Extensive aplasia cutis congenita associated with cephalocranial disproportion and brain extrusion - PubMed (original) (raw)

Case Reports

. 2019 Sep;35(9):1629-1632.

doi: 10.1007/s00381-019-04188-y. Epub 2019 May 28.

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Case Reports

Extensive aplasia cutis congenita associated with cephalocranial disproportion and brain extrusion

Farbod Torkamand et al. Childs Nerv Syst. 2019 Sep.

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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.

Keywords: Aplasia cutis congenita; Cephalocranial disproportion; Intracranial hypertension; Sagittal sinus thrombosis.

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References

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