Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus?another cloacal variant

Abstract

The persistent cloaca is one of the most complex and challenging developmental malformations. It is a rare anomaly occurring only in the female newborn and is represented by the association of urogenital sinus with an anorectal malformation (ARM). Each case is probably unique. We report here one such case of cloaca with the VATER association-tracheoesophageal fistula (TOF) with a urogenital sinus, rectovaginal fistula, and an anteposed stenosed anus, along with preaxial syndactyly of the right hand. The spine, renal, and cardiac systems were normal. Interim management was directed towards repair of the TOF and a right transverse defunctioning colostomy. Despite thorough radioendoscopic preoperative investigations, the complexity of the cloacal anomaly was not delineated until surgery. This case is a rather rare combination of an intermediate form of the cloacal-ARM spectrum. Such patients present with many diagnostic and therapeutic problems. Interval surgery should be directed towards decompression of the affected organ systems, and definitive surgery must be carefully planned and, whenever possible, done in a single stage with simultaneous multisystem repair to correct all significant malformations related to the cloacal complex.

Posterior cloaca—further experience and guidelines for the treatment of an unusual anorectal malformation

“…In our literature review, we found authors referring to this defect as posterior cloaca [2,3,7-9]; but we also found terms such as partial urorectal septal defect and others [10][11][12][13][14] in which the anatomical features, seen in photographs and diagrams, were consistent with the diagnosis of a posterior cloaca.…”

Evaluation and management of rectovaginal fistula in anorectal malformation: an observational study

“…Previously, RVF was believed to be a common clinical variant, with some reports claiming its incidence to be 84% [6]. Later on, it was realized that the vestibular fistula and cloaca were misdiagnosed as RVF [7].…”

Congenital rectovaginal fistula with anorectal agenesis

“…The surgical management of these anomalies is confusing and not standardized [3]. Definitive interval surgical reconstruction should be carefully planned and done in a single stage whenever possible [10]. A well-planned and coordinated approach usually results in a satisfactory outcome [10].…”

“…However, the reported incidence varies widely from 0% to 84% literature [9]. These false high reported incidences have been attributed to the indiscriminate labelling of rectovestibular fistula and cloaca as rectovaginal fistula [10]. In fact, Pena states that an isolated rectovaginal fistula is almost non-existent and higher reported incidence is due to the misdiagnosed cases of rectovestibular fistula and persistent cloaca [10], [11].…”