Multimodal imaging and clinical evolution of an optic nerve head granuloma with prominent overlying vessels in ocular sarcoidosis: a case report and literature review
- 1Department of Ophthalmology, The Second Hospital of Jilin University
Abstract
Background: Sarcoidosis is a multisystem granulomatous disorder of unclear etiology, histopathologically characterized by noncaseating granulomas. Ocular involvement is among the most common extrapulmonary manifestations, whereas optic nerve involvement occurs in fewer than 5% of cases. Direct granulomatous infiltration of the optic nerve represents the rarest form of optic nerve involvement. We report a rare case of a sarcoidosis-associated optic nerve head granuloma with prominent overlying vessels and describe its multimodal imaging features, clinical course, and treatment response, with a review of the clinical spectrum of sarcoid optic nerve head granulomas reported in the English-language literature.
Case presentation: A 31-year-old woman presented with floaters in the left eye. Best-corrected visual acuity was 20/20 in both eyes. Fundus examination of the left eye revealed a white elevated optic nerve head lesion with fine tortuous surface vessels, accompanied by vitreous inflammatory haze, retinal periphlebitis, and candle-wax drippings. Fundus fluorescein angiography demonstrated early filling of lesion-associated vessels with marked late leakage, and optical coherence tomography angiography detected abnormal flow signals corresponding to the lesion. Additionally, chest computed tomography revealed bilateral hilar lymphadenopathy, and transbronchial lung biopsy demonstrated chronic granulomatous inflammation with multinucleated giant cells and no definite necrosis. The overall findings supported the diagnosis of sarcoidosis. The lesion initially progressed after the patient declined corticosteroid therapy, but gradually regressed following systemic corticosteroid treatment, with parallel disappearance of the lesion-associated abnormal flow signals. At the final follow-up, the lesion had completely regressed, leaving only a thin gliotic membrane, while visual acuity remained 20/20 throughout the entire follow-up.
Conclusion: Although rare, sarcoid optic nerve head granuloma is a highly suggestive ocular manifestation that may be the presenting sign of systemic sarcoidosis and can mimic infectious or neoplastic optic disc mass lesions. Multimodal imaging is valuable for early recognition, assessment of treatment response, and longitudinal monitoring. Once the diagnosis is established, corticosteroid-based systemic therapy may be considered. In this case, systemic corticosteroid therapy was associated with anatomic regression of the optic nerve head granuloma, while visual acuity remained stable throughout follow-up.
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BibTeX
@article{xiong2026multimodal,
title = {{Multimodal imaging and clinical evolution of an optic nerve head granuloma with prominent overlying vessels in ocular sarcoidosis: a case report and literature review}},
author = {Jiahao Xiong and Jiyue Tan and Jun Xiao},
journal = {Frontiers in Neurology},
year = {2026},
month = oct,
doi = {10.3389/fneur.2026.1978059},
url = {https://www.frontiersin.org/articles/10.3389/fneur.2026.1978059/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Multimodal imaging and clinical evolution of an optic nerve head granuloma with prominent overlying vessels in ocular sarcoidosis: a case report and literature review
AU - Jiahao Xiong
AU - Jiyue Tan
AU - Jun Xiao
PY - 2026
DA - 2026/10/15
JO - Frontiers in Neurology
DO - 10.3389/fneur.2026.1978059
UR - https://www.frontiersin.org/articles/10.3389/fneur.2026.1978059/full
AB - Background: Sarcoidosis is a multisystem granulomatous disorder of unclear etiology, histopathologically characterized by noncaseating granulomas. Ocular involvement is among the most common extrapulmonary manifestations, whereas optic nerve involvement occurs in fewer than 5% of cases. Direct granulomatous infiltration of the optic nerve represents the rarest form of optic nerve involvement. We report a rare case of a sarcoidosis-associated optic nerve head granuloma with prominent overlying vessels and describe its multimodal imaging features, clinical course, and treatment response, with a review of the clinical spectrum of sarcoid optic nerve head granulomas reported in the English-language literature. Case presentation: A 31-year-old woman presented with floaters in the left eye. Best-corrected visual acuity was 20/20 in both eyes. Fundus examination of the left eye revealed a white elevated optic nerve head lesion with fine tortuous surface vessels, accompanied by vitreous inflammatory haze, retinal periphlebitis, and candle-wax drippings. Fundus fluorescein angiography demonstrated early filling of lesion-associated vessels with marked late leakage, and optical coherence tomography angiography detected abnormal flow signals corresponding to the lesion. Additionally, chest computed tomography revealed bilateral hilar lymphadenopathy, and transbronchial lung biopsy demonstrated chronic granulomatous inflammation with multinucleated giant cells and no definite necrosis. The overall findings supported the diagnosis of sarcoidosis. The lesion initially progressed after the patient declined corticosteroid therapy, but gradually regressed following systemic corticosteroid treatment, with parallel disappearance of the lesion-associated abnormal flow signals. At the final follow-up, the lesion had completely regressed, leaving only a thin gliotic membrane, while visual acuity remained 20/20 throughout the entire follow-up. Conclusion: Although rare, sarcoid optic nerve head granuloma is a highly suggestive ocular manifestation that may be the presenting sign of systemic sarcoidosis and can mimic infectious or neoplastic optic disc mass lesions. Multimodal imaging is valuable for early recognition, assessment of treatment response, and longitudinal monitoring. Once the diagnosis is established, corticosteroid-based systemic therapy may be considered. In this case, systemic corticosteroid therapy was associated with anatomic regression of the optic nerve head granuloma, while visual acuity remained stable throughout follow-up.
SN - 1664-2295
ER -