Complete caudal duplication syndrome – a rare entity

Introdution: Caudal duplication syndrome is a rare anomaly associated with complete/partial duplication of the spine, spinal cord, and of other caudal structures including urogenital and gastrointestinal tract with some degrees of neurological dysfunction.

Case Report: we present the case of the nine-month patient with prenatal diagnosis by ultrasonography of fetal malformation. Cesarean delivery at 39 weeks without intercurrences. After childbirth and during the investigation with ultrasonography, tomography and magnetic resonance imaging was diagnosed the following malformations: Intramedullary cyst at L4 level, cone lipoma and spinal anchorage; doubling of the sacrum; Intestinal duplicity – operated on the fifth day of life due to acute abdomen for enterovesical; fistula was performed Laparotomy with colon-terminus anastomosis and micro bladder raffia; ventricular septal defect, interventricular communication, atrial septal defect, persistent ductus arteriosus – clinical follow up by Pediatric cardiology; Bladder duplication; Four Kidneys; Two penis; two fully formed penis and both functional; two anus being the imperforate left; A paramedian ossified appendage suggestive of a malformed leg; Intramedullary cyst at L4 level, cone lipoma and spinal anchorage. Growth, development, and neurological examination are normal for age. In the eighth month of life was electively operated by the pediatric neurosurgery team, was performed laminectomy L4 + cone lipoma resection + medullary release + intramedullary cyst resection. Intraoperative and postoperative without intercurrences, high with ambulatory follow-up in multidisciplinary follow-up programming and surgery for correction of the osseous and genitalia of the urinary tract.

Discussion: Spinal and spinal cord duplicity span a spectrum of malformations ranging from the simple fibrous bands splitting the cord to complete the duplication of caudal structures. Only when there is associated duplicity of vascular structures and/or organs such as bladder and distal gastrointestinal tract is the term caudal duplication syndrome coined. The embryologic basis of this syndrome is unclear. Some authors believe it results from incomplete division of monozygotic. Most patients have associate moderate to severe neurodeficit.

Finals coments: We did not identify on literature previous reports of complete caudal duplication syndrome with all anatomical structures with malformations.