Childs Nerv Syst

. 2023 Dec;39(12):3341-3348.

 doi: 10.1007/s00381-023-06169-8. Epub 2023 Sep 30. https://pubmed.ncbi.nlm.nih.gov/37776334/

Syndrome of anterior neural stalk, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia related to persistent ventral neurenteric canal: report of two cases

Jason A Chen ^1 2, Joshua D Bernstock ^1 2, Walid Ibn Essayed ^1 2, Woo Do ³, Farokh R Demehri ³, Mark Proctor ¹, Benjamin C Warf ⁴

Affiliations expand

• PMID: 37776334
• DOI: 10.1007/s00381-023-06169-8

Abstract

Purpose: Abnormalities in notochordal development can cause a range of developmental malformations, including the split notochord syndrome and split cord malformations. We describe two cases that appear related to unusual notochordal malformations, in a female and a male infant diagnosed in the early postnatal and prenatal periods, which were treated at our institution. These cases were unusual from prior cases given a shared constellation of an anterior cervicothoracic meningocele with a prominent “neural stalk,” which coursed ventrally from the spinal cord into the thorax in proximity to a foregut duplication cyst.

Methods: Two patients with this unusual spinal cord anomaly were assessed clinically, and with neuroimaging and genetics studies.

Results: We describe common anatomical features (anterior neural stalk arising from the spinal cord, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia) that support a common etiopathogenesis and distinguish these cases. In both cases, we opted for conservative neurosurgical management in regards to the spinal cord anomaly. We proposed a preliminary theory of the embryogenesis that explains these findings related to a persistence of the ventral portion of the neurenteric canal.

Conclusion: These cases may represent a form of spinal cord malformation due to a persistent neurenteric canal and affecting notochord development that has rarely been described. Over more than 1 year of follow-up while managed conservatively, there was no evidence of neurologic dysfunction, so far supporting a treatment strategy of observation.

Keywords: Neurenteric canal; Notochord; Spinal dysraphism; Split cord malformation; Tethered cord.

© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.