Encephaloceles

What is encephaloceles?

Encephaloceles are sac-like protrusions of part of the brain and meninges through openings in the skull. These rare birth defects occur when the neural tube, in which the brain and spinal cord form, fails to close completely during fetal development. Skin or, less often, a thin membrane, covers the sac outside the skull.

Encephaloceles can occur in the base of the skull, the top or back of the skull, or between the forehead and nose. Conditions associated with encephaloceles include hydrocephalus (excess accumulation of cerebrospinal fluid in the brain), developmental delays, microcephaly (an abnormally small head), paralysis and seizures.

Pre-op image of encephalocele

Pre-op image of encephalocele

Post-op image of encephalocele

Symptoms of encephaloceles:

When encephaloceles occurs, it may cause any or all of the following symptoms:

• headache
• nasal drainage
• meningitis
• visual disturbances
• tinnitus

Diagnosis of encephaloceles:

Diagnosing encephaloceles includes an analysis of the nasal fluid for a protein called beta-2 transferrin which is most only found in cerebrospinal fluid. CT and MRI scans may also be require to determine the location and severity of the leakage.

To make an appointment or request a consultation, contact the Johns Hopkins Skull Base Tumor Center at 410-614-0585.