Fellow eye’s revenge: Navigating sympathetic ophthalmia following penetrating keratoplasty

INTRODUCTION

Sympathetic ophthalmia (SO) represents a rare, bilateral granulomatous panuveitis that targets the uveal tract, usually sparked by trauma or surgery to one eye (the inciting eye), prompting an autoimmune attack on the uninjured fellow eye (sympathizing eye).^[1-5]

Clinically, it typically emerges gradually with symptoms including vision blurring, light sensitivity, ocular ache, and redness, often featuring episodes of worsening and improvement.

Pathologically, the condition involves widespread or clustered infiltration of the uvea by lymphocytes and epithelioid cells, occasionally forming distinctive Dalen–Fuchs nodules in the choroid.^[6]

CASE REPORT

A 48-year-old male presented with decreased vision and floaters in the left eye (LE), 3 weeks after undergoing TPK with extracapsular cataract extraction (for a cataractous lens) in the right eye (RE) due to a fungal corneal ulcer. Visual acuity was hand motions positive in the RE and counting fingers at counting fingers distance in the LE. Slit-lamp examination of the RE showed a full-thickness corneal graft secured with multiple interrupted 10-0 nylon sutures; the graft was hazy and edematous with dense fibrinous exudate and pupillary membrane in the anterior chamber [Figure 1]. The LE had 1+ cells in the anterior chamber and a clear lens. Dilated fundus examination of the LE revealed exudative retinal detachment (RD). Wide-field fundus photography showed green–yellow retinal expanse indicative of serous exudative RD, with subretinal fluid causing retinal thickening and shifting; a darker central foveal shadow suggested shallow detachment [Figure 2]. Fundus autofluorescence demonstrated multiple patchy hyper- and hypo-autofluorescent areas at the posterior pole, corresponding to retinal pigment epithelium (RPE) disturbance from serous RD and inflammation, consistent with granulomatous choroidal involvement [Figure 3]. Optical coherence tomography (OCT) confirmed serous (exudative) RD with hyporeflective subretinal fluid and undulating/ wavy RPE/outer retinal layers due to underlying choroidal inflammation [Figure 4]. Fundus fluorescein angiography showed multiple pinpoint hyperfluorescent leaks at the RPE level in early phases; progressive dye pooling in serous RD areas in late phases; disc hyperfluorescence (leakage) due to inflammation; and diffuse choroidal leakage with mottled hyperfluorescence indicating active choroiditis [Figure 5].

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Figure 1: Right eye: Post therapeutic penetrating keratoplasty showing a full-thickness corneal graft secured with multiple interrupted 10-0 nylon sutures; the graft was hazy and edematous with dense fibrinous exudate and pupillary membrane in the anterior chamber. Left eye: Pharmacologically dilated pupil.

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Figure 2: Wide-field fundus photography showed green-yellow retinal expanse indicative of serous exudative retinal detachment, with subretinal fluid causing retinal thickening and shifting; a darker central foveal shadow suggested shallow detachment.

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Figure 3: Fundus autofluorescence demonstrated multiple patchy hyper- and hypoautofluorescent areas at the posterior pole, corresponding to retinal pigment epithelium disturbance from serous retinal detachment and inflammation, consistent with granulomatous choroidal involvement.

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Figure 4: Optical coherence tomography confirmed serous (exudative) retinal detachment with hyporeflective subretinal fluid and undulating/wavy retinal pigment epithelium/outer retinal layers due to underlying choroidal inflammation.

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Figure 5: Fundus fluorescein angiography showed multiple pinpoint hyperfluorescent leaks at the retinal pigment epithelium level in early phases; progressive dye pooling in serous retinal detachment areas in late phases; disc hyperfluorescence (leakage) due to inflammation; and diffuse choroidal leakage with mottled hyperfluorescence indicating active choroiditis.

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Systemic corticosteroid therapy was initiated with intravenous methylprednisolone at a dose of 1 mg/kg/day for 3 days, followed by oral prednisolone at 1.5 mg/kg/day, tapered over 3 months. Mycophenolate mofetil (1 g twice daily) was added concurrently as a steroid-sparing agent. Following treatment, the exudative RD resolved, fluorescein angiographic leakage subsided, and best-corrected visual acuity in the LE improved to 0.2 logMAR.

DISCUSSION

SO following therapeutic penetrating keratoplasty (TPK) is a rare but potentially vision-threatening complication, attributed to significant uveal tissue exposure and immune sensitization in an already inflamed eye. Early systemic immunosuppression is crucial to prevent irreversible visual loss, highlighting the need for close post-operative monitoring of the fellow eye.^[7]

In the present case, SO developed 3 weeks after TPK for fungal keratitis, presenting as exudative RD in the fellow eye without significant anterior segment inflammation. This posterior segment–predominant presentation can delay diagnosis if classical anterior uveitis signs are absent. Multimodal imaging aided early detection, and prompt treatment with systemic corticosteroids and mycophenolate mofetil resulted in complete anatomical resolution and significant visual recovery, with no recurrence on follow-up.

Bhandari et al. similarly reported SO after TPK with an atypical presentation characterized by predominant posterior segment involvement and minimal anterior chamber reaction. Early corticosteroid therapy led to favorable outcomes, reinforcing the importance of high clinical suspicion in such cases.^[8]

Magalhães et al. highlighted the role of imaging, particularly OCT, in demonstrating multilobular serous RD with septations, which may mimic Vogt–Koyanagi–Harada disease and aid in diagnosis.^[9]

The temporal profile described by Damico et al. indicates that most cases occur within the first few months after the inciting event, consistent with the early onset observed in our patient and supporting the need for continued surveillance after TPK.^[10]

Overall, post-TPK SO may present atypically with predominant posterior involvement. Early recognition and prompt immunosuppressive therapy are essential for achieving favorable visual outcomes.

CONCLUSION

Sympathetic ophthalmia after therapeutic penetrating keratoplasty is an uncommon but vision-threatening complication that may present predominantly with posterior segment involvement. Careful postoperative surveillance of the fellow eye, supported by multimodal imaging, and early systemic immunosuppressive therapy are essential for optimizing visual outcomes.