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Caudal duplication syndrome is a very rare anomaly and poses a major challenge for surgeons. The term caudal duplication syndrome is used to describe the association between hindgut duplication, duplication of the genitourinary, and malformations of the spinal cord and vertebrae. A 5-year-old girl is reported with complete duplication of the entire large bowel, cecum, appendix, about 2 cm of the terminal ileum, bladder, urethra, vagina, uterus, and vertebral body deformities. After an extensive evaluation phase, a laparotomy was performed and the duplicated colon was excised completely without removing the adjacent native colon.