Susac syndrome is a rare condition characterized by the clinical triad of central nervous system (CNS) dysfunction, sensorineural hearing impairment, and branch retinal artery occlusion (BRAO). The purpose of this study is to examine the demographics, clinical characteristics, treatment, and long-term prognosis of Susac syndrome. The data recorded for all Susac syndrome patients treated at the Sheba Medical Center between 1998 and 2014 included demographics, clinical signs at presentation and during the disease course, imaging findings, treatment, and prognosis. Susac syndrome was diagnosed in 10 patients (age range 30–45 years). Only 2 patients presented with the full triad and 7 patients developed the full triad during mean follow-up period of 35 months. The average time to full triad was 7 months. Based on our observations at presentation, we divided the disease course into suspected, incomplete, and complete Susac syndrome. All 10 patients were treated at diagnosis with a pulse of high-dose intravenous methylprednisolone. There was improvement in visual acuity and visual field at the end of follow-up compared to baseline, but it was not statistically significant (P = 0.479 and P = 0.053, respectively). Five patients remained with neurological damage, and 5 patients had no improvement of their hearing loss at study closure. In conclusion, Susac syndrome is a rare condition that can mimic other disorders. The diagnosis is challenging because most patients do not initially present with the definitive triad. We suggest a clinical classification for the syndrome that may assist in early diagnosis.

Ocular involvement in leukemia is considered rare. Ocular symptoms can be the presenting signs of leukemia, they can appear after diagnosis has been established, or they can be the first manifestation of a relapse after remission. We report, to the best of our knowledge for the first time, the ocular manifestation of a series of patients with ocular leukemia and the result of their treatment with intravitreal methotrexate (MTX) injections. This is a retrospective cohort study. The medical records of 12 consecutive patients with ocular leukemia (24 eyes, 11 eyes treated with MTX) treated at the Sheba Medical Center from January 2010 to December 2017 were retrospectively reviewed. Details on ocular inflammatory reaction and tumor cell infiltration at presentation and the end of follow-up were recorded as main outcome measures. The 12 patients included 7 women and 5 men (mean age ± standard deviation at diagnosis 25.92 ± 23.91 years, range 2–82 years). Eleven eyes of 6 patients were treated with intravitreal MTX injections. The indication for treatment was biopsy proven, tumor cell infiltration. The mean number of MTX injections was 3.37 ± 5.35 (range 1–18). The mean follow-up was 27.08 ± 36.79 months (range 1–93). All treated eyes showed improvement in the inflammatory reaction and tumor cell infiltration. In conclusion we found that Intravitreal MTX injections may be an effective therapeutic approach for eyes with intraocular leukemic tumor cell infiltration.

To examine the ocular side effects of selected biological anti-cancer therapies and the ocular and systemic prognosis of patients receiving them. We retrospectively reviewed all medical records of patients who received biological anti-cancer treatment from 1/2012 to 12/2017 and who were treated at our ocular oncology service. The following data was retrieved: primary malignancy, metastasis, type of biological therapy, ocular side effects, ophthalmic treatment, non-ocular side effects, and ocular and systemic disease prognoses. Twenty-two patients received biological therapies and reported ocular side effects. Eighteen patients (81.8%) had bilateral ocular side effects, including uveitis (40.9%), dry eye (22.7%), and central serous retinopathy (22.7%). One patient (4.5%) had central retinal artery occlusion (CRAO), and one patient (4.5%) had branch retinal vein occlusion (BRVO). At the end of follow-up, 6 patients (27.27%) had resolution of the ocular disease, 13 patients (59.09%) had stable ocular disease, and 3 patients (13.64%) had progression of the ocular disease. Visual acuity improved significantly at the end of follow-up compared to initial values. Eighteen patients (81.8%) were alive at study closure. Biological therapies can cause a wide range of ocular side effects ranging from dry eye symptoms to severe pathologies that may cause ocular morbidity and vision loss, such as uveitis, CRAO and BRVO. All patients receiving biological treatments should be screened by ophthalmologists before treatment, re-screened every 4–6 months during treatment, and again at the end of treatment. Patients on biological treatment who have ocular complaints should be urgently referred to ocular consultation for early identification and early intervention.

Introduction Naevus of Ota is a congenital condition that may involve the skin, eyeball and even intracranial structures usually in the distribution of the ophthalmic and maxillary divisions of the trigeminal cranial nerve. The purpose of this study was to summarise our experience with the ocular clinical presentation, imaging, outcome, treatment of complications and to offer a new classification of patients with naevus of Ota. Methods We retrospectively reviewed the patients’ medical records and the following parameters were retrieved and analysed: demographics, clinical presentation complications and treatment of complications. Imaging characteristics of patients with naevus of Ota were compared with images from the same period of time of 57 age-matched and gender-matched patients without naevus of Ota (control group). Results The series was composed of 40 patients (18 males, 22 females) whose mean age at diagnosis was 35.27 years (range 0.5–77 years). Thirty-three patients (82.5%) were type I naevus of Ota according to the Tanino classification, three patients (7.5%) were type II, one patient (2.5%) was type III and three patient (7.5%) were type IV (bilateral naevus of Ota). We further classified all cases in according to the ocular involvement extent. Three patients developed malignant transformation to choroidal melanoma and four patients developed glaucoma. Conclusions In this study, a new clinical classification based on the involved ocular component and extent of the involvement (in quadrants) of the globe is suggested first. Further studies are needed to assess whether our clinical ocular classification can assist in identifying patients at risk for developing glaucoma and malignant transformation.

Behçet disease (BD) is a multisystemic disease that commonly involves the eyes. Although it affects patients in all age groups, data on ocular disease by age of onset are limited. This retrospective, multicenter study aimed to compare epidemiology, systemic and ocular manifestations, treatments and outcomes between three age groups: juvenile (<18 years), adult (18–39 years) and late (≥40 years) disease onset. The study included 175 ocular BD patients (303 eyes) from Israel and Palestine: juvenile-onset (n = 25, 14.3%), adult-onset (n = 120, 68.6%) and late-onset (n = 30, 17.1%). Most patients in all groups were male. Systemic manifestations were similar in all groups. Systemic co-morbidities were more common in late-onset patients. Bilateral panuveitis was the most common ocular manifestation in all patients. Non-occlusive retinal vasculitis, peripheral vessel occlusions, cataract and elevated intraocular pressure were found more commonly among juvenile-onset eyes. Anterior uveitis and macular ischemia were most common among late-onset eyes, while branch retinal vein occlusion was most common in adult and late-onset eyes. All patients were treated with corticosteroids. Methotrexate, immunomodulatory combinations and biologic treatments were more commonly used for juvenile-onset patients. All groups had a similar visual outcome. Our study showed that patients with ocular BD have varied ocular manifestations and require different treatments according to age of disease onset, but visual outcome is similar.