What is retinal hamartoma?
Background: Retinal astrocytic hamartomas are glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. Classically they appear as a cream-white, well-circumscribed, elevated lesion that may present as multiple or solitary sites.
What is retinal pigment epithelium?
The retinal pigment epithelium (RPE) is a single layer of post-mitotic cells, which functions both as a selective barrier to and a vegetative regulator of the overlying photoreceptor layer, thereby playing a key role in its maintenance.
What is Chrpe of the retina?
CHRPE is a flat, darkly pigmented spot found in the back of your eye. It can vary in size, ranging from a few to more than 10 mm in diameter. They are composed of enlarged cells with densely packed and larger-than-normal, spherical pigment granules.
What is a retinal hemorrhage?
This includes retinal hemorrhages, which refers to abnormal bleeding within the delicate blood vessels of the retina. When retinal hemorrhages occur, symptoms range from the undetectable to severe vision problems. Vision problems are often temporary, but in some instances, they can be permanent.
What is Bourneville disease?
Tuberous sclerosis (TS), also known as tuberous sclerosis complex (TSC) or Bourneville disease, is a phakomatosis (neurocutaneous disorder) characterized by the development of multiple benign tumors of the embryonic ectoderm (e.g. skin, eyes, and nervous system).
What is hamartoma made of?
Hamartomas are tumours composed of mesenchymal tissues such as cartilage, fat, connective tissue and smooth muscle and can be found in virtually any organ system. These masses commonly develop sporadically, but are also seen in certain syndromes such as tuberous sclerosis or Carney triad.
What causes Chrpe eye?
CHRPE was first described by Blair and Trempe in 1980 [8]. It is commonly caused by a truncating mutation in the codons between 463 and 1387 of the APC gene [14–19]. The global prevalence of CHRPE in individuals with the APC mutation is ninety percent [9].
What kind of tumor is a retinal astrocytic hamartoma?
Background: Retinal astrocytic hamartomas are glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. Classically they appear as a cream-white, well-circumscribed, elevated lesion that may present as multiple or solitary sites.
Can a combined hamartoma of the retina and RPE be detected?
OCT is a helpful tool when evaluating the combined hamartoma of the retina and RPE as it allows one to visualize epiretinal membrane (ERM) commonly associated with the disease. The study by Shields et al examined 11 patients and showed that a distinct ERM with secondary retinal folds and striae was present in almost all patients.
Can a 5 year old have a combined hamartoma?
Peripheral Combined Hamartoma of the Retina and the Retinal Pigment Epithelium in a 5-year-old Boy. Courtesy of Timothy Murray, M.D.
What kind of camera is used for the hamartoma?
This animated GIF file illustrates the height of the hamartoma and is another example of where animated gifs can be a fantastic teaching tool. The left and right stereo images shown were taken with a Zeiss FF-4 Fundus camera by James Gilman of the Moran Eye Center.
