| Hydranencephaly
|
Etiology
•There are many causes of
hydrancephaly including
vascular disease, infection, and trauma.
|
Pathogenesis
•Has been seen in women who
had blunt trauma to the
abdomen during pregnancy, to women exposed to C0 at
high levels during pregnancy, and after a severe viral infection. ,
|
Epidemiology
•An acquired malformation so it is sporadic.
|
General Gross Description
•Hydranencephaly is massive necrosis of the cerebral
hemispheres in utero.
•The basal ganglia and thalamus may still be present but
the cerebral cortex and white matter is represented by a
pial-meningeal membrane
•The brainstem and cerebellum may also be partially
intact.
•Examples:
|
General Microscopic Description
•Cerebral hemispheres are made up of a membrane containing
part of the marginal layer of the cerebral cortex
attached to a fibrotic leptomeninges.
•The basal ganglia and thalamus, if present, are gliotic.
•Examples:
|
Clinical Correlation
•Infants
rarely live past birth.
|
References
• Poirer J et.al. Manual of basic neuropathology. Philadelphia: Saunders, 1990, pp. 198.
•2.Friede. Developmental Neuropathology. New York: Springer-Verlag
1989. pp 31-39.
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| Hydranencephaly
| | Synopsis by: M. L. Grunnet M.D. (TX2000M2100H)[58]
| |