Lens Subluxation and Dislocation

Lens Subluxation and Dislocation

Lens Subluxation and Dislocation

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Lens Subluxation/Dislocation - Wobbling Windows & Why

  • Subluxation: Partial lens displacement; lens edge in pupil. Signs: Iridodonesis, phacodonesis, ↓VA.
  • Dislocation (Ectopia Lentis): Complete displacement; lens outside pupillary area (e.g., vitreous, AC).
  • Mechanism: Zonular fiber weakness/rupture (zonular dehiscence).
  • Etiology:
    • Traumatic:

      ⭐ Most common cause.

    • Hereditary (Bilateral, Symmetrical):
      • Marfan Syndrome: Superotemporal (Up & Out). (Tall, arachnodactyly, aortic issues) 📌 Marfan: UP & Out.
      • Homocystinuria: Inferonasal (Down & In). (Intellectual disability, thromboembolism) 📌 Homo: DOWN & In.
      • Weill-Marchesani Syndrome: Anterior, microspherophakia. (Short stature, brachydactyly)
      • Ehlers-Danlos Syndrome.
      • Sulfite Oxidase Deficiency.
    • Acquired (Unilateral):
      • Pseudoexfoliation syndrome.
      • Hypermature cataract.
      • Chronic uveitis.
      • High myopia.
      • Buphthalmos. Zonular anatomy and structure

Clinical Features - Shaky Sights & Signs

  • Symptoms:
    • Decreased vision (myopic shift, irregular astigmatism)
    • Monocular diplopia (due to lens edge in pupillary area)
    • Glare
  • Signs:
    • Iridodonesis (tremulousness of iris)
    • Phacodonesis (tremulousness of lens)
    • Visible lens edge or equator (aphakic crescent)
    • Unequal anterior chamber (AC) depth
    • Vitreous in AC
    • Cataract formation
    • Secondary glaucoma or uveitis
  • Purkinje Images: Doubling or absence of 3rd & 4th images.

⭐ Iridodonesis (quivering iris) is a key and often early sign of lens subluxation, indicating zonular weakness.

Lens Subluxation with Aphakic Crescent Sign

📌 Mnemonic (Signs): VIP Crescent Unequal AC

  • Vitreous in AC, Iridodonesis, Phacodonesis, Cataract, Crescent (aphakic), Uveitis, Unequal AC depth

Syndromic Associations - The Usual Suspects

  • Marfan Syndrome: Superotemporal (Up & Out) subluxation. FBN1 gene (AD). Systemic: Arachnodactyly, aortic dilatation, MVP.
  • Homocystinuria: Inferonasal (Down & In) subluxation. CBS gene (AR). Systemic: Thromboembolism risk, osteoporosis, intellectual disability.
    • 📌 Mnemonic: HOWN - Homocystine, Osteoporosis, MR (Wit), INferonasal dislocation, thromboembolic.
  • Weill-Marchesani Syndrome: Anterior/Inferior subluxation, microspherophakia. Genes: ADAMTS10, FBN1, LTBP2 (AR > AD). Systemic: Short stature, brachydactyly, glaucoma.
  • Ehlers-Danlos Syndrome: (Briefly) Joint hypermobility, skin hyperextensibility. Lens subluxation less common.
  • Sulfite Oxidase Deficiency: (Briefly) Rare, severe neurological issues.

Ectopia Lentis: Normal vs Dislocated Lens

⭐ Marfan syndrome: Superotemporal lens subluxation. Homocystinuria: Inferonasal lens subluxation.

Comparison: Key Syndromes

SyndromeGene(s)InheritanceSubluxation DirectionKey Systemic Features
MarfanFBN1ADSuperotemporal (Up & Out)Arachnodactyly, aortic dilatation
HomocystinuriaCBSARInferonasal (Down & In)Thromboembolism, osteoporosis, ID
Weill-MarchesaniADAMTS10, FBN1, LTBP2AR > ADAnt/Inf, MicrospherophakiaShort stature, brachydactyly, glaucoma

Complications & Management - Trouble & Treatment

  • Complications:

    • Glaucoma (Pupillary block, Phacolytic, Phacomorphic, Angle recession - if traumatic)
    • Uveitis (lens-induced)
    • Retinal Detachment
    • Amblyopia (in children, if uncorrected)
  • Diagnostic Aids:

    • Slit-lamp: Iridodonesis, phacodonesis, vitreous in AC.
    • Gonioscopy: To assess angle for glaucoma.
    • USG B-scan: For posteriorly dislocated lens.
  • Management:

-   **Conservative**: Observation, regular refraction, optical correction (aphakic glasses/contacts), Mydriatics/Miotics (judicious use), Amblyopia therapy.
-   **Surgical Indications**: Significant ↓ vision, progressive subluxation, lens-induced glaucoma or uveitis, intractable diplopia.
    +   Options: ICCE, ECCE/Phaco + IOL (with Capsular Tension Ring (CTR), Cionni ring, or scleral/iris fixation of IOL), Pars Plana Lensectomy (PPL).

⭐ Management of homocystinuria, a key systemic cause of bilateral ectopia lentis (typically inferonasal), includes high doses of pyridoxine (Vitamin B6).

High‑Yield Points - ⚡ Biggest Takeaways

  • Lens subluxation signifies partial displacement; dislocation indicates complete displacement from the patellar fossa.
  • Trauma is the most frequent cause; systemic associations include Marfan syndrome and homocystinuria.
  • In Marfan syndrome, the lens typically displaces superotemporally (upwards and outwards).
  • In homocystinuria, the lens characteristically displaces inferonasally (downwards and inwards).
  • Key symptoms include ↓ vision, monocular diplopia, iridodonesis (tremulous iris), and phacodonesis (tremulous lens).
  • Major complications are pupillary block glaucoma, cataract formation, and retinal detachment.
  • Management involves optical correction or lens extraction for visually significant or complicated cases.

Practice Questions: Lens Subluxation and Dislocation

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Ectopia lentis in a child is seen in which of the following diseases?

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Flashcards: Lens Subluxation and Dislocation

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_____ syndrome is a type of IOL malposition where intraocular lens subluxes superiorly

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_____ syndrome is a type of IOL malposition where intraocular lens subluxes superiorly

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