Dexamethasone implant for immunogammopathy maculopathy associated with IgA multiple myeloma

Abstract

Background: We describe a case where hyperviscosity retinopathy and immunogammopathy maculopathy were the presenting features of IgA multiple myeloma and report the response of maculopathy to intravitreal injection of dexamethasone implants.

Case presentation: A 56-year-old man presented at the Department of Ophthalmology with the chief complain of reduced vision for the past 10 days in both eyes. Ophthalmic examination revealed central retinal vein occlusion resembling signs with severe macular edema in both eyes with prominent serous macular detachment. After comprehensive evaluation, an IgA type kappa multiple myeloma was diagnosed complicated with hyperviscosity-associated retinopathy and immunogammopathy maculopathy. Patient was treated with multiple sessions of plasmapheresis, systemic chemotherapy, and finally intravitreal implants of dexamethasone with complete restoration of macular edema and serous macular detachment in both eyes. The visual function and the hyperviscosity-associated retinopathy were partially restored.

Conclusion: Ocular manifestation might be the only presenting sign of a life-threatening disease such as IgA multiple myeloma. A high level of suspicion is required to diagnose and treat such cases promptly and effectively.

Keywords: IgA multiple myeloma; chemotherapy; dexamethasone implants; hyperviscosity syndrome; hyperviscosity syndrome–associated retinopathy; immunogammopathy maculopathy; plasmapheresis.

Figure 1.

(a, b) Dilated fundus examination at presentation revealed moderate dilatation and tortuosity of the central retinal vein branches, multiple flame-shaped and dot-blot hemorrhages at the posterior pole and throughout the four quadrants of the fundus and macular edema OU. In addition, scatter cotton-wool spots and Roth’s spots were observed in the mid-periphery. A few yellowish subretinal precipitates could also be observed at the posterior pole. (c, d) Eleven months after diagnosis, and 9 months after dexamethasone implant, a significant improvement in retinopathy can be observed OU with reduction in dilatation and tortuosity of the veins and reduction in the hemorrhages. Cotton-wool spots slightly subsided, while yellowish deposits could be observed in the macula OD (c).

Figure 2.

Optical coherence tomography at presentation demonstrated severe macular edema in the right (a) and in left (b) eyes with prominent serous macular detachment. Fluid accumulation and large cysts with moderately hyperreflective material were observed at the outer retina and mainly at the outer nuclear layer. Furthermore, an amount of hyperreflective deposits were observed in the inner and outer retina, subretinally, adherent to the retinal surface, as well as at the level of retinal pigment epithelium. Two weeks later, after five rounds of plasmapheresis, serous macular detachment persisted while reduction in the intraretinal fluid was noted in both eyes (c, d). Two months after diagnosis, while the patient was under chemotherapy, the serous macular detachment, while decreased, persisted in both eyes (e, f), whereas the intraretinal cysts subsided significantly. The intraretinal hyperreflective deposits remained. One month after dexamethasone administration, a small serous macular detachment remained in both eyes (g, h) without any signs of intraretinal fluid, while the hyperreflective deposits remained. Nine months after dexamethasone administration, there were no signs of intraretinal or subretinal fluid in the macula of both eyes (i, j). The hyperreflective intraretinal deposits persisted, while a disruption in the ellipsoid zone was observed in OD (i).

Figure 3.

(a, b) Fluorescein angiography revealed a slight delay in arterial and venous filling time, blocked fluorescence due to retinal hemorrhages, pinpoint areas of hyperfluorescence from microaneurysms, and vascular staining with mild leakage in the mid-periphery with areas of capillary non-perfusion nasal to the optic disk. No fluorescein leakage was observed at the macula in both eyes.