Abstract
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.
Highlights
Aflatoxins, generated by one genus of a defective fungi, aspergillus could be responsible for the development of this malformation
Frontoethmoidal meningoencephalocele (FEM) is a congenital malformation that encompass the herniation of meninges and cerebral tissue via a defect in the anterior cranium, mostly between the frontal as well as ethmoidal bones[1,2,3]
Thu and Kya proposed that aflatoxins, generated by one genus of a defective fungi, aspergillus could be responsible for the development of this malformation[1, 4]
Summary
Frontoethmoidal meningoencephalocele (FEM) is a congenital malformation that encompass the herniation of meninges and cerebral tissue via a defect in the anterior cranium, mostly between the frontal as well as ethmoidal bones[1,2,3]. Congenital abnormalities such as hypertelorism, middle nasal fissure, wide nasal root, as well as cleft lip or palate is associated in about a third of patients Optic malformations such as anophthalmia/microphthalmia, colobomas, retinal idiosyncrasies, morning glory syndrome, as well as optic nerve or chiasm hypoplasia have been associated with most FEM. "He first presented at a local hospital in Yunnan Province and was referred to West China Hospital for further assessment and management."The obstetric history of this man was unevent-ful and childhood was uneventful His parents did not seek any health intervention initially because he was very well until two years ago when he started having episod-ic seizures. Scheduled out-patient reviews were arranged on every sixth month after operation up to two years
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