Encephalocoele

Radswiki et al.

Encephalocoele, also known as meningoencephalocele, is a form of neural tube defect and a type of cephalocoele where brain tissue and overlying meninges herniate out through a defect in the cranium. 

It should be distinguished from cranial meningocele in which the lesion contains only meninges, and from extracranial brain herniation in which brain herniates through a dural and skull defect (e.g. post trauma or surgery). 

Numerous classification systems exist for encephalocoeles, many which are only of subspecialty interest, and most use very similar terminology, sometimes with different meanings 7. As a result reading about this topic can be frustrating. The term nasal encephalocoele is, for example, variably used, often to encompass both frontoethmoidal and basal encephalocoeles 8

A fairly common classification still widely used is that first described by Suwanwela and Suwanwela in 1972 which divides the encephaloceles according to location 4:

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.

Fronto-ethmoidal encephaloceles manifest as a clinically visible mass along the nose. The intracranial root of most fronto-ethmoidal encephaloceles lies at the foramen 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 bones.

Basal encephaloceles are internal and are not generally externally visible, although they may manifest as a lump or bump in the oropharynx or nasopharynx.

Encephaloceles, when congenital (most common) are a form of neural tube defect and occur due to a failure of fusion during embryogenesis of the cartilaginous neurocranium, the membranous neurocranium or viscerocranium 6

In adults, encephalocoeles are mainly due to traumatic or iatrogenic causes, but are also increasingly recognised in idiopathic intracranial hypertension.

Genetics

Most cases tend to be sporadic.

Associations

Occipital encephaloceles may be associated with a number of additional abnormalities: 

Antenatal ultrasound

An encephalocoele may be seen as a purely cystic mass or may contain echoes from herniated brain tissue. If the mass appears cystic, the meningocoele component predominates, while a solid mass indicates predominantly an encephalocele. Larger encephalocoeles may show accompanying microcephaly.

CT/MRI

MR imaging is the best imaging modality for defining the contents of an encephalocele prior to surgery. High-resolution CT may also be used to display the bone anatomy, but the intracranial connection is best defined with MR imaging. The extent of cerebral tissue in an encephalocele is also better defined with MR imaging, which aids in prognosis and surgical planning. 

The prognosis is variable dependent on the presence of associated anomalies and presence of microcephaly (carries a much poorer prognosis). A Caesarian delivery may be considered to allow for less traumatic birth for the fetal head.

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Article Information

rID: 12522
Section: Pathology
Synonyms or Alternate Spellings:
  • Encephalocele
  • Meningoencephalocele
  • Encephalocoeles
  • Encephaloceles

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    Case 1: frontal
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    Coronal images de...
    Case 2: fronto-ethmoidal-nasal encephalocoele
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    Case 3: fronto-ethmoidal encephalocoele
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    Case 4: occipital
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    Case 5: fronto-ethmoid encephalocele
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    Case 6: medial sphenoid
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    Case 7
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    Case 8
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    Case 9: lateral intrasphenoidal encephalocele
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    Case 10: with frontal mucocoele
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