Intractable Rare Dis Res. 2016;5(2):121-123. (DOI: 10.5582/irdr.2016.01010)

A case of split notochord syndrome: Presenting with respiratory failure in the neonatal period.

Coskun Y, Akman I, Demir MK, Yapicier O, Somuncu S


Split notochord syndrome (SNS) is a very rare congenital anomaly. This report describes a male newborn with a neuroenteric cyst in the posterior mediastinum and multiple vertebrae anomalies presenting with respiratory failure and pulmonary hypertension. This report also discusses the embryological development and the etiologic theories of SNS.

KEYWORDS: Split notochord syndrome, newborn, respiratory failure

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