P. Kumar Rao

Lack of Consensus in the Diagnosis and Treatment for Ocular Tuberculosis among Uveitis Specialists

Abstract Purpose: To assess the approach of specialists to ocular tuberculosis (TB). Methods: The American Uveitis Society (AUS) Listserv was surveyed using two clinical cases and general questions. Results: Of 196 members, 87 responded (44.4%), of whom 64 were affiliated with practices in North America, while 23 were outside of North America. The survey provided normative data on how physicians evaluate patients with uveitis as well as opinions about ocular TB. Responses varied widely on such issues as (1) the pretest probability that a patient with granulomatous panuveitis had TB uveitis (range 1–75%) or that a patient with a risk factor for TB had ocular TB (range 0–90%); (2) the optimal duration of anti-TB therapy; and (3) whether therapy should be discontinued after 2 months in nonresponders. Conclusions: Consensus is lacking among uveitis specialists for the diagnosis or management of ocular TB.

IgG4-associated orbital and ocular inflammation

BackgroundIgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation.FindingsWe report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry).ConclusionsIgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy.

Bilateral macular hole formation in a patient with retinitis pigmentosa.

A 47-year-old woman known to have retinitis pigmentosa (RP) developed macular holes in each eye. Examination and fluorescein angiography demonstrated a partial thickness macular hole in the right eye and a full thickness one in the left eye. It was concluded that macular hole formation can occur in patients with RP, but the exact cause of macular hole development in these patients is uncertain.

Immunohistochemical study of epiretinal membranes in patients with uveitis

BackgroundThe purpose of this study is to report two cases of idiopathic uveitis with secondary epiretinal membrane (ERM) formation in order to describe histologic and immunohistochemical features that may help distinguish uveitic from idiopathic ERMs.MethodsThe study utilized a clinical case series and histopathological and immunohistochemical findings.ResultsThere was no identifiable etiology of inflammation in either case. Histology and immunohistochemistry demonstrated a mixture of abundant inflammatory cells, including lymphocytes, histiocytes, plasma cells, and occasional eosinophils, among a stromal matrix composed of glial elements and condensed vitreous, but no retinal pigment epithelium (RPE) was present. The relative proportions of the various inflammatory cell types were assessed with immunohistochemistry, and among the lymphocyte population, T cells predominated over B cells. In one of the cases, there was an abundance of histiocytes, consistent with granulomatous uveitis, which was later confirmed on histology of the enucleated globe.ConclusionsIdiopathic ERM formation is thought to be secondary to glial cell migration that may require some involvement of RPE cells. The absence of RPE and abundance of inflammatory cells may be used to identify ERMs as secondary to uveitis.

Morphologic Assessment for Glaucoma in the Multicenter Uveitis Steroid Treatment (MUST) Trial

Purpose: To compare Reading Center (RC) cup-to-disc ratio (CDR) assessment from stereoscopic photographs with clinician estimation in a uveitis clinical trial. Methods: Clinical estimation of CDR was performed by ophthalmologists via dilated biomicroscopy. Photographic evaluation was performed at an independent RC by masked, certified evaluators. Quality control was performed by repeat grading of 77 randomly selected images. Results: Among 479 eyes with uveitis, 353 eyes had clinical and photographic grades for CDR. Agreement between clinical and RC grading was fair, with exact agreement of 29%. Agreement within 0.1 and 0.2 CDR was 70 and 93%, respectively (weighted κ = .34). Intergrader reproducibility at the RC was better (weighted κ = .59, ICC 0.74). Conclusion: Morphologic assessment of cup to disc ratio is an important outcome and safety measure for determining glaucomatous damage in clinical trials. Masked RC measurements are more likely to be accurate than biomicroscopic grading in identifying meaningful anatomical change associated with glaucoma.

Extramedullary Relapse of Acute Myelogenous Leukemia Presenting as a Large Serous Retinal Detachment

Background/Aims: To describe the rare presentation of a large, unilateral, serous retinal detachment as an extramedullary manifestation of acute myelogenous leukemia (AML) recurrence without bone marrow or central nervous system involvement after more than 1 year of follow-up. Methods: Case report. Results: A teenage patient with AML, previously treated with multiple courses of systemic chemotherapy, radiation, and bone marrow transplant, presented with acute vision loss. Ophthalmic workup revealed a large, unilateral, bullous, serous retinal detachment. Ultimately, he underwent subretinal fluid biopsy, which was found to be positive for leukemic blast cells. Cytologic markers matched his initial bone marrow biopsy, and therefore were diagnostic of extramedullary AML relapse. Conclusions: Leukemia can cause various ophthalmic manifestations. Autopsy studies suggest that choroidal infiltration is relatively common, but clinical progression to serous retinal detachment is quite uncommon. Furthermore, serous retinal detachment is generally shallow, posterior, and much more often reported in acute lymphocytic leukemia. The ophthalmologist plays a critical role in identifying leukemic ocular involvement. This case demonstrates the potential for ocular biopsy to secure the diagnosis of extramedullary relapse in order to initiate prompt treatment and systemic workup.

Histologic and immunohistochemical features of a vitreous cyst.

PURPOSE To report the histologic and immunohistochemical features of a congenital vitreous cyst. METHODS Interventional case report with immunohistochemical correlation. A 58-year-old woman with known vitreous cyst since childhood had an enlarging visual floater. The vitreous cyst was removed by pars plana vitrectomy and central vision was restored. RESULTS Pathology confirmed a vitreous cyst derived from neural and glial tissue and consistent with retinal origin. CONCLUSIONS Congenital vitreous cysts, derived from neural and glial tissue, can be safely removed using pars plana vitrectomy.