Surgical management of caudal duplication syndrome: A rare entity with a centered approach on quality of life

BACKGROUND: Caudal duplication syndrome (CDS) is associated with complete/partial duplication of the spine, spinal cord, duplication/malformations of other caudal structures, and often neurological dysfunction. CASE DESCRIPTION: A 9-month-old with the prenatal diagnosis of multiple fetal malformations was born through cesarean section at 39 weeks. Computed tomography and magnetic resonance imaging revealed a series of abnormalities: intramedullary cyst at the L4 level with tethering of the conus with a lipoma, a duplication of the sacrum, duplication of the intestines, a ventricular septal defect, bladder duplication, four kidneys, two fully formed functional penises, two anuses (imperforate on the left), and a paramedian ossified appendage suggestive of a malformed leg. The child, at an 8 months of age, underwent a laminectomy at the L4 level for resection of the conus lipoma, release of the tethered cord, and resection of the intramedullary cyst. CONCLUSION: Spine/spinal cord duplication syndromes are typically associated with spine/spinal cord abnormalities and are often associated with moderate/severe neurological deficits. Additional malformations associated with the CDS include duplication of vascular structures/organs (e.g., bladder and distal gastrointestinal tract). Here, we focused on both the patient’s quality of life and the multidisciplinary approach to managing the duplication syndrome associated with multiple system malformations (e.g., neurological/spinal, bladder, and gastrointestinal, others).