Eals disease Full details for. Md exams

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Eales disease retinal vasculitis fundus fluorescein angiography

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Eales Disease — Complete MD Exam Notes


1. Definition

Eales disease is an idiopathic obliterative retinal vasculopathy characterized by:
  • Peripheral retinal periphlebitis (venous inflammation)
  • Retinal ischemia / neovascularization
  • Recurrent vitreous hemorrhage
Primarily affects young healthy males in their 2nd–4th decade. It is predominantly described in the Indian subcontinent, Middle East, and Southeast Asia.

2. Etiology & Pathogenesis

Although labeled idiopathic, the following associations are well-established:
FactorDetails
Mycobacterium tuberculosis (MTB)Most strongly implicated; tuberculin sensitivity found in >80% cases; MTB DNA detected in epiretinal membranes by PCR
Hypersensitivity reactionNot direct infection — rather a Type IV hypersensitivity to tuberculoprotein in retinal vessels
Oxidative stressElevated free radicals damage retinal vascular endothelium
Autoimmune mechanismsAnti-retinal antibodies, T-cell-mediated vasculitis
Other associationsFocal sepsis (dental/tonsillar), sickle cell trait, syphilis (historically)
Pathology: Periphlebitis → venous occlusion → retinal ischemia → VEGF release → neovascularization → vitreous hemorrhage → fibrovascular proliferation → traction retinal detachment.

3. Stages (Standard Classification — Saxena et al.)

StageFeatures
Stage I (Periphlebitis)Ia: Periphlebitis of peripheral small veins; Ib: Periphlebitis of large veins, with or without macular involvement
Stage II (Ischemia)IIa: Capillary non-perfusion (peripheral); IIb: Neovascularization (disc or elsewhere — NVD/NVE)
Stage III (Vitreous hemorrhage)IIIa: Non-clearing vitreous hemorrhage; IIIb: Dense non-clearing vitreous hemorrhage
Stage IV (Fibrosis/Tractional RD)IVa: Firovascular proliferation; IVb: Tractional/combined RD

4. Clinical Features

Symptoms

  • Sudden, painless decrease in vision (due to vitreous hemorrhage) — most dramatic presentation
  • "Floaters" or "cobwebs" in the visual field
  • Recurrent episodes of visual loss
  • Vision may return to normal between episodes

Signs (on fundus examination)

Early (Periphlebitis stage):
  • Perivenous sheathing ("candle-wax drippings" appearance along veins)
  • Retinal hemorrhages (flame-shaped, peripheral)
  • Disc edema (sometimes)
Ischemic stage:
  • Areas of capillary non-perfusion in peripheral retina (best seen on FFA)
  • Cotton-wool spots
Proliferative stage:
  • Neovascularization of disc (NVD) or elsewhere (NVE)
  • Vitreous hemorrhage
  • Pre-retinal hemorrhage
Late:
  • Fibrovascular membranes
  • Tractional retinal detachment
  • Rubeosis iridis (neovascular glaucoma — rare)
  • Complicated cataract

Characteristic pattern: Bilateral but asymmetrical


5. Investigations

1. Fluorescein Fundus Angiography (FFA) — Gold Standard

  • Most important diagnostic tool
  • Shows:
    • Peripheral capillary non-perfusion (hallmark)
    • Venous staining and leakage
    • Neovascularization (hyperfluorescent leakage)
    • Arteriovenous shunts
Eales Disease FFA
Ultra-widefield fundus and FFA showing peripheral vascular sheathing (arrows), inferotemporal NVE (arrowhead), deep intraretinal hemorrhages (asterisk), peripheral capillary non-perfusion (asterisks), and hyperfluorescent neovascular leakage. Post-treatment images show stabilization after anti-tubercular therapy, bevacizumab, and PRP.

2. Tuberculosis workup

  • Mantoux/Tuberculin skin test (positive in >80%)
  • Chest X-ray (calcified hilar lymph nodes, old TB focus)
  • Quantiferon-Gold / IGRA (Interferon Gamma Release Assay)
  • PCR for MTB DNA in vitreous/epiretinal membranes (research)

3. Blood investigations

  • CBC (rule out sickle cell, polycythemia)
  • ESR, CRP (elevated in active inflammation)
  • VDRL/TPHA (rule out syphilis)
  • Blood sugar (rule out DM)
  • Serum protein electrophoresis

4. B-scan Ultrasonography

  • When vitreous hemorrhage precludes fundus view
  • Detects vitreous hemorrhage, fibrous bands, traction RD

5. OCT (Optical Coherence Tomography)

  • Macular edema, epiretinal membrane, subfoveal changes

6. Differential Diagnosis

ConditionDifferentiating features
Branch retinal vein occlusion (BRVO)Older age, hypertension, unilateral, at AV crossing
Sickle cell retinopathySickle cell trait/disease, salmon-patch hemorrhages, "sea-fan" NV
Diabetic retinopathyDM, bilateral, dot-blot hemorrhages, microaneurysms
Sarcoid retinal vasculitisBilateral uveitis, periphlebitis, systemic sarcoidosis findings
Behcet's diseaseOcclusive vasculitis, systemic features (oral ulcers, skin)
Retinal periphlebitis in MSNeurological symptoms, optic neuritis
Familial Exudative Vitreoretinopathy (FEVR)Family history, neonatal onset

7. Treatment

A. Medical Management

DrugIndication / Details
Anti-Tubercular Therapy (ATT)Cornerstone — given to all patients given TB association; 4-drug regimen (HRZE) for 6–9 months
Oral corticosteroidsActive periphlebitis/inflammation (Prednisolone 1 mg/kg/day, tapered)
Periocular/intravitreal steroidsRefractory uveitis/macular edema
AntioxidantsVitamin E, C — adjunctive

B. Laser Treatment

  • Pan-Retinal Photocoagulation (PRP)Treatment of choice for neovascularization/ischemia
    • Applied to ischemic retina to regress NV and prevent vitreous hemorrhage
    • Indicated in Stage IIb and above

C. Anti-VEGF Therapy

  • Intravitreal Bevacizumab / Ranibizumab
  • Used as adjunct to PRP for:
    • Active neovascularization
    • Pre-operative regression of NV before vitrectomy
    • Macular edema

D. Surgical Management

ProcedureIndication
Pars Plana Vitrectomy (PPV)Non-clearing vitreous hemorrhage (>3 months), tractional RD, combined RD
Membrane peelingFibrovascular proliferation causing traction
Scleral bucklingCombined rhegmatogenous + tractional RD
Neodymium:YAG laserVitreous strand lysis

8. Prognosis

  • Good if diagnosed early (Stage I–II) and treated promptly
  • Visual prognosis depends on:
    • Macular involvement (macular edema, ischemia)
    • Extent of vitreous hemorrhage
    • Tractional RD involving macula
  • Recurrences are common; long-term follow-up essential
  • Bilateral involvement occurs in ~50–80% of cases

9. Key Exam Points / High-Yield Facts

PointDetail
Who gets it?Young healthy Indian males, 20–40 years
Most common presentationSudden painless visual loss due to vitreous hemorrhage
Gold standard investigationFluorescein Fundus Angiography (FFA)
Hallmark FFA findingPeripheral capillary non-perfusion
Most common vascular involvedPeripheral retinal veins (periphlebitis)
Best known causative associationMycobacterium tuberculosis (hypersensitivity)
Treatment of active NVPan-Retinal Photocoagulation (PRP)
Medical treatment backboneAnti-tubercular therapy (ATT) + steroids
Surgical treatmentPars Plana Vitrectomy
Named afterHenry Eales (1880) — described recurrent vitreous hemorrhage in young men

10. Mnemonic

"EALES" — Early Periphlebitis → Abnormal FFA (capillary non-perfusion) → Leakage / NV → Excess bleed (vitreous hemorrhage) → Surgery (PPV) if needed

Bottom line for exams: Eales disease = young Indian male + sudden painless vision loss + vitreous hemorrhage + peripheral retinal periphlebitis + positive Mantoux. FFA is gold standard. Treat with ATT + steroids + PRP. PPV for non-clearing vitreous hemorrhage or tractional RD.
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