Romanian Neurosurgery (Nov 2024)
A RARE CASE OF CROUZON SYNDROME ASSOCIATED WITH INTRACRANIAL MENINGIOMA WITH NASAL EXTENSION
Abstract
Introduction Crouzon syndrome is a genetic disorder caused by mutations in the FGFR2 gene, leading to premature fusion of cranial sutures (craniosynostosis) and resulting in distinct craniofacial abnormalities such as a beaked nose, shallow orbits causing proptosis, and midface hypoplasia. These patients often experience complications such as vision and hearing loss, and breathing difficulties due to nasal obstruction. Besides craniofacial anomalies, Crouzon syndrome can be associated with intracranial anomalies like meningiomas, making their management complex and multidisciplinary. Material and Methods We present the case of a 33-year-old female patient with a history of Crouzon syndrome, giant olfactory groove meningioma treated with gamma-knife in 2008 and 2009, chronic infantile encephalopathy, and behavioral disorder. The patient was admitted with complaints of headache, conductive hearing loss in the left ear, and a vegetative tumor formation in the left nasal fossa. Cerebral MRI and CT scans revealed multiple intracranial and nasal formations Results The patient underwent surgical resection of the nasal tumor. Histopathological result was atypic meningioma. Post-operative evolution of the patient was favorable, with no post-operative complications following total ablation of the nasal extension. Conclusions This case highlights the complexity of managing patients with multiple neurocranial, especially in the context of genetic disorders like Crouzon syndrome. While the precise incidence of meningiomas in patients with Crouzon syndrome isn't well-documented, it appears to be a rare complication. Due to its complexity, Crouzon syndrome may be associated with other complex pathologies like aneurysms and intracranial tumors, like it was presented in this case. Therefore, a multidisciplinary collaboration is crucial for optimal treatment and long-term follow-up.
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