Optic nerve Hemangioblastoma with bilateral frontal lobe Oed
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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 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 VF remained in the left eye. A visual evoked potential (VEP) test showed no obvious abnormality in either eye at peak time. The amplitude of the p2 wave in the left eye was lower than that in the right eye.

Magnetic resonance imaging (MRI) showed a mass at the back of the left orbital optic nerve, with isointensity on T1 and hyperintensity on T2/fluid-attenuated inversion recovery (FLAIR) imaging. Heterogeneous enhancement was found on a contrast-enhanced scan. There was no obvious flow void in or around the tumour. The proximal end of the optic nerve on the left side of the orbit was partially enlarged and tortuous, and the optic nerve sheath was dilated. There was no obvious abnormality in the right optic nerve. FLAIR imaging showed hyperintensity of the chiasma, left optic tract, and bilateral frontal lobes.

Endoscopic-assisted intraorbital tumour resection was performed while the patient was under general anaesthesia, during which the medial orbital wall and the anterior part of the optic canal were incised from the nasal cavity. The optic nerve was cut off at the orbital apex, the surrounding tissues of the tumour were separated, and the tumour was completely removed by approaching from the nasal bulbar conjunctiva incision. Pathological examination revealed that the tumor was mainly composed of abundant interstitial cells and capillaries. The cytoplasm of the interstitial cells was rich and lightly stained, showing vacuoles. Immunohistochemical staining showed positivity for CD34, CD31, and CD56 and negativity for progesterone receptor, creatine kinase (AE1/AE3), glial fibrillary acidic protein, S-100, epithelial membrane antigen, and D2–20. The pathological diagnosis was HBL of the left optic nerve.

Three months after the operation, MRI showed that the tumour had been removed, and the signs of bilateral frontal lobe and optic choroid oedema had disappeared.

Source: https://bmcophthalmol.biomedcentral.com/articles/10.1186/s12886-020-01706-4
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