A posterior cloacal variant is a congenital malformation where a urogenital sinus
terminates anterior to a normally placed anus. These are rare malformations with highly
variable anatomy. We report on three cases of a novel phenotype of posterior cloaca
encountered at our institutions between October 2021 and November 2023. Three newborn
girls were referred with ambiguous external genitalia and an anorectal malformation.
In all cases, a midline sac, which are likely fused labioscrotal folds, replacing
the clitoris was noted anterior to the perineal orifices. Two of the three patients
demised as a result of renal failure. The third patient underwent reconstruction and
is well. This posterior cloacal phenotype appears to be frequently associated with
severe renal insufficiency. In survivors of the neonatal period, a good cosmetic outcome
is achievable. Functional outcomes remain to be assessed.
