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Published in: Child's Nervous System 12/2023

30-09-2023 | Diastematomyelia | Case-based Review

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

Authors: Jason A. Chen, Joshua D. Bernstock, Walid Ibn Essayed, Woo Do, Farokh R. Demehri, Mark Proctor, Benjamin C. Warf

Published in: Child's Nervous System | Issue 12/2023

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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.
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Metadata
Title
Syndrome of anterior neural stalk, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia related to persistent ventral neurenteric canal: report of two cases
Authors
Jason A. Chen
Joshua D. Bernstock
Walid Ibn Essayed
Woo Do
Farokh R. Demehri
Mark Proctor
Benjamin C. Warf
Publication date
30-09-2023
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 12/2023
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-023-06169-8

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