Spotlight Case: A Case of White Chorioretinal Lesions Following Retinal Detachment Surgery

  • author profile pictureHeena R. Patel, MD
  • author profile pictureThomas A. Albini, MD, ASRS Website Committee Chair

Case history

A 52-year-old man presented with decreased vision in the left eye for 2 days. 

His medical history was significant for insulin-dependent diabetes mellitus and dialysis treatment, hypertension, and hyperlipidemia. His ocular history was significant for 3 retinal detachment surgical procedures in the right eye. Family history was significant for diabetes mellitus in his father.

Figure 1: Color funduscopic photo of the right eye

Figure 2: Color funduscopic photo of the left eye

On examination, his visual acuity (VA) was no light perception in the right eye and 20/25 in the left eye. Anterior segment exam showed a surgical pupil with neovascularization of the iris in the right eye and 1+ cell in the anterior chamber of the left eye. Funduscopic view of the right eye was hazy with hemorrhages obscuring the view of the posterior pole. The left eye had 1+ vitreous cell and multiple, deep chorioretinal lesions throughout the posterior pole and peripheral fundus. Subretinal fluid was present anterior to the equator inferiorly. Fundus photos, optical coherence tomography (OCT), fluorescein angiography (FA), and indocyanine green (ICG) angiography are shown in Figures 1-5.
 

Diagnosis

Based on clinical presentation and imaging findings, the patient was diagnosed with sympathetic ophthalmia (SO). The recent history of retinal detachment surgery with white chorioretinal lesions on exam, subretinal fluid on OCT, and hypofluorescent lesions on ICG are suggestive of SO.

Figure 3: Montage color funduscopic photo of the left eye.
 

Management

Additional labs were ordered, including:

  • Antinuclear antibody (ANA)
  • Antineutrophil cytoplasmic antibody (ANCA)
  • Angiotensin-converting enzyme (ACE)
  • QuantiFERON-TB Gold (Cellestis Limited, Carnegie, Victoria, Australia)
  • Fluorescent treponemal antibody-absorption test (FTA-ABS)
  • Rapid plasma reagin (RPR)

All were negative. 

The patient was started on oral prednisone 60 mg daily followed with a slow taper. He was started on mycophenolate mofetil for long-term immune regulation in consultation with the patient’s internist. On follow-up, the subretinal fluid resolved (see Figure 6), but the white chorioretinal lesions persisted.
 

Figure 4: OCT images of the left eye, demonstrating subretinal fluid and a thickened choroid

Discussion

SO is a rare, bilateral granulomatous posterior or panuveitis that occurs following either accidental or surgical trauma to one eye. The incidence of SO is estimated at 0.03 out of 100,000/yr.[4] The trauma is frequently penetrating, but cases of non-penetrating trauma have been reported as inciting causes.

Recent reports have shown that ocular surgery, specifically vitreoretinal surgery, has become a major risk factor estimated at 0.06%.[2]

  • Eighty percent of cases occur within 3 months of injury, and 90% occur within 1 year (range: 5 days to 66 years; peak: 4 to 8 weeks).[1]
  • Before treatment was available, 70% of eyes became permanently blind, while 20% of cases ran a relatively mild course with only moderate visual loss.
  • Since the advent of corticosteroids, 65% of patients retain a final VA of 20/60 or better, and 93% retain 20/400 or better.[1]

Exposure to ocular antigens previously sequestered from immune surveillance results in autoimmune destruction of the choroid and retina of both eyes.

Many theories have been postulated to describe the pathogenesis of SO, including delayed-type hypersensitivity reaction to retinal and uveal antigens, specifically to retinal S-antigen. The hypersensitivity reaction has been demonstrated in animal models resembling clinical and histopathologic SO in humans, with inflammation primarily affecting the choroid. However, circulating anti-S-antigen antibodies have not been detected in the serum of humans with SO.[5]

The histocompatibility leukocyte antigen (HLA) haplotype HLA-DRB1*04 is a genetic predisposition to developing SO.[5] Patients with SO and DRB1 were significantly more likely to develop SO within 3 months of last ocular injury and require ≥10 mg prednisone daily to control inflammation.[6]

Figure 5: FA/ICG (side by side) of the left eye, demonstrating multiple hypofluorescent and hyperfluorescent lesions at the level of the retinal pigment epithelium (RPE) and a hyperfluorescent disc on FA with hypofluorescent spots on ICG

Presenting symptoms may be:

  • Blurred vision
  • Decreased near vision due to impaired accommodation
  • Pain
  • Photophobia
  • Epiphora

Signs for diagnosis
 

Anterior segment:

  • Ciliary injection
  • Cell and flare
  • “Mutton fat” keratic precipitates (KP)
  • Anterior and posterior synechiae
  • Iris thickening

Posterior segment:

  • Vitritis
  • Retinal vasculitis
  • Papillitis/optic nerve edema
  • Subretinal yellow-white lesions (Dalen-Fuchs nodules)

        »  Not pathognomonic; found in other forms of granulomatous uveitis

  • Exudative retinal detachment

Management:

  • Prevention by enucleating inciting eye
  • Corticosteroids are first line

        »  Oral prednisone 1 to 2 mg/kg/day for 3 months

        »  If severe presentation, IV methylprednisolone 1g/day for 3 days followed by oral
            prednisone

        »  Rebound inflammation common during tapering, months 3 to 6

        »  Supplemental posterior subtenon’s Kenalog or intravitreal Kenalog (IVK) (Bristol-Myers
            Squibb Company, Princeton, NJ)
 

  • Steroid-sparing agents

        » Mycophenolate mofetil

        » Cyclosporine 5mg/kg/day

        » Azathioprine 2mg/kg/day divided into 3 doses for older patients

        » Chlorambucil[3]

        » Cyclophosphamide

Figure 6: OCT of the left eye, demonstrating improved subretinal fluid

To prevent the onset of SO, enucleation within 2 weeks of trauma is recommended. Once definitive signs of the disease have started in the second eye, enucleation of the injured eye is of little or no value and may be inadvisable except when it is blind or painful.

 

 
  

Take-home points

1. Prevention of SO requires prompt enucleation of inciting eye within 2 weeks of injury.

2. Once definitive signs of the disease have started in the second eye, enucleation of the injured eye is of little or no value.

3. Diagnosis of SO requires prompt treatment with intravenous or oral steroids.

4. SO requires long-term immunomodulatory therapy with mycophenolate mofetil, azathioprine, chlorambucil, or cyclosporine.

 

References

1. Chaithanyaa N, Devireddy SK, Kishore Kumar RV, Gali RS, Aneja V. Sympathetic ophthalmia: a review of literature [published online April 16, 2011]. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;113(2):172-176. doi:10.1016/j.tripleo.2011.01.036.

2. Ozbek Z, Arikan G, Yaman A, Oner H, Bajin MS, Saatci AO. Sympathetic ophthalmia following vitreoretinal surgery [published online July 9, 2009]. Int Ophthalmol. 2010;30(2):221-227. doi:10.1007/s10792-009-9313-z.

3. Patel S, Dodds EM, Echandi LV. Long-term, drug-free remission of sympathetic ophthalmia with high-dose, short-term chlorambucil therapy [published online October 25, 2013]. Ophthalmol. 2014;121(2):596-602. doi:10.1016/j.ophtha.2013.09.009.

4. Galor A, Davis JL, Flynn HW Jr, et al. Sympathetic ophthalmia: incidence of ocular complications and vision loss in the sympathizing eye. Am J Ophthalmol. 2009;148(5):704-710. doi:10.1016/j.ajo.2009.05.033.

5. Chu X, Chan CC. Sympathetic ophthalmia: to the twenty-first century and beyond. J Ophthalmic Inflamm Infect. 2013;3(1):49. doi:10.1186/1869-5760-3-49.

6. Davis JL, Mittal KK, Freidlin V, et al. HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Ophthalmol. 1990;97(9):1137-1142. doi:10.1016/S0161-6420(90)32446-6.


Financial disclosures

Dr. Patel – None.

Dr. Albini - ALLERGAN, INC: Consultant, Honoraria; BAUSCH + LOMB, INC: Consultant, Honoraria; GENENTECH, INC: Grants, Other; THROMBOGENICS, INC: Consultant, Honoraria.

Dr. Hau - SEQUENOM: Speaker, Honoraria; THROMBOGENICS, INC: Other, Honoraria.

Dr. Choudhry - ALLERGAN, INC: Advisory Board, Speaker, Honoraria; BAUSCH + LOMB, INC: Advisory Board, Honoraria; BAYER HEALTHCARE: Consultant, Speaker, Honoraria; NOVARTIS: Advisory Board, Speaker, Grants, Honoraria; OPTOS PLC: Speaker, Honoraria.

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