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Serpiginous Choroiditis

Last updated Dec. 22, 2018

Approved by: Maulik P. Purohit MD, MPH

Serpiginous Choroiditis (SC) is defined as a rare type of posterior uveitis or choroiditis.


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

  • Geographic Helicoid Peripapillary Choroidopathy (GHPC)
  • Geographic Serpiginous Choroiditis
  • Serpiginous Choroidopathy

What is Serpiginous Choroiditis? (Definition/Background Information)

  • Serpiginous Choroiditis (SC) is defined as a rare type of posterior uveitis or choroiditis. The condition affects the pigmented layer of the retina (the retinal pigment epithelium or RPE) and the choroid. Serpiginous Choroiditis falls into a category of disorders known as white dot syndromes
  • Uveitis is inflammation of the uvea, which is a part of the eye structure. The uvea is a pigmented layer that consists of the iris, ciliary body, and choroid. Posterior uveitis is a condition involving the inflammation of the choroid
  • The signs and symptoms of Serpiginous Choroiditis may include alteration of vision, flashes of light in the eye, presence of blind spots, and an absence of pain. There are 2 phases of the condition:
    • Acute stage of Serpiginous Choroiditis: When the eye lesions develop and progress rapidly over time
    • Chronic stage of Serpiginous Choroiditis: When the eye lesions remain stable and do not progress. The lesions usually heal by scarring
  • The cause of Serpiginous Choroiditis is not well-known. However, it is known to be associated with certain conditions that include tuberculosis, herpes infection, and HLA-B7 genetic make-up. Serpiginous Choroiditis is generally a progressive condition that is observed in young and middle-aged adults
  • A healthcare provider can use various physical (eye) exams, blood tests, and imaging studies to diagnose Serpiginous Choroiditis. Upon diagnosis, treatment options, such as steroids or immunomodulators, can be used to treat the condition
  • The prognosis for Serpiginous Choroiditis depends on each individual's signs and symptoms. The condition can lead to permanent blindness, if left undiagnosed or untreated. Complications, such as retinal detachment or choroidal neovascularization, can worsen the prognosis

Who gets Serpiginous Choroiditis? (Age and Sex Distribution)

  • Serpiginous Choroiditis can present itself at any age, but is more likely to occur in young and middle-age adults
  • Both males and females are affected, although a male predominance is noted
  • Individuals of different racial and ethnic backgrounds can be affected
  • It is a rare condition; only 5% of all posterior uveitis is Serpiginous Choroiditis

What are the Risk Factors for Serpiginous Choroiditis? (Predisposing Factors)

The risk factors for Serpiginous Choroiditis may include:

  • The presence of HLA-B7 genetic make-up indicates an increased risk
  • Bacterial infections such as tuberculosis
  • Viral infections caused by herpes family of viruses

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 Serpiginous Choroiditis? (Etiology)

  • Serpiginous Choroiditis is a rare eye condition of unknown cause. It may be the result of an abnormal inflammation that develops due to an underlying condition
  • It has been associated with infections, such as tuberculosis and herpes viral infections, although the cause-effect relationship is not confirmed yet
  • Serpiginous Choroiditis is called so, because there are irregularly-shaped abnormal white dots observed, when an eye specialist examines the fundus of the eye, using an instrument called a fundoscope
  • It is a type of white dot syndrome; white dot syndromes are a group of eye disorders that involve inflammation of the retina and choroid

What are the Signs and Symptoms of Serpiginous Choroiditis?

The signs and symptoms of Serpiginous Choroiditis may vary between individuals. A majority of individuals have no (or mild) signs and symptoms, and thus the condition may go undiagnosed in many. Also, the signs and symptoms may start in one eye, and after a while it may affect the other eye. The time period between this can vary from a few weeks to many years.

The signs and symptoms of Serpiginous Choroiditis may include:

  • The condition is typically bilateral and both eyes are affected; sometimes, it can be unilateral (affecting one eye)
  • Sudden alteration of vision, either in one or both the eyes
  • Serpiginous Choroiditis is usually a painless condition and a majority of individuals do not experience any pain
  • Flashes of light in the eye, or photopsia
  • Presence of blind spots, or scotoma, causing partial vision loss
  • Reduced vision; blurred vision
  • The signs and symptoms may be asymmetrical, which means that:
    • The combination of signs and symptoms in each eye may be different
    • The rate of progression of the signs and symptoms of each eye may be different
    • The severity of signs and symptoms may vary from eye to eye

The signs and symptoms may also be acute or chronic:

  • Acute signs and symptoms has a sudden onset and develops rapidly
  • Chronic signs and symptoms develop slowly over a long period of time

How is Serpiginous Choroiditis Diagnosed?

A healthcare professional may diagnose Serpiginous Choroiditis 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 studies may help detect blind spots or scotoma
  • 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:
    • To check for the presence of antibodies in blood
    • Blood culture for infections
    • Complete blood count (CBC) with differential
    • Erythrocyte sedimentation rate (ESR)
    • Testing to determine the presence of HLA-B7 genetic make-up
  • Other tests to determine any underlying condition causing Serpiginous Choroiditis

If individuals have other signs and symptoms, then the following tests may be performed:

  • Chest X-rays
  • Neuroimaging studies including MRI scan of brain
  • Lumbar puncture: In this procedure, the cerebrospinal fluid is collected and analyzed

Note: In some cases, because of the absence of (or mild) signs and symptoms, there can be a delay in diagnosis that can result in irreversible harm to the eye.

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 Serpiginous Choroiditis?

Complications of Serpiginous Choroiditis may lead to vision abnormalities and may include:

  • Choroidal neovascularization (CNV): An abnormal development of blood vessels in the choroid, which if left untreated can result in permanent loss of vision
  • Retinal detachment: An eye condition wherein the retina gets separated from the eye structures that holds the retinal layers together
  • Glaucoma: A condition that can cause blindness due to higher intraocular pressure
  • Cataracts: When the lens of the eye becomes clouded and cause vision loss
  • If left undiagnosed and/or untreated, the condition can lead to permanent blindness
  • Kidney damage, high blood sugar, and high blood pressure due to steroid treatment
  • Recurrent Serpiginous Choroiditis: In a majority, the condition is known to recur if it is not treated appropriately. With each recurrence, the signs and symptoms may get more severe

How is Serpiginous Choroiditis Treated?

Early diagnosis and treatment of Serpiginous Choroiditis is very crucial in preventing complications that may result in irreversible damage to the eye. With appropriate treatment, the chance of recurrence decreases.

The treatment of Serpiginous Choroiditis may include the following measures:

  • Oral medications (including pain medications)
  • Oral corticosteroids are generally effective and may be given to control inflammation. Steroids may also be administered intravenously depending on the underlying cause
  • Dark glasses may be prescribed for light-sensitivity
  • Addressing underlying conditions that may be contributing to progression of the disorder
  • If the underlying cause is unknown, then decreasing inflammation is the main step towards treating Serpiginous Choroiditis
  • Administration of immunomodulators, which are medications to control dysfunctional immune system
  • If choroidal neovascularization is observed, then laser therapy may be helpful
  • In case of recurrence that fails to get better with medication, surgery to treat the condition may be considered

It is important to note that steroids may not be used in all cases, since it can worsen the condition. A healthcare provider will provide the best treatment options based upon each individual’s specific circumstances.

How can Serpiginous Choroiditis be Prevented?

It may be difficult to prevent Serpiginous Choroiditis, but the risk for the condition may be lowered through the following measures:

  • Educating the individual and family members about the underlying cause and preventing the development of any modifiable risk factors associated with the underlying condition (if possible)
  • Treating any underlying infections and immunologic conditions, as early as possible
  • Maintaining long-term follow-up checkups to watch out for any recurrences

What is the Prognosis of Serpiginous Choroiditis? (Outcomes/Resolutions)

  • The prognosis of Serpiginous Choroiditis generally varies between individuals. In many cases, the prognosis is difficult to predict
  • Also, the prognosis depends upon the severity of the signs and symptoms. Individuals with mild signs and symptoms have better prognosis than those with severe conditions
  • Severe Serpiginous Choroiditis with inadequate treatment can lead to permanent blindness

Additional and Relevant Useful Information for Serpiginous Choroiditis:

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

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

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Oct. 5, 2016
Last updated: Dec. 22, 2018