PXE Guide: Cataracts and Cataract Surgery

What Are Cataracts?

A cataract is clouding of the natural lens inside the eye. Cataracts are common with aging and can also occur earlier because of genetics, eye injury, inflammation, certain medications, diabetes, or other medical conditions.

Cataracts can cause: • blurry or cloudy vision • glare or halos around lights • trouble seeing at night • fading or yellowing of colors • frequent changes in glasses prescription • difficulty reading, driving, or seeing detail

Cataracts are not caused by PXE. People with PXE can develop cataracts for the same reasons other people do.

Can People with PXE Have Cataract Surgery?

Yes. People with PXE can have cataract surgery when it is medically appropriate. PXE itself is not a reason to avoid cataract surgery. However, people with PXE may also have retinal findings such as peau d’orange, angioid streaks, choroidal neovascularization, macular scarring, or retinal atrophy. These retinal findings may affect how much vision improves after cataract surgery.

For that reason, cataract surgery in a person with PXE should include careful preoperative retinal evaluation.

Why Retinal Evaluation Matters Before Cataract Surgery

Cataract surgery treats the cloudy lens. It does not treat retinal disease. In PXE, vision may be affected by changes in Bruch’s membrane and the retina. Angioid streaks are breaks in Bruch’s membrane, and fragile new blood vessels can sometimes grow through these breaks and leak or bleed. This is called choroidal neovascularization.

If a person with PXE has active retinal leakage, bleeding, macular scarring, or atrophy, cataract surgery may still be possible, but the expected visual improvement may be limited. A retina specialist can help determine whether retinal disease is active, stable, or likely to affect the surgical outcome.

Preoperative macular OCT can detect macular problems that may not be obvious on routine examination and may help set realistic expectations before cataract surgery.

Before Surgery

Before cataract surgery, a person with PXE should tell the cataract surgeon they have PXE and ask whether a retinal evaluation is needed.

A reasonable preoperative plan may include: • a dilated eye examination • macular OCT • review of prior retinal imaging • evaluation by a retina specialist if there is known or suspected PXE-related retinal disease • discussion of whether any choroidal neovascularization is active • discussion of realistic visual expectations after surgery

If anti-VEGF injections are being used, the cataract surgeon and retina specialist should coordinate timing.

What to Ask the Eye Surgeon

Useful questions include: • Do I have any macular or retinal findings that may limit the benefit of cataract surgery? • Should I have an OCT before surgery? • Should a retina specialist examine me before surgery? • Do I have active or inactive choroidal neovascularization? • If I receive anti-VEGF injections, how should those be timed around cataract surgery? • What improvement in vision is realistic for this eye? • What symptoms after surgery should make me call urgently?

After Surgery

After cataract surgery, follow the surgeon’s instructions for drops, activity, and follow-up visits.

People with PXE should continue usual retinal monitoring after cataract surgery. Contact the eye doctor promptly for: • new distortion • straight lines looking wavy or bent • a new dark, gray, blurry, or missing spot in central vision • sudden decrease in vision • new flashes, many new floaters, or a curtain-like shadow

These symptoms can have several causes and should be evaluated promptly.

Does Cataract Surgery Cause PXE Eye Disease to Progress?

There is no good evidence that cataract surgery itself causes PXE eye disease to progress. The main issue is that PXE-related retinal disease may already be present and may limit visual improvement.

Because angioid streaks and choroidal neovascularization are important causes of central vision loss in PXE, retinal status should be understood before surgery whenever possible. Angioid streaks are breaks in Bruch’s membrane and can be associated with choroidal neovascularization and central vision loss.

Key Points

• Cataracts are common and are not caused by PXE. • PXE is not a reason to avoid cataract surgery. • Cataract surgery treats the cloudy lens, not the retina. • PXE-related retinal disease may limit how much vision improves after surgery. • People with PXE should tell their cataract surgeon about PXE before surgery. • A dilated retinal exam and macular OCT are often useful before surgery. • A retina specialist should be involved if there is known or suspected PXE-related retinal disease. • Continue retinal monitoring after cataract surgery.