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

Background Teenage 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. Case 15-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. Conclusion Due 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.

Prevalence and Association of Occult Spinal Dysraphism with Anorectal Malformation

Background: Anorectal malformation (ARM) is associated with occult spinal dysraphism (OSD). The guideline for screening for OSD in patients with ARM is still controversial. Objective: 1) To evaluate the prevalence of OSD in each type of ARM and to elucidate which types of ARM should be screened for underlying OSD. 2) To compare the methods used in radiologic screening for OSD, such as plain lumbosacral X-ray, spinal ultrasound (U/S), and spinal magnetic resonance imaging (MRI). Materials and Methods: The medical records of all patients with ARM treated at Siriraj Hospital between 2006 and 2016 were reviewed. Spinal dysraphism screening with lumbosacral X-ray, U/S, or MRI were performed depending on the surgeon’s request. Associated urinary tract anomalies were noted. Data of the surgical treatments, outcomes, and complications of treatments were collected. Results: Among the 204 ARM patients treated, 150 patients were screened for OSD, involving 150 lumbosacral X-ray, 65 U/S, and 39 MRI. The prevalence of OSD (n=33) in all types of ARM was 16.2%. The higher types of ARM had higher prevalence of OSD, except for vestibular fistula (20.5%). The prevalence of OSD in cloaca malformation, rectobladder neck fistula, rectoprostatic urethral fistula, rectobulbar urethral fistula, and perineal fistula were 28.6%, 33.3%, 16.7%, 12.5%, and 9.8%, respectively. Lumbosacral X-ray had a sensitivity of 51.7% and was not a good screening method. Sixteen of 65 U/S revealed OSD (24.6%). Tethered cord and syringomyelia were noted in nine and seven, respectively. Spinal MRI (n=39) revealed 27 OSD (69.2%) comprising 16 tethered cords, 12 spinal lipoma, and 10 syringomyelia. ARM associated with KUB anomalies had a statistically significant correlation with OSD. In 33 OSD, 17 patients (51.1%) were operated on. Fourteen received untethering spinal cord, while five had intradural lipoma excised. Conclusion: Patients with ARM had a high prevalence of OSD and should be screened for. U/S is the first-line method. MRI is strongly recommended in cloaca malformation, rectobladder neck fistula, rectoprostatic urethral fistula, vestibular fistula, and all ARM associated with KUB anomalies. Other types of ARM should be screened by MRI if feasible. Keywords: Spinal dysraphism, Tethered cord, Anorectal malformation

PRIMARY PROCEDURE FOR RECTO-VESTIBULAR FISTULA IN FEMALE CHILDREN: AN EARLY EXPERIENCE AT KHYBER TEACHING HOSPITAL PESHAWAR.

Background: Recto-vestibular fistula is the most common variant of Anorectal malformation in female children. Traditional management of this entity is a three stages procedure i.e. colostomy, PSARP, and colostomy closure. The aim of this study was to manage these patients by a primary definitive surgical procedure without colostomy and to know the procedure feasibility, cost effectiveness, complications, safety and short term functional outcome. Materials and method: Results: A total of 40 female children fulfilling inclusion criteria of the study were included. Age range was 28 days to 8 months. No mortality noted during study period. Mean operative time was 50±15 minutes. Mean Hospital stay in days was 6.65.Procedure related complications were recorded as wound infection 5(12.5%), wound dehiscence 1 (2.5%), posterior vaginal wall injury 5(12.5%).Overall parental satisfaction regarding procedure was 92.5%. Conclusion: Primary single stage procedure either by PSARP or ASARP for the correction of Rectovestibular fistula in female children of ARM was less traumatic to children, acceptable to their parents, having cost effectiveness for poor socioeconomic parents and encouraging functional outcome.

OUTCOME OF SINGLE STAGE SPHINCTER SPARING SCARLESS ( 5S) PROCEDURE FOR RECTOVESTIBULAR FISTULA: A RETROSPECTIVE ANALYSIS

Background: Anorectal malformations (ARM) in girls comprise of a wide spectrum of disease ranging from imperforate anus to common cloaca, a complex malformation. Recto-vestibular fistula (RVF) is the commonest ARM in female patients. Many surgical procedures have been mentioned in the literature but trend is changing from staged to single stage procedure. Objectives: To evaluate post operative results of Single Stage Sphincter Sparing Scarless (5S) procedure for RVF. Methods: It is a retrospective case series of 24 patients with RVF who were admitted in the Department of Paediatric Surgery Jinnah Hospital Lahore and the Children Hospital and the Institute of Child Health Multan from October 2018 to September 2019, between 14 days and 4 years of age , underwent single stage sphincter sparing scarless procedure without any colostomy, anterior or posterior midline incision or division of sphincteric complex. Site of neo-anus was marked with the help of muscle stimulator and all surgeries were done under general anesthesia after meticulous gut preparation. Post operatively patients were kept nil per oral for 5 days to avoid wound contamination due to stool. Follow up was done for six months to evaluate outcome. Authors used a new name for single stage procedure. Results: Mean age was 160 days, operative time 78 minutes and hospital stay 6.8 days. 9 (37.5%) patients were diagnosed with some other associated congenital anomalies like congenital heart disease (CHD), renal anomalies, hemisacrum, syndactyly, talipes equino varus(TEV) and Downs syndrome. As a whole 13(24) presented with complication. 4(16.64%) patients presented with constipation, 3(12.5%) excoriation, 2(8.32%) anal stenosis, 2(8.32%) soiling, 1(4.16%) retraction of rectum and 1(4.16%) superficial wound infection. Patient with retraction was planned for re-do surgery, all others were managed conservatively. Conclusion: Single stage sphincter sparing scarless ( 5S) procedure for recto-vestibular fistula is safe, simple and cost effective technique. Patient suffering is minimum, as there is no colostomy, so no multiple surgeries. Complications are minimum and comparable to staged procedure.

Safety & Cost-Effectiveness of Primary Neonatal Posterior Sagittal Ano-recto Plasty (PNPSARP)

Background: Early repair of Anorectal malformation (ARM) within 6 months may be one of the factors that improve fecal continence. Delayed and multiple-stage repairs require dilatation, strict bowel preparation, fasting and total parenteral nutrition (TPN). PNPSARP requires neither bowel preparation nor parenteral nutrition. We believe it can be achieved within 72 hours of life.Aim: To evaluate the safety, feasibility and cost effectiveness of PNPSARP within 72 hours of life versus delayed or multiple-stage repair of vestibular and perineal fistula. Material & Methods: A retrospective study was carried out of all newborns with ARM at our institute between August 2016 and August 2019. PNPSARP within 72 hours of life was compared with delayed or multiple-stage repair. Neither bowel preparation nor parenteral nutrition was required in the PNPSRP group. Perioperative complications and costs were evaluated. Results: Eight PNPSARP were compared with 7 delayed or multiple stage repair over the study period. Four babies (50%) were operated at day 1 post delivery in the PNPSARP group. Mean operative time (MOT) was 109 minutes (68-155). Mean follow up period was 22 months (12-36). One girl with a vestibular fistula had wound infection. This group had a good outcome with no morbidity, high satisfaction rate and low costs in comparison to delayed or multiple-stage repair.Conclusions: PNPSARP for perineal and vestibular fistula with supportive ancillary services in the first few days of life appears to be safe and cost effective with minimal morbidity. PNPSARP is now the standard technique in our unit.

The outcome of two stage repair in anorectal malformations with rectovestibular fistula.

Wound infection and dehiscence after recto vestibular fistula repair may affect the fecal continent mechanisms. A significant number of children with anorectal malformations have long term social, economic and psychological problems due to fecal incontinence. The role of protective colostomy should not be under estimated especially when you are treating the children from poor socioeconomic settings with compromised nutritional status. Objectives: The aim of this study was to evaluate the outcome of two stage limited posterior sagittal anorectoplasty with protective colostomy in female patients of congenital recto vestibular fistula. Study Design: Prospective study. Settings: Department of Pediatric Surgery, DHQ Teaching Hospital Sahiwal. Period: January 2016 to December 2018. Material & Methods: Thirty four girls with anorectal malformation and recto vestibular fistula underwent two stage anorectoplasty. Divided sigmoid colostomy and limited posterior sagittal anorectoplasty was performed in first stage. Six to eight weeks later stoma was closed in second stage. All the patients were evaluated for fecal continence, constipation, bowel function and complications of stoma formation, definite procedure and stoma closure. Results: The age distribution of patients at the time of surgery ranged from 9 months to 5 years (mean 27.32 months). During the first stage of repair, three patients (8.82 %) developed wound infection. Mucosal prolapse was seen in two patients (5.88%). Anal stenosis was noticed in one patient (2.94%). Seven patients (20.58%) developed peri stoma skin excoriation. Stoma prolapse was observed in three patients (8.82%). Four patients (11.76%) developed wound infection after colostomy closure. During the follow up period, constipation was reported in five patients (14.70%) and soiling in one patient (2.94%). Conclusion: Two stage correction of congenital recto vestibular fistula under the cover of colostomy is safe strategy. It is strongly recommended in a setup with limited resources and weak infrastructure. It is also useful for patients from poor socioeconomic settings.

Sigmoid Colon Atresia in a Female with Imperforate Anus without Vestibular Fistula

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Salvage Laparoscopic-Assisted Anorectoplasty after Failed Vestibular Fistula Repair Using Magnetic Resonance Image Guidance

Patients with vestibular fistula have a good functional outcome after posterior sagittal anorectoplasty (PSARP). While continence is often preserved, close follow-up and management of constipation are often required. Redo anorectal surgery has been associated with worse functional outcomes compared with primary procedures, possibly due to injury and scarring of the pelvic floor musculature and sphincter complex. Our group has a growing experience in the use of intraoperative real-time magnetic resonance imaging (MRI) for anorectal malformation repairs. We present a case of salvage operation of a failed PSARP for vestibular fistula.