Congenital pouch colon (CPC) is an unusual abnormality in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation. It is categorized into four subtypes (Types I–IV) based on the length of normal colon proximal to the colonic pouch. In males, the pouch usually terminates in a colovesical fistula just proximal to the bladder neck. In girls, the terminal fistula opens either into the urethra or in the vestibule, close to the urethral opening. Girls usually have a double vagina with a wide inter-vaginal bridge, a monocornuate uterus on each side, and urinary incontinence due to a widely open bladder neck. Associated major malformations are uncommon with CPC but sometimes, especially in reports from outside India, major abnormalities are present suggesting an early, severe error in embryogenesis. The more severe Types I/II CPC can usually be diagnosed by a large gas shadow or air-fluid level on X-Ray abdomen. For all subtypes of CPC, it is preferable to preserve a segment of the pouch by fashioning a narrow colonic tube for pull-through, the technique known as coloplasty or tubular colorraphy. Girls need additional management of the genitourinary abnormalities. Postoperatively, fecal continence levels are usually poor, especially with Types I/II CPC.

Aims and Objectives: The anatomical types of female anorectal malformation (ARM) are complex and include several rare and regional variants. The aim of this report is to highlight the diagnosis and management of cases of anorectal agenesis with rectovaginal fistula (RVF). Materials and Methods: This is a retrospective chart review of cases of RVF who underwent definitive surgery between November 2000 and September 2016 in a single institution. Results: Of the 244 female patients with anorectal anomalies treated in our institution over a 16-year period, there were 15 cases of RVF with anorectal agenesis giving it an incidence of 6%. All cases of RVF with anorectal agenesis presented with absence of anal opening, varying degree of abdominal distension, and history of passage of fecal matter per vaginum. The mean age of presentation was 3 months (newborn to 1 year). Sigmoid colostomy was done in 13 cases. Distal colostogram demonstrated high RVF which was further confirmed by examination under anesthesia (EUA) and endoscopy before definitive surgery. With the posterior sagittal approach, division of the RVF and anorectoplasty was possible in 14 cases; however, one patient required an abdominal mobilization of the colon for pull through due to a very short distal bowel segment. During a mean postoperative follow-up of 5 years, cosmetic and functional results were satisfactory. Conclusion: RVF with anorectal agenesis is a rare/regional variant of female ARMs. Clinical examination along with distal colostogram, EUA, and endoscopy clinches the diagnosis. Anorectal reconstruction by posterior sagittal anorectoplasty results in a satisfactory outcome.

Background: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. Aim: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy – PSARP – colostomy closure) methodology in a high volume tertiary care institution of a developing country. Patients and Methods: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score. Results: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery. Conclusions: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Introduction: Females with Krickenbeck low-type anorectal malformations - vestibular fistula (VF) and perineal fistula (PF) - are managed either by a primary definitive or conventional three-staged approach. Ultimate outcome in these children may be affected by wound dehiscence leading to healing by fibrosis. Most of the literature favors one approach over other based on retrospective analysis of their outcomes. Whether a statistically significant difference in wound dehiscence rates between these approaches exists needed to be seen. Materials and Methods: A randomized controlled trial for girls <14 years with VF or PF was done. Random tables were used to randomize 33 children to Group I (primary procedure) and 31 to Group II (three-staged procedure). Statistical analysis was done for significance of difference (P < 0.05) in the primary outcome (wound dehiscence) and secondary outcomes (immediate and early postoperative complications). Results: Of the 64 children randomized, 54 (84%) had VF. Both groups were comparable in demography, clinical profile and age at surgery. The incidence of wound dehiscence (39.4% vs. 18.2%; P = 0.04), immediate postoperative complications (51.5% vs. 12.9%; P = 0.001), and early postoperative complications (42.4% vs. 12.9%; P = 0.01) was significantly higher in Group I as compared to Group II. Six of 13 children (46.2%) with dehiscence in Group I required a diverting colostomy to be made. Conclusions: Females with VF or PF undergoing primary definitive procedure have a significantly higher incidence of wound dehiscence (P = 0.04), immediate (P = 0.001) and early postoperative complications (P = 0.01).

Background: Anorectal malformation (ARM) is one of the most common pediatric surgical problems dealt in day-to-day practice. Although the outcome of surgery has improved a great deal over the last three decades fecal incontinence (FI) is still a common long-term morbidity that affects the quality of life of these patients. Bowel wash (BW) program with pelvic floor exercise are standard care of management for these patients. This study was undertaken to assess the role of interferential therapy (IFT) along with BW compared to BW alone in the management of FI. Methodology: Twenty-four children more than 2-year-old age previously operated for malformation and having FI with Kelly score <4 were recruited and assigned to one of the two groups. One group was given standard BW regimen and the other BW with IFT for 3 months. Posttherapy Kelly scoring was done, and the results were compared. Results: IFT with BW provided no added advantage over BW alone for the treatment of FI in patients of ARMs. Conclusion: The time-tested modality of BW is the cornerstone in the management of FI.

Introduction: Anorectal malformation (ARM) associated esophageal atresia (EA) with tracheoesophageal fistula (TEF) spawns special therapeutic propositions. The outcome of these patients banks on numerous factors. We performed this study with an aim to compare the outcome of single-staged simultaneous primary repair of both anomalies versus staged repair of these disorders. Materials and Methods: Retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015 after ethical approval was undertaken. Patients were split into two groups based on whether they underwent staged repair (Group A) or single-staged simultaneous primary repair of ARM with TEF (Group B). Patient's records were analyzed for demography, weight, gestational age, associated anomaly, preoperative and postoperative sepsis screen results, early and late postoperative complications (at least up to 1 year of age). Patient's Kelly score for continence at the age of 3 years or more was compared. Observation: A total of 28 were included in the study. Among these, 17 were managed with staged procedure for ARM (Group A), whereas 11 underwent simultaneous single-stage repair of ARM with TEF (Group B). No difference in continence score was observed in outcome between the two groups (P = 0.96). Overall mortality in Group A at 1-year follow-up was 52.9% and in Group B was 43.4%. Conclusion: The simultaneous single-staged primary repairs result in better long-term outcome in our setup.

Aim: A solitary functioning renal unit in high anorectal malformation may harbor anomalies in the upper/lower urinary tract that further compromise renal function and accelerate hyperfiltration injury. We aimed to analyze the clinical course, management, and outcome of children with a solitary renal unit and high anorectal malformation. Materials and Methods: A cohort of solitary renal units in anorectal malformation managed between 2000 and 2015 with a defined protocol at a pediatric nephro-urology clinic are included in the study. Investigations and management were individualized according to issues in the agenetic/dysplastic unit, solitary functioning unit, or bladder-urethra. Clinical details, management, and outcome were analyzed. Results: Of 53 cases of high anorectal malformation with urologic anomalies, 17 (32%) had single kidneys. Majority (10/17, 59%) had another complicating urinary tract anomaly, four being uremic at presentation. Nearly half had multiple urinary infections including epididymo-orchitis unrelated to the rectourinary fistula and warranted additional stoma (bowel/urinary) or other urinary tract surgery. At a mean follow-up of 70 months, majority (14/17, 82%) have a preserved solitary renoureteric unit while the remaining three await renal replacement therapy. Conclusion: Solitary functioning renal units associated with high anorectal malformation in children need meticulous investigation and surveillance to facilitate renal preservation. The implications of the associated rectourinary fistula, vesicoureteral reflux, urinary infections including epididymo-orchitis, and other urinary tract anomalies on the conventional surgical management are illustrated.

Introduction: In the management of cloaca, there is concern that dissection of the urogenital sinus in early childhood with the aim of total anatomical correction is hazardous. Avoiding such mobilization and providing mitrofanoff channel, when needed, till peripubertal period reduces complications and is technically easier. Materials and Methods: Forty-three cases of cloaca were managed in the period 2004–2016. Case records and radiology were reviewed retrospectively. The follow-up evaluation was done by looking into voiding history, bowel movements, and menstruation history. Results: There were three groups of children, namely, those with no reconstruction done elsewhere except a diverting fecal stoma (Group I, n = 25), those who had undergone anorectal correction elsewhere with no attempt at urogenital reconstruction (Group IIA, n = 13), and tho with attempted bowel and genitourinary reconstruction elsewhere (Group IIB, n = 5). The Group I children (one still awaiting reconstruction) underwent early rectal reconstruction followed by expectant management of the urogenital apparatus. The 18 referred cases had multiple problems, chiefly urogenital, of congenital or iatrogenic origin. While urinary reconstruction included bladder augmentation, ileal neobladder, bladder neck closure, and ureteric reimplantation, the foundation of urinary management was intermittent catheterization through mitrofanoff stoma and the avoidance of any dissection of the cloacal common channel. Surgery on the genital tracts included drainage of hydrocolpos, perineal surgery for low vaginae and abdominoperineal vaginoplasty for high vaginae in the peripubertal period with or without bowel supplementation. Spontaneous voiding was maintained in 17 of 25 (68%) Group I girls (including one death later from intestinal complications), 7 of 13 (54%), Group IIA girls, and 1 of 5 (20%) Group IIB girls. Painless menstruation was noted in eight postpubertal girls, three through the cloacal channel (awaiting reconstruction) and five through the reconstructed vagina. Most of the children are on a bowel management program for fecal cleanliness with washouts through the neoanus or Malone's stoma. Conclusion: We report a nonconventional approach to cloaca based on avoiding dissection of or around the common channel for urethrovaginal reconstruction, opting for mitrofanoff stoma for intermittent catheterization, when needed, and late vaginal reconstruction. We believe this approach has reduced the overall need for intermittent catheterization.

Aim: To assess the modifications in the technique of laparoscopic-assisted anorectal pull-through (LAARP) practiced at our institute and to analyze the postoperative outcome and associated complications. Materials and Methods: A retrospective study was done to analyze the results for LAARP procedure done for high anorectal malformations (ARMs) from January 2001 to May 2016. A total of 68 patients had undergone LAARP, with 62 male and 6 female children. Staged procedure was done in 55 patients and one child with rectovestibular fistula of 5 months of age had a single-stage procedure. Technical modifications such as traction over the fistula which helps in identification and dissection of the puborectalis muscle, dividing the fistula without ligation, railroading with Hegar's dilators over the suction cannula which creates adequate pull-through channel, have helped save time and make the procedure simpler. The patients were followed up with clinical evaluation and continence scoring. Results: All the patients withstood surgery well. One patient with rectovesical fistula required conversion to gain adequate length of the distal rectum, for whom the colostomy was closed and relocated at splenic flexure level. The complications were mucosal prolapse (8 cases), anal stenosis (5), adhesive obstruction (2), distal rectal necrosis (3), and urethral diverticulum (2). The progress has been satisfactory and weight gain is adequate. 71.15% patients had good continence on follow-up. Conclusion: LAARP procedure is safe for high ARMs with good continence and correctable side effects. It has completely replaced posterior sagittal anorectoplasty procedure for high anomalies in our practice.

We are adding two cases of congenital pouch colon with rectal atresia (RA) to the already published five cases. The presentation of RA is often delayed owing to the presence of normal anus, however, clinical evaluation, plain abdominal radiograph, and urine examination for meconuria, may help in detecting this rare association early.

Type V is the rarest form of congenital pouch colon with only four cases reported till date. We report this anomaly in a 6-month-old boy. He was managed successfully with excision of distal pouch and coloplasty of proximal pouch along with abdominoperineal posterior sagittal anorectoplasty. We recommend preservation of proximal pouch in such cases.

We report four female children with externally visible but stenosed fecal orifice which proved to be a high anorectal malformation (Type IV pouch colon). They were managed by an abdominoperineal approach with excellent results. We emphasize the correct recognition of this pathology.