What are the other Names for this Condition? (Also known as/Synonyms)

• Paravenous Chorioretinal Atrophy
• Pigmented Paravenous Chorioretinal Atrophy
• PPRCA (Pigmented Paravenous Retinochoroidal Atrophy)

What is Pigmented Paravenous Retinochoroidal Atrophy? (Definition/Background Information)

• Pigmented Paravenous Retinochoroidal Atrophy (PPRCA) is a rare eye disorder that affects the choroid and retina, which are parts of the eye. The condition is known so, because there is an accumulation of pigment along the retinal vein of the eye
• Many individuals do not have significant signs and symptoms and Pigmented Paravenous Retinochoroidal Atrophy may be an incidental finding during a routine eye exam, when performed using a fundoscope (a special medical instrument)
• The cause of Pigmented Paravenous Retinochoroidal Atrophy is unknown, but it may be due to genetic defects. In some cases, a positive family history has been observed
• In many cases, Pigmented Paravenous Retinochoroidal Atrophy does not present any signs and symptoms. In some cases, it can cause blind spots in the eye or night blindness
• A healthcare provider can use various physical (eye) exams, blood tests, and imaging studies to diagnose Pigmented Paravenous Retinochoroidal Atrophy
• In most cases, no treatment is necessary to manage the condition and the prognosis of Pigmented Paravenous Retinochoroidal Atrophy is typically excellent

Who gets Pigmented Paravenous Retinochoroidal Atrophy? (Age and Sex Distribution)

• Pigmented Paravenous Retinochoroidal Atrophy is a rare disorder that is seen in young adults
• The condition affects both males and females, although a male predominance is observed
• Individuals of different racial and ethnic backgrounds can be affected

What are the Risk Factors for Pigmented Paravenous Retinochoroidal Atrophy? (Predisposing Factors)

• In some cases, Pigmented Paravenous Retinochoroidal Atrophy is known to run in families, indicating that a positive family history may be a risk factor
• However, it has known to be associated with a host of conditions including:
  • Syphilis
  • Tuberculosis
  • Sarcoidosis
  • Behcet’s disease
  • Rubeola
  • Mumps

Note: An association means that there is an increased incidence of the condition that is present with Pigmented Paravenous Retinochoroidal Atrophy. The presence of an associated condition does not mean that it is the cause of PPRCA.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pigmented Paravenous Retinochoroidal Atrophy? (Etiology)

• Pigmented Paravenous Retinochoroidal Atrophy is an eye condition of unknown cause. It is classified as a type of choroidal dystrophy
• It may be a genetic disorder due to the presence of occasional cases of family inheritance. However, the gene that is responsible for the condition is not definitely identified
• Some researchers suspect that CRB1 gene mutation may be responsible for the disorder. However, this has not been conclusively proven yet 

What are the Signs and Symptoms of Pigmented Paravenous Retinochoroidal Atrophy?

The signs and symptoms of Pigmented Paravenous Retinochoroidal Atrophy are observed in young adults.

• A majority of the individuals have no signs and symptoms
• Rarely, individuals may have blurred vision
• If the macula of the eye is involved, then the signs and symptoms may be significantly more pronounced or noticeable
• Occasionally, the presence of blind spots (scotoma) is seen
• Rarely, night blindness (nyctalopia) may be observed
• Partial loss of vision
• The condition affects both the eyes (bilateral presentation)

Additionally, there may be signs and symptoms of any associated condition.

How is Pigmented Paravenous Retinochoroidal Atrophy Diagnosed?

A healthcare professional may diagnose Pigmented Paravenous Retinochoroidal Atrophy using the following tests and procedures:

• Physical examination and analysis of previous medical history
• Eye examination by an eye specialist
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Visual field testing may reveal scotoma (occasionally)
• Slit-lamp examination: Examination of the eye structure using a special instrument called a slit-lamp. In this procedure, the pupils are dilated and the internal eye structure is examined
• Tonometry: Measurement of intraocular pressure or eye fluid pressure, especially to detect conditions such as glaucoma
• Fundus fluorescein angiography (FFA): In this technique, the eye blood vessels are examined using a fluorescein dye
• Fundus autofluorescence (FAF) imaging: It is a diagnostic technique to examine the fundus of the eye using a fluorescent dye
• Indocyanine green (ICG) angiography: It is used to examine the blood vessels of the choroid using a dye, called indocyanine green, particularly to study the choroid
• B-scan ultrasonography: Special ultrasound scan of the eye through a non-invasive diagnostic tool, to assess health of the eye structures
• Electroretinogram (ERG): It is a technique to measure electrical activities in the retinal cells
• Optical coherence tomography (OCT) of eye: Radiological imaging technique to visualize the eye structure
• Blood tests that include:
  • Complete blood count (CBC) with differential
  • Erythrocyte sedimentation rate (ESR)

Note: The pigment deposits in one eye may be more than in the other eye; it can vary from eye to eye and from individual to individual.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pigmented Paravenous Retinochoroidal Atrophy?

The complications of Pigmented Paravenous Retinochoroidal Atrophy may include:

• The presence of heavily pigmented eyes: This may indicate more severe signs and symptoms that is significant enough to cause decreased vision and visual field defects
• Complications due to any underlying or associated condition

How is Pigmented Paravenous Retinochoroidal Atrophy Treated?

• Generally, no treatment is necessary for Pigmented Paravenous Retinochoroidal Atrophy. The healthcare provider may chose to monitor the condition and adopt a ‘wait and watch’ approach
• Symptomatic treatment to address the signs and symptoms may be employed by the healthcare provider, as necessary
• Treatment may be undertaken for any associated or underlying condition

The healthcare provider may recommend the best treatment options based upon each individual’s specific circumstances.

How can Pigmented Paravenous Retinochoroidal Atrophy be Prevented?

Currently, there are no specific methods or guidelines to prevent Pigmented Paravenous Retinochoroidal Atrophy.

What is the Prognosis of Pigmented Paravenous Retinochoroidal Atrophy? (Outcomes/Resolutions)

• The prognosis of Pigmented Paravenous Retinochoroidal Atrophy is generally excellent in a majority of cases
• However, if the findings indicate heavy bilateral pigmentation and severe signs and symptoms, and/or the involvement of the macula, then the prognosis can be much worse and vision may be affected

Additional and Relevant Useful Information for Pigmented Paravenous Retinochoroidal Atrophy:

Pigmented Paravenous Retinochoroidal Atrophy was known formerly by the following terms:

• Chorioeeretinitis striata
• Congenital pigmentation of the retina
• Melanosis of the retina
• Paravenous retinal degeneration
• Pigmented paravenous chorioretinal degeneration
• Pseudoretinitis pigmentosa
• Retino-choroiditis radiate

Please visit our Eye & Vision Health Center for more physician-approved health information:

http://www.dovemed.com/health-topics/vision-center/