Frontoethmoidal encephaloceles are second only to occipital encephaloceles in terms of frequency, representing approximately 15% of all encephaloceles. They represent meninges or brain tissue herniating through cranial defect in the anterior cranial fossa and typically result in facial deformity. 1Although the classification of encephaloceles is very variable, generally fronto ethmoidal encephaloceles are further divided into three subtypes on the basis of location of defect and path through facial structures into : naso-ethmoidal (most common) , naso-frontal and naso-orbial (least common). 1,2,4
The incidence of fronto-ethmoidal encephaloceles is between 0.8 and 4 per 10,000 births and there is no reported sex predominance. They are, however, more common in south-east Asian then Western countries 3.
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 color or pigmentation, soft or firm, may have excessive hair/hypertrichosis) 2. In addition to mass effect encephaloceles may also present with CSF leakage (rhinorrhea) or meningitis.
Treatment and prognosis
Surgical treatment of infant that survive the initial meningitis and shock may improve the short term outcome but few series describe the longer term outcome for the children as well as the ability to live independent life
1. David DJ. Cephaloceles: classification, pathology, and management-a review. J Craniofac Surg. 1994;4 (4): 192-202. Pubmed citation
2. Dhirawani RB, Gupta R, Pathak S et-al. Frontoethmoidal encephalocele: Case report and review on management. Ann Maxillofac Surg. 2014;4 (2): 195-7. doi:10.4103/2231-0746.147140 - Free text at pubmed - Pubmed citation
3. Bhattacharjee A, Chakraborty A, Purkaystha P. Frontoethmoidal encephalomeningocoele with colpocephaly: case report and clinical review. J Laryngol Otol. 2008;122 (03): 321-3. doi:10.1017/S0022215107000308 - Pubmed citation
4. Textbooks of Operative Neurosurgery ( 2 Vol.). B.I. Publications. (2005) ISBN:817225217X.