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Hydranencephaly
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Hydranencephaly

Portion of brainstem, cerebellum, and thalamus with thin pial-glial membrane which represents what remains of the cerebral hemispheres.
Due to intrauterine infection or ischemia.
(Description By:Margaret Grunnet, M.D. )
(Image Contrib. by:Margaret Grunnet, M.D. UCHC )
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.
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.
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.
Hydranencephaly
Synopsis by: M. L. Grunnet M.D. (TX2000M2100H)[58]
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