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Atretic encephalocele
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Atretic encephalocele

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Synopsis

A rare congenital malformation due to failure of fusion of the neural tube, resulting in intracranial structures, both meninges and brain, being unenclosed by or outside of the skull. In the case of atretic encephalocele, formation was interrupted and incompletely developed. Also called abortive encephalocele. Considered generally benign and may or may not be caused by incomplete fusion of the neural tube. May present with a very small, almost unnoticeable protrusion from the scalp of the neonate. Because it is covered by fibrocollagenous tissue, it is easily mistaken for a hamartoma. Of concern are the number and severity of associated birth disorders in the infant.

Management may involve surgery for repair of the encephalocele and/or associated defects. Prognosis may be good if only minor damages occur; however, there is a strong association with developmental delay and vision defects, despite surgery.

Codes

ICD10CM:
Q01.9 – Encephalocele, unspecified

SNOMEDCT:
55999004 – Encephalocele

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Last Updated: 02/26/2016
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Atretic encephalocele
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Atretic encephalocele
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