Castleman's Disease Presenting with Uveal Effusion Syndrome

Abstract

We report a rare case of multicentric Castleman's disease that presented with ophthalmic involvement, along with a review of the literature. A 63-year-old male presented with decreased visual acuity in both eyes. Both eyes had serous elevations of the retinas with shifting subretinal fluid and annular choroidal detachment. No retinal breaks were found. Laboratory tests revealed pancytopenia, hypergammaglobulinemia, and an increased erythrocyte sedimentation rate. Chest and abdominal computed tomographies showed multiple lymphadenopathies in the mediastinum, abdomen, and in both inguinal areas. Histological examination of the inguinal lymph node biopsy was consistent with Castleman's disease. After combination chemotherapy, the serous elevations of both retinas and the annular choroidal detachments of both eyes disappeared. Ophthalmic involvement in Castleman's disease is very rare, and to the authors' knowledge, this is the first report of ophthalmic involvement of Castlemans's disease in Korea.

Clinical Analysis of Castleman’s Disease of the Lacrimal Gland

“…The same treatment principle is applied to CD that has invaded the eyes. Song and Jeong [ 28 ] reported 1 patient with systemic symptoms and invasion of the retinal choroid. With the CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone) scheme, the fundus symptoms disappeared with a significant effect.…”

Bilateral Nanophthalmic Uveal Effusion Syndrome

“…4 It also seems to have a middle-aged male preponderance and commonly occurs bilaterally. 4,5,[12][13][14][15] The association with diabetes may be a coincidence, although the coexistence of diabetes may be significant. 3,5,[16][17] There are no previous reports of diabetes in association with UES.…”

“…6 Although multiple studies suggest computed tomogrpahy and magnetic resonance imaging for accurate preoperative classification and to further evaluate the presence of scleral thickening, the authors believe that ultrasound alone can provide an accurate diagnosis of UES. 15 Occasionally, despite surgery, the disease takes a relapsing and remitting course. 5 Results in this case, however, have been good.…”

Retinal vein occlusion during flare of multicentric Castleman's disease

“…Ophthalmic complications in MCD are very rare with most published cases describing intraorbital and optic nerve lesions 2,3. Intraocular involvement is rarer with one published adult case of uveitis,4 two cases of bilateral,5,6 one case of unilateral serous retinal detachment and choroidal effusion,7 one case of hypertensive choroidopathy,8 one case of choroidal infiltrates,7 and one case of optic nerve drusen and peripapillary choroidal neovascularization in a patient with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome and associated Castleman’s disease 9. One pediatric case describing venous engorgement and tortuosity, intraretinal hemorrhages, cotton wool spots, and neurosensory detachment of the posterior pole was thought to be due to hyperviscosity syndrome which responded to one session of plasmapheresis 10.…”