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Review
. 1989;2(3):166-73.

Encephalocraniocutaneous lipomatosis. Report of two cases and a review of the literature

J S Bamforth  1 , V M Riccardi, P Thisen, D Chitayat, J M Friedman, J Caruthers, J G Hall
Affiliations
  • PMID: 2699567
Review

Encephalocraniocutaneous lipomatosis. Report of two cases and a review of the literature

J S Bamforth et al. Neurofibromatosis. 1989.

Abstract

We describe 2 patients with unilateral facial and cerebral malformations consistent with encephalocraniocutaneous lipomatosis. The characteristic facial appearance seems to develop with age, possibly as a consequence of increased intracranial pressure. However, the clinical data suggest that this condition arises as a result of a disruption of cephalic neural crest and anterior neural tube.

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