Faculty Bibliography

BACKGROUND: Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare, paraneoplastic syndrome characterized by bilateral painless visual loss and proliferation of choroidal melanocytes in association with an underlying systemic malignancy. We report a case of bilateral diffuse uveal melanocytic proliferation associated with an underlying gynecological malignancy that also features the infrequent finding of an iris mass lesion, using multimodal imaging including ultra-widefield imaging, spectral domain and swept-source optical coherence tomography. CASE PRESENTATION: A 59-year-old white female with a prior history of gynecological malignancy in remission presented with progressive bilateral visual loss over several weeks. The patient was noted to have a focal iris mass lesion in her right eye. Ultra-widefield color fundus photography showed a characteristic bilateral 'giraffe pattern' of pigmentary changes extending into the periphery as well as multiple discrete deeply pigmented lesions. Ultra-widefield autofluorescence was useful for visualizing the full extent of involvement. Indocyanine green angiography helped to demarcate the discrete pigmented choroidal lesions. Swept-source OCT clearly delineated the alternating zones of retinal pigment epithelium (RPE) thickening and RPE loss, as well as the prominent choroidal infiltration and thickening. CONCLUSIONS: BDUMP is an important diagnosis to consider in the presence of multiple discrete melanocytic choroidal lesions, diffuse choroidal thickening, characteristic RPE changes, iris mass lesions and exudative retinal detachment. Ultra-widefield imaging may demonstrate more extensive lesions than that detected on clinical examination or standard field imaging. Imaging with SS-OCT shows choroidal and RPE characteristics that correlate well with known histopathology of this entity.

A 20-year-old white woman presented with bilateral acute visual loss (visual acuity: 20/60), panuveitis, and exudative retinal detachments 3 weeks after a second dose of quadrivalent human papillomavirus (HPV4) vaccine. She was treated with oral prednisolone for 6 weeks and responded rapidly. By week 4, vision had normalized and clinical signs resolved. Uveitis after HPV4 vaccination has been reported in two cases. Although the differential diagnosis includes Harada disease, temporal correlation with HPV4 and definitive response to a short course of treatment implicate the vaccine in this case. Vaccine-induced uveitis is rare and difficult to distinguish from coincidental autoimmune disease. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:967-970.].

Purpose To determine the rate of neovascularization (NV) in eyes with acquired vitelliform lesion (AVL) during and following vitelliform collapse. To correlate the optical coherence tomography (OCT) and histopathological characteristics of these neovascular membranes. Methods Retrospective cohort analysis of 112 patients with AVL. Patients that demonstrated evidence of vitelliform collapse, defined as a temporal reduction in the size of subretinal vitelliform material clinically, using OCT and fundus autofluorescence imaging, were included for further analysis. Clinical and OCT characteristics of neovascular membranes were determined. A correlation between OCT and histopathological characteristics of an eye that was clinically diagnosed as non-neovascular but demonstrated a type 1 membrane on post mortem examination was also performed. Results Twenty-six patients (16 males and 10 females) demonstrated evidence of vitelliform collapse, and 7 (26.9%) of these developed NV. 5 of these patients were diagnosed with NV following acute subretinal hemorrhage or exudation. Mean age of patients was 81.1 +/- 11.6 years, and mean period of follow up was 9.0 +/- 4.2 years. All neovascular membranes were type 1. Persistent OCT findings prior to the development of NV included: (1) Irregular elevation of the retinal pigment epithelium (RPE) layer at the site of NV. (2) Separation of the RPE layer and Bruch's membrane (BrM) by a hyporeflective material containing punctate hyper-reflectivity (figure 1). Three patients had fluorescein angiography (FA) within 6 months preceding the diagnosis of neovascular disease that did not demonstrate leakage. Histopathologic examination demonstrated a fibrovascular scar and thick basal laminar deposit (BlamD) under the fovea with hemorrhage between the scar and BrM. These lesions correlated with the split RPE-BrM band on OCT images acquired 8 months before the patient's death (figure 2). Conclusions The rate of Type 1 NV during and following vitelliform collapse in AVLs is significant. In this subgroup of patients, neovascular membranes appear to remain dormant in the anatomic space between BrM and BLamD before the clinical signs of NV, including exudation and hemorrhage, become manifest. Interval review of these patients is therefore indicated as is a prospective study of this topic

IntroductionThe characteristics of type 3 neovascularization (NV), also known as retinal angiomatous proliferation, have been well described clinically, as well as with fluorescein angiography (FA), indocyanine green angiography, and optical coherence tomography (OCT). OCT angiography (OCT-A) is a novel and non-invasive technique for imaging retinal microvasculature by detecting changes, with respect to time, in reflectivity related to blood flow.MethodIn this case series, we describe two patients who presented with type 3 NV and underwent clinical examination and multimodal imaging, including OCT-A.ResultsIn the first patient, OCT-A demonstrated flow within two separate lesions in the same eye, one of which was only weakly detected by FA. In the second patient, sequential OCT-A demonstrated a reduction in intralesional flow following intravitreal therapy.ConclusionsOCT-A may have a role in the early diagnosis of type 3 NV and in assessing the response to treatment. Further studies are needed to determine sensitivity and specificity.

Dome-shaped macula is described as an inward bulge of the macula within a posterior staphyloma in highly myopic eyes. Choroidal neovascularization is a known complication that can cause visual loss in dome-shaped macula. Herein, we describe a patient who presented with features of polypoidal choroidal neovascularization that developed on a background of high myopia with dome-shaped macula.

Juvenile xanthogranuloma is a benign non-Langerhans cell histiocytosis characterized by skin lesions that tend to be self-limited. Ocular lesions can occur in juvenile xanthogranuloma, most commonly presenting as an iris granuloma. Skin lesions of juvenile xanthogranuloma may appear similar to lesions of mastocytosis. Mastocytosis includes a heterogeneous group of diseases characterized by the proliferation and abnormal infiltration of mast cells. Rubbing of cutaneous lesions leads to the release of histamine, causing the lesions to urticate. Juvenile xanthogranuloma and mastocytosis skin lesions occurring concurrently is extremely rare, with only four cases reported. Ocular juvenile xanthogranuloma and cutaneous lesions of mastocytosis have never been described in the same patient in the literature. The authors describe a patient with an ocular juvenile xanthogranuloma presenting at birth with cutaneous mastocytosis developing several years later. [J Pediatr Ophthalmol Strabismus 2014;51:e89-e91.].

BACKGROUND: Age-related macular degeneration (AMD) is a degenerative process that leads to severe vision loss. Wet AMD is defined by choroidal neovascularisation, leading to the accumulation of subretinal fluid (SRF), macular oedema (ME), and pigment epithelium detachments (PED). Purpose To evaluate the initial clinical experience of conversion from bevacizumab or ranibizumab to aflibercept in wet AMD patients. METHODS: Records of 250 consecutive wet AMD patients were retrospectively reviewed. Of 250 patients, 29 were naive (with no previous treatment), and 221 were previously treated with bevacizumab (1/3) or ranibizumab (2/3). On average, converted patients received 14 injections every 6 weeks on a treat-and-extend regimen with Avastin or Lucentis before being converted to aflibercept every 7 weeks on average (no loading dose) for three doses. For the purposes of this study, we concentrated on the patients converted to aflibercept since the number of naive patients was too small to draw any conclusion from. Snellen (as logMar) visual acuities, and optical coherence tomography (OCT) were compared predrug and postdrug conversion. RESULTS: Converted patients did not show a significant difference in visual acuity or average OCT thickness from preconversion values; however, small improvements in ME (p=0.0001), SRF (p=0.0001), and PED (p=0.008) grading were noted on average after conversion to aflibercept. CONCLUSIONS: No significant difference in visual outcome or average OCT thickness was observed when switched from bevacizumab or ranibizumab q6 week to aflibercept 7-week dosing, on average. Mild anatomic improvements did occur in converted patients with regard to ME, SRF and PED improvement, on average, after conversion to aflibercept, and aflibercept was injected less frequently. No serious adverse reactions, including ocular infections or inflammation, as well as ocular and systemic effects were noted.

Mastocytosis is a heterogeneous group of diseases characterized by the proliferation and abnormal infiltration of mast cells in tissue. These collections of mast cells are called mastocytomas. Solitary mastocytomas are cutaneous lesions, most commonly involving the extremities and trunk. Rubbing a cutaneous mastocytoma lesion leads to the release of histamine and other chemical mediators causing the lesion to become elevated and urticarial, similar to an allergic reaction. We describe a rare location of a presumed solitary mastocytoma of the lower eyelid in a young otherwise healthy child.