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An 8-month-old girl presented with vitreous hemorrhage, peripheral retinal arteriovenous anastomosis, and hypoplasia of the thumb. Magnetic resonance imaging of the brain showed multiple hyperintensities, cysts, and calcifications in the white matter. DNA testing showed a mutation in one allele of the CTC gene, supporting the diagnosis of cerebroretinal microangiopathy with calcifications and cysts (CRMCC), or Coats plus disease, despite which designation this condition can present without subretinal exudate. In infants with peripheral retinal vascular disease, neuroimaging can identify the characteristic abnormalities of CRMCC., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe a patient with retinal complications of essential thrombocytosis., Method: Case report. A 24-year-old man with acute vitreous hemorrhage of the right eye underwent ocular and systemic examinations to elucidate the origin of this condition., Results: Fluorescein angiography of the right eye showed an avascular peripheral retina with marked capillary nonperfusion, arteriovenous anastomosis, and sea fan neovascularization. Blood studies showed thrombocytosis without other associated systemic diseases. The peripheral retinal capillary nonperfusion and sea fan neovascularization were treated with dye-laser photocoagulation, and aspirin, 80 mg daily, was given for systemic treatment., Conclusions: In our patient, the avascular retina was associated with essential thrombocytosis, which may cause an avascular peripheral retina with neovascularization and vitreous hemorrhage in otherwise healthy persons.

Objective: The purpose of the study was to demonstrate that the arteriovenous shunt at the arteriovenous crossing could occur in major retinal vascular diseases other than Takayasu disease., Design: Clinical review of consecutive case series., Participants: The authors studied 1885 eyes with retinal vascular diseases such as diabetic retinopathy, branch retinal vein occlusion, central retinal vein occlusion, central retinal artery occlusion, Leber's miliary aneurysms, Eales disease, Behçet disease, and systemic lupus erythematosus retinopathy., Intervention: Fluorescein fundus angiography using a wide-field fundus camera (60 degrees) was performed., Main Outcome Measures: Dye transit from artery to vein through sequential angiography., Results: The arteriovenous shunt at the arteriovenous crossing was found in 8 eyes with proliferative diabetic retinopathy, 27 eyes with branch retinal vein occlusion in the chronic stage, 2 eyes with central retinal vein occlusion, 2 eyes with central retinal artery occlusion, and 2 eyes with miliary aneurysms. The arteriovenous shunt was formed by a direct inflow from artery to vein, showing vasodilation and hyperpermeability, followed by obliteration peripheral to the shunts. The pattern of initial inflow was classified into axial flow and laminar flow, and the inflow became wider and more rapid in the advanced stage., Conclusions: These findings indicate that the arteriovenous shunt at the arteriovenous crossing is not a unique phenomenon in Takayasu disease but rather is a basic pattern of retinal vascular reaction pathologic states.

Microvascular silicone injection, tissue clearing, and histologic examination were used to demonstrate the origin, distribution, and interconnections of newly formed iris and chamber angle blood vessels in four eyes with rubeosis iridis and neovascular glaucoma associated with diabetic retinopathy and central retinal vessel occlusion. The newly formed iris vessels that formed either a tight, evenly distributed (diabetic) or loose, irregularly distributed (central vessel occlusion) network in the iris originated from the normal iris arteries that were branches of either the major arterial circle or of the perforating branches of the anterior ciliary arteries, and drained into the normal iris and ciliary body veins and occasionally into the paralimbal episcleral veins. These newly formed iris vessels appeared to shunt intravascular fluid from arteries to veins. The newly formed anterior chamber angle vessels that formed tufts and arcades at the trabecular meshwork also originated from the roots of the iris arteries and the ciliary body arteries and connected with the peripheral neovascular iris network. In addition, the circumferentially running angle vessels that coursed within the trabecular meshwork branched into and coursed within a fibrosed Schlemm's canal and into two of its intrascleral collector channels. No open communication between these newly formed vessels and the Schlemm's canal-aqueous outflow system was seen.