Korean J Ophthalmol.  2010 Jun;24(3):182-185. 10.3341/kjo.2010.24.3.182.

Castleman's Disease Presenting with Uveal Effusion Syndrome

• Park SH
• Song SJ

• KMID: 945992
• DOI: http://doi.org/10.3341/kjo.2010.24.3.182

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.

Keyword

Choroidal detachment; Giant lymph node hyperplasia; Uveal effusion

MeSH Terms

Aged
Choroid Diseases/etiology
Drug Therapy, Combination
Exudates and Transudates/*metabolism
Fluorescein Angiography
Fundus Oculi
Giant Lymph Node Hyperplasia/*complications/drug therapy
Humans
Hypergammaglobulinemia/complications
Magnetic Resonance Imaging
Male
Syndrome
Tomography, Optical Coherence
Treatment Outcome
Uveal Diseases/diagnosis/*etiology/*metabolism

Figure

• 

  Fig. 1 A fundus photograph of the left eye. Peripheral annular choroidal detachment, shallow detachment of the retina and multiple retinal hemorrhages and exudates due to the underlying diabetic retinopathy are shown.

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  Fig. 2 Fluorescein angiography (FAG) of both eyes revealed numerous fluorescent blotches in the subretinal space. FAG demonstrated hypofluorescence in the early phase and hyperfluorescence in the late phase resulting from leakage of fluorescein dye into the subretinal space, in addition to multiple microaneurysms resulting from diabetic retinopathy. (A) Early phase (right eye). (B) Late phase (right eye). (C) Early phase (left eye). (D) Late phase (left eye).

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  Fig. 3 Optical coherence tomography images through the foveas of both eyes revealed severe cystic changes in the foveal area and a high elevation of the neurosensory retina and the retinal pigment epithelium. (A,B) Retinal map of the right eye. (C,D) Retinal map of the left eye. (E) Retinal thickness map of both eyes. OD=oculus dexter; OS=oculus sinister.

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  Fig. 4 Magnetic resonance imaging. The eyeball size and scleral thickness are normal in both eyes. (A) T1-weighted image, choroidal detachment shown in the left eye. (B) T2-weighted image.

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