NASAL CEREBRAL HETEROTOPIA (NASAL GLIOMA) IN THE ADULT - A RARE CAUSEOF PRIMARY CSF RHINORRHEA - CASE-REPORT AND LITERATURE-REVIEW

Background: Nasal cerebral heterotopia is a congenital lesion that is mainly detected in early childhood. The rare cases of this disorder fo und in adult patients are located intranasally. A common symptom is co mpromised nasal air passage. Clinical findings include polypoid masses in either the nasal cavity or the paranasal sinuses. To our knowledge , primary cerebrospinal fluid (CSF) rhinorrhea has been reported only twice in these patients. Patient: A 64-year-old female patient present ed with CSF rhinorrhea proven by beta 2-transferrin testing. Previous head injury or intranasal manipulation were excluded. Anterior rhinosc opy revealed a watery drainage from the right middle meatus. CT scan s howed a defect in the lateral roof of the right ethmoid sinus, approxi mately 5 mm in diameter; MRI revealed a mass in the right ethmoid and frontal sinuses, penetrating the anterior skull base. The lesion was r esected by an extranasal approach. It showed a fibrous connection to t he frontal lobe. Histologically, the lesion consisted of neural tissue composed of gray and white matter, both with a normal structuring. Du ra and skull base were reconstructed. There were no signs of a CSF lea k postoperatively. Results and conclusions: The differential diagnosis of CSF rhinorrhea includes traumatic events and neoplasms, elevated i ntracranial pressure, and connate lesions as encephaloceles and, in ra re cases, nasal cerebral heterotopia.