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Retinal Tears and Detachments
Published in Amy-lee Shirodkar, Gwyn Samuel Williams, Bushra Thajudeen, Practical Emergency Ophthalmology Handbook, 2019
Non rhegmatogenous retinal detachments: This can be due to a range of diseases described below: Inflammatory disease: Such as Vogt–Koyanagi–Harada (VKH) syndrome, posterior scleritis and other chronic inflammatory processes. This traditionally causes multiple serous detachments.Neoplasia: Choroidal melanoma, metastasis, choroidal haemangioma, multiple myeloma, capillary retinal haemangioma can all present with exudative retinal detachments.Congenital abnormalities: Optic pits, morning-glory syndrome and choroidal coloboma may also present with detachment of the neuro-sensory retina.Vascular: Coats’ disease and malignant hypertension both have exudative retinal detachments.Ocular hypotension: Glaucoma drainage surgery may result in choroidal detachments which can mimic retinal detachments. If the choroidal detachments are not touching they can often resolve once the intraocular pressure has been re-established.
Surgical removal of submacular exudates in Coats’ disease
Published in A Peyman MD Gholam, A Meffert MD Stephen, D Conway MD FACS Mandi, Chiasson Trisha, Vitreoretinal Surgical Techniques, 2019
DellaCroce John T, Ebrahim Shehab A, Peyman Gholam A
The conventional treatment of Coats’ disease includes laser or cryotherapy of peripheral telangiectasis, if possible before and to prevent the onset of retinal detachments. Macular exudates can regress after laser therapy; however, large dense submacular exudates are unlikely to be affected by laser treatment.1 The benefit of direct removal of subretinal exudates in refractory cases, especially those with active proliferative vascular pathology, may warrant a surgical approach. Surgical removal of subretinal exudates associated with Coats’ disease was first performed and described by Peyman et al.2 A similar procedure has been used in the past for removal of hard exudates in patients with diabetic maculopathy.3
A patient with X-linked retinoschisis and exudative retinal detachment associated with a pathogenic hemizygous variant c.304c>T in RS1
Published in Ophthalmic Genetics, 2022
Nathália Nishiyama Tondelli, Beatriz Mello Mencaroni, Carolina Maria Barbosa Lemos, Jefferson Rocha de Sousa, Gabriel Castilho Sandoval Barbosa, André Marcelo Vieira Gomes, Mariana Matioli da Palma
Coats disease is a nonhereditary, sporadic, unilateral condition that affects males more than females and is characterized by vascular telangiectasia, exudation, and exudative retinal detachment (31). The estimated incidence is 0.09 per 100.000 individuals (32). It was originally described by George Coats in 1908. Recently, Shields and colleagues have described a staging classification of Coats disease. Treatment may include observation, photocoagulation, cryotherapy, drainage of subretinal fluid, and scleral buckling, to enucleation in cases of painful rubeotic glaucoma (33). Stage 3 disease or worse was common in the youngest patients and less common in the older ones. Extensive retinal detachment was found mainly in children under the age of three (31,32). Our patient was diagnosed with exudative retinal detachment in the second decade of life reinforcing that he probably has Coats-like disease associated with retinoschisis, rather than two rare retinal dystrophies, Coats disease, and XLRS.
Retinal arteriolar macroaneurysms with supravalvular pulmonic stenosis in the United Arab Emirates
Published in Ophthalmic Genetics, 2022
Arif O. Khan, Francesco Pichi, Piergiorgio Neri, Emad B. Abboud
The ophthalmic phenotype of RAMSVPS is non-inflammatory and distinct. Bilateral retinal arteriolar beading and macroaneurysms along the major trunks seem to develop in childhood whether or not the patient is symptomatic as a child. Retinal arteriolar leakage and bleeding lead to visual loss, including potential submacular scarring. Peripheral capillary remodeling and localized Coats-like dilations are possible. Leaking aneurysms respond to photocoagulation or can spontaneously involute. Because of the potential for rebleeds, regular follow-up every three months is recommended (3). However, because of its rarity, RAMSVPS is often mistaken as other pediatric retinal vascular disorders. The most common misdiagnosis is Coats disease (1–3). However, Coats disease is nonhereditary, almost always unilateral, almost always in boys, and not associated with hemorrhage, retinal arteriolar beading, or retinal arteriolar macroaneurysms (4). Characteristic features of Coats disease include dilated, irregular caliber, small- to medium-sized retinal blood vessels (retinal telangiectasia) with bulbous terminal configurations, associated exudative retinopathy, and potential retinal ischemia.
Ophthalmological Aspects of von-Hippel–Lindau Syndrome
Published in Seminars in Ophthalmology, 2021
Hashim Ali Khan, Muhammad Aamir Shahzad, Fatima Iqbal, Muhammad Amer Awan, Qaim Ali Khan, Ali Osman Saatci, Ahmed Abbass, Fazil Hussain, Syed Arif Hussain, Atif Ali, Wajahat Ali
The differentials of RCH include vasoproliferative tumor (VPT), coats disease, retinal arterial macroaneurysm (RAMA), and other retinal vascular tumors. Coats disease is an idiopathic and unilateral retinal vascular disease that mostly affects younger males.5,46 Lack of systemic association, female gender, prominent areas of retinal telangiectasias, and absence of a defined retinal tumor can help in differentiating retinal exudation between the two conditions. A RAMA is a small retinal vascular lesion usually associated with vitreous hemorrhage and subretinal and intraretinal hemorrhages in elderly patients. RAMA is limited to the posterior pole, does not have a pair of feeding vasculature, and is located on a retinal arteriole.47 VPTs lack a prominent feeder vessel, mostly located in the extreme inferior periphery of the retina, and the absence of systemic features helps to differentiate them from RCH (Table 3).5,34,46,48,49