A Rare Presentation of Caudal Duplication Syndrome in an Adult with No Functional Impairment

Abstract

Abstract:Caudal duplication syndrome is a rare congenital association between malformations and duplications of the gastrointestinal and genitourinary systems and neural tube defects. Less than 100 cases have been reported in literature worldwide, majority being diagnosed at birth. To date only 2 patients have been reported to have presented in adulthood. We hereby report a case of caudal duplication with complete duplication of urethra, bladder, vagina, uterus, entire hindgut and midgut but with no functional abnormalities. Her only complaint being narrow double vaginal vaults with anticipated difficulty in sexual function. As normal pregnancy has been reported in uterus didelphys, a simple vaginal septal resection and posterior vaginal wall lengthening with incorporation of a 'Z-plasty' was done along with vulvaplasty to exclude the abnormal anal opening from the fourchette.

Caudal duplication syndrome: a rare entity

“…5 A classic case of CDS exhibits malformations of the spinal column due to damaged notochord in addition to the duplication of structures which originate from primitive intestine. 1 The complete duplication of colon, bladder, urethra, vagina, cervix and uterus in CDS (as in our case) is rather unusual. 5 The duplication of colon can be cystic or tubular which is mostly seen on mesenteric side with common vascular supply.…”

“…The rare caudal duplication syndrome (CDS) is an association between malformations and duplication of gastrointestinal, genitourinary systems and neural tube defects. 1 Reported prevalence at birth is less than 1 per 100,000 but the condition seems to be more rare. 2 Female to male ratio is approximately 2:1 with no familial or racial predisposition and no other concordant risk factors.…”