Anorectal agenesis with rectovaginal fistula: A rare/regional variant

Abstract

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.

Comparison Between the Pouch‐Perineum Distance in Neonates With a Low‐Type Anorectal Malformation With and Without an Opened Fistula: Pitfall of Measuring the Pouch‐Perineum Distance on Sonography

“…Although a low-type ARM with fistula can be identified by physical examination, the diagnosis of the type of ARM by physical examination alone is sometimes difficult in neonates, such as in female neonates with a rectovaginal fistula and possible passage of fecal matter through the fistula, 21 as well as in neonates with a cloacal malformation and in those with a complex anomaly. 22 Sonography is useful not only to evaluate the pouchperineum distance but also to evaluate the relationship between the puborectalis muscle and the distal rectal pouch, which is reported to be useful to classify the type of ARM.…”

Congenital rectovaginal fistula with anorectal agenesis

“…ARMs are commonly associated with other congenital anomalies [1]. The classification of female ARM is very complex and still evolving because of the difficult anatomy of female ARM and presence of various rare and regional variants [3]. Moreover, there is no clear demarcation between high and low ARM in females [3].…”

“…The classification of female ARM is very complex and still evolving because of the difficult anatomy of female ARM and presence of various rare and regional variants [3]. Moreover, there is no clear demarcation between high and low ARM in females [3]. Rectovaginal fistula is included as one of the rare/regional variants in the Krickenbeck classification (2005) [1], [3], [4].…”

“…Moreover, there is no clear demarcation between high and low ARM in females [3]. Rectovaginal fistula is included as one of the rare/regional variants in the Krickenbeck classification (2005) [1], [3], [4]. Rectovaginal fistula, along with congenital pouch colon (CPC) and H-type fistula are more common in India and some Asian countries [5], [6], [7], [8].…”

“…The incidence of anorectal agenesis with rectovaginal fistula is less than 1% [3]. However, the reported incidence varies widely from 0% to 84% literature [9].…”