Congenital vesicouterine fistulas-A PRISMA-compliant systematic review.

AIMS: Vesicouterine fistulas (VUFs) are infrequent abnormal connections between the bladder and the uterine cavity or cervical canal, being mainly sequelae of repeat Cesarean sections. Exceedingly rare are congenital VUFs. This is a systematic review of available world data aimed to characterize congenital VUFs and better understand the mechanism(s) of their formation. METHODS: The PubMed® database via MEDLINE® search engine was explored from its inception to March 2018. Relevant studies were identified using selected Medical Subject Heading-based terms. This was further supplemented by cross-referencing and handsearching. Retrieved literature was evaluated in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, or PRISMA, guidelines. RESULTS: A total of 6561 articles were identified of which 10 were analyzed. Three VUFs accompanied broader syndromes of congenital defects. A lack of patency at the level of the vagina was present in all assessed cases. Unilateral renal agenesis was confirmed in four of eight (50%) verified patients. Hence, unilateral kidney agenesis was related to a lesser degree (P = 0.0186) than vaginal atresia to VUF. The principal features of these fistulas were as follows: partial or complete vaginal atresia resulting in primary amenorrhea, menouria present since menarche, and urinary continence. CONCLUSIONS: This review provides the first systematic evidence that congenital VUFs are chiefly associated with concomitant vaginal atresia. The symptomatology of such VUFs is consistent with that of type I acquired fistulas.

Anorectal malformation with rectobladder neck fistula: A distinct and challenging malformation.

BACKGROUND: Rectobladder neck fistula is the highest and most complex anorectal malformation in boys and the only one that requires an abdominal approach, open or laparoscopic, for repair. The aim of this study was to describe the unique characteristics of rectobladder neck fistulas that warrant special attention and to describe the associated anatomic variants in the genitourinary tract. METHODS: The database of a tertiary medical center was retrospectively reviewed for all patients treated for rectobladder neck fistula, by our team in 1980-2011. Data on surgical history, associated and functional defects, treatment and outcome were collected by chart review. RESULTS: The study group included 111 patients. The most common anatomic urologic defect was a single kidney in 37 patients (33.3%) and the most common functional urologic defect was vesicoureteral reflux in 40 patients (36%), including 11/37 patients with a single kidney (29.7%). Of the 40 patients who underwent cystoscopy, 16 (40%) had a higher than normal location of the verumontanum. Follow-up ranged from 2 to 290months (median 59). Urinary continence was achieved in 40 of the 61 patients (65.5%) for whom data were available, and fecal continence was achieved in 9 of the 69 patients (13%) for whom data were available. A sacral ratio of 0.4 or less was associated with lower rates of urinary control (23%) and fecal control (0%), relative to higher ratios. Twenty stomas (18%) were found to be located too distally, limiting the availability of the bowel for a pull through. CONCLUSIONS: Rectobladder neck fistula carries a poor prognosis for bowel control and is associated with a high rate of urinary malformations that require long-term care. Pediatric surgeons need to be aware of these complications in order to provide proper treatment and parental counseling. Intra-vesical verumontanum is found in a surprisingly high percentage of patients. The combination of a single kidney with vesicoureteral reflux is common and should be closely followed to avoid renal deterioration. Special attention should be given to colostomy construction to avoid complications and unnecessary procedures. A sacral ratio of 0.4 or less is an indicator of poor fecal and urinary control.

Long term outcomes of laparoscopic-assisted anorectoplasty: a comparison study with posterior sagittal anorectoplasty.

PURPOSE: The aim of this study is to compare the long term outcomes between laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) for children with rectobladderneck and rectoprostatic fistula anorectal malformations (ARM). METHODS: Thirty-two ARM children with rectobladderneck and rectoprostatic fistula who underwent LAARP between October 2001 and March 2012 were reviewed. The outcomes were compared with those of 34 ARM children who underwent PSARP between August 1992 and September 2001. The sacral ratio (SR), age at operation, operative time, postoperative hospital stay and complications were evaluated. Bowel functions were assessed using the Krickenbeck classification. RESULTS: The mean operative time of the LAARP was significantly shorter than that of PSARP group (1.62 ± 0.40 vs 2.13 ± 0.30 h). The postoperative hospital stay was significantly shorter in the LAARP group (5.8 ± 0.65 vs 8.4 ± 0.67 h). The wound infections (11.8% vs 0%) and recurrent fistula (11.8% vs 0%) were more common in PSARP patients. Th e overall morbidity rate of PSARP group was significantly higher than that of the LAARP group (35.3% vs 12.5%, p<0.05). However, 7.5% of the LAARP patients developed rectal prolapse. Twenty-four of 32 patients were followed up for more than 3 years in LAARP group. The median follow up period was 7.5 years (range 4-11) in LAARP patients and 15.5 years (range 11-20) in PSARP patients. The rates of voluntary bowel movement, soiling (grade 1, 2 & 3) were similar in both groups. More patients from PSARP group developed grade 2 or 3 constipation (22.5% vs 0%, P<0.01). CONCLUSIONS: Compared to PSARP, LAARP is a less invasive procedure. The long term functional outcomes after LAARP were equivalent if not better than those of PSARP.

Monti's principle in the treatment of congenital uterovesical fistula.

Congenital uterovesical fistula is rare and generally associated with genital tract abnormalities derived from mullerian ducts or urogenital sinus. Management is usually challenging, and it involves vaginal reconstruction. A 15-year-old female patient presented with a 2-year history of cyclical hematuria. Investigation revealed a bicornuate uterus and complete vaginal agenesis associated with congenital uterovesical fistula. The fistula was repaired, and a neovagina was created using Monti's technique. Postoperative recovery was uneventful with normal voiding and initiation of regular menstruation through the neovagina. We discuss the options of vaginal reconstruction and stress the advantages of the technique used in this case.

Unusual duplicate bladder exstrophy in a female newborn: a case report.

The authors report a rare variant of exstrophy-epispadias complex, a duplicate bladder with normal bladder communicating with an exstrophic bladder by a fistula, in a girl with no genital malformation except for a duplicated clitoris. This variant could be a hybrid form of duplicate bladder exstrophy and superior vesical fistula. It seems easier to repair and has a better prognosis than classic bladder exstrophy.

[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.

A study of the functional aberration of the pouch in anorectal malformation associated with congenital pouch colon.

OBJECTIVE: There are no in vitro studies of congenital pouch colon (CPC) associated with anorectal malformation (ARM) to understand its mechanism of contractility. There is no consensus regarding the utilization of the CPC in repair of anorectal malformation. In view of the above, it was decided to carry out detailed in vitro physiological study of the excised CPC. METHOD: The excised specimens of CPC were taken and 15- to 20-mm long both longitudinal and transverse strips of muscle were obtained both from the proximal and distal part. Contraction was recorded using software chart-5 for windows. Drugs used were acetylcholine, histamine, adrenaline, atropine and pheniramine maleate. The amplitude of contractions was converted to tension gram and then the tension so developed was expressed as tension per unit mass (per gram of wet tissue). RESULTS: There were total of 21 specimens of high ARM with CPC. Five specimens were of the complete pouch, which did not show a response to any drug and the remaining 16 had an incomplete pouch. The mean longitudinal and circular muscle contractions of these 16 samples were statistically higher for the proximal segment than the distal segment both for acetylcholine and histamine. The effect of atropine was not significant but that of adrenaline and pheniramine maleate were significant on the distal segment muscle. CONCLUSION: Congenital pouch colon being deficient or having poorly developed receptors in their wall can not function properly as a reservoir for faeces and, hence, it should be excised to allow adequate function.

The colovesical fistula in congenital pouch colon: a histologic study.

PURPOSE: The aim of this study was to perform a detailed histopathologic examination of the terminal colonic pouch and the colovesical fistula (CVF) excised during surgical management of male patients with the more severe forms (types I/II) of congenital pouch colon (CPC) associated with anorectal agenesis. METHODS: From January 2005 to December 2006, 25 male patients with types I/II CPC underwent abdominal exploration with dissection of the terminal portion of the colonic pouch and associated CVF till the bladder, division-ligation of the fistula, and excision of the colonic pouch. In 6 of the 25 patients, a complete dissection of the fistula to the bladder was possible, and in them, the terminal portion of the colonic pouch and the CVF were subjected to detailed histopathologic examination. The 6 patients included 3 newborns in whom this surgery was performed as a primary procedure, and 3 patients aged 3 months, 15 months, and 2 years, respectively, in whom a window colostomy of the pouch had earlier been performed. After due processing, multiple sections from the specimens were stained using the routine H&E method and examined under the microscope under different magnifications. RESULTS: In 4 specimens, the epithelial lining of the CVF consisted of transitional stratified epithelium with underlying anal/urethral glands. In 2 specimens, obtained from patients 15 months and 2 years old, respectively, the lining was of nonkeratinizing, stratified squamous epithelium. Other findings included aganglionosis in the muscle layers (n = 2), submucosal and subserosal fibrosis (n = 1), and thickening of muscle layers in the fistulous portion, suggestive of the presence of an internal sphincter (n = 2). CONCLUSIONS: The CVF in patients with types I/II CPC shows histologic features of the normal anorectal canal.

Unusual variants of congenital pouch colon with anorectal malformations.

Anorectal malformations are common worldwide. Several classifications were used to describe the different types, but in spite of this, there are rare variants. This report describes 2 unusual cases of congenital pouch colon and anorectal malformations.

Bicornuate uterine horns with complete cervical-vaginal agenesis and congenital vesicouterine fistula.

We present a case of bicornuate uterine horns with complete cervico-vaginal agenesis and associated congenital uterovaginal fistula. The patient presented with cyclical hematuria, amenorrhea, and abdominal lump. The vagina was blind-ending. The cystoscopic examination during cyclical hematuria revealed bloody efflux through a small fistula on the posterior wall of the urinary bladder. The magnetic resonance imaging (MRI) showed bicornuate uterus and cervical agenesis. Hysterectomy and repair of the uterovesical fistula was done. The vagina was reconstructed using an amniotic mould. The report underlies the importance of MRI in diagnosing complexity of such rare anomalies. It also stresses for the need of hysterectomy if cervicovaginal agenesis is present.

Rectourinary perineal fistula--a unique anomaly.

Two patients who presented with a unique anorectal malformation are described. They had what we have named rectourinary perineal fistula. The first patient had a more severe malformation, rectovesicoperineal fistula, and has been followed up for 28 years. The second, with a lesser malformation, rectourethroperineal fistula, has been followed up for 19 years.

"Pouch colon patch graft"--an alternative treatment for congenital short colon.

A patch graft of the pouch colon over the pulled-through ileum in a patient with long-length pouch colon (in whom the entire colon was replaced by a pouch that communicated with the urinary bladder) was tried with good results for the first time. At follow-up the patient was found to have normal bowel patterns and weight gain. Though colon patch grafts have been widely used in patients with total colonic aganglionosis, they have not been tried for the short colon. The authors discuss the clinical setting in which the procedure may prove useful in patients with pouch colon and why these patch grafts may yield comparable or even better results in patients with pouch colon compared with those with total colonic aganglionosis.

Acute retention of urine due to prolapsing stoma in a case of anorectal malformation with bladder neck fistula.

This case report describes an extremely premature infant who was born with a high anorectal malformation requiring a colostomy soon after birth. He later developed multiple episodes of acute urinary retention complicated by bilateral hydronephrosis and acute renal failure. The cause of the retention was found to be the prolapsing stoma, which was kinking the bladder neck.

Tethered cord associated with anorectal malformation.

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.

Umbilical evagination of the bladder with omphalocele minor.

A neonate with umbilical evagination of the bladder (UEB) and omphalocele minor (OM) is reported. The findings in this case support the origin of the urachus from the upper end of the cloaca rather than the allantois. The frequent occurrence of OM with urachal anomalies, including UEB, suggests an embryological association between the two conditions.

[A case of a urachal fistula]. / Su di un caso di fistola dell'uraco.

The authors report a case of urachal fistula diagnosed after repeated omphalitis, this disease was associated with coronal hypospadias. Diagnosis was done thanks to a fistulography; subsequently, during the operation, to evidence the fistula, this was injected with methylene blue, this expedient showed that the fistula was longer than that disclosed from fistulography. To carry out a complete extirpation of the fistula the authors were bound to make a cystotomy. The course after operation was good and the patient discharged after a few days. After 5 years the patient is in perfect health. This case allowed some anatomic, clinical and semeiotic considerations.