Peripheral retinal leakage in POEMS syndrome

Area of Science:

• Ophthalmology
• Hematology
• Vascular Biology

Background:

• POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) syndrome is a rare, multi-systemic blood disorder.
• Elevated plasma cells, platelets, and vascular endothelial growth factor (VEGF) characterize POEMS syndrome.
• Optic disc edema occurs in 52% of patients, potentially threatening vision, with uncertain etiology.

Purpose of the Study:

• To report a rare case of peripheral retinal leakage in a patient with POEMS syndrome.
• To investigate the potential link between VEGF levels and retinal vascular abnormalities in POEMS syndrome.

Main Methods:

• Case presentation of a 60-year-old female with POEMS syndrome and blurred vision.
• Ophthalmic examination revealed bilateral grade 3 disc edema.
• Wide-field fluorescein angiography demonstrated multifocal peripheral retinal vascular leakage.

Main Results:

• The patient presented with bilateral blurred vision and significant optic disc edema.
• Fluorescein angiography revealed extensive peripheral retinal vascular leakage, a previously unreported finding in POEMS syndrome.
• Cerebrospinal fluid analysis and neuroimaging were unremarkable.

Conclusions:

• Increased vascular permeability at the optic disc, secondary to elevated VEGF, is hypothesized to cause disc edema in POEMS syndrome.
• Peripheral retinal leakage represents a novel finding in POEMS syndrome, expanding the spectrum of its ocular manifestations.
• This case underscores the importance of comprehensive retinal imaging in POEMS syndrome patients with visual disturbances.