Child's Nervous System

, Volume 35, Issue 9, pp 1629–1632 | Cite as

Extensive aplasia cutis congenita associated with cephalocranial disproportion and brain extrusion

  • Farbod Torkamand
  • Aryan Ayati
  • Zohreh HabibiEmail author
  • Farideh Nejat
Case Report


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.


Aplasia cutis congenita Cephalocranial disproportion Intracranial hypertension Sagittal sinus thrombosis 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Statement of informed consent

Inform consent has obtained from the parents.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019
corrected publication 2019

Authors and Affiliations

  1. 1.Department of Neurosurgery, Children’s Medical CenterTehran University of Medical SciencesTehranIran

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