Retinal Macrocyst Research Articles

Chronic Pediatric Retinal Detachment with Multiple Macrocysts.

To describe a case of chronic pediatric retinal detachment with multiple macrocysts, its surgical management, and a review of the literature. Case report with fundus photography and optical coherence tomography. We describe a case of an asymptomatic, 11-year-old male with a chronic rhegmatogenous retinal detachment with multiple peripheral macrocysts. The patient had count fingers visual acuity upon presentation. The detachment was successfully surgically repaired with scleral buckling, subretinal fluid drainage, cryotherapy, with a SF6 tamponade. At the 12-month follow-up, the retina remained attached with improvement of visual acuity to 20/100 with resolution of the cysts. Optical coherence tomography revealed loss of macular ellipsoid zone. Genetic testing revealed a heterozygous dominant COL11A1 mutation. To the authors' knowledge, this is the first reported case of chronic retinal detachment presenting with multiple peripheral macrocysts in a pediatric patient with Stickler's Syndrome. More research is needed into the cause and significance of retinal macrocysts, particularly in the pediatric population.

Unusual anterior and posterior segment features of coats disease.

To report the clinical course and management of unusual anterior and posterior segment features of Coats disease and their relation to the age of the patients to increase the awareness towards these rare clinical features rarely described in the current literature. A retrospective descriptive review of 45 eyes of 45 patients affected by Coats disease was conducted at the Retinoblastoma Referral Center and Ophthalmology Unit of the University of Siena in Italy analyzing data from 2000 to 2022. Medical records and images were revised to find some cases presenting unusual anterior and posterior segment features in patients affected by Coats disease.We identified therefore 4 unusual clinical conditions: retinal macrocysts, anterior chamber cholesterolosis, fovea-sparing Coats disease and secondary vasoproliferative tumor. Two patients presented with retinal macrocyst (2/45 = 4.4%), one with anterior chamber cholesterolosis (1/45 = 2.2%), two with fovea sparing Coats disease (2/45 = 4.4%) and one with vasoproliferative tumor associated (1/45 = 2.2%) for a total of six (6/45 = 13.3%) patients manifesting unusual anterior or posterior segment features in Coats disease. Unusual anterior and posterior segment features of Coats disease such as retinal macrocyst and anterior chamber cholesterolosis have been more frequently reported in younger children while fovea-sparing and vasoproliferative tumors have been more commonly described in older patients. Age is then a strong prognostic marker which allows to distinguish two different phenotypes of Coats disease: patients younger and older than 3 years old with more aggressive and milder phenotype respectively.

Ultrasound imaging of extensive peripheral macrocysts in chronic total combined rhegmatogenous-traction retinal detachment

PurposeTo report a case of a 42-year-old male with proliferative diabetic retinopathy (PDR) complicated by tractional retinal detachments (TRD) bilaterally with a rare finding on B-scan ultrasonography. ObservationsOn B-scan ultrasound, a total combined rhegmatogenous-TRD was observed, accompanied by extensive retinal macrocysts that appeared nearly confluent for 360°. Conclusions and ImportanceRetinal macrocysts are rare but important clinical entities that help drive management, as their presence typically suggests a chronic retinal detachment.

Hemorrhagic Retinal Macrocyst with Retinal Detachment

Purpose: To report a satisfactory clinical outcome of hemorrhagic retinal macrocyst with retinal detachment after pars plana vitrectomy and silicone oil injection. Case summary: A 33-year-old man visited our clinic with a 1-week history of visual disturbance in his left eye. His ocular and medical history were unremarkable, and best-corrected visual acuity (BCVA) in the left eye was 20/40. Fundoscopic examination of the left eye showed a hemorrhagic retinal macrocyst that was well-demarcated, semi-transparent, dome-shaped, and larger than 8 disc diameters at the superonasal location, with macular-off retinal detachment. B-scan ultrasonography also confirmed the presence of an intra-retinal cystic lesion with internal mobile echogenic signals representing blood. The patient underwent 23-gauge pars plana vitrectomy, barrier laser photocoagulation around the retinal tear and boundary of the cyst, and silicone oil injection, without any other additional procedures for the hemorrhagic retinal macrocyst. After the surgery, the patient stayed in the face down position for two weeks. Three months postoperatively, a completely collapsed hemorrhagic retinal macrocyst with successful retinal reattachment was observed. Six months later, the BCVA in the left eye had improved to 20/30. One year later, even after silicon oil removal, the collapsed hemorrhagic retinal macrocyst and reattached retina remained stable.Conclusions: A hemorrhagic retinal macrocyst with retinal detachment was successfully treated with pars plana vitrectomy and silicone oil tamponade.

Resolution of exudative retinal detachment and regression of retinal macrocyst post-laser in Coats disease

A 15-year-old boy presented with painless progressive blurring of vision in the right eye for 1 year in duration. His visual acuity in the right eye was hand movement. The right fundus showed presence of extensive subretinal exudates at the posterior pole and a retinal macrocyst at the temporal periphery. It was associated with exudative retinal detachment at the inferior periphery of the retina. Fundus angiography revealed telangiectatic retinal vessels at the superotemporal retina. Based on clinical and angiographic findings, a diagnosis of Coats disease was made. He was treated with retinal laser photocoagulation. There was resolution of the exudative retinal detachment, reduction of subretinal exudates, and regression of the retinal macrocyst with improvement of visual acuity to 1/60 post-laser therapy.

Hemorrhagic retinal macrocysts, simulating choroidal melanoma: a case report

IntroductionHemorrhagic retinal macrocysts are extremely rare retinal lesions that can be mistaken for malignancy with subsequent enucleation. Such a case was diagnosed, by a retina specialist based on ultrasonography, as a choroidal melanoma with exudative retinal detachment and the patient was advised to have brachytherapy.Case presentationA 15-year-old Caucasian boy suffered sudden visual loss in the left eye and exam revealed vitreous hemorrhage. Magnetic resonance imaging revealed the mass as hyperintense on T1-weighted images and isointense on T2-weighted images with no enhancement after gadolinium dye. Following scleral buckle, the hemorrhagic retinal macrocyst collapsed gradually over a period of 5 weeks. The patient recovered visual acuity of 6/7.5 at the 1-year follow up.ConclusionA hemorrhagic retinal macrocyst can be erroneously diagnosed as choroidal melanoma. Hints for the presence of retinal macrocysts include: egg shape; cyst wall configuration; no attachment to the choroid; and presence of retinal detachment.

Picture of the Month—Quiz Case

A 31-MONTH-OLD HISPANIC BOY PRESENTED for evaluation of possible retinoblastoma in the right eye. At 22 months of age, strabismus had developed. There was no history of trauma. Three months later, examination showed total retinal detachment with the retina visible immediately posterior to the lens (Figure, A and B). Visual acuity was reduced to light perception in the right eye. With the left eye, he could fix and follow an object normally. The anterior segment of the right eye was unremarkable except for xanthocoria (yellow pupil) from an exudative retinal detachment. Examination of the left eye was unremarkable. He was treated with anterior chamber injections of bevacizumab on a monthly basis for 5 months to resolve the retinal detachment. Following the injections, visual acuity was unchanged. Intraocular pressures were normal in each eye (13 mm Hg). No conjunctival hyperemia or corneal opacity was seen. The anterior segment of the right eye was filled with refractile yellow crystals (Figure, C and D). There was no view of the fundus but B-scan ultrasonography revealed total retinal detachment with a retinal macrocyst. There was no intraocular tumor or calcification.

Hemorrhagic intraretinal macrocyst: Differential diagnoses and report of an unusual case

Retinal ‘cysts’ may be single or multiple, ranging from two-to-ten disc diameters in size, and occur in eyes with longstanding retinal detachment. The authors describe a retinal macrocyst larger than ten disc diameters, with a blood-filled cavity, and its ultrasound findings. Improved retinal nourishment following retinal reattachment gradually reverses the process responsible for cystic degeneration, with the eventual collapse of the cyst (within days or weeks). Surprisingly, this giant cyst did not collapse for almost three years despite retinal reattachment. The internal mobile echogenic contents were suggestive of blood. The possible reason of blood in the cyst could be rupture of the retinal blood vessels in the cyst cavity. This could be a recurrent phenomenon, which did not allow the cyst to collapse. The Hemorrhagic Intraretinal Macrocyst needs to be differentiated from mimicking clinical conditions, namely, retinoschisis, choroidal melanoma, subretinal abscess, choroidal hemangioma, and the like. It could take up to a few years to collapse spontaneously, following successful retinal reattachment.

A Case of Coats' Disease Accompanying A Retinal Macrocyst

Purpose: To report a case of laser photocoagulation treatment for the patient of Coats' disease accompanying a retinal macrocyst. Case summary: A three-year-old boy visited the hospital whose chief complaint was visual acuity decrease of his left eye. Fundus examination showed macular scar, foveal hard exudates and inferior retinal cystic lesion in his left eye. Two months later, examination under anesthesia (EUA) and fluorescein angiography (FAG) was performed. The results revealed inferior retinal macrocyst, nasal avascular retina and telangiectasia around the retinal macrocyst. Laser photocoagulation was performed around the retinal macrocyst and at the nasal avascular retina. One year after the laser photocoagulation, retinal macrocyst did not further progress and the retina was stabilized. Conclusions: Laser photocoagulation was done around the retinal macrocyst and at the nasal avascular retina of the Coats' disease accompanying a retinal macrocyst and the lesions did not further progress and the retina was stabilized.

Internal Drainage of a Retinal Macrocyst With an Nd:YAG Laser to Aid Primary Retinal Reattachment

Retinal macrocysts are an occasional finding in patients with long-standing retinal detachments. The cysts usually do not require any specific treatment during procedures to repair retinal detachment and resolve spontaneously after retinal reattachment. However, if the cyst is adjacent to a retinal break and prevents its closure, the retinal reattachment procedure can fail. We describe a technique using the Nd:YAG laser to perforate the retinal cyst postoperatively, which allows for subsequent break closure and retinal reattachment.

Chronic Retinal Detachment with Secondary Retinal Macrocyst and Peripheral Neovascularization

Purpose: To report the association of a retinal macrocyst with peripheral retinal neovascularization that occurred secondary to a chronic, subclinical rhegmatogenous retinal detachment and to illustrate the utility of wide-field fundus photography. Methods: Case Report Results: A 37 year-old male was diagnosed with chronic retinal detachment on routine eye examination. Further evaluation revealed intraretinal hemorrhages and a retinal macrocyst. Diagnosis was confirmed with wide-field fundus photography and fluorescein angiography. Surgery was recommended to repair the retinal detachment, to allow spontaneous resolution of the macrocyst. Conclusion: Chronic, subclinical retinal detachments may rarely be associated with retinal macrocysts and retinal neovascularization. This altered retinal morphology can be delineated on wide-field fundus imaging, which aids in diagnosis and management.

Laser Treatment of Coats' Disease

A 9-year-old boy presented with an exudative retinal detachment, vascular telangiectasias, subretinal lipid, and retinal macrocyst formation. He underwent three sessions of large-spot diode laser for the treatment of Coats' disease. Serial examinations and fundus photography documented an excellent involutional response with an improvement in visual acuity.

Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture

PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines. METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease. RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass. CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.

Classification and management of Coats disease: the 2000 Proctor Lecture

PURPOSE: To review the methods and results of management in a large series of patients with Coats disease, to determine risk factors for poor visual outcome and enucleation, and to propose a practical classification of Coats disease. METHODS: In a retrospective consecutive series in 150 patients, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with management, including observation, laser photocoagulation, cryotherapy, and various techniques of retinal detachment surgery and enucleation. The anatomic outcome, complications of treatment, visual results, and reasons for enucleation were tabulated. Factors predictive of poor visual outcome (20/200 or worse) and enucleation were determined using Cox proportional hazards regression models. Based on these observations, a staging classification of Coats disease, applicable to treatment selection and ocular prognosis, is proposed. RESULTS: In 117 patients (124 eyes) with a mean follow up of 55 months (range, 6 months to 25 years) primary management was observation in 22 eyes (18%), cryotherapy in 52 (42%), laser photocoagulation in 16 (13%), various methods of retinal detachment surgery in 20 (17%), and enucleation in 14 (11%). Anatomic improvement or stability was achieved in 76% of eyes, and final visual acuity was 20/50 or better in 17 eyes (14%), 20/60 to 20/100 in eight (6%), 20/200 to finger counting in 30 (24%), and hand motion to no light perception in 49 (40%) Enucleation was ultimately necessary in 20 eyes (16%). Risk factors predictive of poor visual outcome (20/200 or worse) included postequatorial ( P = .01), diffuse ( P = .01), or superior ( P = .04) location of the telangiectasias and exudation, failed resolution of subretinal fluid after treatment ( P = .02), and presence of retinal macrocysts ( P = .02). The main risk factors for enucleation were elevated intraocular pressure (greater than 22 mm Hg; P less than or equal to .001) and iris neovascularization ( P less than or equal to .001). Coats disease was classified into stage 1, telangiectasia only; stage 2, telangiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation) stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage disease. Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coats disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7% of stage 3, 78% of stage 4, and 0% of stage 5 disease. CONCLUSIONS: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes. However, poor visual outcome of 20/200 or worse commonly results. Patients who present with stages 1 to 3 Coats disease have the best visual prognosis, and patients with stages 4 and 5 have a poor visual prognosis.

HEMORRHAGIC RETINAL MACROCYSTS IN ADVANCED COATS DISEASE

The authors report a case of advanced unilateral Coats disease with associated hemorrhagic retinal macrocysts. Fluorescein angiography showed the macrocysts to be intensely hypofluorescent due to absence of perfused retinal blood vessels in the inner wall and presence of intracavitary blood blocking outer wall and choroidal fluorescence. Around the cystic lesions and in other areas of the peripheral retina, characteristic telangiectatic retinal blood vessels were evident. The mechanism responsible for the development of hemorrhagic retinal macrocysts in Coats disease appears to be coalescence of microcystic spaces in edematous and degenerated chronically detached retina.

Experimental retinal vascular occlusion. III. An ultrastructural study of simultaneous occlusion of central retinal vein and artery.

The retinal changes following the simultaneous occlusion of retinal vessels were examined at an ultrastructural level. The preservation of endothelial cells that prevented leakage of blood and horseradish peroxidase tracer material in the surrounding retinal tissues explained the lack of hemorrhages observed clinically. Mueller cell death resulted in severe structural disorganization of the retina, with disruption of the inner limiting membrane, involvement of the outer retinal layers, and fleurette formation. The retinal macrocysts were derived from ischemic infarction.