Rectovestibular Fistula Research Articles

Does presence of a VACTERL anomaly predict an associated gynecologic anomaly in females with anorectal malformations?: A Pediatric Colorectal and Pelvic Learning Consortium Study

BackgroundVACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM). MethodsThis study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher's exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. Results834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p<0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p<0.001). ConclusionsVACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system. Level of evidenceIII. Retrospective comparative study

Successful Resolution of Secondary Infertility following Treatment of Fourth-Degree Perineal Tear through Neglected Recto-Vestibular Fistula in an Adult Female with Anorectal Malformation

Anorectal malformations (ARM) are rare occurrences characterized by the absence or abnormal localization of the anus and usually diagnosed and treated in the neonatal period.

Recto-vestibular fistula with colonic duplication: report of three cases and review of literature

BackgroundAnorectal malformations associated with colonic duplications are a rare condition. Double terminations of tubular colonic duplication in the perineum are even rarer. Colonic duplication may have different presentations according to its location and size and incidence of less than 15% of all gastrointestinal duplications. We hereby report three such cases of recto-vestibular fistula along with underlying tubular colorectal duplication over a period of 10 years (2010–2020) managed successfully by different surgical approaches. These cases also illustrate the diagnostic challenge and variable presentation of colonic duplications in female patients with recto-vestibular fistula.Case presentationCase 1 is a patient with flank heteropagus with absent anal opening and recto-vestibular fistula, while cases 2 and 3 presented as suspected H-type recto-vestibular fistula with normal anal opening. All the cases were however managed with a specific surgical approach on case basis. Duplicated colon was excised in case 1, but in case 2 and case 3, duplicated colon was converted into single channel using proximal and distal staplers. Further definitive repair of female recto-vestibular fistula was done.ConclusionsColorectal duplication is a rare congenital malformation, and their association with anorectal malformations is even rarer. This association can have varied presentation, and surgical approach should be according to the congenital anatomical variations on case-to-case basis. Colonic duplications should always be ruled out in a female newborn child with history of passage of stools both from the vestibule and anus.

Quality of defecation in patients with anorectal malformation following posterior sagittal anorectoplasty (PSARP) in Arifin Achmad General Hospital of Riau Province, Indonesia

Background: Anorectal malformation is a congenital abnormality that shows imperfect anus or absence of anus. The most common complication in patients with anorectal malformations after posterior sagittal anorectoplasty (PSARP) are disorders in defecation, especially constipation. Method: The purpose of this study was to determine the correlation of quality of defecation measured with the presence of constipation with the type of PSARP surgery in Arifin Achmad Hospital of Riau Province. This study used an analytical retrospective method from November 2018 to March 2019. Total sampling was the sampling method used in this study, which accounted for a total of 28 subjects. The population of this study was all patients with anorectal malformation and fistula in the Pediatric Surgery Division of Arifin Achmad General Hospital. Result: This study showed that among 28 cases found, there were 19 female patients (68%) and 9 male patients (32%). Based on the lesion level, there were 17 cases (60,7%) of intermediate lesions and 11 cases (39,3%) of high lesions. In this study, rectovestibular fistula had the highest incidence among other types of fistula (53,6%). Constipation were found in 53.6% patients with p-value of 0.025 (OR 6.40, 95% CI 1.183-34.613). Conclusion: There was a significant correlation between the PSARP method and constipation in patients who underwent anorectal malformation surgery, which constipation was a sign of the quality of defecation.

Expanding the PURA syndrome phenotype with manifestations in a Japanese female patient

We report on a 15-year-old Japanese female patient with hypotonia and global developmental delay from the neonatal period who was revealed to carry a known pathogenic PURA variant (NM_005859.5:c.697_699del, p.Phe233del) by whole-exome sequencing. She had previously unreported clinical features, including a rectovestibular fistula, extremely short stature, and underweight, expanding the known phenotype of PURA syndrome.

Rectovestibular fistula: Which surgical approach is suitable? A randomized controlled trial.

The management of a vestibular fistula is a challenge for pediatric surgeons. We compared four different operative techniques in terms of postoperative complications, continence, and cosmetic appearance. This prospective, randomized, comparative study included female children with rectovestibular fistulae who were selected from patients with Anorectal Malformations (ARMs) treated between January 2016 and July 2020. The patients were randomly divided into four groups based on the operative technique: Trans-Sphincter Anorectoplasty (TSARP), Posterior Sagittal Anorectoplasty (PSARP), Classic Anterior Sagittal Anorectoplasty (ASARP), and modified ASARP. The incidence of vestibular fistulae among all patients with ARMs was 13.4%. The total number of patients with vestibular fistula was 112, including eighty-four (75%) with rectovestibular fistulae and twenty-eight (25%) with anovestibular fistulae. Associated congenital anomalies were found in nineteen (22.6%) patients. The percentage of parents satisfied with the cosmetic appearance and continence of their children was the highest after TSARP. PSARP had the lowest incidence regarding vaginal wall injuries. TSARP is the best operative technique for handling rectovestibular fistulae and is suitable for infants and children. In the TSARP technique, the external sphincter muscle can be preserved following complete dissection of the rectum without the need for a midline skin incision. A midline skin incision is required in the modified ASARP technique.

Surgical Complications and Functional Outcomes in Children with Anorectal Malformations with Vestibular Fistula

Background: Vestibular fistula is one of the most common types of anorectal malformations in female infants. Colostomy prior to definitive anorectoplasty is still controversial. Objective: To compare the complication rates and functional outcomes of the surgical treatments of vestibular anus between colostomy prior to anorectoplasty and treatment without colostomy. Materials and Methods: The medical records of 40 patients with vestibular fistula that underwent definitive surgical treatments at Siriraj Hospital between January 2006 and December 2018 were reviewed. Demographic data, operative records, functional outcomes, and complications were analyzed. Results: In the 40 vestibular fistula patients, colostomy (C) prior to vestibular fistula repair was performed in 18 patients, while vestibular fistula was repaired without colostomy (NC) in the other 22 patients. The C group had more associated anomalies than the NC group, including GI tract anomalies at 23.5% versus 9.1%, spinal anomalies at 47.1% versus 9.1%, and KUB anomalies at 52.9% versus 9.1%. The definitive anorectoplasty for vestibular fistula were anal transposition (31, 77.5%), PSARP (5, 12.5%), and other operations (7, 10%). Anal transpositions were performed in both the NC (90.9%) and C (61.1%) groups, respectively. NC had higher complications than C at 36.4% versus 22.2%. The recurrence of fistula occurred in 9.1% of NC cases versus 0% in C. Anal stricture was found in 18.2% of NC cases versus 5.6% in C. The wound infection rates of these two groups were the same. The complication rate with colostomy was 22.2% with prolapsed colostomy in 11.1% of cases and wound infection in 5.6%. The NC group had higher complication rates of anorectoplasty than the C group, but the overall complications, including colostomy complications, of these two groups were comparable. The Krickenbeck scoring system was used to evaluate the functional outcomes. In the median follow-up period of 1,336 days and 23 patients were evaluated. Even though the NC group had higher complication rates of anorectoplasty than the C group, the functional outcomes of these two groups were the same. Conclusion: Vestibular fistula repair with prior colostomy had lower complication rates of anorectoplasty than primary vestibular fistula repair, but the functional outcomes of these two groups were the same. Keywords: Vestibular; Rectovestibular; Anovestibular

Trans-fistula Anorectoplasty (TFARP) in the Management of Anorectal Malformation (ARM) with Recto Vestibular Fistula (RVF) in Females: A Retrospective Study

Aim: To determine the outcome of trans-fistula anorectoplasty (TFARP) in female children having anorectal malformation with recto-vestibular fistula.&#x0D; Study Design: A retrospective study.&#x0D; Place and Duration: Department of pediatric surgery during the period of February 2018 to February 2020.&#x0D; Methodology: A total of 87 female children who have imperforate anus with recto-vestibular fistula from the age of 3 months to 6 years were included in this study. Initially, we have started this technique with the covering sigmoid colostomy, then TFARP after 12 weeks interval &amp; once new anus healed and hegar dilatation achieved then sigmoid colostomy closed after 24 weeks of the first procedure. When we were experienced then we start TFARP as a single-stage procedure. Post-operative data regarding early complications of local wound infections, prolapse of the rectal mucosa, and late complications for anal stenosis, rectal prolapse, and scarring in the perineal body were collected up to 2-3 months respectively.&#x0D; Results: A total of 87 patients were included in this study. The average operative time was 40 minutes. The total days of hospital stay were 3 to 5 days. Regarding early complications, skin excoriation was present in 21 (24%) followed by vaginal tear in 17 (19%) cases. Mucosal prolapse was a late complication observed in 17 (20%) cases. Continence was good (Score 0) in 33 (38%) cases, while it was fair (score 1) in 36 (41%) cases.&#x0D; Conclusion: Primary repair of recto-vestibular fistula by Trans-fistula anorectoplasty in children is a feasible procedure that has a good cosmetic appearance and anal continence. Single-stage reconstruction is possible in children with satisfactory results. It produces less morbidity and is a more efficient procedure.

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study

BackgroundOutcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. MethodsA multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4–7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups ‘poor’ ≤ 11, and ‘fair’ 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. ResultsThe study included 111 RVF-patients. Median BFS was 16 (range 6–20). The ‘below normal’ group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. ConclusionsAlthough median BFS at 4–7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. Level of EvidenceLevel III.

Complete tubular colonic duplication ending in a common channel as a recto vestibular fistula: A case report and literature review of management options

Colon duplication (CD) is a rare congenital abnormality with varied presentations. We present a neonatal case with complete tubular CD terminating uniquely as a common channel, a single rectovestibular fistula. We summarize surgical management options in CD as well as factors that will help to decide the best surgical treatment in individual cases.

Laparoscopic-Assisted Anorectoplasty for Rectovestibular Fistula: A Comparison Study with Anterior Sagittal Anorectoplasty.

Posterior or anterior sagittal anorectoplasty (ASARP) is the mainstream for correcting rectovestibular fistula (RVF). However, the intermediate RVF has the potential risk of wound complications when applying ASARP due to its high rectal pouch, long fistula tract, and difficulty separating the rectum and vagina. We developed laparoscopic-assisted anorectoplasty (LAARP) for surgical correction of RVF, which has acceptable preliminary outcomes. The purpose of this study is to evaluate the safety and efficacy of LAARP in comparison with ASARP for patients with RVF. Twenty-five patients with RVF who underwent LAARP between October 2017 and December 2020 were retrospectively reviewed. The outcomes were compared with 43 patients who underwent ASARP between April 2015 and August 2018. The age, weight at operation, sacral ratio, operative time, and postoperative hospital stay were evaluated. The results were assessed for complications, perineum appearance, and bowel function. The two groups were comparable in terms of demographics. The median operative time of the LAARP group was significantly longer than that of the ASARP group (113 vs. 95 minutes; p = 0.015). The mean length of the resected rectum in the LAARP group was also longer than that in the ASARP group (6.75 ± 5.07 vs. 3.31 ± 3.06 cm; p = 0.001). Compared with the LAARP group, complications in the ASARP group were more frequent (4.0 vs. 27.9%, p = 0.036). No intraoperative or postoperative wound-related complications occurred in the LAARP group. However, in the ASARP group, one patient had an intraoperative vaginal injury and four had postoperative anastomosis-related complications. The incidence of redo operation in the ASARP group was significantly higher than that in the LAARP group (p = 0.000). Cosmetic satisfaction was higher in the LAARP group (96.0 vs. 76.7%; p < 0.05). In terms of voluntary bowel movement, soiling, and constipation, the LAARP group had similar results compare with the ASARP group. The LAARP technique has shown several unique strengths in treating intermediate type RVF, including lower risks of complications, and minimal muscular injury, with a comparable bowel function.

Vestibular and Perineal Fistula: Outcomes of Single-stage Repair with Early Feeding and Limited Postoperative Antibiotics

Vestibular and Perineal Fistula: Outcomes of Single-stage Repair with Early Feeding and Limited Postoperative Antibiotics

Vestibular anus, a surgical dilemma

It is a pleasure for me to draw attention in this issue of your admirable journal to recto-vestibular fistula. Vestibular anus means congenital presence of the anus in the vestibule behind the vaginal orifice. It shares a common wall between the vagina and the rectum. In addition to an abnormal cosmetic appearance, it leads to recurrent attacks of urinary tract and vaginal infections. In adult female patients, it leads to dyspareunia. Several techniques have been described for the treatment of this type of anomaly, however; the postoperative continence is not always satisfactory. Optimal surgical repair would be easy to assess minimum dissection to spare pelvic nerves and guide the rectal pouch through the muscles of continence and intact sphincter [1]. The surgeon must cautiously during surgical separation of the posterior vaginal wall from the anterior rectal wall to avert rectal wall tear.

Early results of different types of anorectoplasty for anorectal malformations

Objective: To document our experience with the technique of surgical repair in anorectal malformations (ARM) and the short term anatomical and functional outcomes. Methods: This study included total 31 babies. Fourteen were perineal fistula cases with age range between 1 to 4 days. Ten patients had vestibular fistula and seven had rectourinary fistula. Primary diverting colostomy was done for vestibular and rectourinary fistula patients. On the other hand low imperforate anus with anoperineal fistula without associated major anomalies had undergone fistulectomy and simple anoplasty. Results: Simple anoplasty was done for fourteen perineal fistula cases. Anterior sagittal anorectoplasty (ASARP) was done for 10 vestibular fistulae and posterior sagittal anorectoplasty (PSARP) was done in 10 recto-urinary fistula cases. Simple anoplasty and vestibular anus scored good (5-6) in 70% to 71% while PSARP scored fair in 58% of the cases. Postoperative mucosal prolapse, anal stenosis and retraction occurred in two, six and one patient respectively. Redo surgery was done in mucosal prolapse and retraction cases. Four responded to anal dilation and the other needed redo surgery by a simple cutback technique for anal stricture. Continence was assessed in 23 patients whose follow-up periods were longer than 3 years. Twenty one patients had a good score and two had a fair score. No patients had a poor score. Conclusion: Our approach has the following advantages: (i) The operative technique is simple and easy to perform. (ii) Minimal complication rate with good cosmetic results. Mediscope 2021;8(2): 75-79

To Study the Outcome of Posterior Sagittal Anorectoplasty in Anorectal Malformations

Aim: To assess the effectiveness of posterior sagittal anorectplasty for the repair of anorectal malformations in terms of postoperative complications and functional outcomes. Study design: Descriptive prospective study. Place and duration of study: Sahiwal Teaching Hospital, Sahiwal. From 1st January 2016 to 31st December 2019. Methodology: Forty-two children with high/intermediate anorectal malformations, who underwent posterior sagittal anorectoplasty, were included in the study. Patients with low variety anorectal malformations, recto vestibular/ano vestibular fistula, persistent cloaca and patients with sacral dysraphism, spinal dysraphism, myelomeningocele spina bifida occulta and menifesta were excluded. Demographic information included age, gender, weight, type of anorectal malformation, type of surgery performed, operation time and hospital stay. Immediate post-operative complications were also noted. Bowel habits, constipation and faecal incontinence were evaluated in all patients during the follow up period. Results: Thirty-one (73.80%) were males and 11(26.19%) were females and mean age was 1.72 years. The mean weight at the time of operation was 11.00 kg. High variety anorectal malformations were 14(33.33%) and intermediate were 28(66.66%). Rectourethral fistula 25(59.52%) was most common variety seen in males. Recto bulber urethral fistula in 17(40.47%) and recto prostatic urethral fistula in 8(19.04%) patients were seen. Anal agenesis without fistula 7(16.66%) was most common anomaly in females. Overall complication rate was 15(35.71%). Constipation 11(26.19%) was most common post-operative complication. True fecal incontinence was seen in 3(7.14%) patients. Strong and effective squeeze of anal sphincter was seen in 39 (92.85%) patients and satisfactory bowel habits were present in 37 (88.08%) patients. Conclusion: Posterior sagittal anorectplasty is a useful procedure in the management of high/intermediate anorectal malformations, precise identification and reconstruction of muscle complex result in good anatomical and functional outcome. Immediate post-operative complication rate is also low. Keywords: Anorectal malformations, Posterior Sagittal Anorectoplasty, Postoperative complications, Functional outcomes

National Medical Research Center for Children’s Health

Abstract Introduction. Anorectal malformations are one of the most numerous groups of proctologic pathology in children. The incidence is 1 per 5,000 live births. Perineal ultrasound, distal colostography, and MRI of the pelvic organs and sacrococcygeal region are used to clarify the anatomico-topographic features of the malformation and identify concomitant malformations (presacral masses, distal spinal cord pathology). This article presents a clinical case of surgical treatment of an 11-year-old girl who underwent primary surgery for anorectal malformation with rectovestibular fistula in infancy. In the postoperative period for 10 years, the parents practically did not carry out rehabilitation therapy. These factors led to persistent decompensation of the lower intestine in the form of a giant megarectum, which required repeated anorectoplasty with resection of pathologically dilated parts of the intestinal tube. The article presents description of the repeated surgery with intraoperative electromyoidentification of the perineal muscles, data of follow-up 9 months after the repeated anorectoplasty with instrumental diagnostic methods (irrigography, functional study of the closing apparatus of the rectum).Discussion. This clinical observation illustrates complications following surgical treatment of anorectal malformation with rectovestibular fistula. Lack of intraoperative electromyoidentification of the muscles during primary anorectoplasty often resulted in ectopia of the neoanus relative to the center of the perineal muscle center. This circumstance was one of the causes of persistent chronic constipation in the postoperative period.Conclusion. The use of electromyostimulation during primary proctoplasty for reliable determination of the sphincter-levator complex topography is mandatory. Long sequential rehabilitation in the postoperative period including neoanus bougienage, provision of regular full defecation, physiotherapeutic treatment, as well as regular follow-up examination in the in-patient department is also an integral part of the treatment.

Complete tubular duplication of colon presenting as rectovestibular fistula: A case report

Background: Duplication of the gastrointestinal tract is a rare congenital disorder in pediatric patients. Complete resection in case of a total tubular duplication that requires total or subtotal colectomy is undesirable in children.Case Presentation: This case presented a simple surgical technique for treating complete colonic duplication without colonic resection. A 2 months old female baby with abdominal distention and stool complaint came out from her vaginal vestibular and normal anus. A digital rectal exam showed a palpable mass in the posterior region. Abdominal CT Scan showed a rounded mass with size 2x2 cm in the left posterolateral of rectum region. The initial diagnosis was suspected sacrococcygeal tumor type 4 with rectovestibular fistula. Intraoperative findings showed duplication of the caecum, appendix, total colon (ascending to sigmoid) and rectum (one directed to the vagina and the other to anus). The ileostomy was done 20 cm from the ileocaecal junction as a temporary treatment to relieving distention with definitive repair planned in the following months. The second stage repair was performed after 1-year-old with posterosagital anorectoplasty followed by distal separation of the duplicated colon using a 12 cm linear stapler. For this case, the third stage repair was done one year after the previous surgery to close the remaining ileostomy. The patient’s outcome showed good results with normal defecating function and no complication.Conclusion: Complete tubular duplication is a rare case without specific clinical symptoms, but the assessment of the diagnosis can be assisted by radiology such as abdominal CT and barium enema. We hope this article could illustrate management for complete tubular duplication of colon presenting as rectovestibular fistula, thus reflect how important clinical judgment and initial diagnostic in our health care system.

12. Does presence of a VACTERL anomaly predict an associated Gynecologic anomalies in females with Anorectal Malformation?: A Pediatric Colorectal and Pelvic Learning Consortium Study

<h3>Background</h3> VACTERL association (as defined by 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb) is present in around a third of patients with anorectal malformations (ARM). In female ARM patients' gynecologic anomalies are seen in conjunction with 1 in 5 rectovestibular and 1 in 20 rectoperineal fistulae. This study hypothesized that the presence of VACTERL association would predict an increased risk of gynecologic anomalies and this information will help to guide evaluation and management protocols. <h3>Methods</h3> This study is a cross-sectional, retrospective analysis from the prospectively-collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). Subjects with inflammatory bowel disease were not eligible for the PCPLC registry. The 316 female subjects with a perineal or vestibular fistula who were enrolled in the registry by January 14, 2020 were included in this study. The relationship of VACTERL association, ARM plus an anomaly in two or more VACTERL systems, with presence of a genital anomaly was evaluated with Fisher's exact test (Table 2). The relationship of each individual VACTERL system with presence of a genital anomaly was analogously assessed. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. <h3>Results</h3> 316 patients with rectoperineal (n=162, 51.3%) or rectovestibular (n=154, 48.7%) fistulae underwent VACTERL screening and gynecologic evaluation. 83 (26.3%) of patients with rectoperineal fistulae or rectovestibular fistulae had gynecologic anomalies. (Table 1). Among subjects with VACTERL association, 70.4% have a spine anomaly, 68.7% have a renal anomaly, and 70% have a cardiovascular anomaly. Genital anomalies were present in 40.9% vs. 17.9% of subjects with vs. without VACTERL association (p < 0.001). Genital anomalies were present in 31.0% vs. 16.5% of subjects with vs. without an anomaly in at least one VACTERL system (p = 0.006) Table 2. <h3>Conclusions</h3> Presence of VACTERL association in patients with rectoperineal or rectovestibular fistula correlates with an increased risk of gynecologic anomalies and should prompt early involvement of pediatric gynecology in their care.

Correlation of anorectal malformation complexity and associated urologic abnormalities

BackgroundPatients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses. MethodsA retrospective review was performed of patients with ARM treated at our pediatric colorectal center. All patients underwent protocolized urologic screening. ARM subtypes were ordered with increasing severity as follows in males: perineal, bulbar, prostatic and bladder neck fistulae. Females were similarly categorized as perineal, vestibular and vaginal fistulae followed by cloaca with <3 cm common channel and cloaca with >3 cm common channel. The following urologic diagnoses were assessed to determine whether a correlation existed with the severity of the ARM subtype: hydronephrosis, vesicoureteral reflux (VUR), solitary kidney, renal ascent anomalies (ectopic or pelvic), renal fusion anomalies (horseshoe or cross fused kidney), duplex kidney, hypospadias and undescended testicle. ARM subtypes were defined by distal rectal anatomy. ResultsA total of 712 patients were included in our study with a mean age of 4 years and of whom 45% were male. The overall rate of urologic anomalies was greater in males than females (65% vs 56% p < 0.026). In both sexes, the rate of urologic anomalies increased with increasing severity of ARM subtype (p<0.00010) finding that males with bladder neck fistula and females with cloacal malformations, particularly with long common channels, being the highest incidence. In males and females, the rate of hydronephrosis increased as the complexity of ARM increased and this correlated significantly (p < 0.0001 vs p < 0.0003 respectively). Similarly, the incidence of VUR also increased as complexity of ARM increased in both males and females (p = 0.01 and p<0.0001 respectively). The remaining urologic diagnoses were not significantly correlated with severity of ARM. ConclusionsUrologic anomalies occur at a high rate in children with ARM and appear to increase in frequency with increasing complexity of ARM subtype. These findings stress the importance of proper ARM screening and proactive collaboration with a clinician with expertise in pediatric urology early in the management of such children to improve early recognition of urologic diagnoses. Level of evidenceLevel III.

15-Year-old with neglected recto-vestibular fistula in western Uganda: a case report

BackgroundTeenage and late presentation of anorectal malformations are not uncommon in developing world. Some of the reasons for late presentation include but not limited to illiteracy, poverty, lack of awareness, and limited trained pediatric surgeons. In rural areas, neonates with ARMs are considered cursed and are marginalized.Case15-Year-old African girl (a munyankole by tribe in Uganda) from western Uganda presented at 15 years of life with colostomy and uncorrected anorectal malformation. Never went to school due to social stigma.ConclusionDue to limited number of trained pediatric surgeons in most of African Countries, many children in addition to living with a colostomy or untreated malformation, may also be undiagnosed with chronic constipation. Improved awareness and advocacy would promote early presentation and treatment.