Sphenoethmoidal encephalocele: a case report with review of the literature.

A rare case of sphenoethmoidal encephalocele with detailed pathological studies is reported. Only a few cases have been documented and were reviewed. The etiology and embryology of the congenital malformation remains speculative with an agreement on their development at an early embryonic stage. The mass presenting in the nasopharyngeal cavity remains often undetected unless causing respiratory embarrassment and is frequently misdiagnosed. Thorough radiological investigations detect the bone defect and outline the extent of the herniated mass and associated cerebral abnormalities. Small lesions have been successfully repaired. Larger ones prove less amenable to surgery and carry a high mortality.