RESUMO
Split notochord syndrome has been described in several previous case reports; however, we recently treated two patients with a previous undescribed variant of this syndrome. A 2,800 g male baby was admitted to the neonatal patients room with a non covered mass on the lumbo-sacral region, and a portion of intestine, with meconium being passed attached to it the legs were equine-varus and the anus was absent. Radiographs were consistent with a double spine defect (lumbar and sacral split notochord), and the patient was taken to the operating room for closure. A 2,600 g male baby was first seen in other hospital, and was admitted with a circular defect in the mid-line of the lumbosacral region. This defect was conformed by knee, leg, ankle and fingers. Radiographs showed bony structures consistent with incomplete lower extremity. During surgery, the skin overlying the mass was excised, showing a wide spine defect including stomach, duodenum, jejunum, ileum and colon, covered by a peritoneal membrane; there was not communication between this structure and the normal digestive tract.
