Kawasaki Disease (KD) is an acute, self-limited vasculitis predominantly affecting children under five years old. Named after Dr. Tomisaku Kawasaki, who first described the condition in Japan in 1967, KD is now recognized worldwide. While the disease primarily involves medium-sized arteries, particularly the coronary arteries, it can also affect various other organs, including the eyes. Understanding the ophthalmic manifestations of Kawasaki Disease is crucial for early diagnosis and effective management.

Kawasaki Disease is characterized by inflammation of blood vessels throughout the body. The exact cause remains unknown, though it is believed to involve a combination of genetic predisposition and environmental triggers, possibly infectious agents. The disease progresses through three phases:

1. Acute Phase (1-2 weeks): High fever lasting more than five days, rash, conjunctivitis, red and swollen hands and feet, red lips, and a “strawberry” tongue.
2. Subacute Phase (2-4 weeks): Symptoms subside, but the risk of coronary artery aneurysms increases.
3. Convalescent Phase (6-8 weeks): Clinical symptoms resolve, but laboratory abnormalities may persist.

Ophthalmic manifestations of Kawasaki Disease are often among the earliest signs and can provide vital clues for diagnosis. They include:

1. Bilateral Non-Exudative Conjunctivitis

• • Presentation: One of the hallmark features of KD, occurring in over 90% of cases. It usually appears within the first few days of the disease.
  • Characteristics:
    • Redness in both eyes without pus or discharge
    • Absence of significant itching or discomfort
  • Significance: This type of conjunctivitis helps differentiate KD from other causes of fever and rash in children, such as viral infections and bacterial conjunctivitis.

2. Anterior Uveitis

• • Presentation: Less common but significant, affecting approximately 70% of patients with KD. It typically manifests within the first week.
  • Characteristics:
    • Inflammation of the iris and ciliary body
    • May cause photophobia (sensitivity to light) and mild discomfort
    • Often diagnosed through slit-lamp examination
  • Significance: Anterior uveitis can aid in the diagnosis of KD when accompanied by other systemic symptoms.

3. Subconjunctival Hemorrhage

• • Presentation: Occurs less frequently but is noted in some cases.
  • Characteristics:
    • Small blood spots under the conjunctiva
    • Usually painless and does not affect vision
  • Significance: While not exclusive to KD, its presence alongside other symptoms may support the diagnosis.

4. Keratitis

• • Presentation: Rare in Kawasaki Disease but has been documented.
  • Characteristics:
    • Inflammation of the cornea
    • Symptoms may include eye pain, redness, and vision changes
  • Significance: Although rare, keratitis can indicate a severe inflammatory response and may require additional ophthalmic intervention.

5. Vitritis

• • Presentation: Another rare manifestation of KD.
  • Characteristics:
    • Inflammation of the vitreous humor (gel-like substance within the eye)
    • Can lead to floaters and blurred vision
  • Significance: Vitritis is unusual in KD but highlights the disease’s potential to cause widespread inflammation.

6. Retinal Changes

• • Presentation: Very rare but can include findings such as retinal vasculitis or cotton-wool spots.
  • Characteristics:
    • Inflammation of retinal blood vessels
    • Microinfarcts in the retina
  • Significance: Retinal involvement is typically seen in more severe cases of KD and warrants close monitoring.

Diagnosing Kawasaki Disease, particularly its ophthalmic manifestations, involves a combination of clinical criteria and laboratory tests:

• Clinical Criteria: Presence of fever lasting more than five days along with at least four of the following: bilateral non-exudative conjunctivitis, changes in lips and oral cavity, polymorphous rash, changes in extremities, and cervical lymphadenopathy.
• Laboratory Tests: Elevated inflammatory markers (ESR, CRP), elevated white blood cell count, and sometimes thrombocytosis (increased platelet count).

Ophthalmic Examination:

• Slit-lamp Examination: Essential for detecting anterior uveitis and keratitis.
• Fundoscopy: May reveal retinal changes and vitritis.

Management of Kawasaki Disease focuses on reducing inflammation and preventing coronary artery complications:

1. Intravenous Immunoglobulin (IVIG): Mainstay of treatment, given within the first 10 days to reduce the risk of coronary artery aneurysms.
2. Aspirin: Used in high doses initially for its anti-inflammatory effects, followed by lower doses for antiplatelet effects.
3. Corticosteroids: Sometimes used in refractory cases or those at high risk for coronary complications.
4. Ophthalmic Care: Specific treatments for ocular manifestations include topical steroids for anterior uveitis and supportive care for conjunctivitis.

The prognosis for children with Kawasaki Disease is generally good with timely treatment. Most ocular manifestations resolve without long-term sequelae. However, untreated KD can lead to serious complications, including coronary artery aneurysms and myocardial infarction.

Ophthalmic manifestations are a crucial aspect of Kawasaki Disease, often providing early clues for diagnosis. Bilateral non-exudative conjunctivitis and anterior uveitis are the most common ocular signs. Recognizing these manifestations and understanding their implications can lead to timely intervention, significantly improving outcomes for affected children. Comprehensive management, including ophthalmic care, is essential for preventing complications and ensuring a good prognosis.

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