OBJECTIVE: To examine the clinical characteristics, diagnosis and treatment, and prognosis of ocular amyloidosis in a Chinese population.
METHODS: A retrospective case series study was conducted. The clinical data of 37 patients with ocular amyloidosis were collected and the clinical characteristics, diagnosis and treatment, and prognosis were summarized and analyzed.
RESULTS: The 37 patients included 12 males and 25 females ranging in age from 22 to 75 years, with median age of 49 years. The clinical signs and symptoms included a conjunctival mass in 37 patients (100%), periorbital discomfort or pain in 29 patients (61.9%), ptosis in 18 patients (23.8%), exophthalmos or eyeball displacement in 3 patients (14.3%), restricted eye movement in 2 patients (9.52%), vision loss in 1 patient (4.76%), and diplopia in 1 patient (4.76%). A total of 29 patients had only conjunctival involvement and 8 patients had concomitant orbital and conjunctival involvement. The main treatment for patients with conjunctival involvement was surgical resection. Thirty-one patients had stable disease, 4 patients progressed or relapsed, and 2 patients were lost to follow-up.
CONCLUSIONS: Ocular amyloidosis most commonly presents as an eyelid or conjunctival mass or diffuse thickening and can also present as an orbital mass. Diagnosis is mainly dependent on histopathological examination. Surgery is the main treatment and is done to confirm the diagnosis to guide further treatment, preserve function, and prevent complications that threaten visual acuity. Close postoperative follow-up is necessary.

OBJECTIVE: This study investigated the safety and efficacy of sclerotherapy with intralesional bleomycin injection (IBI) for retrobulbar orbital low-flow vascular lesions under multi-slice computed tomography (CT) guidance.
METHODS: Between January 2010 and September 2021, consecutive patients with retrobulbar orbital low-flow vascular lesions who underwent CT-guided IBI at a tertiary centre in Taiwan were enrolled. Their medical records and imaging data were retrospectively collected.
RESULTS: This study enrolled 13 patients (7 male and 6 female patients; age range: 1-57 years; mean age: 25.9 years) with lymphatic malformation (LM, n = 4), venolymphatic malformation (n = 1), and venous malformation (VM, n = 8). The overall radiological response rate was 76.9% (10 of 13); the radiological response rate was 75.0% in the VM group (6 of 8) and 75.0% in the LM group (3 of 4). Moreover, 3 patients (23.1%) had minor complications and 1 (7.7%) had a major complication. The mean clinical and radiological follow-up was 8.3 months and no recurrence or progression was reported.
CONCLUSIONS: CT-guided IBI is an effective and relatively safe minimally invasive treatment for retrobulbar orbital low-flow vascular lesions, with an overall radiological response rate of 76.9% in a mean of 1.5 sessions and a low complication rate.
CONCLUSIONS: CT-guided sclerotherapy with IBI is a relatively safe, effective, and feasible alternative treatment option for retrobulbar orbital low-flow vascular lesions.

Pseudolymphoma (PSL) of the orbit is a benign lymphoid hyperplasia (LH). It is a rare disease with an extensive range of known causative agents. LH is further classified into \"reactive\" (RLH) and \"atypical\" (ALH) types. It clinically presents as a single or a few plaques and/or nodular lesions, particularly on the head, neck, and upper trunk. It must be differentiated from orbital malignant lymphoma. In this report, we present a case of a 58-year-old Pakistani female with an asymptomatic recurrent right periorbital swelling for three years. It was clinically diagnosed as an angiotensin-converting enzyme (ACE) inhibitor-induced angioedema as it responded to stopping the ACE inhibitor; however, after four months, the patient again started to develop right periorbital swelling. An incisional biopsy revealed perivascular and periadnexal infiltration of lymphocytes, plasma cells, and a few neutrophils along with pigmentary incontinence. The formation of multiple lymphoid follicles and infiltration by monomorphic lymphoid cells in deeper skeletal muscle fibers were also observed. Immunohistochemistry (IHC) showed polyclonality and low Ki-67 labeling (20%), corresponding to periorbital RLH. Our objective in this study is to highlight the importance of considering PSL as a differential diagnosis in periorbital swelling. We also suggest that recurrent angioedema may lead to PSL.

UNASSIGNED: Localized amyloidosis can affect numerous tissues throughout the body and can also affect a variety of peri-ocular tissues including the conjunctiva, extra-ocular muscles, peri-orbital soft tissue, and lacrimal gland. We report two cases of amyloidosis presenting with eyelid involvement.
UNASSIGNED: The first case represented a more subtle presentation of skin thickening with a pre-septal cellulitis, while the second case had a dramatic presentation of edema evolving into tissue dehiscence and spontaneous hemorrhage with ongoing angioedema and systemic coagulopathy.
UNASSIGNED: The two cases of biopsy-proven orbital/peri-ocular amyloidosis demonstrate the different clinical presentations that may go from the subtle to dramatic, depending on which peri-ocular tissues are affected and to what degree. Standards for treatment of amyloidosis remain conservative initially with surgery or radiation recommended only for refractory cases, but additional therapies are under investigation. Clinicians should have high clinical suspicion for amyloidosis with findings such as skin thickening or significant periorbital edema and should always consider tissue biopsy and further workup for amyloidosis if the findings worsen or do not resolve with treatment of more common conditions such as cellulitis.

BACKGROUND: Retinoblastoma (RB) is one of the most curable childhood cancers if early detected and treated. Late presentation complicates the management of RB results in dismal treatment outcome. Objective: The aim of this study is to report the clinical presentation pattern of retinoblastoma patients seen at Jimma University Medical center (JUMC).
METHODS: The study was a retrospective review of retinoblastoma cases managed at JUMC between August 2016 and July 2020.
RESULTS: Among pediatric oncology cases registered retinoblastoma, accounting 8.5 % (36/423) of all childhood cancer patients in the study period, 29 (90.6%) of them had unilateral retinoblastoma and 3(9.4%) of them had bilateral retinoblastoma. The average age at presentation for bilateral and unilateral retinoblastoma patients was 17 (range 3-30) months and 37.5 months (range 8-84) respectively. The first symptom observed by the family was leukocoria in 21 (65.6%) of the patients but 24(75%) of the patients presented with advanced stage (proptosis and fungating orbital mass) of the disease. The longest and the shortest lag time of presentation from the first symptom was 17 months and 2 weeks respectively with the mean lag time of 1.4 months in bilateral and 6 months in unilateral cases. Clinically, the majority of the eyes 24/35(68.6%) were extraocular tumors involving orbital tissues at presentation.
CONCLUSIONS: Most of retinoblastoma patients presented at advanced stage of the disease and presented very late after the family observed the disease. Early detection strategies must be designed by the government and responsible stakeholders in mitigating the effects of late presentation.

Smoking is a well-established risk factor for several eye disorders including cataracts and age-related macular degeneration. While many individuals are informed of the various adverse health effects, there is limited research into patients\' awareness of the relationship between smoking and eye disease and the potential impact this might have on reducing smoking behaviour. Our findings document the low level of awareness of the risk of blindness from smoking at a tertiary eye unit in the United Kingdom and highlight the need for increased involvement from eye care professionals, alongside health campaigns to educate the public of this consequence of smoking.

Ocular adnexal extranodal marginal zone lymphoma (OA-EMZL) and immunoglobulin G4-related ophthalmic disease (IgG4-ROD) may exist on a continuum. Presence of immunoglobulin light-chain restriction and clonal gene rearrangement suggests presence of lymphoma; whereas bilateral, infraorbital nerve and systemic involvement accompanied by elevated serum IgG4 levels may indicate synchronous IgG4-ROD. Although steroids have been the mainstay for the treatment of IgG4-ROD, radiotherapy (RT) has been used occasionally. The reported RT doses range between 24 and 30 Gy, which can result in acute and late toxicities. A low-dose regimen of four Gy has not been previously described. We describe a patient with bilateral OA-EMZL arising from IgG4-ROD successfully treated with low dose \'boom-boom\' radiotherapy. In addition, we review the literature for the association between these two conditions and the role of RT in their management.

OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory disease that can affect almost any organ system. We aimed to present our single-center experience of pediatric patients with IgG4-RD, a rare disease in children.
METHODS: Pediatric patients diagnosed with IgG4-RD at the Hacettepe University between June 2014 and September 2020 were evaluated retrospectively. Patients with definite, probable, or possible diagnosis of IgG4-RD were included.
RESULTS: A total of eight patients with a median age of 13.4 (IQR 9.5-15.0) years were included in the study. Clinical presentations were IgG4-related ophthalmic disease in six patients, IgG4-related lymphadenopathy in one patient, and IgG4-related sialadenitis and lymphadenopathy of several lymph nodes accompanied by pancreatitis, ulcerative colitis, and pulmonary manifestations in one patient. Elevated serum IgG4 was detected in three of eight patients (37.5%). The main histopathological feature was fibrosis and lymphoplasmacytic infiltrates. Corticosteroids were used as first-line treatment in almost all patients with or without steroid-sparing agents. Azathioprine, methotrexate and rituximab were used as steroid-sparing agents. Relapse occurred in two of seven patients. Radiotherapy was used as the last resort in one patient with severe orbital disease.
CONCLUSIONS: IgG4 RD mainly presents with orbital manifestations in pediatric population but has wide phenotypic clinical variability. Although rare, early recognition and treatment are essential for a better outcome in these patients.

UNASSIGNED: Alemtuzumab is a humanized monoclonal antibody used as a treatment of multiple sclerosis (MS) and chronic lymphocytic leukemia. It decreases T cell count leading to significant immunosuppression, with increased risk of systemic and ocular infections. Herein, we report a unique case of bilateral acute retinal necrosis (ARN) caused by varicella-zoster virus (VZV) in a patient affected by MS under treatment with alemtuzumab.
UNASSIGNED: A 36-year-old man with a relapsing-remitting MS under treatment with alemtuzumab developed bilateral visual loss. Anterior segment examination displayed granulomatous keratic precipitates and 3+ cells in the anterior chamber, while fundoscopy showed bilateral 1+ vitritis and peripheral retinal necrosis, complicated by retinal detachment in the left eye. The high viral load for VZV in aqueous humor samples had a univocal interpretation for viral reactivation. In addition to systemic therapy with acyclovir, the patient was treated with bilateral intravitreal injections of foscarnet and underwent pars-plana vitrectomy and silicone oil tamponade for retinal detachment in the left eye.
UNASSIGNED: This report shows a unique case of bilateral ARN caused by VZV associated with alemtuzumab. Any visual loss in MS patients under biologic therapy should not be underestimated, performing an accurate differential diagnosis with optic neuritis.

A 5-year-old Doberman Pinscher dog was presented with sudden onset left-sided periocular bleeding following third eyelid gland replacement surgery. Left-sided exophthalmos and 360-degrees subconjunctival hemorrhage were present. The ophthalmic examination revealed blindness with absent direct and consensual pupillary light reflexes. A superficial exposure ulcerative keratitis due to exophthalmos was also present. Computed tomography (CT) revealed a large left-sided retrobulbar mass lesion surrounding the optic nerve, compatible with a retrobulbar hematoma. Due to absence of orbital fractures and no history of trauma, a coagulopathy was strongly suspected. The buccal mucosal bleeding time (BMBT) was prolonged (>4 minutes) consistent with a primary hemostatic defect in the absence of thrombocytopenia. Von Willebrand factor antigens levels were decreased (24%- Normal Range: 50%-150%). Surgical drainage of the retrobulbar hematoma was performed uneventfully thirty minutes after subcutaneous injection of desmopressine acetate, Minirin® (1 µg/kg, SC). Von Willebrand disease type I gene mutation was confirmed by PCR amplification of the DNA encoding von Willebrand factor. This case report demonstrates that hemostatic disorders, including von Willebrand Disease (vWD), are a risk factor for orbital bleeding following surgical trauma. Retrobulbar hematoma should be considered as a differential diagnosis in any dog with exophthalmos.