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Case Report
Ophthalmic artery ischemia in hemophagocytic lymphohistiocytosis: A case report and review of current literature
1 MBBS, Junior Doctor, Whittington Hospital, London, United Kingdom
2 MBBS, Junior Doctor, University College Hospital, London, United Kingdom
3 MD, Consultant Ophthalmic Surgeon and Vitreoretinal Surgeon, Ophthalmology Department, Royal Free Hospital, London, United Kingdom
4 PhD, Consultant Ophthalmic Surgeon and Neuro-ophthalmologist, National Hospital for Neurology & Neurosurgery, Neuro-ophthalmology Department, Queen Square, London, United Kingdom
5 BM, MD, Associate Professor and Consultant Ophthalmologist and Vitreoretinal Specialist, Ophthalmology Department, Royal Free Hospital, London, United Kingdom
Address correspondence to:
Ayushi Gupta
Whittington Hospital, Magdala Avenue, London N19 5NF,
United Kingdom
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Article ID: 100040Z17AG2024
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How to cite this article
Gupta A, Shah N, ElHousseini Z, Bremner F, Asaria R. Ophthalmic artery ischemia in hemophagocytic lymphohistiocytosis: A case report and review of current literature. J Case Rep Images Opthalmol 2024;7(1):5– 13.ABSTRACT
Introduction: This case report describes the ocular manifestations of hemophagocytic lymphohistiocytosis in a 35-year-old male with suspected Adult-onset Still’s Disease. Documented ocular presentations of hemophagocytic lymphohistiocytosis are variable, and the pathophysiology remains unclear. A complete review of the current literature suggests that posterior segment findings, including retinal and vitreous hemorrhage, are most common.
Case Report: Here we describe the first published case of bilateral combined choroidal and retinal ischemia in a patient with ocular hemophagocytic lymphohistiocytosis. This is suggestive of complete ophthalmic artery occlusion driving proliferative retinopathy. Mechanistic hypotheses include anemia, thrombocytopenia, coagulopathy, and histiocytic infiltration.
Conclusion: In this case, pre-retinal hemorrhage, retinal hemorrhage, and vitreous hemorrhage were treated with panretinal photocoagulation, pars plana vitrectomy, and bevacizumab at different stages in each eye. Unusually for ocular hemophagocytic lymphohistiocytosis, the patient developed a unilateral tractional retinal detachment, later thought to be due to early administration bevacizumab prior to vitrectomy in the left eye. Final visual acuity in this patient remains poor, and worse in the left eye. We therefore suggest early vitrectomy to maintain adequate views of the fundus before bevacizumab or panretinal photocoagulation in this patient cohort.
Keywords: Choroidal ischemia, Ocular hemophagocytic lymphohistiocytosis, Ophthalmic artery occlusion, Retinal ischemia
SUPPORTING INFORMATION
Author Contributions
Ayushi Gupta - Substantial contributions to conception and design, Acquisition of data, Interpretation of data, Drafting the article, Final approval of the version to be published
Neal Shah - Interpretation of data, Final approval of the version to be published
Zine ElHousseini - Interpretation of data, Final approval of the version to be published
Fion Bremner - Interpretation of data, Final approval of the version to be published
Riaz Asaria - Interpretation of data, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2024 Ayushi Gupta et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
