S-Space College of Medicine/School of Medicine (의과대학/대학원) Dept. of Medicine (의학과) The Seoul Journal of Medicine The Seoul Journal of Medicine Vol. 23 No.3 (1982)
Encephalocele(Two Autopsy Cases Report)
- 서정욱; 손진희; 지제근; 이규은; 김현철; 강영호
- Issue Date
- 서울대학교 의과대학
- Seoul J Med, Vol.23 No.3, pp. 385-391
- Two autopsy cases of encephaloceles were reported.
The first case was a female baby died in one day
and had a huge occipital mass containing posteri"r
two thirds of cerebrum. midbrain, pons and medulla
oblongata. An unexplained polypoid mass of cerebral tissue was seen at parasagittal area of left parietal
lobe. Tentorium cere belli was absent. Subarachnoid
vessels were congested. The encephalocele was covered
by dura and skin. Occipital bone had a huge defect.
The subcutaneous tissue over the encephalocele had
multiple nests of neuroglial cells without connection
to the brain proper. Cut sections of the brain showed
enlargement of left lateral ventricle. Focal hemorrhage
and calcification were present at occipital lobe.
The second case was a female baby died soon after
birth. She had a cystic, polypoid mass at right
forehead. Right eye was replaced downward and
nares were separated apart. Median cleft lip was also
present. Right half of frontal bone was completely
defective, a small portion of through which frontal
lobe was herniated. Cystic cavity of the frontal
mass was a continuation of right lateral ventricle.
Falx cerebri was hypoplastic, thalami fused to each
other and Arnold-Chiari type I anomaly was
associated. Cut sections showed bilateral hydrocephalus
especially at right lateral ventricle. The
encephalocele was covered by skin and dura. Skeletal
muscle was partly incooperated and was splitted.
Anterior lobe of pituitary gland showed abnormal
capsulation with fibre-collagenous capsule and septation.
Classical theories explaining the pathogenesis of
encephaloceles were proposed by Geoffrey-SaintHillaire(
1827) and Spring(l853). They were incomplete
separation of ectodermal germ layers and
increased intracranial pressure. But disputes on the
pathogenesis are still present. Major disputes are
1. Whether the brain tissue in encephalocele is
herniated tissue or heterotopic tissue?
2. Is there any evidence explaining the classical
theory of Geoffrey-Saint- Hillaire?
3. Is the increased intracranial pressure a primary
defect or secondary phenomenon?
4. Local ischemia in skull and adjacent soft tissue
makes the defect to give harniating pathway, is it
We reviewed the literature and tried to explain
them based on the findings of our cases.