Retinal Arterial Macroaneurysm with Supravalvular Pulmonic Stenosis – Retinal Disorders

The retina is a layer of tissue in the back of your eye that senses light and sends images to your brain. In the center of this nerve tissue is the macula. It provides the sharp, central vision needed for reading, driving and seeing fine detail.

Retinal disorders affect this vital tissue. They can affect your vision, and some can be serious enough to cause blindness. Examples are

• Macular degeneration – a disease that destroys your sharp, central vision
• Diabetic eye disease
• Retinal detachment – a medical emergency, when the retina is pulled away from the back of the eye
• Retinoblastoma – cancer of the retina. It is most common in young children.
• Macular pucker – scar tissue on the macula
• Macular hole – a small break in the macula that usually happens to people over 60
• Floaters – cobwebs or specks in your field of vision

NIH: National Eye Institute

• Amaurosis fugax (Medical Encyclopedia)
• Central serous choroidopathy (Medical Encyclopedia)
• Electroretinography (Medical Encyclopedia)
• Fluorescein angiography (Medical Encyclopedia)
• High blood pressure and eye disease (Medical Encyclopedia)
• Home vision tests (Medical Encyclopedia)
• Intravitreal injection (Medical Encyclopedia)
• Retinal artery occlusion (Medical Encyclopedia)
• Retinal vein occlusion (Medical Encyclopedia)

Retinal arterial macroaneurysm with supravalvular pulmonic stenosis (RAMSVPS) is a disorder that affects blood vessels in the eyes and heart. The condition generally becomes apparent in infancy or childhood.RAMSVPS damages the arteries in the light-sensitive tissue at the back of the eye (the retina). These arteries gradually develop multiple small bulges called beading. Eventually, larger bulges in the blood vessel walls (macroaneurysms) occur. These macroaneurysms can tear (rupture), leading to bleeding that can spread into other areas of the eye and cause vision loss.People with RAMSVPS also have a heart condition called supravalvular pulmonic stenosis. Pulmonic stenosis is a narrowing that affects the pulmonic valve between the heart and the lungs. The term “supravalvular” means that the narrowing occurs just above the valve, in a blood vessel called the pulmonary artery. Supravalvular pulmonic stenosis impairs blood flow into the lungs, where blood normally picks up oxygen for distribution to cells and tissues throughout the body. As a result, less oxygen is carried through the bloodstream, leading to signs and symptoms that include shortness of breath; a rapid heartbeat; fatigue; and swelling in the face, feet, or abdomen.