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Congenital Hypertrophy of the Retinal Pigment Epithelium, referred to as CHRPE (“chirpy”), a form of freckling inside the eye has been associated with a hereditary condition known as Familial Adenomatous Polyposis (FAP) or Gardner's Syndrome. 80% of patients with FAP have CHRPE.
Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a rare benign lesion of the retina, usually asymptomatic and detected at routine eye examination. It results from a proliferation of pigmented epithelial cells, well defined, flat, does not cause visual symptoms if they do not reach the macula.
H35.52ICD-10-CM Code for Pigmentary retinal dystrophy H35. 52.
H35.30ICD-10 code H35. 30 for Unspecified macular degeneration is a medical classification as listed by WHO under the range - Diseases of the eye and adnexa .
It can be detected in an eye exam by your primary optometrist, ophthalmologist, or retina specialist. This is a congenital condition meaning you are born with it, but it may go undetected until later in adulthood. In the vast majority of cases, CHRPE is a benign finding that never causes a problem with vision or life.
Pigment epithelial detachment (PED) is a pathological process in which the retinal pigment epithelium separates from the underlying Bruch's membrane due to the presence of blood, serous exudate, drusen, or a neovascular membrane.
Retinitis pigmentosa: It is the most commonly seen retinal dystrophy. RP is a progressive rod-cone disease with rods affected first and has a high level of clinical and genetic heterogeneity. The age of presentation and the prognosis depends on the type of inheritance.
In macular degeneration, clumps of yellowish material gradually accumulate within and beneath the retinal pigment epithelium. These deposits are visible to a doctor who looks inside the eye. The clumps appear as small yellow spots known as drusen (singular: druse).
Macular mottling is evident at an early age with attenuation and narrowing of the retinal arterioles. The pigmentary changes are salt-and-pepper in appearance but there are also areas of RPE atrophy with relative sparing of the fovea. Pigment clumping in the shape of bone spicules has been observed in the periphery.
A condition in which parts of the eye cells degenerate, resulting in blurred vision and ultimately blindness. A condition in which there is a slow breakdown of cells in the center of the retina (the light-sensitive layers of nerve tissue at the back of the eye).
Vision with macular degeneration Wet macular degeneration is a chronic eye disorder that causes blurred vision or a blind spot in your visual field. It's generally caused by abnormal blood vessels that leak fluid or blood into the macula (MAK-u-luh).
ICD-10 code H54. 8 for Legal blindness, as defined in USA is a medical classification as listed by WHO under the range - Diseases of the eye and adnexa .
Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a typically benign, asymptomatic, pigmented fundus lesion. It is a congenital hamartoma of the retinal pigment epithelium (RPE) and occurs in three variant forms: solitary (unifocal), grouped (multifocal) and atypical. Atypical CHRPE is associated with familial adenomatous ...
CHRPE lesions associated with FAP are typically smaller in diameter (50-100 μm) than solitary lesions. Clinically they appear as multiple oval, spindle, comma or fishtail-shaped lesions haphazardly distributed across the fundus. Retinal invasion and proliferation of RPE, capillaries and glial cells are typical. Larger lesions may contain depigmented lacunae and may be surrounded by depigmented haloes, mottled RPE and small, pigmented satellite lesions. Bilateral lesions occur in 78% of patients.
Most solitary and grouped CHRPE lesions are characterized by a monocellular layer of hypertrophied RPE cells, densely packed with large, round macromelanosomes. The underlying Bruch’s membrane may be thickened and the overlying photoreceptor layer degenerates with increasing age. The choroid, choriocapillaris and inner retinal layers are unaffected. Glial cells replace the RPE and photoreceptor layer in areas of depigmented lacunae.
The diagnosis of CHRPE is usually made clinically and no diagnostic procedures are generally necessary. Color fundus photography is useful for documentation and follow up of lesions and wide-field scanning-laser ophthalmoscopy has been recommended as a screening tool.