1. Sympathetic Ophthalmia
بسم الله الرحمن الرحيم
Sympathetic Ophthalmia
F. Kianersi M.D 1390 / 7 / 21

2. Sympathetic Ophthalmia
RED EYE and TRAUMA
Sympathetic Ophthalmia
SO is a Rare, Bilateral, Diffuse Granulomatous, Autoimmune Panuveitis that may develop after either surgical or accidental trauma to 1 eye (the Exciting eye), followed by a latent period and the appearance of Uveitis in the uninjured fellow eye (the Sympathizing eye).
Sympathetic ophthalmia is always a consideration in major ocular trauma. Note that you have only about 5-7 days to decide whether or not to remove the eye for once the uveitis has begun, enucleation is no longer beneficial. 2

3. History
The first written reference to appeared about 1000 A.D. Stating that "the right eye, when diseased, often gives suffering to the left. “
In the 16th century, Bartisch wrote in his textbook of ophthalmology that after injury in one eye, "the other good eye is in great danger.”

4. History Louis Braille was born on 1809.
At the age of 3, Braille injured his left eye with a stitching awl from his father's workshop.
This destroyed his left eye, and sympathetic ophthalmia led to loss of vision in his right eye.
Braille was completely blind by the age of 4.

5. History
The term Sympathetic Ophthalmia was coined by William MacKenzie in 1840.
In 1905, Ernest Fuchs described the classical microscopic findings in SO.
Since then the disease has became well recognized in ophthalmology.

7. Incidence
A century ago, the reported incidence of SO was about 2% following injury to one eye.
Significant improvements in the management of ocular trauma, together with the more widespread use of immuno - modulatory therapy, has led to an overall decrease in the SO.

8. Incidence
The most recent minimum incidence is 3 out of every 10,000,000 cases of penetrating injury or surgery resulted in SO.
It is thus obvious that the incidence of SO is extremely low.

9. Time of Onset after Trauma
The time between the injury in the “exciting” eye and the onset of SO ranges from 5 days to 66 years.
In recent reports, 1/3 of patients developed SO within 3 months and less than 1/2 did so within 1 year of injury.

11. Clinical Features
SO is manifested by a gradual onset with a progressive course marked by frequent periods of worsening.
Patients with SO typically present with asymmetric bilateral Panuveitis, with more severe inflammation in the exciting eye than in the sympathizing eye, at least initially.

12. Clinical Features
Signs and symptoms in the sympathizing eye vary in their severity and onset, ranging from minimal problems in near vision, mild photophobia, and slight redness to severe granulomatous anterior uveitis.

13. Clinical Features
Both eyes may show cellular infiltration in AC with mutton-fat KPs, thickening of the iris from lymphocytic infiltration, and PS formation.

14. Clinical Features
The most common ocular findings in the anterior chamber are mutton fat KPs and variable degrees of inflammatory reaction.

15. Clinical Features
The posterior segment involvement is the most characteristic and may include:
Vitreous Cells,
Retinal Vasculitis,
Papillitis,
Choroiditis, and
Serous RD.

16. Clinical Features
The most classic findings, are whitish-yellowish infiltrates which are usually seen in the peripheral choroid and are named Dalen-Fuchs nodules.
They are seen in about one third of patients with SO.

17. Fluoresceine Angiography
The F/A reveals multiple hyper-fluorescent sites of leakage at the level of the RPE during the venous phase, which persists into the late stage.
There is often late staining of the optic nerve.

18. Histopathologic Features
Diffuse, Granulomatous, Non - necrotizing infiltration of the Choroid with a predominance of Lymphocytes.
Absence of inflammatory involvement of the Choriocapillaris and Retina.

19. Histopathologic Features
Dalen-Fuch's nodule is made up of epitheloid cells and lymphocytes and is found between the RPE and the Bruch's membrane.

21. Etiology The precise etiology of SO is unknown.
In the overwhelming majority of patients, there is a history of penetrating ocular injury complicated by incarceration of uveal tissue.
SO may result from altered T-Iymphocyte responses to ocular self-antigens such as retinal S-antigen or to other retinal or choroidal melanocyte antigens.

23. Etiology
There maybe a genetic predisposition to the development of the disease, as patients with SO are more likely to express HLA-DR4, DRw53, and -DQw3 haplotypes.

24. Surgical Procedures that may lead to SO
Deep Vitrectomy
Iridencleisis
Trabeculectomy
YAG laser Cyclodestruction
Cyclocryotherapy
Proton beam and helium ion irradiation for Choroidal Melanoma
Secondary IOL placement
Cataract extraction, particularly when the iris is entrapped within the wound
Scleral Buckling

25. Differential Diagnostic
Any causes of panuveitis, including:
VKH,
TB,
Sarcoidosis,
Syphilis,
Fungi,
Traumatic or Postoperative Endophthalmitis.

26. Differential Diagnostic
The clinical presentations of SO and VKH may be strikingly similar; however, a history of prior ocular injury is typically absent in patients with VKH.

28. Clinical Course
The course of SO is chronic, with frequent exacerbations, and if left untreated, leads to loss of vision and Phthisis Bulbi.

29. Complications
Structural complications of SO include:
Cataract,

30. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,

31. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,

32. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,
CNV,

33. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,
CNV,
Retinal Detachment,

34. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,
CNV,
Retinal Detachment,
Retinal and Optic Nerve Atrophy,

35. Fluoresceine Angiography in Late Cicatricial Phase
The areas correspond to Dalen-Fuchs nodules become atrophic, and appear on the angiogram as window defects.

36. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,
CNV,
Retinal Detachment,
Retinal and Optic Nerve Atrophy,
Subretinal Fibrosis and Choroidal Atrophy,

37. Complications Structural complications of SO include: Cataract,
Secondary Glaucoma,
CME,
CNV,
Retinal Detachment,
Retinal and Optic Nerve Atrophy,
Subretinal Fibrosis and Choroidal Atrophy,
Phthisis Bulbi.

39. Management
The course of SO is chronic, with frequent Exacerbations, and if left untreated, leads to loss of vision and Phthisis Bulbi.
Every attempt should be made to salvage eyes with a reasonable prognosis for useful vision with meticulous and prompt closure of penetrating injuries.

40. Management
Enucleation within 2 weeks of injury to prevent the development of SO should be considered in grossly disorganized globes with no discernible visual function.
Although controversial, Enucleation may still be preferred to Evisceration as the operation of choice for the removal of ocular contents in severely injured eyes.

41. Management
Enucleation of the exciting eye once SO has become established has not been shown to be beneficial in altering the disease course of the sympathizing eye.

43. Treatment
The mainstay of therapy for SO is immunomodulatory therapy, initially with systemic Corticosteroids.
Azathioprine,
Methotrexate,
Mycophenolate mofetil,
Cyclosporine,
Chlorambucil,
Cyclophosphamide.

44. Treatment
Topical corticosteroids, together with cycloplegic/mydriatic agents, are essential in the treatment of acute anterior uveitis associated with SO.
Periocular Corticosteroids are used in the management of inflammatory recurrences and CME.

46. Prognosis
SO remains a serious disease that may result in a very poor visual outcome without therapeutic intervention.
With prompt and aggressive aggressive systemic immunomodulation, the visual prognosis of SO is good, with 50% of patients achieving a final visual acuity of 20/40 or better in at least 1eye.

47. SYMPATHETIC OPHTHALMIA
RED EYE and TRAUMA
SYMPATHETIC OPHTHALMIA
(Bilateral Granulomatous Panuveitis after Trauma)
Onset: 5 days to 66 years after penetrating trauma
Onset: 33% at 3 mo., <50% after 1 year
Cause: antigen-antibody interaction
Risk: Very low
Removal of injured eye after onset does not help
Treatment: Aggressive Immunosuppressive therapy
Sympathetic ophthalmia is always a consideration in major ocular trauma. Note that you have only about 5-7 days to decide whether or not to remove the eye for once the uveitis has begun, enucleation is no longer beneficial. 47

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