Frontoethmoidal encephalocoele

Frontoethmoidal encephalocoeles are second only to occipital encephalocoeles in terms of frequency, representing approximately 15% of all encephalocoeles.  They represent meninges or brain tissue herniating through cranial defect in the anterior cranial fossa and typically result in facial deformity. 

Although the classification of encephalocoeles is very variable, generally frontoethmoidal encephalocoeles are further divided into three subtypes on the basis of location of defect and path through facial structures into 1,3,5

  • nasoethmoidal (most common)
  • nasofrontal
  • naso-orbial (least common)

The incidence of fronto-ethmoidal encephalocoeles is between 0.8 and 4 per 10,000 births and there is no reported sex predominance. They are, however, particularly common in South East asian countries 4

Presentation depends on location and size of the defect and protrusion. If large the obvious facial deformity will be present with a mass of variable appearance (sessile or pedunculated; normal skin colour or pigmentation, soft or firm, may have excessive hair – hypertrichosis) 3

In addition to mass effect encephalocoeles may also present with CSF leakage (rhinorrhea) or meningitis.

The multilayer reconstruction surgery is a method which exhibits excellent results with high success rate and is advocated as a treatment for such conditions.

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Article information

rID: 48453
Section: Pathology
Synonyms or Alternate Spellings:
  • Frontoethmoidal meningoencephalocele

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Cases and figures

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    Case 1: nasoethmoidal
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    Case 2: nasofrontal
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    Coronal images de...
    Case 3: naso-orbital
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