Fuchs' Endothelial Dystrophy — Symptoms, Diagnosis and Treatment

Fuchs' endothelial corneal dystrophy is the most common indication for corneal transplantation in developed countries, including Australia. Despite this, many patients are not diagnosed until the condition is quite advanced. Understanding the natural history of Fuchs' dystrophy allows better planning of treatment and helps patients make informed decisions about timing of surgery.

What Is Fuchs' Dystrophy?

Fuchs' dystrophy is a slowly progressive condition affecting the innermost layer of the cornea — the endothelium. The endothelial cells are responsible for pumping fluid out of the corneal stroma, keeping it thin, clear, and transparent. In Fuchs' dystrophy, these cells die prematurely, and the surviving cells develop guttata — small, wart-like excrescences on Descemet membrane that are visible on slit-lamp examination.

As the endothelial cell count drops, the pump fails. Fluid accumulates in the stroma, causing corneal oedema (swelling). In advanced cases, fluid collects in the epithelium (the outer layer), forming painful blisters called bullae — a condition known as bullous keratopathy.

Stages of Fuchs' Dystrophy

Stage 1 — Preclinical: Guttata visible on examination, but no symptoms. Corneal thickness normal. No treatment required; monitoring only.

Stage 2 — Symptomatic stromal oedema: Morning blurring that clears during the day. Corneal thickness increased on specular microscopy. Visual acuity may still be 6/6. Observation; some patients use hypertonic saline drops to help draw fluid from the cornea.

Stage 3 — Decompensation: Persistent corneal clouding throughout the day. Corrected visual acuity reduced. Surgery should be considered at this stage.

Stage 4 — Bullous keratopathy: Painful blistering of the corneal surface. Urgent surgical intervention needed.

Non-Surgical Management

In early stages, several measures can slow symptom progression:

• Hypertonic saline drops or ointment (e.g. Muro 128) draw water out of the cornea by osmosis, temporarily reducing morning oedema
• Reducing humidity in the bedroom — a fan directed toward the face during sleep can help accelerate overnight corneal drying
• Avoiding eye rubbing, which can damage already-stressed endothelial cells
• UV protection — some evidence suggests UV exposure may accelerate endothelial cell loss

These measures manage symptoms but do not halt progression or replace damaged cells.

Surgical Treatment — DMEK

The definitive treatment for Fuchs' dystrophy is endothelial keratoplasty — selectively replacing only the diseased inner layer with healthy donor cells. DMEK (Descemet Membrane Endothelial Keratoplasty) is now the gold standard technique in experienced hands.

DMEK transplants an ultra-thin graft (approximately 10–15 microns) of Descemet membrane and endothelial cells. It is performed through a small incision, under local or general anaesthetic, as a day procedure. Most patients can return to light activity within a week, and vision continues to improve over 3–6 months as the new cells repopulate.

Combined Surgery with Cataract Extraction

A significant proportion of patients with Fuchs' dystrophy also have cataracts — both conditions are more common with advancing age. If cataract surgery is performed in an eye with existing Fuchs' dystrophy, the procedure may accelerate corneal decompensation.

Performing cataract surgery and DMEK at the same time — the "triple procedure" — avoids two separate operations and produces excellent visual outcomes. For patients with moderate Fuchs' changes and a visually significant cataract, combined phaco-DMEK is typically the preferred approach.

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Dr Ross MacIntyre consults at Northern Eye Consultants in Bundoora and Bass Coast Eye Centre in Wonthaggi. Book a corneal assessment →