Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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February 13, 2020
Atretic Parietal Cephalocele
- Background:
- Cephaloceles are congenital herniations of brain tissue through a skull defect.
- Atretic parietal cephaloceles (APCs) are involuted cephaloceles consisting of dura, fibrous tissue, and dysplastic brain tissue. They are typically sporadic and have a more favorable prognosis than true or noninvoluted cephaloceles.
- Clinical Presentation:
- Scalp lesion that may enlarge with crying or Valsalva
- Neurologic development may be normal or may be delayed if the atretic cephalocele is syndromic and associated with other intracranial anomalies.
- Key Diagnostic Features:
- MRI is the best imaging modality to depict this entity and other intracranial malformations.
- An atretic cephalocele presents as a scalp lesion with an associated bone defect at the cranial vertex. Persistent vertical embryonic positioning of the falcine sinus is seen in most cases.
- Other typical signs: focal interruption of the superior sagittal sinus at the APC, prominence of the superior cerebellar cistern and suprapineal recess, superior peaking of the posterior tentorium and "spinning top" configuration of the tentorial incisura
- Differential Diagnoses:
- Sinus pericranii: Abnormal communication between the intracranial and extracranial venous system; enhance to the same degree as cerebral venous structures; may be associated with APC
- Dermoid cyst: Typically firmer than atretic cephalocele and covered by reddened skin; calvarial defects seen with dermoid cysts demonstrate a scalloped morphology; MRI demonstrates internal fat signal without enhancement after the administration of contrast
- Treatment:
- The prognosis of atretic cephalocele is generally good in the absence of other intracranial abnormalities.
- The treatment is surgical excision of the cyst and oversewing of the tract formed by the dura.
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