Concurrent juxtapapillary hemorrage and optic disc edema as initial presentation of POEMS syndrome

Abstract

POEMS syndrome is a rare complex multisystem disorder of which typical features are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS). The pathogenesis of this syndrome is not well understood. In recent years, it has been recognized as a paraneoplastic syndrome caused by an underlying plasma cell neoplasm. 1,2 Because its symptoms are complex and can mimic those of other disorders, it is difficult to diagnose. 3-6 POEMS syndrome has a rapidly progressive course, so making an early, accurate diagnosis can contribute to higher possibilities of survival. Optic disk edema (ODE) is an early and common ocular manifestation of POEMS syndrome, but being asymptomatic, it is likely to be misdiagnosed. Here, a case of POEMS syndrome that presented as ODE and juxtapapillary subretinal hemorrhage is described, and the potential diagnostic problems will be discussed to raise awareness of this disease to ophthalmologists. A 45-year-old female had experienced numbness and weakness in the lower limbs for 3 months, coinciding with weight loss (15 kg in 3 months), and blurred vision with metamorphopsia predominantly in her left eye for 1 week. The patient's blood pressure was 134/90 mm Hg, and body

Retinal Manifestations in Patients with Myeloproliferative Disorders

“…Makula ödemi, seröz makula dekolmanı, venöz sinüs trombozu, diskte neovaskülarizasyon, peripapiller neovaskülarizasyon da görülen bulgular arasındadır. [19,20,21] PMF geç evrede ise kemik iliği yetmezliğine bağlı olarak çıkan anemi, trombositopeni ve lökopeni görülmektedir. Bu aşamadaki bulgular daha çok anemiye bağlı ortaya çıkar ve iskemik retinopati, neovaskülarizasyon, retinal hemorajiler, eksudalar ve atılmış pamuk lekeleri görülür.…”

Recent Advances in the Treatment and Supportive Care of POEMS Syndrome

“…About 30% of the patients may present with papilledema, and it may be one of the signs of increased intracranial pressure. Juxtapupillary hemorrhage and optic disc edema may also be seen [ 126 ]. Despite patients being asymptomatic for most of the times, a formal ophthalmologic exam is recommended [ 127 ], and treatment is necessary when intraocular pressures are very high.…”