Rectovestibular Fistula Research Articles

A study on Etiology and incidence of formation of types of Anorectal malformation: A prospective study

Background: Anorectal malformations are one of the most common congenital defects. This study was undertaken to study the hospital incidence of anorectal malformations (ARM), frequency of various types of defects, their sex distribution and the spectrum of anomalies associated with ARM. Materials and Methods: Ninety consecutive children attending the paediatric surgery department were included in this study. A detailed history was taken, and examination was performed for the primary as well as the associated defects. Appropriate investigations like invertogram, cologram were done wherever indicated. Management was as per the standard protocol. The data was recorded and analyzed. Results: Out of the 90 patients, 52 (57.77%) male patients and 38 (42.22%) female patients. Most of our patients presented within first 24 hours of life. Patients who presented after 72 hours were either female patients with anovestibular malformation or male patients with anocutaneous fistula. In the present study, High anomalies are the most common anorectal malformation in this study. Cardiac and Urological abnormalities are the most common associated anomalies with anorectal malformation. In this study we have performed ultra sound in all and voiding cystourethrogram in87/90 patients. In 29 patients ultrasound was abnormal. VCUG was abnormal in 25 patients. Conclusions: Anorectal malformations occurred equally in males and females. Females had intermediate defects more frequently, rectovestibular fistula being the commonest. Males were more likely to have high lesions; anorectal agenesis without fistula was the commonest defect. The most common associated defects seen were vesicoureteric reflux and esophageal atresia. Complications were seen more commonly in males with high lesions. There was a significant association between presence of an associated defect and mortality and morbidity.

Application of a Lateral Pedicled Cranial Bone Flap for the Treatment of Secondary Zygomaticomaxillary Defects.

This study aimed to evaluate the effect of laterally-based pedicled cranial bone flap (LBPCBF) for the repair of secondary zygomaticomaxillary defects. Between December 2014 and December 2016, 6 patients with unilateral zygomaticomaxillary defects were selected; of these, 5 had trauma, and 1 was exposed to titanium mesh in the infraorbital area due to radiotherapy following total maxillectomy for right maxillary squamous cell carcinoma. Preoperatively, 3 patients suffered from an intraoral vestibular groove fistula, 2 suffered from an extraoral fistula, and 1 from local exposure of titanium mesh. Surgical treatment was implemented in all 6 patients, of which, the 5 trauma patients underwent debridement, reduction, and fixation of periorbital fracture, followed by repair of the defects in the infraorbital margin, anterior wall of maxillary sinus, and zygomatic body with LBPCBF, and then, reconstruction of the orbital floor with titanium mesh. The other patient with exposed titanium mesh underwent repair of defects in the infraorbital margin and anterior wall of the maxillary sinus with LBPCBF after titanium mesh trimming. Postoperative review at 6 months revealed disappearance of intraoral fistula, wound healing, and improvement of facial deformity in all 6 patients, of which 2 patients demonstrated postoperative ectropion. The application of LBPCBF can simultaneously repair bone defects in the infraorbital margin, anterior wall of maxillary sinus, and zygomatic body, as well as, supplement the soft tissue, thereby indicating a satisfactory treatment effect.

Delayed diagnosis of anorectal malformations in neonates.

Anorectal malformations (ARM) are common congenital abnormalities of the terminal hindgut. Ideally, ARM should be diagnosed at, or shortly following, birth by careful physical examination of the perineum. Delayed diagnosis has been implicated as a risk factor for complications, including intestinal perforation. This study aimed to determine the rate of delayed diagnosis and associated intestinal perforation in ARM. A retrospective review was performed for all ARM patients managed at The Royal Children's Hospital over a 16-year period (2000-2015). Data collected included ARM type, timing of diagnosis and complications. Delayed diagnosis was defined as being at more than 24 h of age. A total of 243 ARM patients (male 146/243, 60%) were included. The most frequent ARM types were perineal fistula (83/243, 34%) and rectovestibular fistula (40/243, 16%). Diagnosis was delayed beyond 24 h of age in 92 of 243 (38%) patients. The ARM type most commonly delayed in diagnosis was perineal fistula (37/83, 45%). Two patients in whom diagnosis was delayed suffered an intestinal perforation. Delayed diagnosis in ARM patients remains a common, and potentially fatal, occurrence. Improved assessment of newborns is required to ensure timely diagnosis of ARM, and avoidance of complications associated with delayed diagnosis.

Middle-term bowel function and quality of life in low-type anorectal malformation

BackgroundLow-type anorectal malformations (ARMs) are considered benign; however, in China, data regarding such conditions are limited. Thus, this study aimed to assess the middle-term bowel functions and quality of life (QOL) among children with low-type ARM.MethodsChildren > 3 years of age who underwent treatment for low-type ARM (vestibular fistula [VF] and perineal fistula [PF]) during 2013 and healthy children were included. The children were interviewed during their outpatient visits. The primary outcome measures were bowel function, as assessed using the Baylor Continence Scale (BCS), and QOL, as measured using the Pediatric Quality of Life Inventory (PedsQL 4.0).ResultsA total of 82 patients responded; mean patient age was 6.8 (range, 3–12) years. Mean BCS score in the control group (7.94 ± 4.74) was significantly lower than that in the VF (18.69 ± 11.11, P < 0.001) and PF (15.47 ± 6.50, P < 0.001) groups. However, there were no differences in PedsQL 4.0 scores among the groups. The patients scored the lowest for emotional function and the highest for physical function. Nearly all measurements of QOL significantly decreased with increased BCS score.ConclusionsChildren with low-type ARM can achieve good bowel control and QOL. However, although ARMs are benign, several children with this condition suffer from anal function problems that affect QOL. Redo operations, mislocated anus, and incorrect constipation treatment are the iatrogenic causes of fecal incontinence.

Single-stage reconstruction of third-degree perineal lacerations in horses under general anesthesia: Utrecht repair method.

To describe perioperative management, surgical procedure, and outcome in mares with third-degree perineal lacerations (TDPL) treated with a single-stage repair, the Utrecht repair method (URM). Retrospective study. Twenty mares with TDPL. Medical records of mares with TDPL reconstructed with a URM were reviewed for perioperative management; surgical outcome; and postoperative fertility, athletic performance, and complications. Mares ranged in age from 3.5 to 11 years. Long-term follow-up was available for 13 mares. Mean duration of follow-up was 9 years (median, 9.5; range, 2-215 months (17.9 years)). Standardized perioperative fasting and postoperative refeeding protocols were used. Only five mares received supportive gastric medication. Reconstruction of the rectovestibular shelf was successful in 18 of 20 mares. Two of 20 mares developed a small rectovestibular fistula after the initial repair, which was successfully repaired with a second surgery. Other postoperative complications were observed in 13 mares and consisted of mild postanesthetic myositis, facial nerve paralysis, esophageal obstruction, rectal obstipation, partial perineal dehiscence, and rectal or vestibular wind-sucking. Six of seven mares that were subsequently bred became pregnant. One mare was successfully used for embryo recovery, and five of six mares foaled without recurrence of a TDPL. Nine of 13 mares were used for riding at various levels. The alternative single-stage reconstruction for TDPL was successful in 18 of 20 mares after a single surgery. No major complications related directly to the technique were noted. The URM is a valid alternative surgical technique for repairing TDPL in mares.

The rare case of a true rectovaginal fistula and its repair

Abstract Background Within the spectrum of anorectal malformations, congenital rectovaginal fistulae are a rare but treatable entity. These less common variates can be treated much in the same fashion as other malformations such as imperforate anus or rectovestibular fistulae are repaired. Summary In the case presented here, diagnosis of a rectovaginal fistula was delayed despite repeated examinations until the patient reached a size where the fistula was easily identifiable. The patient had no other anorectal malformation and was otherwise in good health. Her repair was done as a same day surgery and the patient recovered without complication. Conclusion A rectovaginal fistula is a highly unusual clinical entity but one amenable to repair in the same fashion as other similar malformations.

Screening for associated anomalies in anorectal malformations: the need for a standardized approach.

Anorectal malformations (ARM) are common congenital abnormalities of the terminal hindgut. The high incidence of associated anomalies necessitates systematic screening, which should include renal and spinal ultrasonography, spinal radiography and an echocardiogram. This study aimed to determine the incidence of associated anomalies in ARM, and whether screening protocols were appropriately applied. A retrospective review was performed of all ARM patients managed at The Royal Children's Hospital, Melbourne over a 16-year period (2000-2015). Data collected included ARM type, presence of associated anomalies, as well as utilization of renal and spinal ultrasonography, spinal radiography and echocardiography. A total of 243 patients (male 146/243, 60%) were reviewed. The most frequent ARM types were perineal fistula (83/243, 34%) and rectovestibular fistula (40/243, 16%). Full screening was performed in 153/243 (63%), while 18/243 (7%) received no screening. In fully screened patients, associated anomalies were diagnosed in 143/153 (93%), with cardiovascular, renal and musculoskeletal anomalies being most frequent. The high incidence of associated anomalies identified in fully screened ARM patients highlights the importance of systematic screening. Clinically significant anomalies may have been overlooked in the more than one-third of ARM patients in whom screening was absent or incomplete. Standardized screening protocols for ARM patients have now been implemented.

Anterior sagittal anorectoplasty as a technique for the repair of female anorectal malformations: A twenty two-years-single-center experience

BackgroundAnorectal malformations (ARMs) are major congenital malformations occurring in female children in approximately 1 out of every 4000–5000 births. Posterior sagittal anorectoplasty (PSARP) was introduced for their treatment in early 1980s by Pena and Devries. However, anterior sagittal anorectoplasty (ASARP) was first introduced by Okada as a modification of the previous technique. PurposeThe aim of this study was to report our experience and the long-term follow-up results of 594 female patients with anorectal malformations treated by anterior sagittal anorectoplasty (ASARP) in a single center over 22 years. We chose this approach as the incision is smaller, with minimal cutting of the external sphincter and easier dissection of the vagina and rectum.The type of our study was retrospective study. ResultsThe study included 594 patients; 342 of them were less than 1 year old. About 526 patients had rectovestibular fistula, anterior perineal anus in 55 patients, anocutaneous fistula in 6 patients and rectovaginal fistula in 7 patients. All of these patients had been subjected to ASARP technique which had been done in one stage without proceeding colostomy in about 470 cases and in staged fashion with a protective colostomy in 124 patients. Postoperative follow up revealed that four patients (0.7%) developed anal retraction, 30 patients (5%) developed anal stricture, 20 patients (3.4%) had rectal prolapse and 40 patients (6.7%) developed anterior anal migration. Constipation was observed in 161 patients (27%) by the end of 1st postoperative year reduced to 41 patients (7%) after one year. 474 patients were assessed for continence by Kelly's continence score and 453 patients showed good result, 16 patients showed fair result and about 5 patients had poor continence outcome. ConclusionAnterior sagittal anorectoplasty is a procedure with optimal functional outcome especially at if done at an early age in one stage without preliminary colostomy.

Validity and Responsiveness of the Visual Vertigo Analogue Scale.

People with visual vertigo have dizziness provoked by visual stimulation. We have developed a Visual Vertigo Analogue Scale (VVAS) to evaluate their symptoms and response to rehabilitation. Our goal was to validate the VVAS against the Situation Characteristics Questionnaire (SITQ) score and determine its responsiveness to treatment. Participants (n = 115) completed 3 questionnaires: Dizziness Handicap Inventory (DHI), VVAS, and the SITQ at their initial and final sessions of vestibular rehabilitation. The SITQ was analyzed using the Space Motion Discomfort (SMD1) outcome measure and by calculating the average score of all the items (SMDavg). The results were stratified into those who had a significant clinical change in their DHI score posttreatment and those who did not. Associations of the VVAS with SMD scores and change scores were determined by Pearson and Spearman correlations. Nonparametric t tests were used to compare the 2 DHI groups on the clinical outcomes. There were significant associations between VVAS scores (P < 0.0001) and both SMD1 (βVVAS = 0.02) and SMDavg scores (βVVAS = 0.03). Significant differences existed between the 2 DHI groups for all scores: VVAS (P = 0.0002), SMD1 (P = 0.02), and SMDavg (P = 0.0001). VVAS scores correlated well with SMD1 and SMDavg scores. Changes in VVAS pre- and posttreatment scores corresponded to the changes seen in SMD1 and SMDavg scores. The VVAS shows validity and responsiveness to change. The VVAS can be used to detect clients with visual vertigo and to verify the progression of the client's symptoms.Video Abstract available for more insights from the authors (see Video, Supplemental Digital Content 1, http://links.lww.com/JNPT/A258).

The impact of perioperative care on complications and short term outcome in ARM type rectovestibular fistula: An ARM-Net consortium study

BackgroundThe impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown. MethodsAn ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered. ResultsOverall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%).Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation. ConclusionsDifferences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions. Type of studyTreatment study. Level of evidenceIII

Can fecal continence be predicted in patients born with anorectal malformations?

PurposeThe purpose of this study was to identify factors associated with attaining fecal continence in children with anorectal malformations (ARM). MethodsWe performed a multi-institutional cohort study of children born with ARM in 2007–2011 who had spinal and sacral imaging. Questions from the Baylor Social Continence Scale were used to assess fecal continence at the age of ≥4 years. Factors present at birth that predicted continence were identified using multivariable logistic regression. ResultsAmong 144 ARM patients with a median age of 7 years (IQR 6–8), 58 (40%) were continent. The rate of fecal continence varied by ARM subtype (p = 0.002) with the highest rate of continence in patients with perineal fistula (60%). Spinal anomalies and the lateral sacral ratio were not associated with continence. On multivariable analysis, patients with less severe ARM subtypes (perineal fistula, recto-bulbar fistula, recto-vestibular fistula, no fistula, rectal stenosis) were more likely to be continent (OR = 7.4, p = 0.001). ConclusionType of ARM was the only factor that predicted fecal continence in children with ARM. The high degree of incontinence, even in the least severe subtypes, highlights that predicting fecal continence is difficult at birth and supports the need for long-term follow-up and bowel management programs for children with ARM. Type of StudyProspective Cohort Study. Level of EvidenceII.

Treatment using diffuse laser energy of cochlear and vestibular fistulas caused by cholesteatoma.

To measure the outcomes of laser treatment of cholesteatoma covering cochlear and vestibular fistulas. Cholesteatoma matrix over the fistula was denatured; the power density was sufficient only to gradually heat, but not vaporise, the keratin-forming matrix. The denaturing speed was controlled so that the integrity of the fistula cover was maintained. The change in bone conduction threshold and the residual rate of cholesteatoma at the fistula were measured. Thirty-six fistulas were assessed. There were seven cochlear fistulas. All were 5 mm or less in maximum length. For the entire group, the average change in bone conduction threshold was -0.3 dB. For cochlear fistulas, the average change in bone conduction was + 0.2 dB. The distribution of hearing results for the entire group was Gaussian; the apparent changes in hearing could be attributed to errors associated with testing. All patients underwent second-stage surgery. In all cases, the cholesteatoma was completely cleared from the fistula site. There were no facial palsies. Laser denaturing of cholesteatoma matrix over fistulas measuring 5 mm or less of vestibular apparatus and the cochlea is effective at eliminating cholesteatoma, and is not associated with cochlear hearing loss or facial palsy.

Unusual Recto-colonic Tubular Duplication in a Female Neonate

Complete tubular colonic duplication is exceedingly rare. A second ectopic opening in the perineum other than a normally cited anus could be an unusual presentation. We report an unusual case of recto-colonic duplication in a 16-day-old girl who presented with fecal discharge from a vestibular opening in addition to a normally situated anus. The diagnosis of total recto-colonic tubular duplication associated with a rectovestibular fistula and a normal anus was confirmed by barium enema and computed tomography scan with double contrast. At operation, we performed a long transanal incision of the common septum to create an anastomosis between the normal and duplicated colon with complete submucosal excision of the rectovestibular fistula. The patient was doing well at 3-year follow-up examination.

Unusual Recto-colonic Tubular Duplication in a Female Neonate

Complete tubular colonic duplication is exceedingly rare. A second ectopic opening in the perineum other than a normally cited anus could be an unusual presentation. We report an unusual case of recto-colonic duplication in a 16-day-old girl who presented with fecal discharge from a vestibular opening in addition to a normally situated anus. The diagnosis of total recto-colonic tubular duplication associated with a rectovestibular fistula and a normal anus was confirmed by barium enema and computed tomography scan with double contrast. At operation, we performed a long transanal incision of the common septum to create an anastomosis between the normal and duplicated colon with complete submucosal excision of the rectovestibular fistula. The patient was doing well at 3-year follow-up examination.

Хирургическая коррекция атрезии прямой кишки с ректовестибулярным свищом

Хирургическая коррекция атрезии прямой кишки с ректовестибулярным свищом

Anorectal malformations and perineal hemangiomas: The Arm-Net Consortium experience

AimPerineal hemangiomas rarely occur in patients with anorectal malformations (ARMs), but they can pose a significant challenge and warrant special attention. Surgical incision of posterior sagittal anorectoplasty (PSARP) may involve the hemangioma site resulting in hemorrhage, damage to blood supply, leading to complications and adversely affecting outcome. The aim of this study was to review the experience of the ARM-Net Consortium in the management of perineal hemangioma associated with ARM and evaluate treatment strategies. Materials and methodsData on all patients with ARM and a perineal hemangioma located in the planes of the PSARP dissection who were managed at participating ARM-Net centers were collected retrospectively by questionnaire, as follows: ARM type, hemangioma distribution and penetration, imaging findings, medical/surgical management, timing of definitive repair, complications and outcome. ResultsTen patients from eight centers were included. Three patients each had a rectobulbar or rectovestibular fistula, 2 had a rectoperineal fistula, and one had a rectoprostatic fistula; in one patient, the hemangioma was too disfiguring to determine malformation type. Mean follow-up time was 36.6 months (median 29 months). Colostomies were performed before definitive repair in 8 patients. Five patients received systemic beta-blockers before PSARP: 3 were operated uneventfully following partial/complete involution of the hemangioma, and 2 are awaiting surgery. The two patients with rectoperineal fistula were managed expectantly. The remaining 3 patients underwent surgery with no preoperative medical treatment, and all had complications: mislocated neoanus in three and complete perineal dehiscence in one. ConclusionAttempting PSARP in the presence of a perineal hemangioma may lead to complications and adversely affect outcome. This study confirms the benefits of beta blocker treatment before surgical reconstruction. Level of evidenceTreatment study, level III.

ANTERIOR SAGGITAL ANORECTOPLASTY

… Anorectal malformation is the common congenital malformation. Ectopic anusand vestibular fistula are Intermediate types of anorectal malformations (ARM), which are themost common in female babies. Many surgical procedures have been described for the treatmentof ARM. Anterior Saggital Anorectoplasty (ASARP) is not only convenient for the anesthetist formaintenance of anesthesia but also gives better exposure of surgical structures during surgery.Objectives: To determine the technical suitability and outcome of ASARP in intermediate typesof ARM in female children. Design: This Descriptive study with prospective collection of dataaccording to a set protocol. Setting: The study was carried out at the department of PediatricSurgery, Military Hospital, Rawalpindi, Pakistan. Period: November 2010 to March 2014, overthe period of 3 years and 5 months. Patients and Methods: The data of all female patientspresenting with intermediate types of ARM and undergoing ASARP, during the study periodwere analyzed, with respect to age, type, associated anomalies, complications and cosmeticoutcome. Results: A total of 36 patients of intermediate variety underwent ASARP. Age rangedfrom 6 months to 22 years. All patients had colostomy prior to this procedure. During surgery,posterior vaginal wall tear occurred in 2 patients (5.5%). Postoperatively, 2 patients (5.5%) hadretention of urine, 2 patients (5.5%) developed wound infection with superficial disruption, analstenosis occurred in 2 patients (5.5%) and 1 patient (2.7%) had rectal mucosa prolapse. Noneof them required re_ do surgery. Cosmetic outcome was excellent in 31 patients (86.1%), while itwas satisfactory in 5 (13.8%) patients. Conclusions: Anorectoplasty through anterior approachis not only technically easy but has good cosmetic results in intermediate type of imperforateanus in female children.

Comparative study of outcome following primary posterior sagittal ano-rectoplasty and primary anterior sagittal ano-rectoplasty for vestibular fistula

Background: To compare the postoperative complications and bowel function following primary PSARP and primary ASARP.Methods: This prospective study was carried out over a period of 4 years. Patients above 4 months, who needed surgical repair for vestibular fistula were included in study. They were randomly allocated into PSARP group and ASARP group. After surgical intervention, patients of both groups were compared with respect to post-operative complications, voluntary bowel control, constipation, need for laxatives.Results: 44 patients were enrolled in the study. Of these, 22 patients were allocated to primary PSARP group while remaining patients underwent ASARP. One patient from each group was lost to follow‑up and hence, excluded from the final analysis. The two groups were comparable with respect to age, maturity at birth, weight at the time of surgery, blood investigations. Total 16 patients had associated congenital anomalies. During the postoperative period, four patients from PSARP group and two patients from ASARP group had superficial wound infection of perineal incision which was managed conservatively. One patient in PSARP group had a major breakdown of perineal wound with retraction of pulled rectum which required colostomy. There was no recurrence of fistula in any patient. No patient had stenosis of neo-anus or anterior displacement of rectum. Functional assessment of bowel function was done in 25 patients who completed 3 years of age. Voluntary bowel movements were observed in 75% of cases in the ASARP group compared to 38 % in PSARP group. 15% patients of PSARP group and 7.5% patients with ASARP had soiling. Though the difference was not statistically significant, nearly 38% of the patients after PSARP, needed laxative for normal bowel habit compared to 8% patients in ASARP group.Conclusions: ASARP promises many advantages in the treatment of vestibular fistula in comparison to PSARP. Comparable post-operative complications, good cosmetic results, excellent continence with less need for laxatives are the advantages of ASARP.

One-stage repair of anorectal malformations in females with vestibular fistula: a systematic review and meta-analysis.

Females with recto-vestibular fistula (RVF) can be managed either by one-stage sagittal anorectoplasty (SARP) or by conventional multi-stage approach with colostomy followed by SARP. Our aim was to define which approach, one-stage or multi-stage, is safer and more beneficial. Using a defined search strategy, two investigators identified all comparative studies on the mentioned procedures. The study was conducted under PRISMA guidelines. The meta-analysis was performed using RevMan 5.3. Data are mean ± SD. Of 649 titles/abstracts screened, 13 full-text articles were analyzed. Three studies were included (156 females). One-stage SARP was associated with increased risk of wound infection (24.3 ± 8.7%) compared to multi-stage approach (10.9 ± 2.5%; p < 0.01) and increased risk of wound dehiscence (16.2 ± 4.8% vs. 2.4 ± 1.1%, respectively; p < 0.01). The incidence of anorectal stenosis was higher following one-stage repair (33.3%) vs. multi-stage approach (10.7%; p < 0.05). No differences were found with regards to redo SARP in both groups (12.9 ± 7.3% vs. 4.8 ± 0.8%; p = ns). At follow-up, the prevalence of soiling and constipation were similar after one-stage (19.7 ± 10.3% and 29.5 ± 5.4%) and multi-stage repair (13.7 ± 8.9% and 28.7 ± 4.4%; p = ns). In females with RVF, the SARP performed without protective colostomy increases the risk of postoperative complications. However, this one-stage approach seems not to be associated with reduced fecal continence.

Managing adults with previously treated anorectal malformations

Abstract Background Although rare, it is not unheard of to encounter patients whose anorectal malformations have been ignored until adulthood in resource-limited environments. However, it is extremely uncommon to be faced with patients whose early primary management was directed toward a permanent stoma. In cases where the proximal rectum is all but sacrificed, the management for definitive repair and reconstitution of intestinal continuity is a challenge. Little information exists in the literature. Methods The management of two adult female patients born with rectovestibular fistulas in a resource-limited environment is reviewed. Their management was complicated by inadequate repair, followed by resection of the distal intestine, and creation of a permanent end stoma. Both patients were seeking definitive repair of the inadequately treated anorectal malformation and reconstitution of intestinal integrity. The existing literature is reviewed. Results Both patients are doing well three years after the final surgical interventions. Neither patient has fecal incontinence. One initially had issues with constipation, but has successfully overcome the problem using dietary measures. Neither has issues with the genitourinary system. Review of the literature revealed numerous articles describing the delayed presentation of patients with anorectal malformations, but none describing this specific group of patients in whom the initial management specifically targeted a permanent stoma. Conclusion Adults with inadequately repaired rectovestibular fistulas whose initial surgical management specifically targets resection of the proximal rectum and creation of a permanent end colostomy are rare. Their management is a challenge, especially in resource-limited environments. When the anorectal malformation is of a favourable type, appropriate investigation coupled with meticulous surgical planning of the definitive repair may yield excellent outcomes.