Syndrome of anterior neural stalk, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia related to persistent ventral neurenteric canal: report of two cases.
Childs Nerv Syst
; 39(12): 3341-3348, 2023 Dec.
Article
em En
| MEDLINE
| ID: mdl-37776334
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.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Cistos
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Hérnias Diafragmáticas Congênitas
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Malformações do Sistema Nervoso
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Defeitos do Tubo Neural
Limite:
Female
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Humans
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Infant
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Male
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article