Lamellar Sclerectomy with Sclerostomy for Choroidal Hemangioma in Sturge-Weber Syndrome: A Case Report

Sturge-Weber syndrome (SWS) is a rare phakomatoses without any hereditary predisposition. The choroidal hemangioma and glaucoma are frequently present on the same side as facial nevus flammeus. We report a case of choroidal hemangioma with glaucoma not responding to medical therapy and managed with four quadrants lamellar sclerectomy with sclerostomy.

Some aspects of diagnosis and treatment of a сircumscribed form of choroidal hemangioma complicated by macular edema

Introduction. The interest are rare cases of a сircumscribed choroidal hemangioma located juxtapapillary (JP) on the nasal side of the optic nerve disc (OND) and complicated by macular edema. Purpose. Studying the possibility of timely diagnosis and evaluating the effectiveness of treatment of a сircumscribed choroidal hemangioma complicated by macular edema. Material and methods. A retrospective analysis of the diagnostics and treatment results of 5 patients (5 eyes) with JP сircumscribed choroidal hemangioma, localized on the nasal side of the optic disc, complicated by macular edema, was carried out. Results. As a result, in all patients, already 1 month after the session of selective laser transpupillary thermotherapy (TTT), an increase in visual acuity and quality was observed, which was accompanied by pronounced resorption of intraretinal cysts and / or subretinal fluid in the macula. Conclusion. The purposeful use of modern diagnostic methods and the use of a sparing method of laser TTT makes it possible to identify and cure patients with CPCH complicated by macular edema without loss of visual functions. Key words: сircumscribed choroidal hemangioma, selective transpupillary thermotherapy, cystic macular edema.

Pseudoretinoblastoma: Spectrum of Pathology and Frequency in Different Age Groups. Analysis of 14 years of Experience

Purpose. To evaluate the spectrum and the occurrence frequency of lesions simulating retinoblastoma in different age groups. Material and methods. A retrospective study of 608 patients (871 eyes) with suspected retinoblastoma was performed in the Ocular Oncology and Radiology Department of the Fyodorov Eye Microsurgery Federal State Institution in the period from 2007 to 2020. The mean patient age at presentation was 21 months (from 1 to 209 months). In 475 patients (78.1%) (715 eyes), the diagnosis of retinoblastoma was confirmed after a comprehensive assessment of the eye. 133 patients (156 eyes) (21.9%) had symptoms mimicking retinoblastoma-pseudoretinoblastomas. All patients with pseudoretinoblastomas were divided into different groups based on the simulating condition and age: from 0 to 1 year, from 1 to 2 years, from 2 to 5 years, older than 5 years. Results. There were 25 conditions simulating retinoblastoma. The most common conditions were Coats' disease (n=17; 12.8%), combined hamartoma of retina and retinal pigment epithelium (n=12. 9%), retinal detachment (n=11; 8.3%), vitreous opacities caused by intrauterine uveitis (n=11; 8.3%), choroidal hemangioma (n=11; 8.3%), vasoproliferative tumor (n=10; 7.5%), retinopathy of prematurity (n=7; 5.3%), morning glory syndrome (n=7; 5.3%). Most patients had unilateral lesions (n=116; 87.2%). Pseudoretinoblastomas differed based on age at presentation, because children 1 year of age or younger were most likely to have vitreous opacities caused by intrauterine uveitis (n=9; 21.4%) and retinopathy of prematurity (n=7; 16.7%); children aged from 1 to 2 years – Coats' disease (n=4; 20%) and choroidal hemangioma (n=3; 15%), from 2 to 5 years – Coats' disease (n=8; 21.6%), older than 5 years – Coats' disease (n=5; 14.7%) and vasoproliferative tumors (n=7; 20.6%). Conclusion. Timely referral of children with suspected retinoblastoma in specialized ocular oncology centers helps to avoid unnecessary treatment for retinoblastoma, enucleation and improve the vital prognosis.

Choroidal Hemangioma Associated with Hepatic Hemangioma: A Case Report and Literature Review

Circumscribed choroidal hemangioma (CCH) is a rare congenital ocular tumor type that is usually benign and asymptomatic. CCH has only been reported once previously in the literature in association with a visceral neoplasm. Here, we present a newly described association between CCH and hepatic hemangioma (HH). We report the case of a 57-year-old woman diagnosed with asymptomatic HHs who presented with a 2- to 3-month history of central scotoma and blurred vision in her left eye. Assessment identified an orange-red elevated lesion with a central serous retinal detachment and subretinal edema. Complementary tests suggested a CCH. To our knowledge, CCH has only been reported once previously in association with a visceral neoplasm, making this newly described association between CCH and HH of clinical relevance. Ophthalmologists should be aware of the possible association between CCH and other visceral tumors as this association offers an opportunity for the early detection of this pathology.