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

Yesim Coskun; Ipek Akman; Mustafa Kemal Demir; Ozlem Yapicier; Salih Somuncu

doi:10.5582/irdr.2016.01010

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

Intractable Rare Dis Res. 2016 May;5(2):121-3. doi: 10.5582/irdr.2016.01010.

Authors

Yesim Coskun¹, Ipek Akman¹, Mustafa Kemal Demir², Ozlem Yapicier³, Salih Somuncu⁴

Affiliations

¹ Department of Pediatrics, Goztepe Medical Park Hospital, School of Medicine, Bahcesehir University, Istanbul, Turkey.
² Department of Radiology, Goztepe Medical Park Hospital, School of Medicine, Bahcesehir University, Istanbul, Turkey.
³ Department of Pathology, Goztepe Medical Park Hospital, School of Medicine, Bahcesehir University, Istanbul, Turkey.
⁴ Department of Pediatric Surgery, Goztepe Medical Park Hospital, School of Medicine, Bahcesehir University, Istanbul, Turkey.

Abstract

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.

Publication types

Case Reports