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Pediatrics Congenital Anomalies of the CNS f39f1191

Which of the following is not true about encephalocoele?

A
It is a neural tube defect
B
Common in the frontal region
C
Can be associated with hydrocephalus
D
It is protrusion of neural tissue through a defect
High-Yield Explanation
Ans. is 'b' i.e., Common in the frontal region o Encephalocele-There is protrusion of brain tissue (cerebral cortex, cerebellum or brainstem) through a cranial defect (cranium bifidum). Most common site is occipital region. Hydrocephalus can occur due to aqueductal stenosis, chiari malformation or Dandy-Walker syndrome.Encephalocoeleo Encephalocoeles, also known as meningoencephalocele, are a type of cephalocoelewhere brain tissue herniates out through a defect in the cranium.Terminologyo They should be distinguished from cranial meningoceles in which the lesion contains only meninges and extracranial brain herniation, in which brain herniates through a dural and skull defect (e.g. post trauma or surgery).Epidemiologyo They are thought to occur in one of every 4,000 live births. Fronto-ethmoidal encephaloceles are more common in South and Southeast Asian populations. There is no recognised gender predilection.Clinical presentationo Fronto-ethmoidal encephaloceles manifest as a clinically visible mass along the nose. The intracranial root of most fronto-ethmoidal encephaloceles lies at theforamen caecum, a small ostium located at the bottom of a small depression anterior to the crista galli and formed by the closure of the frontal and ethmoid boneso Basal encephaloceles are internal and are not generally externally visible, although they may manifest as a lump or bump in the oropharynx or nasopharynx.Pathologyo It is thought to occur due to a failed closure of the rostral end of the neural tube in early developmentLocationo Many encephalocoeles are midline in position# Occipital encephalocoele: ~75% of cases# Other locations include:# Fronto-ethmoidal encephalocoele / nasal encephalocoele: ~15% of cases# Basal encephalocoele:-10%# Parietal encephalocoele

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