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Related Experiment Videos

Retinal detachment in Marfan syndrome.

A Loewenstein1, I S Barequet, E De Juan

  • 1The Wilmer Institute, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.

Retina (Philadelphia, Pa.)
|August 19, 2000
PubMed
Summary

Successful retinal detachment (RD) repair in Marfan syndrome patients has improved. Recent surgeries show good outcomes regardless of lens status, unlike earlier procedures where aphakic eyes fared poorly.

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Area of Science:

  • Ophthalmology
  • Genetics
  • Retinal Surgery

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue, often leading to ocular complications.
  • Retinal detachment (RD) is a serious complication that can occur in patients with Marfan syndrome.
  • Surgical outcomes for RD in Marfan syndrome patients can vary.

Purpose of the Study:

  • To evaluate and compare postsurgical outcomes of retinal detachment (RD) repair in patients with Marfan syndrome.
  • To assess the impact of lens status (phakic, aphakic, pseudophakic) on RD repair success in Marfan syndrome patients.

Main Methods:

  • Retrospective review of two cohorts of Marfan syndrome patients who underwent RD surgery.
  • Cohort 1: 12 patients (15 eyes) operated recently at the Wilmer Institute.

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  • Cohort 2: 16 patients (24 eyes) operated several years earlier elsewhere.

    • Main Results:

    • In the recent cohort, all 5 phakic eyes achieved a final visual acuity (VA) of 20/80 or better with a flat retina.
    • In the recent cohort, 60% of 10 aphakic eyes had VA 20/80 or better after RD repair.
    • In the earlier cohort, only 29% of 17 aphakic/pseudophakic eyes had a flat retina and VA 20/80 or better, with 71% having no light perception.

    Conclusions:

    • Past RD repair outcomes in Marfan syndrome patients were significantly worse in aphakic eyes.
    • Recent surgical approaches appear to offer a good prognosis for RD repair in Marfan syndrome patients, irrespective of lens status.
    • Improved surgical techniques and management likely contribute to better outcomes in contemporary RD repair for Marfan syndrome.