Optic Disc Edema in Syphilis

Abstract

Background:Syphilis is an uncommon cause of optic nerve head edema; however, differentiating syphilis from other etiologies of optic nerve head swelling may be challenging. We describe 4 cases of ocular syphilis presenting with swollen optic nerve head(s) without overt signs of intraocular inflammation to better define the phenotypic presentation of this condition to allow its early recognition and treatment and discuss potential pathophysiological mechanisms of syphilitic optic neuropathy.Methods:Retrospective case series of patients presenting to a tertiary neuro-ophthalmology practice with a swollen optic nerve head(s) but no overt signs of intraocular inflammation, which was eventually determined to be secondary to syphilis.Results:Four patients were included in the study. The mean age was 43 years, 2 were women and 2 had bilateral involvement. Two patients had a recent history of skin rash, and one patient was investigated for abdominal pain and elevated liver enzymes. Two patients presented with photopsias and preserved visual function, whereas 2 presented with vision loss. Although chorioretinitis was present in all cases, it was very subtle in all and was only appreciated on fundus autofluorescence (FA) in 3 of 4 cases. Three patients demonstrated evidence of optic perineuritis on neuro-imaging. All patients were treated with a course of intravenous penicillin with a variable degree of visual recovery.Conclusions:Systemic symptoms are common in patients with syphilic optic neuropathy. Optic disc edema as a manifestation of syphilis is usually accompanied by subtle chorioretinitis, which is best appreciated on FA. Optic perineuritis is common in patients with syphilitic optic neuropathy, with its pathophysiology likely similar to meningitis seen in neurosyphilis.

The case of a 26‐year‐old male with subacute progressive visual loss

“… Bilateral optic disc edema with profoundly elevated and blurred margins of both eyes could result from increased intracranial pressure, which is called “papilledema,” but also should raise suspicion for other possible causes such as infectious/inflammatory, demyelinating, toxic‐metabolic/nutritional, and hypertensive emergency in the appropriate cases. Systemic condition and past medical history could help to narrow down the possible etiologies of blurred vision with bilateral optic disc edema. Syphilitic optic neuropathy is a rare manifestation of late syphilis. The hallmark of this condition is optic disc edema without association of hemorrhage or exudate in patients who do not have evidence of increased intracranial pressure 2–4 The proper diagnosis and prompt treatment of syphilitic optic neuropathy leads to better vision outcomes. …”

“…The hallmark of this condition is optic disc edema without association of hemorrhage or exudate in patients who do not have evidence of increased intracranial pressure. 2 , 3 , 4 The proper diagnosis and prompt treatment of syphilitic optic neuropathy leads to better vision outcomes. …”

“…The hallmark of this condition is optic disc edema without association of hemorrhage or exudate in patients who do not have evidence of increased intracranial pressure. 2 , 3 , 4 …”