| Encephalocoele
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Etiology
•Failure of the anterior neural tube to close for
genetic, toxic or infectious reasons.
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Pathogenesis
•Infections, toxins and genes can be the cause of
failure of the anterior neural tube to close.,
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Epidemiology
•Anterior neural tube closure defects are seen in
1:1000 infants.
•Encephalocoeles in which part of the
brain is outside the skull in a skin covered sac are
less comman than anencephaly.
•Occipital encephalocoeles
are much more comman than parietal or frontal ones.
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General Gross Description
•In encephalocoeles a distorted portion of brain hangs
out of the skull in a skin covered sac.
•The sac may be large containing a large portion of
disorganized cerebral hemispheres or it may be small
containing only gliotic cerebellum and meninges.
•The
disorganized brain in the sac is usually attached to
the remainder of the brain and may contain cerebral
spinal fluid.
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General Microscopic Description
•The brain in the sac is disorganized with little
formation of cerebral cortex and white matter and
marked gliosis.
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Clinical Correlation
•If the encephalocoele is large and their is a large
volume of abnormal brain in the sac, then the patient
may be profoundly mentally retarded. If it is very
small, then the patient may be normal mentally. In
either case the sac must be excized so that the skin
does not erode and cause a meningitis.
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References
• Poirer J et.al. Manual of basic neuropathology. Philadelphia: Saunders, 1990, pp.197.
• Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1301.
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| Encephalocoele
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| Synopsis by: Dr ML Grunnet (TX2000M21660)[407]
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