The split notochord syndrome: alpha and omega

Two cases of the split notochord syndrome are described. Each patient presented with different clinical features that can be attributed to similar embryological defects. One infant presented with a covered lumbosacral meningocele, caudal to which lay isolated, exposed small-bowel mucosa. This is the second such case described in the literature. The second child presented with respiratory difficulties and was found to have a long thoraco-abdominal bowel duplication. Each child had surgical treatment of the abnormalities and both remain well with no neurological deficits at 6 and 3 years, respectively. The abnormalities are explained as consequences of a division of the notochord resulting in abnormal endo-ectodermal adhesion. Surgery is advocated for excision of an unacceptable skin lesion, spinal dysraphism, and because of the risks of haemorrhage and neoplasm.