Pseudoexfoliation

CASE REPORT: CATARACT SURGERY IN THE SETTING OF PSEUDOEXFOLIATION SYNDROME

Slit-lamp examination revealed 3+ nuclear sclerosis and 2–3+ cortical changes bilaterally. The anterior lens capsule showed classic flaky, white deposits in the mid-periphery consistent with pseudoexfoliation syndrome (PXF). Fundus examination showed symmetric discs with a cup/disc ratio of 0.3 in both eyes. Corneal topography demonstrated a regular astigmatic pattern, and macular OCT was normal in both eyes. Ocular dominance testing confirmed left eye dominance.

SURGICAL PLANNING AND RISK DISCUSSION

Given the presence of PXF, the patient was counseled on the elevated intraoperative risks, including zonular weakness, potential capsular instability, and the possible need for adjunctive devices such as a capsular tension ring (CTR). After discussing visual goals, the patient elected for mini-monovision using enhanced monofocal IOLs, targeting plano in the dominant left eye and -1.00 D in the right eye.

SURGICAL COURSE 

Both surgeries were performed using the Catalys Femtosecond Laser for precise capsulotomy and lens fragmentation, and phacoemulsification with the Alcon Centurion system.

• Left Eye: Intraoperatively, significant zonular laxity was observed, and a capsular tension ring was placed to ensure adequate stabilization of the bag.
• Right Eye: No complications were noted.

In both eyes, IOLs were inserted without difficulty, and postoperative positioning was stable.

POSTOPERATIVE OUTCOME

At 1 week postoperatively:

• Left Eye (plano target): UCVA was 20/25
• Right Eye (-1.00 target): UCVA was 20/40 for distance and J3 for near

The patient achieved excellent binocular vision for both distance and functional near tasks and expressed high satisfaction with the outcome.

CASE DISCUSSION

This case highlights the importance of preoperative recognition and planning in patients with pseudoexfoliation. The use of femtosecond laser-assisted cataract surgery and a CTR contributed to a stable surgical course and favorable visual outcomes, despite underlying zonular compromise. The mini-monovision approach provided an excellent range of vision while preserving contrast sensitivity and minimizing dysphotopsias.

PSEUDOEXFOLIATION AND CATARACT SURGERY: OVERVIEW

Pseudoexfoliation syndrome (PXF) is a common age-related systemic condition characterized by the abnormal production and accumulation of fibrillar extracellular material on ocular structures, particularly the lens capsule, zonules, iris, and trabecular meshwork. It has significant implications for cataract surgery due to increased intraoperative and postoperative risks.

Note that the stained pseudoexfoliated material (arrows) on the lens confirms the classic appearance with amorphous, eosinophilic deposits. (Stejar LD, et al. Histopathological Analysis of Pseudoexfoliation Material in Ocular Surgeries: Clinical Implications. Diagnostics. 2024 Jan;14(19):2187.)

CLINICAL FEATURES OF PXF

• Gray-white flaky deposits on the anterior lens capsule, pupil margin, and corneal endothelium
• Poorly dilating pupil due to iris sphincter atrophy
• Weak zonular fibers, leading to phacodonesis or lens subluxation
• Associated with increased intraocular pressure (IOP) and secondary open-angle glaucoma
• Often asymmetrical, though it is a bilateral disease

PREOPERATIVE CONDITIONS

• Comprehensive assessment of zonular stability (e.g., lens decentration, phacodonesis)
• Pupil dilation may be poor—prepare for mechanical dilation (iris hooks or Malyugin ring)
• Glaucoma evaluation: IOP measurement and optic nerve imaging, as PXF glaucoma is often more aggressive
• Consider capsular tension rings (CTR) if zonular weakness is suspected

INTEROPERATIVE CHALLENGES

• Poor dilation: Use mechanical pupil expansion devices when needed
• Zonular instability:
• Gentle phacoemulsification technique with minimized stress on the zonules
• Use of capsular tension rings (CTR), modified CTRs, or capsule hooks
• Avoid excessive rotation or manipulation of the nucleus
• Capsulorhexis may be more difficult due to poor red reflex or capsule instability
• Risk of vitreous loss if zonular dehiscence occurs

POSTOPERATIVE CONSIDERATIONS

• Capsular phimosis or anterior capsule contraction may occur – monitor closely
• Higher risk of IOL decentration or dislocation, even years after surgery
• Inflammatory response may be exaggerated – monitor for prolonged inflammation
• Glaucoma monitoring: PXF eyes are at risk for postoperative IOP spikes and long-term progression

SURGICAL PEARLS

• Anticipate challenges and plan accordingly (e.g., have CTRs, pupil devices on hand)
• Use a high-viscosity cohesive OVD for chamber stability and pupil management
• Avoid aggressive maneuvers that stress the zonules
• Consider placing a three-piece IOL in the sulcus with optic capture if the capsular bag is compromised

Long-term follow-up is essential due to risk of late complications

SYSTEMIC ASSOCIATIONS OF PSEUDOEXFOLIATION SYNDROME (PXF)

While PXF is primarily recognized as an ocular disorder, it is increasingly understood to be a systemic microfibrillopathy, with the deposition of abnormal fibrillar material in multiple organs and tissues.

EXTRAOCULAR DEPOSITION SITES

• Fibrillar pseudoexfoliative material has been identified in various non-ocular tissues, including:
• Skin
• Heart
• Lungs
• Kidneys
• Blood vessels

This supports the concept of PXF as a systemic disorder, not limited to the eye. There is an increased risk of systemic diseases:

1. Cardiovascular Disease

• • PXF is associated with systemic vascular endothelial dysfunction, a key contributor to:
    • Atherosclerosis
    • Coronary artery disease (CAD)
    • Peripheral vascular disease
  • Pathophysiologic features include:
    • Reduced nitric oxide activity
    • Increased oxidative stress
    • Vascular stiffening and impaired perfusion
      

2. Cerebrovascular Disease / Stroke

•  Some population-based studies (especially in Northern Europe) report an increased incidence of ischemic stroke in individuals with PXF.
• Other studies have found no significant association after adjusting for age and comorbidities.
• A meta-analysis in Ophthalmology (2016) suggested a possible link but emphasized limitations due to study heterogeneity and confounding factors.

3. Aortic Aneurysm

• Multiple studies, particularly from Scandinavian and Icelandic cohorts, have found a higher prevalence of abdominal aortic aneurysm (AAA) in patients with PXF.
• One prominent Icelandic study reported that patients with PXF had twice the prevalence of AAA compared to those without.

4. Hypertension

• Hypertension is the most consistently reported systemic association with PXF.
  • Findings across multiple studies:
  • Increased prevalence of systemic hypertension in PXF patients compared to age-matched controls.
  • This association persists after adjusting for other cardiovascular risk factors.
• Likely mechanisms include vascular stiffness and connective tissue dysregulation secondary to LOXL1 gene involvement.

CLINICAL FINDINGS:

While routine systemic screening for all PXF patients (e.g., CT angiograms or full cardiac workups) is not standard of care, you should consider further investigation if:

• The patient has other cardiovascular risk factors (e.g., family history, hypertension, smoking, high cholesterol).
• There are symptoms suggestive of vascular disease (e.g., bruits, pulse deficits, chest/abdominal pain).
• The patient is undergoing major ocular surgery and systemic risk could influence management.

A practical approach could include:

• Referral to family physician or internist for cardiovascular risk stratification.
• Ultrasound screening for AAA in elderly men with PXF and risk factors.

While PXF is primarily managed by ophthalmologists and optometrists, its presence may be a biomarker for systemic elastosis. It is reasonable to consider broader systemic evaluation in select patients, particularly for vascular disease and aneurysms, guided by the clinical context.

FINAL THOUGHTS:

Cataract surgery in patients with pseudoexfoliation syndrome (PXF) presents distinct challenges due to the condition’s impact on zonular integrity, pupil dynamics, and postoperative stability. Success hinges on thorough preoperative evaluation, the use of advanced surgical technologies such as femtosecond laser-assisted cataract surgery, and readiness to deploy adjunctive tools like capsular tension rings or pupil expansion devices.

Importantly, PXF is more than just an ocular condition—it is increasingly recognized as a systemic disorder with fibrillar material found in organs such as the heart, lungs, kidneys, and blood vessels. This systemic association, supported by LOXL1 gene mutations, is linked to a higher risk of cardiovascular and cerebrovascular disease, hypertension, and aortic aneurysms.

With proper surgical strategy, including mini-monovision planning, careful zonular support, and close postoperative monitoring, excellent visual outcomes and patient satisfaction are attainable. However, due to the long-term risks of IOL decentration, glaucoma progression, and systemic vascular implications, lifelong ophthalmic and medical follow-up is strongly recommended for patients with pseudoexfoliation.