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Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review. Materials and Methods: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score. Results: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had “good” continence score compared to four children (three having anal canal) in Group 2. Conclusion: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.

Context: To our knowledge, there is no description of anterior sagittal anorectoplasty (ASARP) with external anal sphincter preservation and passing neorectum in the middle of muscle complex under direct vision for the treatment of recto-vestibular fistula (VF). Aim: This study evaluates a new modification combining ASARP with under vision sphincter preservation. Subjects and Methods: This prospective study was conducted on thirty female infants with VF. Procedure starts with a vertical midline incision extending from ectopic opening to posterior limit of external sphincter. Sharp dissection of the fistula is carried out. Artery forceps is passed at the center of muscle complex under vision, then neorectum is placed in the middle of the muscle complex. We introduced a new scoring system based on parental interview assessing functional outcome. Each patient was given a score between 0 and 20; good: 14–20, fair: 7–13, and poor: 0–6. Results: ASARP was performed at a mean age of 2.6 months, a mean weight of 5.2 kg, a mean operative time of 102 min, and a mean hospital stay of 3.6 days. Wound infection occurred in four cases, seven cases had perianal excoriations, six cases had anal stenosis, and only one case complained of anal displacement. Patients followed for a mean of 18.8 months. Majority of our patients (50%) had good score (mean = 16.8), normal frequency, no or mild soiling, normal anal position with no or mild stenosis. Fourteen patients had fair score (mean = 10.5). Only one had poor outcome with severe soiling and perineal excoriation. Conclusion: Our modification offers optimal correction, with minimal sphincteric damage, without additional complexity or difficulties. Scoring system is simple, practical, and truly reflects early functional and parent satisfaction after surgery.

The two main surgical procedures for high and intermediate anorectal malformations (ARM), namely, Stephens′ and Peρa′s procedure, are compared in terms of their outcome. Materials and Methods: Fifty-eight patients who had Stephens′ procedure and 28 patients who had posterior sagittal anorectoplasty (PSARP) are clinically analyzed in terms of associated anomalies, procedural complications, anatomical reconstruction and functional results. Functional results were assessed by Kelly score, voluntary bowel movements and sensation. Statistical analysis of data was done by Chi-square test. Results: There were 34 high and 52 intermediate ARM. Associated anomalies were noted in 32%. Procedure-related complications of urethral injury, bladder injury, neurogenic bladder, anal stenosis and mucosal prolapse were seen in both procedures. Ectopic positioning of anus was seen in 25% of PSARP and 19% of sacroperineal mobilization (SPM). Good circular sphincter creation was seen in 43% of PSARP and 40% of Stephens′. Noncontractile sphincter was found more with SPM. In functional results, when assessed by Kelly score, VBM and sensation, there was no difference for high ARM, whereas results were better with SPM for intermediate anomalies. Discussion: A few reports are available in literature comparing PSARP and SPM. Procedural complications of urethral injury and neurogenic bladder are slightly more with PSARP. Ectopic positioning, poor contraction of sphincter are associated with poor results, and creation of good circular sphincter with good squeeze is associated with good results. Functional assessment by Kelly score, VBM and sensation doesn′t reveal any difference between two procedures for high ARM, whereas for intermediate anomalies, Stephens′ procedure seems to give better functional results.