Lattice dystrophy is a rare, inherited corneal disorder that affects the structure and transparency of the cornea, leading to progressive vision impairment. It is part of a group of corneal dystrophies and is characterized by the presence of abnormal, lattice-like deposits of amyloid in the corneal stroma, which over time, can cause corneal scarring and visual disturbances. This article provides an in-depth exploration of lattice dystrophy, including its causes, symptoms, diagnosis, management, and prognosis.

Lattice dystrophy is one of the many forms of corneal dystrophies and belongs to the category of stromal dystrophies. These conditions are generally progressive and affect the stroma, which is the thick middle layer of the cornea responsible for its strength and clarity.

Lattice dystrophy is typically inherited in an autosomal dominant pattern, meaning a person only needs one mutated gene from either parent to develop the condition. Mutations in the TGFBI gene (Transforming Growth Factor Beta Induced) are the primary cause of lattice dystrophy. This gene is responsible for encoding proteins that play a role in corneal structure and function. Mutations in this gene lead to the abnormal deposition of amyloid proteins in the cornea.

There are several types of lattice dystrophy, classified based on the onset, severity, and appearance of the lattice lines:

• Type I (Classic Lattice Dystrophy): This is the most common type, usually beginning in early adulthood, around the second decade of life. It is characterized by thin, lattice-like lines that develop in the corneal stroma and gradually become denser.
• Type II (Meretoja Syndrome): This form is associated with systemic amyloidosis and has a later onset, usually in middle age. In addition to ocular manifestations, patients may experience peripheral neuropathy and facial weakness.
• Type III: A rare variant that presents in older adults and is characterized by thick, ropy lattice lines. Unlike the other types, this variant tends to have a slower progression.
• Type IIIA: A late-onset form that is similar to Type III but without associated systemic amyloidosis.

The primary symptoms of lattice dystrophy include:

• Blurred Vision: As the amyloid deposits grow and affect the cornea’s transparency, vision becomes increasingly blurry.
• Corneal Erosions: Recurrent corneal erosions (RCE) are common in lattice dystrophy, causing sharp eye pain, tearing, redness, and light sensitivity. These erosions occur when the corneal epithelium (the outermost layer) becomes damaged or loosens.
• Dry Eyes: Patients may experience dryness and discomfort in the eyes, especially as the cornea becomes increasingly irregular in shape.
• Light Sensitivity: Photophobia, or sensitivity to light, can occur as a result of the corneal surface’s compromised integrity.

Lattice dystrophy is diagnosed through a comprehensive eye exam, with several diagnostic tools available:

• Slit-Lamp Examination: During this test, an ophthalmologist uses a special microscope to examine the cornea in detail. The lattice-like lines in the corneal stroma are typically visible on slit-lamp examination, even in the early stages.
• Corneal Topography: This imaging technique maps the surface curvature of the cornea, helping to detect any irregularities or changes in shape due to dystrophy.
• Genetic Testing: Genetic tests can confirm mutations in the TGFBI gene, aiding in the diagnosis and providing information about the specific type of lattice dystrophy present.

There is no cure for lattice dystrophy, but treatments focus on managing symptoms and preserving vision. The treatment options depend on the severity of the condition and its progression:

• Lubricating Eye Drops: Artificial tears or lubricating gels can help alleviate dry eye symptoms and reduce discomfort from recurrent corneal erosions.
• Bandage Contact Lenses: Soft contact lenses can protect the corneal surface and promote healing of corneal erosions.
• Phototherapeutic Keratectomy (PTK): This laser procedure is used to remove superficial amyloid deposits and smooth the corneal surface, reducing symptoms of recurrent erosions and improving vision.
• Corneal Transplant (Keratoplasty): In advanced cases where vision is significantly impaired due to corneal scarring, a corneal transplant may be necessary. The two primary types of transplants are:
  • Penetrating Keratoplasty (PK): A full-thickness corneal transplant.
  • Lamellar Keratoplasty: A partial-thickness corneal transplant that preserves the healthy layers of the cornea.
• Antibiotics and Pain Management: When corneal erosions occur, antibiotics may be prescribed to prevent infection, and pain medications may be recommended to manage discomfort.

The progression of lattice dystrophy varies depending on the type and severity of the condition. Most patients experience a gradual decline in vision over time due to the accumulation of amyloid deposits and the development of corneal scars. However, with appropriate management and treatment, vision can be preserved for many years.

Ongoing research into the genetic basis of lattice dystrophy is leading to better diagnostic techniques and potential treatments. Gene therapy holds promise as a future treatment option by addressing the underlying genetic mutations responsible for the condition. Advances in corneal transplantation and regenerative medicine may also improve outcomes for patients with severe disease.

Lattice dystrophy is a complex and rare corneal disorder with significant implications for vision health. Early diagnosis and ongoing management are essential for minimizing symptoms and preserving visual function. Patients with lattice dystrophy should maintain regular follow-ups with an ophthalmologist to monitor the progression of the disease and address any complications. As research continues to evolve, new treatment modalities may offer hope for improved quality of life for those affected by this condition.

By understanding the nature of lattice dystrophy and exploring available treatment options, patients can take an active role in managing their condition and maintaining their vision health.

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