AUTHOR=Zhuang Xiaonan , Gao Fengjuan , Sun Zhongcui , Ding Xinyi , Xu Gezhi 

TITLE=Case report: Sympathetic ophthalmia after vitrectomies in a patient with Von Hippel–Lindau syndrome

JOURNAL=Frontiers in Medicine

VOLUME=Volume 10 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1118913

DOI=10.3389/fmed.2023.1118913

ISSN=2296-858X

ABSTRACT=Background: Sympathetic ophthalmia (SO) is a rare but sight-threatening uveitis and most observations have been made after typical manifestations occur. This report focuses on choroidal changes detected by multimodal imaging at the presymptomatic stage of SO, which is implicated in early recognition of SO.
Case Presentation: A 21-year-old woman suffered decreased vision in the right eye and was diagnosed with retinal capillary hemangioblastomas associated with von Hippel-Lindau syndrome. The patient underwent two 23-G pars plana vitrectomies (PPVs), soon after which typical signs of SO manifested. SO resolved quickly after oral administration of prednisone and remained stable during follow-up of more than 1 year. The retrospective analysis revealed preexisting bilaterally increased choroidal thickness, ischemic spots on the choroid and choriocapillaris en-face slabs in optical coherence tomography angiography (OCTA) after the first PPV, which were all reversed by corticosteroids treatment.
Conclusions: The case report highlights the involvement of the choroid and choriocapillaris at the presymptomatic stage of SO after the first inciting event. Abnormally thickened choroid accompanied by choroid and choriocapillaris ischemia suggested that inflammatory responses have started and an ensuing surgery would run the risk of exacerbating SO. OCTA scanning of both eyes should be ordered routinely for patients with a history of trauma or intraocular surgeries, especially before the next surgical intervention. The report also suggests that non-human leukocyte antigen gene variation may also regulate the progression of SO, which requires further laboratory investigations.