Optic nerve Hemangioblastoma with bilateral frontal lobe Oedema: a case report
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CASE REPORT
Open Access
Optic nerve Hemangioblastoma with bilateral frontal lobe Oedema: a case report Shujia Xu1, Qian Li1, Bingyang Bian1, Hongli Zhou2 and Dan Li1*
Abstract Background: Hemangioblastomas are rare benign tumours that are most commonly detected in the subtentorium or spinal cord. Optic nerve hemangioblastoma is very rare and is most commonly associated with Von HippelLindau (VHL) syndrome. Case presentation: Here, we report a case of hemangioblastoma of the optic nerve with bilateral frontal lobe oedema without VHL syndrome, which has not yet been reported. A 51-year-old woman presented with progressive and painless deteriorating vision in the left eye. Magnetic resonance imaging showed a mass at the back of the left orbital optic nerve. Endoscopic-assisted intraorbital tumour resection was performed successfully. The pathological diagnosis was left optic nerve hemangioblastoma. Conclusions: This is the first reported case of optic nerve hemangioblastoma (HBL) with bilateral frontal lobe oedema. Keywords: Optic nerve, Hemangioblastoma, VHL syndrome
Background Hemangioblastomas (HBLs) are benign tumours of the central nervous system that are often associated with Von Hippel-Lindau (VHL) syndrome. The main ophthalmological manifestation is retinal HBL, and it is very rare for HBLs to occur in the optic nerve. At present, only 35 cases of optic nerve HBLs have been reported in the literature. In this report, we describe a case of HBL of the optic nerve with bilateral frontal lobe oedema, a previously unreported manifestation of optic nerve HBL. Case presentation A 51-year-old female patient presented with progressive and painless deteriorating vision in the left eye for 1 month and was treated in April of 2018. Her past medical history included 10 years of hypertension and diabetes and 6 years of heart disease. Her family medical history was also recorded. After fundus examination, her * Correspondence: [email protected] 1 The First Hospital of Jilin University, Changchun, China Full list of author information is available at the end of the article
symptoms were diagnosed as ischaemic optic neuropathy in the left eye. After vasodilation treatment, visual acuity decreased further, accompanied by left exophthalmos. The patient returned for treatment on June 8, 2018. The ophthalmological examination results were as follows: best corrected visual acuity was 20/20 in the right eye; finger counting/50 cm in the left eye; and exophthalmos was 13 mm in the right eye and 16 mm in the left eye. The orbital distance was 97 mm. The cornea was transparent in both eyes. The pupils were 3 mm, and were of the same size and roundness in both eyes with light reflex. The crystalline lens and vitreous body were nebulous. The fundus examination results were as follows: no abnormality in the right eye; the boundary of the optic disc in the left eye was not clear (instead, it was pale); and the cup/disc ratio was 0.9. The visual field (VF) results revealed no abnormality in the right VF, while only the inferior nasal
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