Misdiagnosis of Congenital Posterior Urethroperineal Fistula and Comparison With Urethral Duplications and Rectourethral Fistula.

Congenital posterior urethroperineal fistula (CUPF) is a urothelium-lined tract between the posterior urethra and perineum. This condition is rare and has been proposed to be a urethral duplication variant. A case of CUPF that was misdiagnosed and surgically treated as a rectourethral fistula is presented. The clinical presentation, diagnosis, and treatment of CUPF are discussed and compared with those of Y-type urethral duplications and H-type rectourethral fistulas.

Congenital anterior urethrocutaneous fistula: A systematic review.

Congenital anterior urethrocutaneous fistula (CAUF) is a rare anomaly characterized by fistulization of penile urethra to skin. It's usually seen as an isolated deformity or may accompany genitourinary or anorectal malformations. We aim to define the common properties of patients mentioned in literatures by systematic review. A comprehensive search of PubMed, Embase, Web of Science, and Cochrane Library was performed including cross-referencing independently by two assessors. Selections were restricted to human studies in English. Based on the systematic review, 63 patients in 34 articles were included in the study. Most common fistula site was subcoronal in 29 (46.0%) patients. Chordee was in 8 (14.5%) and associated genitourinary anomaly was detected in 19 (30.2%) of patients. Fistula recurrence ratio was 6/59 (11.3%) using different surgical techniques and 3/6 was closed spontaneously. CAUF is frequently located in subcoronal level and usually an intact urethra distal to it. Success rates are high with the principles of hypospadias surgery.

Laparoscopic dissection and division of distal fistula in boys with rectourethral fistula.

BACKGROUND: Congenital rectourethral fistula (RUF) is the most common form of anorectal malformations found in boys. The aim of this study is to review our experience with dissection and division of distal fistula using laparoscopic surgery in the management of RUF, especially rectourethral bulbar fistula. METHODS: One hundred and two consecutive boys with congenital RUF who underwent conventional or single-incision laparoscopic surgery between July 2008 and June 2015 were enrolled in the study. The dissection of the distal fistula was performed along submucosal layer to a level 0.5 cm proximal to the urethra. Rectal mucosa of the fistula was dissected to the distal most point and completely transected flush with the posterior urethra. The residual muscular cuff was ligated with Hem-o-Lock clip or 5-0 PDS suture. Voiding cystourethrography and pelvic magnetic resonance imaging were performed at 3 mo, 6 mo, and 1 y postoperatively. RESULTS: All patients successfully underwent laparoscopic surgery without conversion. The mean age at the time of operation was 4.3 ± 2.9 mo. The operative times for the rectoprostatic fistula and rectobulbar fistula were similar (118.2 versus 119.4 min, P = 0.082). There was no significant difference in average operative time between conventional laparoscopic surgery group and single-incision laparoscopic surgery group (118.8 versus 119.1 min, P = 0.281). There was no injury to the urethra or vas deferens. The urethral catheter was removed on postoperative day 10. All patients were followed up. The median follow-up period was 3.3 ± 1.8 y. No recurrent fistula or urethral diverticulum was detected on voiding cystourethrography and pelvic MRI at 1 y. CONCLUSIONS: Submucosal dissection and division of distal fistula using a laparoscopic approach is safe, feasible, and effective for congenital RUF, especially bulbar fistula, in boys.

Rare association of congenital penile urethrocutaneous fistula with Y-type urethral duplication.

A 24-year-old man presented with symptoms of difficulty in passage of urine, mixed with gas and stool from penoscrotal junction since birth. He had a history of surgery (posterior sagittal anorectoplasty) for imperforate anus in first week of his life. On physical examination, external meatus was adequate with small 5×5 mm fistulous opening noted at penoscrotal junction with normal anal tone and opening. On evaluation with retrograde urethrography, voiding cystourethrogram, cystoscopy and barium enema, he was found to have penile urethrocutaneous fistula of 0.5×0.5 cm with Y-type incomplete urethral duplication. Urethroanal fistula (Y-type urethral duplication) was of small size (<5 mm), so closed primarily through perineal route with interposition of ischiorectal fat. Penile urethrocutaneous fistula (4 mm) too was closed primarily and covered with tunica vaginalis flap in single sitting. It has been 6 months since surgery and the patient is fully continent and voiding well with no urinary or faecal leak.

Congenital anterior urethrocutaneous fistula: 3 new cases and review of literature.

OBJECTIVE: Congenital anterior urethrocutaneous fistula, is a rare anomaly characterised by fistulisation of penile urethra to skin and presence of a concomitant normal or hypospadiac external urethral meatus. It may be seen as an isolated anomaly or may accompany genitourinary or anorectal malformations. We aim to present 3 new cases and define the common properties of patients stated in literature. METHOD: Information of 3 patients aged 2, 3 and 6 with this diagnosis were reviewed retrospectively and features of 51 patients in 25 articles with literature search. RESULTS: From the patients we operated, 2 had midpenile and 1 had subcoronal fistula. Urethral meatus was at tip of glans in all with 1 stenotic meatus. Two-layered primary repair was performed in 3 patients and deep ventral incision on urethral plate with meatotomy were added to fistula repair in one with stenotic meatus. Fistula recurred in this patient but resolved spontaneously after dilatations. In literature, most common fistula site was subcoronal in 27 (52.9%). Hypospadias was in 11.8% and associated genitourinary anomaly was detected in 21.5% of patients. Fistula recurrence ratio was 7.8% using different surgical techniques. CONCLUSION: Congenital anterior urethrocutaneous fistula is frequently located in subcoronal level and usually a normal urethra distal to it. Physical examination is important to detect additional anomalies. Success rates are high with primary repair techniques.

Congenital anterior urethrocutaneous fistula at the penoscrotal junction with proximal penile megalourethra: A case report.

Congenital anterior urethrocutaneous fistula and megalourethra are both rare anomalies. These anomalies are commonly associated with other anorectal or genitourinary anomalies and evaluated with voiding cystourethrography. We examined a 34-month-old boy who presented with a fistula at the penoscrotal junction. A voiding cystourethrogram showed a jet of urine coming through the fistula and proximal saccular dilatation of the penile urethra. We present the imaging findings of the first case of an association between a congenital anterior urethrocutaneous fistula at the penoscrotal junction and a proximal penile megalourethra. We also discuss the etiology, management, and differential diagnosis of this entity, and review the literature.

Imperforate anus with rectopenile fistula: a case report and systematic review of the literature.

BACKGROUND: Although anorectal malformations (ARMs) are frequently encountered, rare variants difficult to classify have been reported. METHODS: This study describes a patient with ARM and rectopenile fistula. The literature was reviewed systematically to assess the anatomical characteristics, clinical presentations and operations of this rare type of ARM. RESULTS: Eight patients were reported in the six included articles. In three patients, the fistula extended from the rectum to the anterior urethra without communication with the skin. In one patient, the fistula, located deep in corpus spongiosum, opened to the ventral aspect of the penis without communication with the urethra. In the remaining four patients, the fistula extended from the rectum to the cutaneous orifice in the ventral aspect of penis, with communication or a short common channel with the urethra. CONCLUSIONS: Imperforate anus with fistula extending into the penis is a rare variant of anorectal malformation. Unawareness of this lesion resulted in a delay of correct diagnosis and appropriate management. A thorough examination, including colonourethrography and fistulography, should be performed in all patients with a fistula opening in the ventral aspect of the penis.

[An Adult Case of Transperineal Repair of Congenital Rectourethral Fistula Using Gracilis Muscle Flap Interposition].

A man in his 50s was referred to our hospital after recurrent severe urinary tract infection. He had undergone anoplasty for anorectal malformation during early infancy. He noticed urinary leakage from the anus for a long time. Under diagnosis of congenital rectourethral fistula, we performed fistula closure. The fistula was transsected via transperineal incision and each stump was closed. A gracilis muscle flap approximately 30 cm long was harvested from the left thigh, brought into the deepest part between the separated rectum and urethra through a subcutaneous tunnel and fixed there. The urinary leakage from the anus disappeared, and the infection resolved. Application of gracilis muscle flap for congenital diseases is rare, but was useful in the present case.

One stage operation through modified posterior sagittal approach preserving the sphincter intact for anal agenesis with rectovestibular fistula.

PURPOSE: To describe the surgical technique and outcomes of an one stage operation through modified posterior sagittal approach (PSAP) preserving the sphincter intact for anal agenesia with rectovestibula fistula. PATIENTS AND METHODS: 57 patients suffering from anal agenesis with rectovestibular fistula were operated by a one-stage operation through a modified PSAP preserving the external sphincter intact from 2002 to 2010. The operation was performed in one-stage through a posterior sagittal approach with three modifications: The external sphincter complex was not opened on the posterior side, the dissection was carried out outside the rectal pouch, the rectal pouch was not tapered and was pulled through the center of the external sphincter identified by muscle stimulator. RESULTS: Patients age varied from 3 days to 30 days (mean: 21±9 days). The mean operative time was 57±8 min (range, 35-90 min). There were no intraoperative complications. There were no intraoperative or postoperative deaths. There were no early postoperative complications. Follow up from 40 months to 140 was obtained in 52 (91.2%) patients. Constipation has seen in 3 patient, 46 patients (88.5%) had 1-2 defecations per day, 2 patients (3.85%) had 3-4 defecations per day, 1 patients (1.9%) had more than 4 defecations, and 3 patients(5.8%) had one defecation every 2-3 days. Rectal mucosal prolapse occurred in 7 patients who required a second operation. CONCLUSION: One stage operation through modified PSAP is feasible, is safe and provides good continence outcomes for anal agenesis with rectovestibular fistula.

Congenital posterior urethroperineal fistula: a review and report of the 25th case in literature.

Duplications of the urethra are rare, but the congenital posterior urethroperineal fistula (CUPF) is an even rarer anomaly. CUPF resembles type II A2, Y-duplication described by Effmann but differs significantly because it has a normal functional dorsal urethra and a ventral hypoplastic accessory urethra. Excision or fulguration of the accessory urethra results in the resolution of patient's symptoms. The 25th case in English literature is reported with a review of literature; the addition of CUPF to Effmann classification as "type II A2, Y-hypoplastic ventral urethra" is proposed.

[Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias. A case report]. / Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal. reporte de un caso clínico.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

The diagnostic value of MRI fistulogram and MRI distal colostogram in patients with anorectal malformations.

Contrast fistulogram (FG) and distal pressure colostogram (DPCG) are standard diagnostic methods for the assessment of anorectal malformations. Pelvic magnetic resonance imaging (MRI) earned a place among essential diagnostic methods in preoperative investigations after the Currarino syndrome and a high incidence of associated spinal dysraphism were described. The aim of our study was to evaluate the possibility of substituting FG and DPCG by a modified pelvic MRI, e.g. MRI fistulogram (MRI-FG) and MRI colostogram (MRI-DPCG). The prospective study involved 29 patients with anorectal malformations who underwent a modified pelvic MRI. The length and course of fistulas and rectum, and the presence of sacral anomalies were studied on MRI images and compared with images obtained by radiologic examinations. Modified MRI brought identical results as contrast studies in 25 patients when related to the fistula and rectum length and course. MRI was more accurate for the detection of sacral anomalies. MRI-FG was the only imaging method used in the four most recent patients. The results support the assumption that conventional contrast examinations for the assessment of anorectal malformations can be replaced by MRI, thus reducing the radiation dose.

Perinatally discovered complete tubular colonic duplication associated with anal atresia.

Complete tubular colonic duplication (CTCD) is exceedingly rare. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy. We present 3 cases of surgically proven neonate CTCD discovered at laparotomy for anal atresia. We reviewed the mode of clinical presentation, the imaging, and laparotomy findings. Our series illustrates that this rare disease presents perinatally in association with anal atresia, with or without other associated anomalies.

Rotation of the amputated fistula tract for the management of congenital urethral-enteric fistula with severe urethral stenosis: a novel technique with long-term outcomes.

OBJECTIVE: Urethral-enteric fistulae with hypoplastic/atretic distal urethra in boys with anorectal malformations are amenable to management via sequential dilation of the distal urethra (P.A.D.U.A.) and subsequent repair of the fistula, but failure of this technique occasionally requires complex reconstruction. We present a novel surgical approach, along with long-term results, that incorporates rotation of the amputated fistula tract (RAFT) in boys with H-type urethral-enteric fistulae. METHODS: The charts of four patients undergoing the RAFT procedure were reviewed. All had previously failed P.A.D.U.A. Surgical principles were similar in all cases: the fistula tract was amputated as close to the bowel as possible. A tubularized or on-layed urethra was then fashioned from preputial skin and anastomosed to the distal end of the urethral fistula. The distal end of the neourethra was then brought to the tip of the penis, or anastomosed to the proximal end of the patent distal urethra. RESULTS: Mean age was 12.3 months, and there was a mean follow up of 10.2 years. All four patients had a rectourethral fistula as a component of VACTERL, with a urethral deficit of 7-11 cm. All had a functionally intact urethra on reconstruction, with normal continence and bladder neck closure. Two patients needed further bladder augmentation with a Mitrofanoff channel for poor bladder compliance. Both boys who were post-pubertal in this series report normal sexual function with antegrade ejaculation. CONCLUSIONS: The RAFT technique represents a viable reconstructive option for congenital H-fistulae with distal urethral stenosis, with excellent long-term results. It provides boys with normal urethral function, along with intact urinary continence and antegrade ejaculation. This technique may be of particular utility in patients after failed P.A.D.U.A., or in whom a staged buccal onlay graft is not feasible.

Fístula rectouretral tras tratamiento prenatal de paciente Con valvas de uretra posterior / Rectourethral fistula after prenatal treatment of posterior urethra valves

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Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome.

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.

Urethral reconstruction in patients suffering from aphallia: a reconstructive challenge.

OBJECTIVE: To document our experience (at a tertiary health care center) of reconstruction of the urethra in 3 male patients suffering from aphallia and congenital urethrorectal fistula as well as their long-term follow-up. METHODS: Extensive single-stage urethroplasty in 3 patients suffering from aphallia was performed. Prior to that, neophallus reconstruction was done in all 3 patients after puberty. In a single stage, we used oral mucosa and pedicled penile skin onlay for anterior urethra reconstruction and groin-based pedicle (Singapore) flap for posterior urethra substitution surgery. The current follow-up is between 2 and 5 years. During the last follow-up, the patients also answered questions from a validated patient-reported outcome measure and quality of life questionnaire. RESULTS: All the 3 patients are voiding well in the standing position with a mean Q(max) of 14 ml/s. None of them has any problems related to the neourethra as per their last follow-up. All 3 patients were satisfied with the surgical procedures related to the aphallia correction and stated that the surgery did not interfere with the quality of their lives. CONCLUSION: Reconstruction of the urethra from pedicled skin flap combined with buccal mucosa graft urethroplasty gives durable and satisfactory long-term results.

Laparoscopic management of congenital H-type urethroanal fistula.

Laparoscopic management in a case of congenital urethroanal fistula with a normal anterior urethra in a 5-month-old male child is reported. A congenital H-type urethroanal fistula is a rare entity. Management of such fistula has always been surgical, ranging from perineal dissection, posterior saggital approach, to abdomino-perineal approach. We describe a minimally invasive laparoscopic method for approaching such fistula, which has not been described in the literature and is being reported for the first time.