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- 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
Chen, Jason A.; Bernstock, Joshua D.; Essayed, Walid Ibn; Do, Woo; Demehri, Farokh R.; Proctor, Mark; Warf, Benjamin C.
- 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.
- Subjects
DIAPHRAGMATIC hernia; CYSTS (Pathology); SPINAL cord; HUMAN abnormalities; GENETICS
- Publication
Child's Nervous System, 2023, Vol 39, Issue 12, p3341
- ISSN
0256-7040
- Publication type
Article
- DOI
10.1007/s00381-023-06169-8