Unilateral Atresia of Cervix Uteri and Uterus Didelphys in a Girl with Operated Congenital Pouch Colon

Abstract

This case report describes a girl with uterus didelphys with unilateral hematometra and hematosalpinx with congenital pouch
colon (CPC) condition, where all or part of the colon is replaced by a pouch-like dilatation (5-15 cm in diameter), which
communicates distally with the urogenital tract through a large fistula and associated with anorectal agenesis (supralevator
anorectal malformation). CPC is seen in Asia and more specifically in northern India, Pakistan and Nepal. The management
summary of this condition is creation of diverting colostomy at birth with or without the excision of pouch followed by pull
through (abdominoposterior sagittal anorectoplasty) later on. The girl underwent similar procedure at birth and subsequent
definitive surgery (pull through), to create a neoanus at 15 months of age. Subsequently since menarche, the child had severe
cyclical abdominal pain, which on investigation was found due to hematometra and hematosalpinx in right-sided uterus.
Laparotomy with removal of right-sided uterus and right fallopian tube was performed. Right-sided uterus had atresia of cervix
uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated
gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.