First Author: M.Haruta JAPAN
Co Author(s): C. Taguchi R. Yamakawa
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Primary vitreoretinal lymphoma is an uncommon neoplastic disorder characterized by infiltration of the vitreous and retina by lymphoma cells. It is considered to be a subset of primary central nervous system lymphoma, which is most often diffuse large B-cell lymphoma. The purpose of the current study was to report a case of primary vitreoretinal lymphoma in which a macular hole developed after the 1st vitrectomy. Although intravitreal injections of Methotrexate may have a negative effect on macular hole closure, the 2nd vitrectomy with internal limiting membrane peeling and sulfur hexafluoride gas tamponade successfully closed the macular hole.
Maculopathy has been reported to occur in patients with primary central nervous system lymphoma as a complication of blood-brain barrier disruption therapy. However, we found no previous report on the formation of macular hole in patients with primary vitreoretinal lymphoma.
Medical records of a patient with primary vitreoretinal lymphoma who developed a macular hole after the 1st vitrectomy were reviewed. Measurement of IL-6 and IL-10 and cytologic analysis of the vitreous were performed using undiluted vitreous specimen. Phacoemulsification and intraocular lens implantation was done in the left eye prior to the 1st diagnostic vitrectomy to avoid cataract progression. Triamcinolone acetonide was injected into the vitreous cavity during vitrectomies for the visualization of the vitreous. The internal limiting membrane was stained with Brilliant Blue G, and peeled off using vitreous forceps. The patient was instructed to maintain a prone position for ten days after the 2nd vitrectomy.
A 65-year-old female presented with worsening vision in the left eye. Her best-corrected visual acuities were 1.5 OD and 0.2 OS. Fundus examination of the left eye showed 3+ vitreous haze and multifocal, yellow-white infiltrates in the retina and beneath the retinal pigment epithelium. She underwent a diagnostic 25 gauge vitrectomy followed by intravitreal Methotrexate injections in the left eye. Undiluted vitreous specimen showed an increased IL-10 level (1470 pg/mL) with an elevated IL-10 to IL-6 ratio of 15.1, and the cytologic analysis of the vitreous showed atypical lymphoid cells with large irregular nuclei and scanty cytoplasm. The results of contrast-enhanced brain MRI showed no signs of a malignant neoplasm. The retinal infiltrates in the left eye responded well to the intravitreal Methotrexate injections but a macular hole developed 5 days after the 1st vitrectomy. Following 2 additional intravitreal Methotrexate injections, the 2nd pars plana vitrectomy with internal limiting membrane peeling and sulfur hexafluoride gas tamponade successfully closed the macular hole. Twelve weeks after the 1st vitrectomy of the left eye, ophthalmic examination showed no evidence of active retinal infiltrates with her best-corrected visual acuities of 1.5 OD and 0.2 OS.
Clinical features of primary vitreoretinal lymphoma include homogenous vitreous cells and multifocal, yellow-white infiltrates in the retina or beneath the retinal pigment epithelium. However, diagnosis of primary vitreoretinal lymphoma is often difficult because primary vitreoretinal lymphoma can masquerade as uveitis. Diagnostic vitrectomy was useful in the current case to clarify the presence of primary vitreoretinal lymphoma and to differentiate primary vitreoretinal lymphoma from uveitis. Macular hole may develop after vitrectomy performed as a treatment for primary vitreoretinal lymphoma. Although the exact pathogenesis of macular hole formation in the current case remains unclear, mechanical stress of vitrectomy, retinal infiltrates including the macula, and tangential retinal traction mediated by acute scarring of retinal infiltrates by intravitreal Methotrexate injection may all have contributed to the formation of macular hole in the current case. Intravitreal injections of Methotrexate may inhibit the proliferation of glial cells which is considered to be necessary for macular hole closure after vitrectomy. However, the 2nd vitrectomy with internal limiting membrane peeling and sulfur hexafluoride gas tamponade could successfully close the macular hole in the current case.