OCULAR MANIFESTATIONS OF MONOCLONAL IMMUNOGLOBULIN LIGHT CHAIN DEPOSITION DISEASE.

PURPOSE: To demonstrate unusual retinal findings in a patient with progressive renal failure due to idiopathic monoclonal immunoglobulin light chain deposition disease, using multimodal imaging. METHODS: Observational case report of a 43-year-old white man with renal failure due to light chain deposition disease. His course over 6 years was documented with multimodal imaging including fundus photography, fundus autofluorescence, fluorescein angiography, and spectral domain optical coherence tomography. Additional evaluations included ocular ultrasound, electroretinography, positron emission tomography, serum protein electrophoreses, skeletal surveys to detect osteolytic lesions, and renal, liver, and rectal biopsies in search of amyloid. RESULTS: The patient's ocular course mirrored the severity of his renal dysfunction for which he required a renal transplant. Changes observed in the native kidney recurred in the transplant 2 years later, as evidenced by immunohistochemistry, revealing thick linear deposits of kappa chains, with no complement, overlying the glomerular basement membrane. The systemic workup was negative for amyloid but showed an overwhelming ratio of kappa to lambda light chains on serum protein electrophoreses and no clinical signs of plasma cell dyscrasias, all consistent with idiopathic light chain deposition disease. The patient presented with a generalized, bilateral "leopard-spot" fundus appearance on fundus autofluorescence, striking globular subretinal deposits on spectral domain optical coherence tomography, and subfoveal subretinal fluid without retinal pigment epithelium detachment or choroidal effusions. The subfoveal fluid did not respond to intravitreal injections of antiangiogenic agents or steroids but resolved after renal transplantation. A temporary posttransplant visual improvement was associated with lessening of the subretinal drusenoid deposits demonstrated by multimodal imaging. The terminal vision deterioration was associated with amorphous, vitelliform-like material deposition and atrophic changes. CONCLUSION: This case may illustrate a resemblance in the renal glomerulus basement membrane and retinal pigment epithelium-Bruch membrane complex, because the authors observed deposits of excess monoclonal kappa chains manifesting as extracellular, proteinaceous aggregates on the basement membrane of the glomerulus, and striking, globular subretinal deposits that overlay a thickened retinal pigment epithelium-Bruch membrane complex. The ocular lesions' refractoriness to intravitreal treatments could be attributed to the fact that they represent proteinaceous aggregates similar to those documented in the glomeruli. This is the first report of generalized, large, subretinal drusenoid deposits and their course, as documented through multimodal imaging, paralleling the chronology of systemic changes in a patient with light chain deposition disease.

Idiopathic giant retinal cyst.

The authors describe a case report of idiopathic asymptomatic giant retinal cyst with underlying hyperpigmentation. Low- and high-frequency ultrasound (ultrasound biomicroscopy) revealed an anechoic giant cyst with two hyper-reflective traction bands between the ciliary body and the anterior base of the retinal cyst. There was no retinal detachment.

An outbreak of fungal endophthalmitis after intravitreal injection of compounded combined bevacizumab and triamcinolone.

IMPORTANCE: Our experience may be useful to other practitioners using compounded intravitreal agents, those suspecting infectious outbreaks, and those managing fungal endophthalmitis. OBJECTIVE: To describe a series of patients with fungal endophthalmitis following intravitreal injection of combined bevacizumab and triamcinolone acetonide prepared by the same compounding pharmacy. DESIGN AND SETTING: Noncomparative case series. PARTICIPANTS: Eight eyes of 8 patients who received an intravitreal injection of compounded combined bevacizumab-triamcinolone in a period of 3 weeks had subtle, nonspecific findings that were later diagnosed as fungal endophthalmitis. MAIN OUTCOME MEASURES: Visual acuity, response to antimicrobial therapy, and number of vitreoretinal surgical operations after diagnosis of fungal endophthalmitis. RESULTS: Eight patients developed endophthalmitis 41 to 97 days after receiving the intravitreal injection, which was prepared by the same compounding pharmacy. The injections occurred at the same location in New York. Treatment was based on clinical examination findings and knowledge of the etiology of the endophthalmitis. Eventually, all patients were treated with oral voriconazole. Five of 8 patients were initially treated with intravitreal antimicrobial agents. After 3 months of follow-up, visual acuities ranged from 20/50 to hand motions. Local, state, and federal health department officials were involved in investigating the source of the outbreak. CONCLUSIONS AND RELEVANCE: In the current study, we report a fungal endophthalmitis outbreak after intravitreal injection of contaminated, compounded combined bevacizumab-triamcinolone. In this series, Bipolaris hawaiiensis was the identified causative agent. The challenge of medical diagnosis, identification of the source of the outbreak, and management experience are highlighted in our series. Our experience may be useful to other practitioners using compounded intravitreal agents, those suspecting infectious outbreaks, and those managing fungal endophthalmitis.

Unusual presentation of metastatic carcinoma to the eye.

PURPOSE: To describe four patients with an unusual presentation of metastatic carcinoma to the eye, diagnosed by histopathologic and immunohistochemical evaluation of vitrectomy material. METHODS: Retrospective case series of four patients with metastatic carcinoma to the retina/vitreous. The New York Eye and Ear Infirmary database was searched to identify patients with metastatic carcinoma to the retina or vitreous, diagnosed from 1995 to 2006. Diagnoses were established through histopathologic and immunohistochemical evaluation of vitreous fluid obtained via pars plana vitrectomy. RESULTS: The New York Eye and Ear Infirmary Pathology database search resulted in 19,149 vitrectomy specimens, 4 of which were positive for metastatic carcinoma. The diagnosis was clinically unsuspected in three patients. The oncologic work-up revealed the origin of metastases to be from the breast in two patients and from the lung in two patients. CONCLUSION: Metastatic carcinoma to the eye can present with clinically innocuous metastases, masquerading as epiretinal membrane and macular hole, central serous chorioretinopathy, rhegmatogenous retinal detachment, retinal granuloma, and endophthalmitis. These findings suggest that submission of vitrectomy specimens for pathologic evaluation should be considered in patients with an oncologic history or in those with atypical clinical presentation.