This patient-education article is written by the cornea service at Suraj Eye Institute, Nagpur.

What are Stromal Corneal Dystrophies?

The stromal corneal dystrophies are a group of inherited, bilateral, slowly progressive disorders in which abnormal protein or carbohydrate deposits accumulate in the middle layer of the cornea — the stroma. Over years, these deposits scatter light and reduce vision, and some types also cause repeated breakdown of the corneal epithelium (recurrent erosion).

Three classical forms account for most cases seen in clinic. Their slit-lamp appearance is distinctive and is the first step in diagnosis.

Stromal Corneal Dystrophies — Slit-Lamp Appearance

Slit-Lamp Appearance: Lattice, Granular and Macular Dystrophy

LATTICE Branching refractile lines (amyloid deposits)

GRANULAR Discrete white deposits with clear intervening stroma (hyaline)

MACULAR Diffuse cloudy haze across the cornea (glycosaminoglycan)

Figure 1. The three classical stromal corneal dystrophies have distinctive slit-lamp appearances. Lattice dystrophy shows branching, refractile linear lines (amyloid deposits). Granular dystrophy shows discrete white snowflake-like deposits in clear intervening stroma (hyaline material). Macular dystrophy shows a diffuse cloudy haze that involves the cornea from limbus to limbus (glycosaminoglycan deposits).

The Three Forms at a Glance

Feature Lattice Granular Macular
Inheritance Autosomal dominant Autosomal dominant Autosomal recessive
Gene TGFBI TGFBI CHST6
Deposit Amyloid Hyaline Glycosaminoglycan
Onset First decade Childhood / teens First decade
Recurrent erosions Common Less common Less common
Vision loss Gradual, by 4th decade Gradual, often later Earlier, more severe

Symptoms

Patients have a slowly progressive decline in vision over decades. Those with lattice dystrophy also experience repeated episodes of sharp eye pain, watering and light sensitivity on waking (recurrent corneal erosions) caused by spontaneous breakdown of the corneal epithelium over the deposits.

How Stromal Dystrophies are Diagnosed

Diagnosis is clinical — the slit-lamp pattern is usually distinctive, and a family history supports the diagnosis. Anterior segment OCT defines the depth of the deposits, which is essential for surgical planning. Genetic confirmation is occasionally requested in unusual cases.

Treatment

Treatment is offered when vision is significantly reduced or recurrent erosions are frequent and disabling:

  • Lubricants and a bandage contact lens for recurrent erosion episodes.
  • Excimer laser phototherapeutic keratectomy (PTK) for shallow anterior deposits — smoothes the surface and improves vision in selected eyes.
  • Deep anterior lamellar keratoplasty (DALK) for deeper deposits. DALK preserves the patient’s own endothelium and has lower long-term rejection risk than penetrating keratoplasty.
Recurrence in the graft is well recognised — especially in lattice dystrophy. Deposits can reappear in transplanted tissue years later, sometimes requiring a repeat procedure. Long-term follow-up is essential.
✔ Corneal Dystrophy Care at Suraj Eye Institute

Our cornea service offers slit-lamp pattern recognition and anterior segment OCT to define the depth of dystrophic deposits, family screening for affected relatives, and the full range of treatment — from bandage contact lenses and excimer laser phototherapeutic keratectomy (PTK) for superficial disease to deep anterior lamellar keratoplasty (DALK) for deeper involvement.

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Frequently Asked Questions

Are my children at risk?
It depends on the type. Lattice and granular dystrophies are autosomal dominant — each child has a 50 % chance of inheriting the gene. Macular dystrophy is autosomal recessive and is much less likely to affect children unless both parents carry the gene.

Will a corneal transplant cure the disease?
A transplant restores vision very effectively, but the disease is genetic so the deposits can slowly reappear in the donor tissue over many years. This is most likely in lattice dystrophy. Long-term follow-up allows further treatment when needed.

Can I avoid surgery?
Many patients with milder forms never need surgery and manage well with spectacles or lubricants. Surgery is offered only when daily activities are significantly affected or when recurrent erosions are frequent.

Is there a medical cure?
No medical treatment dissolves the deposits. Research into gene therapy is active but not yet available in clinical practice. Lubrication, bandage contact lenses, PTK and DALK are the established treatments.

How is the depth of the deposits measured?
Anterior segment OCT (AS-OCT) provides a high-resolution cross-section of the cornea and shows exactly how deep the deposits extend. This determines whether PTK can be used or whether deeper surgery (DALK) is needed.

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