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Unilateral Anophthalmia and Congenital Frontal Cranioschisis Associated with Extradural Neuroglial Heterotopia: new Insights into a Possible New Malformative Spectrum

Abstract Background: Neuroglial heterotopia, characterized by mature neuroglial tissue outside the central nervous system, has not been previously associated with cranioschisis. Case Report: A 4-year-old female patient, with left congenital… Click to show full abstract

Abstract Background: Neuroglial heterotopia, characterized by mature neuroglial tissue outside the central nervous system, has not been previously associated with cranioschisis. Case Report: A 4-year-old female patient, with left congenital anophthalmia, had a nasofrontal neuroglial heterotopia protruding through an ossification defect. Discussion: Nasofrontal cranioschisis may be associated with neuroglial heterotopias. The combination of anophthalmia and neuroglial heterotopia, previously described only once in the literature, may be part of a broader malformation spectrum that has not been properly characterized to date.

Keywords: unilateral anophthalmia; neuroglial heterotopia; heterotopia; anophthalmia congenital; cranioschisis

Journal Title: Fetal and Pediatric Pathology
Year Published: 2022

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