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  • Title: Extensive aplasia cutis congenita associated with cephalocranial disproportion and brain extrusion.
    Author: Torkamand F, Ayati A, Habibi Z, Nejat F.
    Journal: Childs Nerv Syst; 2019 Sep; 35(9):1629-1632. PubMed ID: 31139904.
    Abstract:
    Aplasia cutis congenita (ACC) is a rare congenital disorder which can be linked with a variety of other abnormalities. However, the association of this anomaly with cephalocranial disproportion and brain extrusion is rarely reported. In this report, we present a neonate with an extensive ACC with exposed dura mater and sagittal sinus, who later presented with brain extrusion from the defect and an acrocephalic-like feature required decompressive surgery during the first month of life. Theories regarding etiology and progression of acrocephalic feature and brain protrusion in this case have been discussed.
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