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Updated: Jul 9, 2026

Biological Preparation and Mechanical Technique for Determining Viscoelastic Properties of Zonular Fibers
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Published on: December 16, 2021

Pathophysiology of zonular diseases.

Pascal Dureau1

  • 1Fondation Ophtalmologique Adolphe de Rothschild, Pediatric Ophthalmology Department, Paris, France. pdureau@fo-rothschild.fr

Current Opinion in Ophthalmology
|December 20, 2007
PubMed
Summary

Zonular diseases, like ectopia lentis, are linked to fibrillin gene mutations causing lens instability. Management focuses on refractive errors, glaucoma, and retinal detachment, guided by genetic insights.

Area of Science:

  • Ophthalmology
  • Genetics
  • Molecular Biology

Background:

  • Zonular diseases encompass a range of inherited conditions affecting the eye's structural integrity.
  • Ectopia lentis, or lens displacement, is a key clinical manifestation.
  • These conditions often stem from genetic defects impacting connective tissues.

Purpose of the Study:

  • To review the mechanisms underlying clinical manifestations of zonular diseases.
  • To highlight the role of fibrillin in these conditions.
  • To discuss associated complications and current management strategies.

Main Methods:

  • Literature review of studies on zonular diseases and ectopia lentis.
  • Analysis of molecular mechanisms involving fibrillin.

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  • Synthesis of findings on clinical manifestations and complications.
  • Main Results:

    • Fibrillin mutations are implicated in a spectrum of heritable diseases causing zonular stretching.
    • Common complications include refractive errors (myopia), glaucoma (various types), and retinal detachment.
    • Anterior lens displacement can lead to pupil block glaucoma.

    Conclusions:

    • Genetic and molecular understanding aids in genetic counseling for affected families.
    • Treatment for myopia and glaucoma is mechanism-dependent.
    • Continuous improvement in lens surgery techniques is ongoing.