Lori Vollmer

The diagnosis and management of ocular lymphoma.

Purpose Lymphoma is the most common malignancy of the ocular adnexa. Most of the ocular adnexa lymphomas are non-Hodgkin B-cell lymphomas. The most common type of ocular adnexa lymphoma is primary extranodal marginal zone B-cell lymphoma of the MALT (mucosa-associated lymphoid tissue). Most of these neoplasms are primary extranodal lymphomas, although 10% to 32% are secondary tumors from disseminated disease. Case Report A 58-year-old woman presented for a comprehensive examination, with the chief complaint of ocular discomfort in both eyes. Anterior segment examination revealed bilateral salmon-colored lesions of the inferior and superior conjunctivae. The patient was referred for systemic evaluation and histopathology of the conjunctival lesions. She was diagnosed as having marginal zone lymphoma of the MALT and underwent radiation therapy (RT). Conclusions Ocular lymphoma may present on routine examination or with mild symptoms. Although most commonly a primary extranodal neoplasm, the condition may be associated with disseminated lymphoma and requires thorough evaluation and staging of the disease for determination of appropriate treatment. The primary eye care provider plays an important role in the identification and staging of the disease, as well as managing complications from RT. It is also important to recognize that concurrent conditions requiring treatment with topical medications, such as glaucoma, may be complicated after treatment because of the inflammation and ocular surface irritation after RT. The necessity and benefit of the addition of intraocular pressure medications during that time should be measured on a case-by-case basis. Patients should be followed closely after treatment for relapse of disease and identification of complications from ocular RT.

Proliferative vitreoretinopathy as a late complication of blunt ocular trauma.

BACKGROUND Blunt ocular trauma to the eye can manifest in many ways. However, it is unknown how long patients are at risk for certain complications after the initial injury. Proliferative vitreoretinopathy is one possible complication of ocular trauma. This condition generally results from severe ocular trauma, chronic retinal detachment, or retinal surgery. The nidus for proliferation is unclear; however, once formed, proliferative membranes create traction at the vitreoretinal surface, leading to further complications and ocular morbidity. CASE REPORT A 34-year-old man presented emergently with decreased vision in the left eye for 3 weeks and pain for 3 days. The patient denied any recent trauma or systemic disease but did report trauma to his left eye approximately 5 years earlier. His best-corrected acuities were 20/20 in the right eye and light perception in the left eye. The anterior segment evaluation found a nearly complete hyphema, iris neovascularization, and superior uveal prolapse through scleral perforation of the left eye. Retinal detachment and proliferative vitreoretinal disease were also discovered, necessitating surgical repair. CONCLUSION Proliferative vitreoretinopathy is associated with severe ocular trauma. In this case, the patient had a markedly delayed onset of proliferative retinopathy resulting from blunt trauma with partial perforation 5 years before. Clinicians should be aware of the potential for the development of proliferative vitreoretinopathy as a long-term complication of blunt ocular trauma as well as the treatment options for this disease.

Use of multifocal ERG and OCT for diagnosing Stargardt's disease

Stargardt’s disease (Stargardt’s macular dystrophy, juvenile macular degeneration) was originally described by Stargardt in the early 1900s and is known as the most common congenital macular dystrophy, with an incidence of one in every 10,000. Stargardt’s disease (STGD) is characterised as a widespread lipofusin storage disease of the retinal pigment epithelium (RPE). Typically, it presents between the first to third decades of life and is associated with a progressive, bilateral decrease in visual acuity leading to legal blindness and central visual acuity of 6/60 to 6/120. Though it is typically associated with an autosomal recessive (AR) inheritance pattern, autosomal dominant (AD) cases have been reported with later onset and less severe acuity loss. Classic findings include bilateral macular changes that begin as granular mottling with progression toward a ‘beaten bronze’ maculopathy. The late stage of the disease is associated with macular atrophic degeneration. Yellow-white flecks in the posterior pole may be noted; they are described as tri-radiating ‘fish tail’ (pisciform) flecks. Traditionally, Stargardt’s disease has been diagnosed through clinical evaluation and fluorescein angiography (IVFA), which demonstrates a dark or ‘silent’ choroid due to the blockage of the fluorescein by the lipofusin deposition in the RPE. Though fluorescein angiography has been considered the ‘gold standard’ in verifying the presence of the disease, the characteristic dark choroid pattern is seen in only 85 per cent of patients with Stargardt’s disease. There have been reports of patients with autosomal dominant inheritance pattern who fail to demonstrate this classic fluorescein angiography finding. Newer and minimally invasive procedures, such as the multifocal electroretinogram (mfERG) and optical coherence tomography (OCT), may aid in diagnosing and managing patients with the disease. These ancillary tests may be used in the event that fluorescein angiography cannot be performed or used in conjunction with traditional fluorescein angiography as a complementary procedure. Multifocal electroretinography (mfERG) is a minimally invasive procedure that has been shown to be helpful in the diagnosis of Stargardt’s disease. Unlike traditional flash electroretinography (fERG), this technology has proved extremely reliable in diagnosing and monitoring various forms of macular dystrophy. In the case of Stargardt’s disease, the patient presents with significant variation in presentation; from normal to reduced acuity, minimal to significant fundus findings and normal to slightly reduced full field flash electrophysiological findings. Regardless of the presentation, Stargardt’s patients typically have a significant reduction of the foveal response and central approximately 10 degrees in both eyes on mfERG, even early in the disease process with relatively good visual acuity and minimal fundus findings. In addition to electrophysiology, retinal imaging technology such as OCT has proved effective in correlating structural damage with functional visual loss. The OCT findings of patients with Stargardt’s disease include decreased thickness of the retina, most notably in the foveola. The OCT also reveals photoreceptor loss and external nuclear layer changes, as well as abnormalities in the retinal pigment epithelium.

Rapid onset phacolysis.

BACKGROUND Phacolysis involves the breakdown of a hypermature cataract, causing an antigenic reaction to the lens proteins released into the anterior chamber with subsequent inflammation. To date, the time it takes for a crystalline lens to reach hypermaturity and induce a phacolytic response has never been clearly detailed. It is believed that cataract maturation is a slow process. The process by which the lens proteins begin to leak is thought by many to be similarly slow. However, the immune-related inflammatory process that develops when the lens proteins begin to leak may be quite rapid. It may be an error to consider this aspect of the phacolytic process to be slow. METHODS We present a case with a clear, timed delineation of the phacolytic process. A mature cataract became hypermature with subsequent phacolysis and inflammatory pressure rise over the course of 17 days. It appears that this is the first published account of the time involved in the development of phacolysis and, we believe, the fastest onset of the process. CONCLUSION While cataract maturation is generally considered to be a slow, insidious process, it should be recognized that the phacolytic process might not be slow. Once a lens reaches hypermaturity, phacolysis could occur quite rapidly over the course of several days. This case could be an important consideration in management of the removal of advanced cataracts. This case may be the shortest reported time from diagnosis of a mature cataract to the development of inflammatory phacolysis and secondary glaucoma, occurring over a period of only 17 days.

Atypical retinal vaso-occlusion with structural and functional resolution.

Purpose The purpose is to report a patient with primary open-angle glaucoma that developed sudden painless unilateral vision loss, a sequential ophthalmoscopic appearance with features of both central retinal artery and later central retinal vein occlusion, and objective visual system dysfunction in the form of a relative afferent pupil defect, who spontaneously recovered vision along with complete resolution of the pupillary defect over several weeks. Case Report A 50-year-old woman with a long-standing history of glaucoma presented with acute, painless vision loss in one eye, a pallid retina with a cherry red macula, diffuse retinal hemorrhages, and a relative afferent pupil defect. Spectral domain optical coherence tomography and fluorescein angiography were essentially normal with neither retinal edema nor retinal ischemia to account for the visual dysfunction. Over the course of 2 months, the patient regained vision and the relative afferent pupil defect, typically a permanent manifestation of retinal destruction, resolved. Conclusions Not all retinal vaso-occlusive phenomena can be completely attributed to a central retinal vein or artery occlusion. In the patient presented, there was no objective diagnostic testing that revealed a cause for the patient’s vision loss or relative afferent pupillary defect. This combined with the complete recovery of vision and resolution of the relative afferent pupillary defect underscores a lack of comprehensive understanding of retinal vaso-occlusive disease.