Eales’ Disease

1. Eales’ Disease
Dr Gyanendra Lamichhane,
Dr I. Kansakar
Lumbini Eye Institute
Bhairahawa,Nepal

2. References:
•Retina and vitreous –AAO (2004-2005)
•Prospective study on idiopathic retinal vasculitis (Joshi S.N)
•Clinical ophthalmology- 5
th
edition (Jack J Kanski)
•Retina 3
rd
edition (Stephen J Ryan)
•Atlas of ophthalmology (R.K. Parrish)
•Principle and practice of ophthalmology (Peymen)
•Eales disease – An update Major Review (J. Biswas)
Survey of ophthalmology 2002

3. Introduction:
•In 1880 and 1882, Henry Eales -“primary recurrent retinal
hemorrhage”.
•Similar conditions of retinal and vitreous hemorrhage were
described under the name of Eales’ Disease.
•Eales didn’t mention any inflammatory signs preceding or
accompanying the hemorrhages.

4. •In 1887 Wadsworth reported on signs of inflammation of the
retinal vasculature - Eales’ disease and periphlebitis
•Elliot initially suggested that the disease be called
“periphlebitis retinae”.

5. •Currently, Eales’ disease is considered to be an idiopathic
inflammatory venous occlusion that primarily affects the peripheral
retina.
•Retinal changes include perivasculitis, mainly periphlebitis, and
peripheral non-perfusion.
•This inflammation induced vascular occlusion can lead to a
proliferative vascular retinopathy, with sequelae such as recurrent
vitreous hemorrhage and traction retinal detachment.

6. Aetiopathogenesis:
•Recognized as primary vasculitis of unknown etiology
occurring in young adults.
•Retinal vasculitis and peripheral retinal neovascularisation
associated with various systemic and ocular disease could
mimic Eales’ disease.

7. Systemic disease associated with Eales’
disease:
Systemic disorders associated with Eales’ disease:
Tuberculosis
Hypersensitivity to tuberculoprotein
Thromboangitis obliterans
Neurologic disease
Hematological abnormalities

8. Tuberculosis:
•The assumption of tubercular aetiology is based on active or
healed tuberculosis in some patient with Eales’ disease.
•Ophthalmoscopic evaluation in patient with active or healed TB
showed 1.3% had Eales’ disease.

9. Tuberculosis: cont…d
•Presence of Tubercular bacilli DNA in epiretinal
membrane (Madahavan et al)
•2010 eyes with active pulmonary or extra pulmonary TB
– no Eales’ disease (Biswas J et al)
•32 patient with Eales’ disease were followed up for 37
years, only one patient had active tuberculosis (William
et al)

10. Hypersensitivity to tuberculoprotein:
•Allergic reaction to tuberculosis has been reported by
many authors till date.
•Positive Mantoux reaction which is as high as 90% in
some series.

11. Hypersensitivity to tuberculoprotein:
cont…d
•Ashton – retina sensitized against tuberculoprotein and
re-exposure leads to retinal vasculitis.
•Eales’diease has been reported in Mantoux negative
patients and mantoux test positive in 67-90% of healthy
individuals.

12. Systemic disease:
•Several studies have shown association between neurological
and hematological disease.
•bilateral hearing loss 48% (Renie et al) , 25% (William et al).
•2 pt with Eales’ disease had progressive worsening of
neurological deficit (Rodier G).
•Myelopathy with Eales’ disease has been described by many.

13. Immunological studies in Eales’ disease:
•Immune mediated mechanism has been suggested by many
authors as a possible cause of Eales’ disease.
•Acute onset, steroid responsiveness, lymphocytic infiltration
and abnormal immunological parameters all indicate an
immunological basis of disease.

14. Immunological studies in Eales’ disease:
cont…d
•Altered immune response of type III and/or IV reaction to an
infectious agent (Muthukaruppan et al).
•Raised IgG and IgA levels (Johnson et al), elevated levels
of circulating immune complexes and antiretinal antibody
(Kasp et al) , immunophenotyping predominant T cell CD4
•Higher frequencies of HLA B5(B51), DR1 and DR4 (Biswas
et al)

15. Biochemical studies in Eales’ disease:
•Raised alpha-globulins and reduced albumin levels in
the serum samples.
•PDGF, IGF1, EDF, TGFa and TGFb play a key role in
neovascularisation.
•Raised serum alpha1 acid glycoproteins in 27 patients
of Eales’ disease (Sen et al).

16. Stages of Eales’ disease
•Stage I (inflammatory stage)
Localized areas of peripheral
retinal edema with sheathing of
the smaller caliber vascular
branches.
Minute retinal hemorrhages as
well as minute vascular brackets
or hooklets connecting two
adjoining vessels.
active periphlebitis with subhyaloid hemorrhage
over the macula.

17. Active periphlebitis with tortuosity of veins as well
as multiple superficial retinal hemorrhage

18. Montage fundus photograph showing an active perivasculitis involving
predominantly the peripheral retina of an Eales disease patient.

19. •Stage II (ischemic stage)
Involvement of larger vessels
and extend more posteriorly
Veins as well as arterioles may
be sheathed
Widespread retinal hemorrhages
and vitreous looks hazy

20. •Stage III (stage of
neovascularisation)
Peripheral new blood
vessels with numerous
vitreous and retinal
hemorrhages.
The hemorrhages
frequently recurs.

21. Sea fan–like neovascularisation of the retina.

22. •Stage IV (complicated stage)
Massive retinal proliferans
associated retinal and massive vitreous
hemorrhage.
With this advanced disease the
neovascular frond can cause tractional
rhegmatogenous retinal detachment.

24. Clinical features:
•Usually occurs in young , healthy people, with a peak
incidence between the ages of 30 and 40 years.
•It occurs more frequently in males 80-90%.
•75% cases it presents before 49 years.
•Can be unilateral or bilateral.90% bilateral (Duke Elder)
56.14% had bilateral retinal vasculitis( O.K Malla and
coworkers)

25. •Vitreous floaters or blurring of vision, symptoms
attributable to recurrent vitreous hemorrhages.
•80% between the age of 20-40 years and 95% were
male (O.K Malla and co workers)
54.34% between 20-30 years and 94.73% male
•Rare in more developed countries.

26. •More commonly reported from Indian subcontinent. The
reported incidence in India is 1 in 200-250 patient
•Anterior uveitis/Vitritis
•Active perivasculitis with exudates around the veins in
one or more quadrants. Arterioles may be affected.

27. Healed perivasculitis as sheathing of the veins
Macular changes uncommon
Peripheral retinal neovascularisation reported in 36-84% of cases
Recurrent vitreous hemorrhages, the hall mark of the disease
Some vitreous hemorrhages resolve, some do not
( organize with multiple VR adhesions & RRD/TRD)
Some patient specially with multiple sclerosis are asymptomatic.

28. Proliferative stage

29. Vitreous hemorrhage

30. Eales’ disease
Exacerbations and remissions
quiescent
Rubeosis iridis
Hemorrhagic glaucoma
cataract
Loss of eye
Tractional RD
Macular distortion
Detachment
Cystoid macular degeneration
and
Macular holes
Tractional retinal breaks and
Rhegmatogenous RD

31. Healed perivasculitis with anastomotic arteriovenous shunt

32. Fibrovascular proliferation causing tractional retinal detachment

33. Healed perivasculitis with sclerosed vein and multiple
chorioretinal atrophic patches

36. Fundus fluorescein angiography
•To delineate areas of capillary nonperfusion, peripheral
retinal nonperfusion is present in all patients with Eales’
disease.
•Retinal or disc neovascularisation
•Macular edema
•Helps in monitoring the regression and disappearance of
new vessels during treatment and follow up.

38. FFA following laser photocoagulation of neovascular frond

39. Multiple veno venous shunts in late AV phase

40. Pathology:
•Patchy perivascular or intramural infiltration of lymphocytes or
granulation tissue sometimes with or without giant cells
•Plasma cells are occasionally present.
•Veins are primarily affected
•The vascular changes are usually seen on retinal periphery.

41. •Hyalinization and thinning of vein wall
•Narrowing and obstruction of the lumen
•Endothelial cell proliferation
•Thrombosis and rupture of the vein
•Intravitreal new vessel formation and
•Marked thickening of internal limiting membrane have been
reported.

42. Diagnostic studies performed on patients with
Eales’ disease
I.To rule out leukemia and hematological condition:
Hemoglobin and hematocrit
Total RBC count
Total WBC and Differential count

43. II. Others tests:
Platelet count
ESR
Reticulocyte count
Blood sugar
Stool analysis
Mantoux test
Basic coagulation test
Sickle cell preparation

44. Hemoglobin Electrophoresis
VDRL and TPHA test
Anti nuclear antibody
Serum ACE
Lysosome

45. Sarcoidosis Wegener Granulomatosis
III. Radiological tests:

46. Differential diagnosis:
Vasculitis mimicking Eales’ disease
Systemic Ocular
Behcet’s disease Birdshot retinochoroidopathy
Leukemia Coat’s disease
Lyme Borreliosis Pars planitis
Multiple sclerosis Viral retinitis
Sarcoidosis
Systemic lupus erythematosus
Toxocariasis
Toxoplasmosis
Tuberculosis
Wegener’s granulomatosis

47. Proliferative vascular retinopathy
mimicking Eales’ disease:
Systemic Ocular
Diabetes mellitus BRVO
Sarcoidosis CRVO
Sickle cell disease Coats’ disease
Pars planitis
ROP

48. Sarcoidosis
Sarcoid nodules Bilateral hilar lymphadenopathy

49. Candle wax dipping

50. Vitritis and snowballPeripheral neovascularisation

51. leukemia

52. Sickle cell retinopathy
Seafan neovascularisation

54. Behcet disease
Aphthous ulceration Erythema nodusum like lesions

55. Dermatographism Hypopyon

56. Occlusive vasculitis
Retinal exudation and vascular occlusion

57. Treatment:
•Symptomatic treatment.
•Treatment aim
reducing retinal perivasculitis and associated vitritis;
reducing risk of vitreous hemorrhage from new vessels
by retinal ablation and surgical removal of non resolving
vitreous hemorrhage and/or vitreous membranes.

58. Treatment of Eales’ disease:
•Observation.
•Medical
•Corticosteroids
•Antituberculosis drugs
•Immunosuppressive drugs.


•Retinal ablation
•Photocoagulation
•cryotherapy
•Surgical
•vitrectomy

59. Observation:
•Patient with inactive retinal vasculitis
•Follow up 6 months to 1 year interval.
•Patient with fresh vitreous hemorrhage if retina is found
to be attached.
•Such vitreous hemorrhage usually clears by 6 to 8
weeks.

60. Medical therapy:
•Corticosteroids are mainstay of therapy in active perivasculitis
stage of Eales’ disease.
•Majority of cases 1mg/kg body weight, tapered to 10mg/week
over 6 to 8 weeks.
•Maintenance 15 to 20mg/day for 1 to 2 months.
•Periocular depot steroid injection may be added for associated
macular edema.

61. •Systemic and Periocular steroid useful in patient having
3 quadrants involvement with macular edema.
•Systemic steroid only if less than 3 quadrant
involvement.
•No difference in response between Mantoux positive
and negative cases.

62. •Immunosuppressive therapy in patient unresponsive or
have unacceptable side effects.
(Azathioprine and cyclosporine)
•Some investigators have recommended ATT
(Rifampicin and Isoniazid) for 9 months.

63. Photocoagulation:
•Mainstay of therapy in proliferative stage of Eales’ disease.
•The aim
Regulate the circulation
To obliterate surface neovascularisation and
Close leaking intraretinal microvascular abnormalities.

64. •Sectoral laser for capillary non
perfusion and PRP for
neovascularisation of disc.
•Occasional massive
hemorrhage can occur.
•After laser, regressing
neovascularisation can cause
macular distortion and retinal
tear.
Laser not advised in active inflammatory stage

65. FFA following laser photocoagulation of neovascular frond

66. Vitreoretinal surgery:
•Vitrectomy alone or combined with other vitreoretinal surgical
procedures is often required.
•Nonresolving vitreous hemorrhage with obscuration of
central vision of 3 mo duration may be subjected to
vitrectomy.

67. •Vitrectomy done between 3 to 6 mo has better results
than done after 6 months (Kumar et al).
•Early vitrectomy in patient with TRD, extensive vitreous
membranes or epimacular membranes.
•Endolaser can be given along with vitrectomy.

68. Tractional radial retinal fold after vitrectomy

69. Summary and conclusions:
•Characteristic clinical findings and angiographic pattern.
•Mimic several ocular or systemic disease presenting as
retinal vasculitis or proliferative retinal vasculopathy.
•Hypersensitivity to tubercular protein has been considered a
prime cause of Eales’ disease.

70. •Probable multifactorial etiology.
•HLA, retinal autoimmunity, mycobacterium genome, free
radical mediated damage.
•Corticosteroids in active disease and laser photocoagulation in
ischemic and proliferative stage.
•Results of vitrectomy in non resolving vitreous hemorrhage
with or without retinal detachment are satisfactory.