A Silent Congenital Ethmoidal Encephalocele Progressing into Frontoethmoidal Meningoencephalocele with Episodic Seizures in Adulthood: A Case Report and Literature Review

Xiang Yang, Xiaolin Ai, Seidu A. Richard, Jianguo Xu


Background: Frontoethmoidal meningoencephalocele (FEM) is rare congenital malformations that has specific geographic distribution. This condition is depicted with herniation of brain tissue as well as meninges via a defect in the cranium at the frontal, orbital, nasal as well as ethmoidal regions. Although several cases have been reported, none of them have a silent and progressive nature with episodic seizures. Case Presentation: We present a 20-year-old man with a swelling on the nasal bridge which was notice a few years after birth. The swelling initially progressed slowly without any symptoms from childhood through adolescents. Both CT scan and MRI revealed a defect in cranium at the frontal and ethmoidal regions with herniation of meninges and brain matter into the nasal cavity but no fistula. We successfully reduced the encephalocele as well as watertight closed the dura mater. The skull defect repaired, and left orbital defect also reconstructed via surgery. Conclusions: Aflatoxins, generated by one genus of a defective fungi, aspergillus could be responsible for the development of this malformation. We suggest that pregnant women in areas with high incidence of the malformation be screen thorough with ultrasound scan to detect the malformation earlier. We also suggest that further studies on the malformation be geared toward using 3D ultrasound to study the pathophysiology of this malformation.


Frontoethmoidal, Meningoencephalocele, Seizures, Aflatoxins, Hypertelorism

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DOI: https://doi.org/10.7575/aiac.abcmed.v.6n.3p.20


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