Faculty Bibliography

PURPOSE/UNASSIGNED:To describe a rare case of severe anterior and posterior scleritis that led to a diagnosis of high-risk myelodysplastic syndrome. METHODS/UNASSIGNED:We present a case report along with a literature review on cases of paraneoplastic scleritis to highlight commonalities in etiology, presentation, treatment response, and outcomes. RESULTS/UNASSIGNED:A 59-year-old male presented with a severe case of unilateral anterior and posterior scleritis with ciliochoroidal detachment and secondary angle closure. His workup revealed severe macrocytic anemia and thrombocytopenia. A subsequent bone marrow biopsy demonstrated cytogenetic analysis consistent with high-risk myelodysplastic syndrome (MDS). The patient was started on high-dose prednisone but was unable to be tapered below 30 mg without recurrence. Rituximab infusions were initiated followed by resolution of ocular symptoms and a successful taper of prednisone. He initiated chemotherapy and was to receive allogenic bone marrow transplantation. However, while on chemotherapy, he clinically deteriorated and passed away. CONCLUSION/UNASSIGNED:This case of severe scleritis was suspected to be a paraneoplastic syndrome secondary to myelodysplastic syndrome. Paraneoplastic syndromes represent a rare etiology of scleritis. Patients with hematologic abnormalities such as unexplained cytopenias, severe manifestations at presentation, or steroid refractory scleritis may warrant comprehensive evaluation for malignancy, expedited systemic workup with attention to blood counts, and interdisciplinary collaboration between ophthalmology, hematology, and internal medicine. If an underlying malignancy is identified, definitive treatment of the scleritis involves treatment of the cancer.

A 58-year-old woman presented with painless progressive bilateral blurred vision, worse in the right eye, over several days. Two months prior, she developed a diffuse pruritic rash that spared her face. On examination, she was found to have reduced visual acuity bilaterally, optic nerve edema, anterior uveitis, and scattered intraretinal and peripapillary nerve fiber layer hemorrhages. Diagnostic evaluation demonstrated optic nerve head enhancement on MRI. Her serum workup showed positive antibodies for Borrelia burgdorferi with a negative Lyme disease Western blot and positive Treponema pallidum antibodies with an rapid plasma reagin titer of 1:1,024. CSF showed normal protein, a slight elevation in nucleated cells with lymphocytic predominance, and a negative Venereal Disease Research Laboratory. We examine the differential diagnosis for bilateral optic nerve edema and uveitis and explore challenges around interpreting diagnostic testing for a neuro-ophthalmic pathology of ongoing public health interest.

PURPOSE/OBJECTIVE:To report a case of a full thickness macular hole (FTMH) after exposure to an extremely powerful handheld laser pointer. METHODS:We evaluated a 14-year-old male with a laser induced FTMH one month after a momentary exposure to a 5000 mW blue laser pointer. Imaging modalities including fundus color, autofluorescence, and spectral domain optical coherence tomography (SD-OCT), acquired both at our clinic and by the referring physician soon after the injury, are used to describe the clinical evolution of the case. RESULTS:Soon after the injury an intensely white, circular opacification of the retina approximately 400 µm in diameter was seen in the fovea. Early SD-OCT images showed full thickness hyperreflectivity, likely representing tissue necrosis. One month later, a FTMH and eradication of the retinal pigment epithelium at its base were evident in the fundus color, autofluorescence and SD-OCT images. CONCLUSION/CONCLUSIONS:High power laser pointers have become easily available online. The presenting findings after exposure to such high-power devices are distinct from those reported after exposure to weaker laser pointers. While long exposure to weaker lasers typically produces extensive, calligraphic figures and yellow placoid lesions involving only the outer retina, in our case a very brief exposure led to focal, full-thickness injury of the fovea.

Retinal detachment occurs more frequently in patients with pigment dispersion syndrome. We evaluated the incidence of peripheral retinal abnormalities known to predispose to rhegmatogenous retinal detachment in a consecutive series of 60 patients with pigment dispersion syndrome with or without glaucoma. Lattice degeneration was present in at least one eye of 12 patients (20%). Seven patients had bilateral lesions. Full-thickness retinal breaks were found in seven patients (11.7%) and two patients (3.3%) had asymptomatic rhegmatogenous retinal detachments that required scleral buckle procedures. The incidence of lattice degeneration and full-thickness retinal breaks appears to be increased in this group of patients, and may be responsible for the increased risk of rhegmatogenous detachment.