J Korean Ophthalmol Soc.  2023 Nov;64(11):1109-1114. 10.3341/jkos.2023.64.11.1109.

A Case of Orbital Infarction Syndrome in a Patient with Hemophagocytic Lymphohistiocytosis

• Choi JM
• Kwon MS
• Kang YK
• Kim DW
• Son BJ

• KMID: 2547876
• DOI: http://doi.org/10.3341/jkos.2023.64.11.1109

Abstract

Purpose
To report a case of orbital infarction syndrome in a patient with hemophagocytic lymphohistiocytosis (HLH).
Case summary
A 70-year-old woman with diabetes mellitus and hypertension was referred to the Department of Ophthalmology for sudden-onset left upper eyelid ptosis after being diagnosed with HLH. After 3 days, the best corrected visual acuity in the right eye was 0.8, while there was no light perception in the left eye. In the left eye, the ocular motility examination showed limitations in all fields of gaze. On fundus examination, optic disc pallor, retinal hemorrhage, and narrowed retinal arteries were observed in the left eye. Fluorescein angiography showed no blood flow in the retinal arteries and veins in the left eye. On neck computed tomography angiography, the left distal internal carotid artery was narrowed. Orbit computed tomography showed exophthalmos and extraocular muscle hypertrophy in the left eye. Orbit magnetic resonance imaging confirmed optic nerve edema, enhancement of the optic nerve sheath, and high signal intensity of the intraocular fat in the left eye. Slit-lamp examination revealed ischemia of the anterior segment and ischemic necrosis of the eyelid in the left eye. The patient was diagnosed with orbital infarction syndrome.
Conclusions
Several factors may lead to ischemia of the orbital tissues in patients with HLH, including coagulopathy, an increased incidence of blood clots, and inflammation in the orbit. It is necessary to consider the possibility of orbital infarction syndrome when HLH patients present with visual loss, ocular movement limitations, and anterior segment ischemia.

Keyword

Anterior segment ischemia, Hemophagocytic lymphohistiocytosis, Ophthalmoplegia, Optic perineuritis, Orbital infarction syndrome