Approach Considerations
Recurrent erosions are managed conservatively with medical treatment. If recurrences are frequent or debilitating, laser phototherapeutic keratectomy can be considered.
Decreased visual acuity is treated surgically when indicated based on the patient’s visual acuity complaints. Surgical options include laser phototherapeutic keratectomy to remove superficial opacities and lamellar or full-thickness cornea transplantation. In either case, lattice deposits may recur, requiring repeat treatment.
Medical Care
When recurrent erosions occur with lattice corneal dystrophy, they are treated similarly to any other form of recurrent erosions. Under the care of an ophthalmologist, a bandage contact lens along with antibiotics can be prescribed. Alternatively, patching with an antibiotic ointment can be used. Once the acute episode of recurrent erosions has resolved, preventative treatments may include Muro 128 drops, lubrication drops, and lubricating ointment at bedtime. If recurrent corneal erosions occur despite medical therapy, excimer laser phototherapeutic keratectomy (PTK) may be considered. [7, 8]
Future approaches to management of autosomal-dominant corneal dystrophies may include gene therapy.
Surgical Care
Excessive corneal erosions or mild visual decreases can be treated with laser phototherapeutic keratectomy (PTK). The excimer laser removes anterior opacities, smooths the corneal surface, and allows the epithelium to re-adhere more tightly. In PTK, enough tissue must be removed from the anterior cornea to eliminate the opacities. This can be highly effective for the treatment of superficial opacities. Treatment of deep corneal opacities risks excessive flattening of the cornea and stromal haze. If corneal opacities recur after treatment with PTK, repeat treatment is possible unless the cornea becomes too thin to allow further ablation.
If visual acuity worsens and opacities are deep, a lamellar or full-thickness corneal transplant can be performed. Corneal transplants are usually not necessary until after age 40 years. [1] Although the success rate for a corneal transplant is very high, amyloid deposits can recur in the grafted tissue 2-14 years later.
A recent study examined the histopathological and ultrastructural correlate of delayed epithelial healing in eyes with lattice corneal dystrophy; [9] the study concluded that histopathological findings may correspond to reduced cell matrix interactions and may help explain the delayed healing.
Complications
Recurrent erosion syndrome is a risk factor for infectious keratitis. Patients who present with an acute erosion and an epithelial defect are treated with prophylactic topical antibiotics.
The most frequent complication of phototherapeutic keratectomy is recurrence of amyloid deposits. In this case, further treatment can be considered. However, repeat treatments or the treatment of deep stromal opacities risks excessive flattening of the cornea and unacceptable refractive outcomes. Treatment of the deep corneal stroma also risks haze formation or progressive corneal ectasia. In these cases, corneal transplantation may be indicated.
Corneal transplantation is a highly successful procedure; however, amyloid deposits can also recur in the grafted tissue. PTK can be applied to treat opacities in the transplanted tissue. Repeat corneal transplantation may ultimately be required. Corneal transplantation is associated with refractive error that may require glasses or a contact lens for correction. Less-common risks include graft rejection, graft failure, infection, glaucoma, and vision loss.
Consultations
Patients with lattice corneal dystrophy type II (systemic amyloidosis) require specialty care for the systemic management of this rare condition.
Diet
Diet is not known to affect the onset or progression of lattice corneal dystrophy.
Activity
Activity modification is not known to affect the onset or progression of lattice corneal dystrophy.
Prevention
Lattice corneal dystrophy is an inherited condition without known preventive measures.
Long-Term Monitoring
Patients with lattice corneal dystrophy should undergo regular long-term monitoring with their ophthalmologist. Follow-up care focuses on monitoring for progression and assessing the need for treatment of recurrent erosions or decreased visual acuity.
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Lattice corneal dystrophy. Image courtesy of James J. Reidy, MD, FACS, Associate Professor of Ophthalmology, State University of New York, School of Medicine & Biomedical Sciences, Buffalo, New York.
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Slit lamp image of lattice corneal dystrophy. Image courtesy of James J. Reidy, MD, FACS, Associate Professor of Ophthalmology, State University of New York, School of Medicine & Biomedical Sciences, Buffalo, New York.
Tables
Feature |
Granular Dystrophy |
Macular Dystrophy |
Lattice Dystrophy |
|---|---|---|---|
Age of onset |
First decade of life |
First decade of life |
First decade of life |
Heredity |
Autosomal dominant |
Autosomal recessive |
Autosomal dominant |
Reduced vision |
Fourth or fifth decade of life |
First or second decade of life |
Second or third decade of life |
Erosions |
Uncommon |
Common |
Frequent |
Opacities |
Discrete, intervening stroma Clear, not to limbus |
Indistinct margins, intervening Stroma hazy, extends to limbus |
Refractile lines and dots Usually not to limbus |
Material |
Hyaline |
Glycosaminoglycans |
Amyloid |
