Iris-Claw IOL Implantation May Help Treat Marfan Syndrome–Associated Ectopia Lentis

In children with Marfan syndrome, long-term data suggest that lens subluxation treatment with anterior chamber iris-claw IOL lens implantation provides good outcomes with low corneal endothelial cell count (ECC) loss.

Study Design

This was a retrospective chart review of 23 children (43 eyes) with Marfan syndrome–associated ectopia lentis treated with lensectomy and primary anterior chamber iris-claw IOL implantation over a 13-year period. All patients were operated on by a single surgeon at a single medical center in the Netherlands.

Outcomes

Data on ECCs were available for 17 patients (33 eyes). During the follow-up period (median 6.2 years), the mean annual ECC decline was 0.71%. Six surgery-related complications were identified, including iris bombe due to blockage of peripheral iridectomy (3 eyes) and lens implant dislocation due to trauma (3 eyes).

Limitations

Capturing useful specular imaging was difficult due to the young age of the patient population, effectively resulting in cross-sectional vs longitudinal ECC data. The median age at surgery was 6.1 years, making the median 6.2-year follow-up time relatively short and necessitating continued long-term ECC count monitoring.

Clinical Significance

In this study, anterior chamber iris-claw IOL lens implantation did not appear to adversely impact the corneal endothelium. However, it should be noted that patients could be treated with lensectomy and aphakic spectacles or contact lenses and get similar visual benefits, with a lower risk of iris bombe and no risk of lens dislocation.

Financial Disclosures: Dr. Jagger Koerner discloses no financial relationships.