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.

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.

[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.

Adult isolated congenital anterior urethrocutaneous fistula.

Isolated congenital anterior urethrocutaneous fistula (CAUF) is an extremely rare deformity and few cases have been reported in the English language literature. Moreover, adult CAUF has not been reported up to now. We present a rare adult patient with this unusual isolated CAUF deformity. The possible etiology and treatment strategy are discussed.

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.

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.

MRI of congenital urethroperineal fistula.

We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning.

[Imaging of anorectal malformations in the neonatal period]. / Imagerie des malformations anorectales en période néonatale.

PURPOSE: The classification of anorectal malformations (ARM) as high or low is based on clinical and anatomical characteristics. It has an impact on the surgical management but also on the functional prognosis. In the absence of consensus, our goal was to determine the value of imaging in the neonatal period for diagnosis and management of infants with ARM. MATERIALS AND METHODS: Retrospective study of 43 infants imaged over a 9 year period. The different imaging studies (abdominal and pelvic ultrasound, radiographs, percutaneous opacifications and fistulograms) performed for each infant were collected and analyzed then correlated to clinical and surgical findings. RESULTS: Clinical evaluation could classify 30 ARM as low and 4 ARM as high while 9 ARM without fistula remained indeterminate. Imaging findings were mixed: on ultrasound, the rectal cul-de-sac to perineum distance did not appear to be determinant, contrary to published data. Pelvimetry showed limitations, irrespective of the technique. Morphological evaluation provided the following data: presence of fistula, sphincter anatomy, rectal cul-de-sac to perineum distance. CONCLUSION: Classification relies on clinical evaluation in most cases. Opacification techniques and ultrasound remain useful in some cases. MRI could become the preferred imaging modality.

Congenital urethrovaginal fistula with imperforate hymen: a first case report.

BACKGROUND: Congenital urethrovaginal fistulas are rare, and all five reported cases have been associated with urogenital abnormalities. We describe a case of congenital urethrovaginal fistula with an imperforate hymen and no other urogenital abnormalities. CASE: A 25-year-old female sought medical advice regarding cyclical hematuria, dyspareunia, and infertility of four years' duration. Investigations disclosed a urethrovaginal fistula with an imperforate hymen. The patient was successfully treated with excision of the hymen and closure of the urethrovaginal fistula. CONCLUSION: In this instance, the diagnosis of congenital urethrovaginal fistula was delayed until adulthood because the presence of urinary incontinence, the usual presentation of a urethrovaginal fistula, was concealed by the imperforate hymen. We could find no previously reported case of urethrovaginal fistula with imperforate hymen.

Fetal urogenital sinus with consecutive hydrometrocolpos because of labial fusion: prenatal diagnostic difficulties and postpartal therapeutic management.

OBJECTIVE: To elucidate the differential diagnoses of tumorous dilations in the fetal pelvic region detected by prenatal ultrasound and the postnatal management demonstrated on a fetus with 29 weeks of gestation with a retrovesical located bottle-like cystic structure measuring 54 x 31 x 27 mm within the pelvis. Postnatal findings were a labial fusion and a consecutive hydrometrocolpos due to a urethrovaginal fistula. METHOD: Case report of a fetus affected by an intricate cloacal anomaly. RESULTS: The long-term prognosis for this nonsyndromic form of hydrometrocolpos without any other structural defects or organic failures after operative sanitation is excellent. Final reconstruction is planned at about 12 months of age. CONCLUSION: Prenatal diagnosis of tumorous dilations in the fetal pelvic region often involves difficulties because of numerous differential diagnoses and possible presentation in late pregnancy. Magnetic resonance imaging could be a useful complementary tool for assessing these anomalies when ultrasonography is inconclusive. In some cases, the final diagnosis cannot be confirmed until after delivery.

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.

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.

Case report: endoscopic treatment of isolated congenital urethroperineal fistula.

Isolated congenital urethrocutaneous fistula is an extremely rare congenital anomaly. Management is sometimes complex and depends on the location. We report our endoscopic treatment in a man with a urethroperineal fistula.

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.

Covered exstrophy with visceral sequestration: case report and review of literature.

We report a case of a male newborn with asymmetrical epispadic diphallia and congenital urethral fistula, and exomphalos minor. A segment of bowel was attached in the region of the pubis; with no communication to the underlying bowel. This was excised; histological examination revealed this to be a colonic remnant. The excision of the examphalos minor and approximation of the pubis and the rectus sheath over the bladder was also done; urethroplasty is planned at a later date. A comprehensive review of the available literature revealed only eight such cases before this; the possible embryology is discussed.

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.

Genital abnormalities in females with Bardet-Biedl syndrome.

The major manifestations of the Bardet-Biedl syndrome are digital anomalies, tapetoretinal degeneration, obesity, renal abnormalities, and hypogenitalism (described mainly in males). We report on 2 girls with Bardet-Biedl syndrome who also had vaginal atresia. A similar association in females with Bardet-Biedl syndrome was suggested in published reports of 11 affected individuals who had structural genital abnormalities, (some of which were missed in childhood), including persistent urogenital sinus, ectopic urethra, hypoplasia of the uterus, ovaries and fallopian tubes, uterus duplex, and septate vagina. The association of atresia of the vagina and other malformations of female genital structures in individuals with Bardet-Biedl syndrome has often been missed in childhood and should be looked for more systematically.