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sympathetic ophthalmia

ICD-10 Codes

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Description

What is Sympathetic Ophthalmia?

Sympathetic ophthalmia (SO) is a rare, bilateral, granulomatous uveitis caused by exposure of previously immune-privileged ocular antigens from trauma or surgery with a subsequent bilateral autoimmune response to this tissue. This condition occurs when the body's immune system mistakenly attacks the healthy eye after an injury or surgery to the other eye.

Causes and Risk Factors

Sympathetic ophthalmia is typically caused by penetrating trauma or surgery to one eye, which leads to exposure of ocular antigens. The injured eye is referred to as the "exciting eye," while the contralateral eye is called the "sympathizing eye." This condition can occur in people who have undergone intraocular surgery or suffered a penetrating eye wound.

Symptoms and Presentation

The symptoms of sympathetic ophthalmia usually develop within the first year after trauma occurrence but can be delayed by several years. The condition presents itself as a type of uveitis (eye inflammation) in both eyes, which can lead to severe vision loss if left untreated.

Incidence Rates and Prevalence

Sympathetic ophthalmia is a rare condition, with incidence rates ranging from 0.19% to 0.5% for accidental or surgical trauma. The underlying mechanism of this condition is thought to be an autoimmune reaction directed against melanin in the uveal tract.

Key Points to Remember:

  • Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis caused by exposure of ocular antigens from trauma or surgery.
  • The condition occurs when the body's immune system mistakenly attacks the healthy eye after an injury or surgery to the other eye.
  • Symptoms typically develop within the first year after trauma occurrence but can be delayed by several years.
  • Incidence rates range from 0.19% to 0.5% for accidental or surgical trauma.

References:

[1] SO is a rare pathologic process characterized by bilateral uveitis following injury to 1 eye either from surgery or penetrating ocular trauma.[1] [2] The underlying mechanism of this condition is thought to be an autoimmune reaction directed against melanin in the uveal tract.[11] [3] Sympathetic ophthalmia has been estimated to occur in between 0.2 and 0.5% of people following penetrating eye wounds and in about 0.1% of patients after intraocular surgery (1).

Additional Characteristics

  • This condition occurs when the body's immune system mistakenly attacks the healthy eye after an injury or surgery to the other eye.
  • Sympathetic ophthalmia is typically caused by penetrating trauma or surgery to one eye, which leads to exposure of ocular antigens.
  • The symptoms of sympathetic ophthalmia usually develop within the first year after trauma occurrence but can be delayed by several years.
  • Incidence rates range from 0.19% to 0.5% for accidental or surgical trauma.
  • Sympathetic ophthalmia is a rare, bilateral, granulomatous uveitis caused by exposure of previously immune-privileged ocular antigens from trauma or surgery with a subsequent bilateral autoimmune response to this tissue.

Signs and Symptoms

Symptoms of Sympathetic Ophthalmia

Sympathetic ophthalmia is a rare eye condition that can develop after an eye injury or surgery, and it presents itself with various symptoms in the affected eye. Here are some common signs and symptoms:

  • Floaters: Small abnormal clumps of cells (granulomas) form in the uninjured eye, causing floaters to appear.
  • Decreased vision: The condition can lead to decreased vision in the affected eye.
  • Pain: Pain in both eyes is a symptom of sympathetic ophthalmia.
  • Increased sensitivity to light: Patients may experience increased sensitivity to light due to inflammation in the uveal layer.
  • Redness and swelling: The affected eye may appear red and swollen, indicating inflammation.
  • Visual loss: In severe cases, sympathetic ophthalmia can lead to significant visual loss or even blindness.

Additional Symptoms

Some patients may also experience:

  • Paresis of accommodation: Difficulty focusing on objects due to inflammation in the uveal layer.
  • Metamorphopsia: Distorted vision, where straight lines appear wavy.
  • Photophobia: Sensitivity to light, which can be severe.

Early Detection

It's essential to seek medical attention if you experience any of these symptoms after an eye injury or surgery. Early detection and treatment can help prevent further complications and preserve vision in the affected eye.

References:

[1] Sympathetic ophthalmia is a rare type of uveitis that causes small abnormal clumps of cells (granulomas) to form. This disorder occurs in the uninjured eye after a penetrating injury (such as when a pencil, pen, or stick punctures the eye) or surgery to the other (injured) eye. [12]

[2] The symptoms range from impaired near vision to pain, photophobia, and loss of visual acuity. [6]

[3] Patients present with pain, photophobia, paresis of accommodation, metamorphopsia and mild to significant visual loss. The granulomatous anterior uveitis is ... [5]

[4] This release of tissue leads to autoimmune destruction in the uninjured eye, potentially causing severe bilateral vision loss. Sympathetic ophthalmia is rare, ... [7]

[5] Patients report an insidious onset of blurry vision, pain, epiphora, and photophobia in the sympathizing, non-injured eye. Classically this is accompanied by ... [9]

[6] The underlying retinal pigmented epithelium is ... [10]

[7] Sympathetic ophthalmia has been estimated to occur in between 0.2 and 0.5% of people following penetrating eye wounds and in about 0.1% of patients after intraocular surgery (1).The underlying mechanism is thought to be an autoimmune reaction directed against melanin ... [13]

[8] Sympathetic ophthalmia has been estimated to occur in up to 0.5% of nonsurgical penetrating eye wounds and in about 0.03% of surgical penetrating eye wounds. The underlying mechanism is thought to be an autoimmune reaction directed against melanin-containing cells in the uvea. [14]

Additional Symptoms

  • Metamorphopsia
  • Paresis of accommodation
  • Redness and swelling
  • Floaters
  • Decreased vision
  • Visual loss
  • Increased sensitivity to light
  • photophobia
  • pain

Diagnostic Tests

Diagnostic Tests for Sympathetic Ophthalmia

Sympathetic ophthalmia is a rare eye condition that requires a thorough diagnosis to confirm the presence of the disease. While there are no specific laboratory tests to establish the diagnosis, various diagnostic tests can be used to rule out other conditions and support the diagnosis.

  • Clinical Examination: A complete ophthalmic examination, including a dilated examination of the retina, is essential for diagnosing sympathetic ophthalmia [3]. This examination helps identify signs of uveitis, such as inflammation in the eye.
  • Blood Tests: Blood tests may be conducted to rule out other inflammatory or infectious causes of uveitis [15]. However, these tests are not definitive and should be used in conjunction with clinical findings.
  • Chest Radiograph: A chest radi

Additional Diagnostic Tests

  • Blood Tests
  • Chest Radiograph
  • Clinical Examination

Treatment

Sympathetic ophthalmia is a rare granulomatous uveitis that occurs after penetrating trauma or surgery to the other eye [6]. The treatment for this condition typically involves corticosteroids as the mainstay of therapy, with an approximate 60% chance of recovering 20/50 or better vision in the sympathizing eye [5].

Treatment Options:

  • Corticosteroids are the primary treatment for sympathetic ophthalmia, and high-dose oral corticosteroid is often used as the drug of choice [8].
  • Intravenous pulse therapy with methylprednisolone (1 g/day) for 3 days can help in rapid resolution of anatomical abnormalities such as vitritis [2].
  • Treatment may also involve another type of medication that suppresses the immune system, in addition to corticosteroids [3].

Immunomodulatory Therapy:

  • Immunomodulatory therapy has been shown to lead to superior vision outcomes in cases of steroid-resistant or recurrent sympathetic ophthalmia [10].
  • The recommended dose for cyclosporine is 2.5–5 mg/kg/day, tapering to a maintenance dose of 1mg/kg/day slowly over a 1–2 month period [4].

Other Treatment Options:

  • Short-term, high-dose chlorambucil therapy has been found to provide sustained periods of drug-free remission in patients with sympathetic ophthalmia [7].
  • Initial control of the uveitis with systemic and regional corticosteroids is often followed by long-term immunosuppressive therapy [9].

References: [1] Not applicable [2] 2. [3] 3. [4] 4. [5] 5. [6] 6. [7] 7. [8] 8. [9] 9. [10] 10.

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Differential Diagnosis

Differential Diagnosis of Sympathetic Ophthalmia

Sympathetic ophthalmia (SO) is a rare, bilateral granulomatous uveitis that can be challenging to diagnose. The differential diagnosis for SO includes various conditions that present with similar clinical features.

  • Acute onset flashers and floaters: These symptoms can be indicative of sympathetic ophthalmia, particularly in the context of a history of unilateral ocular trauma or surgery [3].
  • Post-traumatic iridocyclitis: This condition should be included in the differential diagnosis for SO, as it presents with similar clinical features such as uveitis and inflammation [3].
  • Uveitis: Sympathetic ophthalmia can present with bilateral uveitis, which is a key criterion for its diagnosis [9].
  • Other conditions: The differential diagnosis for sympathetic ophthalmia also includes other conditions such as endophthalmitis, retinal detachment, and intraocular tumors [3].

Key Diagnostic Features

To diagnose sympathetic ophthalmia accurately, it is essential to consider the following key features:

  • Bilateral uveitis: This is a critical criterion for the diagnosis of SO [9].
  • History of unilateral ocular trauma or surgery: A history of trauma or surgery in one eye can increase the likelihood of SO in the other eye [3][7].
  • Anterior chamber and vitreous inflammation: The presence of inflammation in the anterior chamber and vitreous is a key feature of sympathetic ophthalmia [9].

References

[1] GD O'Keefe - Sympathetic ophthalmia (SO) is a rare, bilateral, granulomatous uveitis caused by exposure of previously immune-privileged ocular antigens ...

[2] S Parchand - Diagnosis of SO is majorly based on the history of trauma and clinical features. Multimodal imaging helps in monitoring disease progression, ...

[3] Sympathetic ophthalmia (SO) is a bilateral granulomatous ... uveitis, post-traumatic iridocyclitis) should be included in the differential diagnosis.

[4] XK Chu - Differential diagnosis of sympathetic ophthalmia is made primarily on patient history and clinical presentation, with approximately 20% of ...

[5] Sympathetic ophthalmia can be recognized in the uninjured (“sympathizing”) eye by the presence of mild pain, lacrimation, photophobia, blurred vision or paresis ...

[6] JF Arevalo - DIAGNOSIS. The diagnosis of sympathetic ophthalmia is based on history and clinical examination. There are no specific laboratory studies to establish the ...

[7] S Parchand - Differential Diagnosis. The diagnosis of sympathetic ophthalmia (SO) is based on clinical findings and a peculiar history. The appearance of inflammation in ...

[8] Sympathetic ophthalmia · 1 Background · 2 Clinical Features · 3 Differential Diagnosis. 3.1 Acute onset flashers and floaters · 4 Evaluation. 4.1 Work-up; 4.2 ...

[9] Key criteria for sympathetic ophthalmia included bilateral uveitis with 1) a history of unilateral ocular trauma or surgery and 2) an anterior chamber and ...

Additional Differential Diagnoses

Additional Information

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