ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Atretic encephalocele
Print
Other Resources UpToDate PubMed

Atretic encephalocele

Print Images (1)
Contributors: Bo Hoon Lee MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Atretic encephaloceles are rare malformations resulting in herniation of meninges and the brain through an opening in the skull. They are thought to be in the spectrum of defects of neural tube closure. The etiology is unknown.

Atretic encephaloceles occur most commonly in the parietal, occipital, or frontal region and may present with an otherwise benign-appearing subcutaneous nodule or mass. Alopecic lesions over the defect can occur as well. They have been associated with intracranial anomalies including interhemispheric cysts, abnormalities of the corpus callosum, ventriculomegaly, cystic malformations, and venous anomalies.

Related topic: Encephalocele

Codes

ICD10CM:
Q01.9 – Encephalocele, unspecified

SNOMEDCT:
55999004 – Encephalocele

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Hamartoma
  • Cephalohematoma
  • Dermoid cyst – typically firmer and present at midline
  • Sinus pericranii – classically decreases in size when patient stands up

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 10/15/2019
Last Updated: 11/15/2019
Copyright © 2020 VisualDx®. All rights reserved.
Atretic encephalocele
Print 1 Images
Atretic encephalocele : Scalp edema
Copyright © 2020 VisualDx®. All rights reserved.