ABSTRACT

Split notochord syndrome (SNS) is an extremely rare spinal anomaly, associated with anomalies of the vertebrae, central nervous system, and gastrointestinal tract. So far less than 30 neonatal cases with SNS have been published in the literature. Duplications of the gastrointestinal tract are rarely seen and rectal duplications (RDs) account for 5% of alimentary tract duplications. This case of SNS was associated with RD, posterior stenotic anus, meningomyelocele, Arnold Chiari malformation, hemiver-tebrae, short neck, and pes equinovarus. Postmortem investigations have also revealed uterus didelphus and bilateral hypoplastic kidneys. This case was presented to highlight the fact that there are rare associations with SNS, together with a literature review.