Fertility and the Reproductive Tract in a Woman with Caudal Duplication Syndrome

Background: Caudal duplication syndrome is a rare entity characterized by complete duplication of the genitourinary system and hindgut structures. There have been 13 case reports of women with genitourinary duplication; only 3 had complete caudal duplication and only 1 had a successful term pregnancy. There are no case reports to date of patients having more than 1 successful pregnancy with this syndrome. Case: A 28-year-old multiparous woman with known caudal duplication syndrome presented with recurrent urinary tract infections and hydronephrosis. This patient was interested in having a unification of her two vaginas. An examination under anesthesia revealed two gluteal clefts, three labia minora, two vaginas widely separated by >4 cm, a bifid clitoris, two normal cervices, two urethras, a right perforate anus, and a left imperforate anus. Exploratory laparotomy revealed uterine didelphys, 2 bladders, 2 ureters, and 2 kidneys. Results: Excision of the left bladder and ureteral reimplantation resulted in complete resolution of her hydronephrosis. Vaginoplasty was not recommended in this case, because the separation between the vaginas was >4 cm. Conclusions: Caudal duplication syndrome is an extremely rare condition most often recognized in utero, at birth, or during infancy, with complete duplication of the genitourinary and hindgut structures. Many of these patients have the ability to achieve successful gestation, but have an elevated risk of pregnancy and/or urologic complications. Collaboration with a gastroenterologist, urologist, and gynecologist is critical for optimizing care.