Carcinoma-associated retinopathy in a young teenager with immature teratoma of the ovary.

A 14-year-old African American girl presented with diminished vision in both eyes 1 week after undergoing an oophorectomy for a right ovarian mass. Systemic metastatic work-up was negative. Visual acuity was 20/40 in the right eye and 20/50 in the left eye. Slit-lamp biomicroscopy was unremarkable in both eyes. Fundus examination showed diffuse patchy areas of retinal pigment epithelial atrophy in the macula and peripheral retina bilaterally. Color vision had decreased in each eye. Electroretinography revealed nondetectable rod and cone responses. Both pattern and flash visual evoked potential (VEP) testing showed delayed latency in both eyes. She was treated with pulse intravenous methylprednisolone for 3 days along with intravenous immunoglobulins and rituximab, followed by systemic prednisolone and biweekly intravenous immunoglobulins and rituximab for 3 months. Antiretinal autoantibodies against 48-kDa (arrestin) and 64-kDa and 94-kDa proteins were positive, suggestive of carcinoma-associated retinopathy. After 3 months, visual acuity was 20/40 in each eye with improvement in color vision and VEP findings.

Bilateral ischemic maculopathy in acquired immune deficiency syndrome.

BACKGROUND: This brief report aims to report a case of bilateral macular ischemia as a cause of sudden decreased vision in a patient with acquired immune deficiency syndrome (AIDS). FINDINGS: A 26-year-old male with disseminated cryptococcal meningitis, Candida thrush, Pneumocystis jiroveci pneumonia, and positive human immunodeficiency virus (HIV) infection with CD4 count of 4 cells/µl complained of sudden blurred vision in both eyes while on treatment with systemic antiviral, antifungal, and antibiotic medications. Ocular examination revealed HIV retinopathy changes with significant macular ischemia in both eyes, which was confirmed by fluorescein angiography. One dose of intravitreal foscarnet (1.2 mg/0.1 cc) was injected in both eyes. Laboratory work-up of serum and vitreous samples showed negative cytomegalovirus (CMV) titers. At 2 weeks of follow-up, he was started on treatment with atripla, a combination anti-retroviral therapy for AIDS. At 6 weeks of follow-up, there was an improvement in visual acuity and clinical findings. CONCLUSIONS: Noninfectious HIV retinopathy in AIDS is common, but bilateral macular ischemia is a rare presentation. It is important to rule out CMV retinitis as it is a major cause of visual morbidity among AIDS patients.

Intravitreal implant migration into anterior chamber in a post-vitrectomy eye with central retinal vein occlusion and persistent macular edema.

The authors report the case of a 76-year-old man with a history of central retinal vein occlusion with persistent macular edema in the pseudophakic left eye, which was vitrectomized after complicated retinal detachment surgery. Two weeks after treatment with an intravitreal dexamethasone implant, the implant migrated into the anterior chamber. Visual acuity was hand motion in the right eye and 20/40 in the left eye with corneal edema. One week later, the implant relocated back into the vitreous cavity without surgical intervention, with a marked decrease in corneal edema and improved visual acuity (20/30) in the left eye. Weak zonules and posture change may have caused implant migration in this patient.

Dry eye syndrome in aromatase inhibitor users.

BACKGROUND: Aromatase inhibitors are frequently used as an adjuvant therapy in the treatment of breast cancer. We observed that several patients taking aromatase inhibitors presented with severe dry eye symptoms, and we investigated whether there is a relationship between aromatase inhibitors and dry eyes in these patients. DESIGN: Retrospective chart review. PARTICIPANTS: Forty-one women. METHODS: A computerized search of health records was performed to identify patients using anastrazole, letrozole and exemestane seen by the Cornea Service from August 2008 to March 2011. The results were compared with age-matched controls. MAIN OUTCOME MEASURES: Ocular surface changes among aromatase inhibitors users. RESULTS: Of the 41 women, 39 were Caucasians. Thirty-nine patients had breast cancer (95%), one patient had ovarian cancer (2.5%) and one had an unknown primary cancer. Mean age was 68 ± 11.3 years (range 47-95). Most common presenting symptoms were blurred vision in 28 (68%) patients, irritation/foreign body sensation in 12 (29%) patients, redness in 9 (22%) patients, tearing in 6 (22%) patients and photosensitivity in 2 (5%) patients. Mean Schirmer's test measurement was 11 ± 5.8 mm (range 0.5-20 mm). Blepharitis was noted in 68 of 82 eyes (73%), decreased or poor tear function in 24 eyes (29%), conjunctival injection in 18 eyes (22%) and superficial punctate keratitis in 12 eyes (29%). Among an age-matched population (45-95 years), dry eye syndrome was found in only 9.5% of patients. CONCLUSIONS: Because the prevalence of ocular surface disease signs and symptoms appears to be higher in study group than control patients, aromatase inhibitors might be a contributing factor to the dry eye symptoms.

Vitreoretinal lymphoma: changing trends in diagnosis and local treatment modalities at a single institution.

UNLABELLED: In this retrospective study on vitreoretinal lymphoma, there was significant change in diagnosis and treatment trends over 17 years at a single institution. Fine needle aspiration biopsy had replaced vitrectomy to collect vitreous sample and external beam radiotherapy in combination with systemic chemotherapy was replaced by intravitreal methotrexate and rituximab, which regressed vitreoretinal lymphoma (VRL) with no relapses or major ocular complications. INTRODUCTION: The purpose of this study was to report the changing trends in treatment (external beam radiotherapy [EBRT] and intravitreal chemotherapy) of VRL and treatment outcomes at a single institution. MATERIALS AND METHODS: A retrospective chart review of vitreous biopsy proven patients was performed. The data analysis included demographics, systemic lymphoma status, ocular symptoms, clinical and immunocytological findings, treatment methods, and response (intravitreal methotrexate 300 µg/0.05 mL, 1000 µg/0.1 mL of rituximab and EBRT 36-45 Gy) and ocular and systemic lymphoma outcomes at last follow-up. RESULTS: Twelve eyes of 8 patients had intraocular B-cell lymphoma (median age, 61 years; range, 50-83). Central nervous system non-Hodgkin's lymphoma (CNS-NHL) was present in 7 of 8 patients. Most common ocular symptoms were diminution of vision in 4 and floaters in 3 patients. Iritis and uveitis were found in 6 eyes and vitritis in 11 eyes. Retinal infiltrates were present in 8 eyes. Immunocytology revealed elevated levels of interleukin (IL)-10 (12,783.5 pg/mL), IL-6 (26.7 pg/mL), and IgH gene rearrangement. Three patients were treated with EBRT, 6 eyes with intravitreal methotrexate (median, 9.5; range, 2-15), and 2 eyes with intravitreal rituximab injections (median, 4; range, 2-6). Two patients developed marked keratitis because of methotrexate toxicity. At median follow-up of 33.5 months (range, 4-96), VRL had resolved in 7 eyes and persistent in 5 eyes. One patient died because of advanced CNS-NHL. CONCLUSION: Intravitreal chemotherapy provided good control rates for VRL patients in our limited series. Patients with associated CNS-NHL had poorer outcomes.

Clinical and image-guided chorioretinal findings in long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.

The purpose of this study is to report clinical, optical coherence tomography (OCT), and fluorescein angiogram/indocyanine green angiography (FA/ICG) findings in patients with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) enzyme deficiency in two siblings. A 13-year-old girl and her 14-year-old brother presented with progressive decrease in central vision. Clinically, there were blond-looking fundi, diffuse retinal pigment epithelial (RPE) disruption/atrophy in the macula and peripheral retina with choriocapillaris atrophy in both of them. OCT showed RPE irregularity and diffuse disruption of the RPE layer. FA/ICG imaging demonstrated transmitted choroidal fluorescence secondary to diffuse RPE atrophy with no evidence of leakage. Electroretinogram and electrooculogram findings were suggestive of primary abnormality of pigment epithelium. The boy died of cardiac/respiratory illness, whereas his sister is alive at the last follow-up. Abnormal chorioretinal findings in LCHAD patients should be carefully followed. Regular follow-up is recommended to monitor the ocular and systemic status.

Laser pointer induced macular damage: case report and mini review.

To report laser pointer induced damage to retina and choroid and briefly review literature. A case report of a 13-year old Caucasian boy developed blurry central vision and central scotoma in right eye (OD). He was exposed for one minute to class IIIA green laser pointer of 650 nm wavelength and 5 mW power. Clinical examination showed a grayish lesion in foveal region. Ancillary testing revealed disruption of the retinal pigment epithelial (RPE) layer in foveal region and indocyanine green angiography demonstrated evidence of choroidal hypofluorescence suggestive of choroidal infarction in OD. Visual acuity improved from 20/100 to 20/60 in one day and he was treated with tapering doses of oral prednisolone (40 mg) for 3 weeks. Laser pointer with a power of >5 mW caused damage to RPE in the macula. Children should not be given laser pointers as toys especially those with label of danger instructions.

Does fingolimod in multiple sclerosis patients cause macular edema?

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system and is the common cause of optic neuritis. Fingolimod, an immunosuppressive agent, is used in MS to prevent acute exacerbations. We report a case of relapsing-remitting MS treated with fingolimod. The patient presented with an acute decrease in vision in the left eye. Eye examination showed clinical macular edema (ME) in the left eye, which was confirmed on fluorescein angiogram and optical coherence tomography (OCT). After discontinuation of fingolimod and treatment with topical corticosteroid medication, there was complete resolution of the ME. The ME as a side-effect of fingolimod is reversible after discontinuing, which was seen on OCT.

Vitreous hemorrhage as the initial manifestation of idiopathic thrombocytopenic purpura.

PURPOSE: To report vitreous hemorrhage as the initial manifestation in idiopathic thrombocytopenic purpura, an uncommon presentation. METHODS: Case report. RESULTS: A 69-year-old white woman presented with diffuse vitreous hemorrhage as the initial manifestation of idiopathic thrombocytopenic purpura, 12 years after plaque radiotherapy for choroidal melanoma. CONCLUSION: Unexplained and recurrent vitreous hemorrhage should be evaluated for idiopathic thrombocytopenic purpura.

Second malignant neoplasms following chemoreduction with carboplatin, etoposide, and vincristine in 245 patients with intraocular retinoblastoma.

BACKGROUND: To evaluate the occurrence of second malignant neoplasms (SMN) following chemoreduction (CRD) with carboplatin, vincristine, and etoposide (CEV) as frontline therapy in patients with retinoblastoma (RB). PRODECURE: We conducted a two-institution retrospective chart review of 245 patients with intraocular RB treated with six cycles of vincristine, carboplatin, and etoposide for treatment of intraocular retinoblastoma. Cumulative incidence of SMN was calculated with adjustment for the competing risk of death. RESULTS: There were 187 patients with germline retinoblastoma and 58 with non-germline disease. External beam radiotherapy was subsequently utilized in 46 (24%) of germline cases and six (10%) of non-germline cases. Mean follow-up of germline and non-germline patients was 80 and 70 months, respectively. Seven subsequent cancers were found in six patients for an overall incidence of 3% at a mean of 11 years. For germline cases, following CEV alone (n = 156), SMN were found in 4% following the RB diagnosis. We found no SMN in patients with non-germline RB. One patient developed pineoblastoma. SMN included osteosarcoma (n = 3), rhabdomyosarcoma (n = 1), orbital and conjunctival melanoma (n = 1), low-grade glioma (n = 1), and acute promyeloctic leukemia (n = 1). Five of the six patients with a second malignancy survive at mean of 46 months (range 15-71 months). CONCLUSIONS: At a mean of 11 years, 4% of children with germline RB treated with CEV as frontline therapy developed SMN's. No SMN was found in non-germline patients. Concerns regarding CEV-induced second cancers should not deter clinicians from using life and vision preserving therapy in patients with retinoblastoma.

Giant fornix syndrome: a case series.

PURPOSE: To describe the demographics, characteristics, and treatment of giant fornix syndrome, a rare cause of chronic purulent conjunctivitis in the elderly. METHODS: Retrospective chart review of five patients with giant fornix syndrome evaluated by the Cornea Service, Oculoplastics and Orbital Surgery Service and the Department of Pathology at the Wills Eye Institute. RESULTS: The median age of the 5 female patients was 75 years (mean 80, range 70-95). The median duration of eye symptoms before presentation was 2 years (mean 2.4, range 1-4). Before referral, the chronic conjunctivitis was treated with topical antibiotics in all 5 cases and with additional dacryocystorhinostomy in one case. The right eye was affected in 2 cases, and the left eye was affected in the other 3 cases. Floppy eyelids were present in 2 cases. The superior fornix was involved in 4 cases, and the inferior fornix was involved in one case. Pseudomembranes and superficial punctate keratitis (SPK) were seen in 3 cases. Diagnosis of giant fornix syndrome was made in all 5 cases. Conjunctival culture grew methicillin-resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa, and S. aureus in singular cases. Case 1 was treated with topical moxifloxacin, Case 2 was treated with topical vancomycin and repair of the upper eyelid, Case 3 was treated with topical besifloxacin, and Case 4 was treated with dacryocystorhinostomy and topical vancomycin. Case 5 was treated with reconstruction of the left upper eyelid. The median duration of follow up was 4 months (mean 21.6, range 1-84). CONCLUSIONS: Giant fornix syndrome can lead to chronic relapsing conjunctivitis in the elderly. Deep conjunctival fornices in affected patients can be a site for prolonged sequestration of bacteria causing recurrent infections. Removing the infected debris from the superior fornix and reconstruction of the upper eyelid may prevent the recurrent chronic persistent infection.

Choroidal metastasis from hepatocellular carcinoma, diagnosed by fine needle aspiration biopsy and treated by iodine-125 brachytherapy.

PURPOSE: To report a rare case of choroidal metastasis from hepatocellular carcinoma. METHODS: A 63-year-old man with known hepatocellular carcinoma (HCC) and treated clavicular metastasis noted reduced visual acuity in the right eye. He was found to have an amelanotic choroidal tumor measuring 18 × 14 × 9.3 mm and with retinal detachment. Fine needle aspiration biopsy confirmed metastatic HCC. RESULTS: The choroidal metastasis was treated with 5 days of iodine-125 plaque brachytherapy (3500 cGy to apex). Tumor regression to 2.6 mm thickness with resolution of retinal detachment and preservation of visual acuity at 20/40 at the 4-month follow-up was documented. CONCLUSIONS: HCC metastasis to the choroid can be treated with focal plaque brachytherapy, with preservation of vision and quality of life.

Bilateral uveal melanoma in an arc welder.

PURPOSE: To report sequential bilateral uveal melanoma in an arc welder. METHODS: Case report. RESULTS: A 57-year-old Caucasian male, with a 15-year profession of arc welding, was found to have an iridociliary mass in his left eye (OS), measuring 14 × 10 × 4 mm, and proven on fine needle aspiration biopsy to be spindle B-cell melanoma. A coincidental small choroidal nevus was observed in the right eye (OD). There was no ocular melanocytosis. Plaque radiotherapy was applied OS, with regression of the iridociliary melanoma. Four years later, the choroidal nevus OD enlarged into melanoma measuring 8 × 7 × 2.7 mm and was treated successfully with plaque radiotherapy. There was no evidence of systemic metastasis at 56-month follow-up. CONCLUSION: Arc welding is a known environmental risk for unilateral uveal melanoma, and possibly predisposed our patient to bilateral uveal melanoma.

Prognosis of uveal melanoma in 500 cases using genetic testing of fine-needle aspiration biopsy specimens.

PURPOSE: To determine the relationship between monosomy 3 and incidence of metastasis after genetic testing of uveal melanoma using fine-needle aspiration biopsy (FNAB). DESIGN: Noncomparative retrospective case series. PARTICIPANTS: Five hundred patients. METHODS: Fine-needle aspiration biopsy was performed intraoperatively immediately before plaque radiotherapy. The specimen underwent genetic analysis using DNA amplification and microsatellite assay. Systemic follow-up was obtained regarding melanoma-related metastasis. MAIN OUTCOME MEASURES: Presence of chromosome 3 monosomy (loss of heterozygosity) and occurrence of melanoma metastasis. RESULTS: Disomy 3 was found in 241 melanomas (48%), partial monosomy 3 was found in 133 melanomas (27%), and complete monosomy 3 was found in 126 melanomas (25%). The cumulative probability for metastasis by 3 years was 2.6% for disomy 3, 5.3% for partial monosomy 3 (equivocal monosomy 3), and 24.0% for complete monosomy 3. At 3 years, for tumors with disomy 3, the cumulative probability of metastasis was 0% for small (0-3 mm thickness), 1.4% for medium (3.1-8 mm thickness), and 23.1% for large (>8 mm thickness) melanomas. At 3 years, for tumors with partial monosomy 3, the cumulative probability of metastasis was 4.5% for small, 6.9% for medium, and [insufficient numbers] for large melanomas. At 3 years, for tumors with complete monosomy 3, the cumulative probability of metastasis was 0% for small, 24.4% for medium, and 57.5% for large melanomas. The most important factors predictive of partial or complete monosomy 3 included increasing tumor thickness (P = 0.001) and increasing distance to optic disc (P = 0.002). CONCLUSIONS: According to FNAB results, patients with uveal melanoma demonstrating complete monosomy 3 have substantially poorer prognosis at 3 years than those with partial monosomy 3 or disomy 3. Patients with partial monosomy 3 do not significantly differ in outcome from those with disomy 3.

A case series of neuroendocrine (carcinoid) tumor metastasis to the orbit.

PURPOSE/BACKGROUND: To report the clinical and radiographic features and treatment outcome of neuroendocrine tumor (carcinoid) metastasis to the orbit. MATERIALS AND METHODS: Retrospective chart review of four cases. RESULTS: Mean patient age at the time of diagnosis of the primary neuroendocrine tumor and orbital metastasis was 58 and 66 years, respectively, with a mean duration of 8 years between diagnosis of primary tumor and orbital metastasis. Primary neuroendocrine tumor sites were gastrointestinal tract (n = 2), lung (n = 1), and testicle (n = 1). The most common presenting symptom was diplopia (three cases). Magnetic resonance imaging revealed orbital tumor in all cases. Octreotide scan was positive in one case. Treatment was tumor excision in three cases followed by external beam radiotherapy in two cases and one patient was followed without treatment. Tumor cells showed immunoreactivity to chromogranin, synaptophysin, and neuron-specific enolase in all cases. Mean follow-up after orbital tumor diagnosis was 39 months. Three patients had known systemic extraorbital metastasis before orbital involvement (mean interval of 5.9 years) and one case had immediately after development of orbital metastasis. One patient had multiple recurrences of orbital metastasis and eventually underwent exenteration. Two patients died of disseminated metastasis between 2 and 3 years after diagnosis of orbital metastasis. CONCLUSION: All four patients with orbital metastasis from neuroendocine tumor had evidence of systemic extraorbital metastasis. Aggressive metastatic neuroendocine tumors of orbit can lead to local recurrence even after surgical excision and radiation. Imaging tests were helpful in allowing early diagnosis and for monitoring after treatment.

Retinocytoma shows lack of response to chemoreduction.

The authors report a case of retinocytoma showing no response to chemoreduction. A 30-month-old girl presented with Group B multifocal retinoblastoma in the right eye and Group E retinoblastoma in the left eye. After the first cycle of chemotherapy (vincristine [0.9 mg/m(2)], carboplatin [336 mg/m(2)], and etoposide [90 mg/m(2)]), there was remarkable reduction in the tumor size in the left eye, whereas the right eye tumors did not regress and were diagnosed as retinocytomas/retinomas. Retinocytoma/retinoma shows little to no response to chemoreduction.