On careful examination of the perineum, a small opening in the anoderm was noted approximately 0.5 cm below the anocutaneous junction (Fig 2). A small amount of urine could be expressed from this opening after the patient had voided. A sinogram was performed, and showed the fistulous tract entering the membranous urethra. The patient’s past medical history was unremarkable except for the UTIs. There were no symptoms of constipation or diarrhea. The anus was normal in position, size, and sphincter tone. Examination of the genitalia was also normal. During urethrocystoscopy methylene blue was injected from the perianal opening and dye was observed coming out of the membranous urethra. The fistula was dissected and excised via an anterior perineal approach, with suture ligation of the tract adjacent to the urethra. The patient’s postoperative course was unremarkable and he is currently asymptomatic after 15 months. Bowel movements are normal, and there has been no recurrent infection or fistula. Histological examination of the specimen showed a tract lined with stratified squamous epithelium with chronic inflammation (Fig 3). Case
2
The second patient came to the attention of the pediatric surgery service at the age of 4 years. He had a history of recurrent UTIs since the newborn period that required multiple hospitalizations and numerous visits to the emergency department. During that time, an extensive workup of his urinary tract was obtained. Investigations showed a single pelvic kidney with short double ureters.
BNORMAL connections between the anorectum and the urethra in males and the anorectum and the vagina in females are usually associated with imperforate anus. Fistulae associated with normal anal anatomy have been reported almost exclusively in females.1*2 We describe two male patients with urethral fistulae without any other anorectal malformation. Diagnosis was delayed in both patients, with consequent disastrous results in the second.
From the Depattments of Pediatric General Surgery and Pediatric Urology, Montreal Children’s Hospital, McGill University, Montreal, Quebec. Presented at the 23rd Annual Meeting ofthe Canadian Association of Paediatric Surgeons, Quebec City, Quebec, September 19-22, 1991. Address reprint requests to L.T. Nguyen, MD, Department of Pediatric General Surgery The Montreal Children’s Hospital, 2300 Tupper St, Montreal, Quebec H3H IP3, Canada. Copyright o 1992 by W. B. Saunders Company 0022-3468192/2710-0005$03.00l0 1278
Fig 1. Patient 1. VCUG at time of diagnosis. Journal of Pediatric Surgery,
Vol 27,No
10 (October),
1992: up
1278-l 280
CONGENITAL
Fig 2.
URETHRAL FISTULA WITH NORMAL
ANUS
1279
Patient 1. Small perianal opening (arrow) of urethral fistula.
He underwent numerous diagnostic tests including several VCUGs and urethrocystoscopies. He developed chronic renal failure because of recurrent UTIs. A gastrocystoplasty was performed with the goal of improving bladder compliance. Postoperatively, the patient had fever and positive urine cultures. Findings on an abdominal computed tomography scan suggested an intraabdominal abscess and he underwent exploratory laparotomy. No abscess was found at the time of operation. Before surgery. a Foley catheter was inserted but the balloon could not be palpated in the bladder once the abdomen was opened. Further examination after closure showed the balloon to be in the rectum (Fig 4). Once the patient had recovered from his operation, a repeat VCUG was performed, and a fistula was demonstrated between the membranous urethra and the anus 1.5 cm above the dentate line (Fig 5). A review of the VCUG that had been obtained in the newborn period showed that contrast present in both the bladder and the rectum, but the significance of this finding had not been realized (Fig 6). At the time of repair, an 8F cystoscope was easily passed from the urethra through the short fistula into the rectum. An anterior perineal approach was used to excise the fistula and repair the urethra and the recta1 wall. The patient’s recovery was uneventful and he also remains without recurrent infection or fistula 9 months after surgery. Microscopy of the specimen revealed a fistulous tract lined with fibrinous material, with anal mucosa at one end and transitional epithelium at the other (Fig 7).
Fig 3.
Patient 1. Photomicrograph
of operative specimen
Fig 4. Patient 2. Foley catheter going through fistula and exiting from rectum.
DISCUSSION
Fistulae between the anus and the urethra in association with a normal anus are rare.133 In males, these malformations have been called N type or I-I type anourethral fistulae.4J Brem et al6 recently reviewed the literature and found a total of 21 cases of anourethral fistula in males. Of these, 14 had normal anal anatomy and 7 had mild anal anomalies. The diagnosis of an anourethral fistula can be difficult to make in these cases. Patients can present at birth with meconium in the urine, or later in life
Fig 5. Patient 2. VCUG at 5 years of age showing short urethroanal fistule (arrow).
HONG ET AL
1280
Fig 7. Patient 2. Photomicrograph of specimen showing part of fistulous tract and transitional epithelium.
Fig 6. Patient 2. VCUG from newborn period. Contrast can be seen in both the bladder (b) and the rectum (arrow).
with complaints of passage of urine per rectum or recurrent UTI. Careful physical examination is important. The perianal opening in our first patient was missed for severa months even after the VCUG had demonstrated the fistula. The most useful diagnostic test is a VCUG, which should demonstrate the fistula between the proximal urethra and the low anus. In most cases, if the fistula is not associated with other
anomalies of the urethra, excision of the fistula with primary repair of the urethra via an anterior perineal approach is possible.5 A temporary diverting colostomy might be necessary in those cases in which the intestinal opening is high in the rectum. In our review of the literature we have not found any cases similar to our patient with the urethralperianal fistula. The tract cannot be considered a urethral duplication because of the squamous epithelial lining. Stephens4 and more recently van der Putte,’ have offered embryological theories for these anomalies. Our two cases illustrate that N type anourethral fistulae can be easily missed, sometimes with disastrous consequences. A thorough investigation is indicated in any male with symptoms of recurrent UTIs or abnormal urination. ACKNOWLEDGMENT The authors thank Dr Ian Neilson, former surgical fellow, for providing the slide for Fig 2, and Dr Haj-Shahine, Department of Pathology, for reviewing the pathology and providing the photomicrographs.
REFERENCES 1. Chattejee SK: Double termination of the alimentary tract: A second look. J Pediatr Surg 15:623-627,198O 2. Tsuchida Y, Saito S, Honna T, et al: Double termination of the alimentary tract in females: A report of 12 cases and a literature review. J Pediatr Surg 19:292-296,1984 3. Smith ED: The bath water needs changing, but don’t throw out the baby: An overview of anorectal anomalies. J Pediatr Surg 22:335-348,1987
4. Stephens FD, Donnellan WL: “H-type” urethroanal fistula. J Pediatr Surg 12:95-102,1977 5. White JJ, Haller JA, Scott JR Jr, et al: N type anorectal malformations. J Pediatr Surg 13:631-637,1978 6. Brem H, Guttman FM, Laberge J-M, et al: Congenital anal fistula with normal anus. J Pediatr Surg 24~183-1851989 7. van der Putte SCT: Normal and abnormal development of the anorectum. J Pediatr Surg 21:434-440,1986
