From Vitreoretina Services (MA, SPC, RN, KA), Dr. Shroff’s Charity Eye Hospital, New Delhi; and Ocular Pathology Services (JB), Sankara Nethralaya, Chennai, India.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Manisha Agarwal, MS, DNB, Dr. Shroff’s Charity Eye Hospital, 5027, Kedar Nath Road, Daryaganj, New Delhi-110002, India.
We report the case of a 9-year-old boy with an atypical presentation of posterior sympathetic ophthalmia supported by fundus fluorescein angiography and optical coherence tomography findings and confirmed on histopathology of the exciting eye.
A 9-year-old boy presented with decreased vision in the left eye for 3 days. He had a history of cataract surgery with intraocular lens implantation at the age of 9 months followed by membranectomy in the right eye. He also had a history of blunt trauma with a finger to the right eye causing retinal detachment for which he underwent pars plana vitrectomy with intraocular lens repositioning. The patient was subsequently diagnosed as having recurrent total retinal detachment for which no intervention was advised.
On examination, the best-corrected visual acuity was inaccurate projection of rays in the right eye and 6/24, N24 in the left eye. Goldmann applanation tonometry recorded normal intraocular pressure in both eyes. Slit-lamp examination of the right eye showed organized blood clot in the anterior chamber with vascular ingrowth (Fig. 1) and the left eye showed clear lens with retrolental cells and flare. There was no view of the fundus in the right eye and the fundus examination of the left eye showed a pocket of subretinal fluid at the macula.
Figure 1. Right Eye Showing Organized Blood Clot in the Anterior Chamber with Vascular Ingrowth.
Ultrasound B-scan of the right eye showed reverberations of the intraocular lens with total retinal detachment. Fundus fluorescein angiography of the left eye showed multiple sites of choroidal leakage with coalescence of dye under serous detachment of the retina (Fig. 2). Optical coherence tomography of the left eye showed multiple pockets of subretinal and intraretinal fluid with hyperreflective echoes underneath the serous retinal detachment (Fig. 3).
Figure 2. (A–E) Color Fundus Photograph and Fundus Fluorescein Angiography of the Left Eye Showing Multiple Pinpoint Sites of Choroidal Leakage with Coalescence of Dye Under Serous Detachment of the Retina.
Figure 3. Optical Coherence Tomography of the Left Eye Showing Multiple Pockets of Subretinal and Intraretinal Fluid with Hyperreflective Echoes Underneath the Serous Retinal Detachment.
The child was treated with intravenous methylprednisolone 1 mg/kg body weight for 3 days followed by a tapering dose of oral prednisolone 1 mg/kg along with topical prednisolone acetate 1% six times a day and atropine 1% two times a day in the left eye. Enucleation of the right eye was advised, which was refused by the father of the child.
At 3 days of follow-up, there was no change in the visual acuity of the left eye. Fundus examination of the left eye and repeat optical coherence tomography showed a decrease in the subretinal fluid at the macula.
Best-corrected visual acuity in the left eye at 1 week of follow-up was 6/9, < N36 (pharmacologically dilated pupils under atropine 1%). Goldmann applanation tonometry recorded an intraocular pressure of 16 mm Hg in the left eye. Fundus examination of the left eye showed a residual pocket of shallow subretinal fluid.
At 1 month of follow-up, the best-corrected visual acuity in the left eye was 6/6, N9P. Goldmann applanation tonometry recorded an intraocular pressure of 38 mm Hg. Slit-lamp examination of the left eye showed no signs of inflammation. Fundus examination and optical coherence tomography of the left eye showed no evidence of subretinal fluid at the macula. Steroid-induced glaucoma was well controlled with oral and topical anti-glaucoma medications. Topical prednisolone 1% was stopped and oral prednisolone and anti-glaucoma medicines were tapered and stopped.
At 2 months of follow-up, the child presented with decreased visual acuity in the left eye recorded as 6/12P, N18P. Fundus examination and optical coherence tomography showed recurrence of sub-macular fluid.
The child was treated with intravenous methylprednisolone 1 mg/kg body weight for 3 days followed by oral and topical prednisolone along with anti-glaucoma medication. Oral azathioprine 1.5 mg/kg body weight was started after a systemic evaluation and complete blood counts. Enucleation of the right eye was done after an informed consent was obtained. Histopathology of the enucleated eye showed organized hemorrhage in the anterior and posterior chamber with histiocytic and foreign body giant cell and granulomatous reaction. Moderate chronic inflammation with retinal detachment and no evidence of malignancy were seen (Fig. 4).
Figure 4. Histopathology of the Enucleated Right Eye Showing Organized Hemorrhage in the Anterior and Posterior Chamber with Histiocytic and Foreign Body Giant Cell (black Arrow) and Granulomatous Reaction (hematoxylin–Eosin, Original Magnifcation ×200).
Sympathetic ophthalmia, also known as sympathetic uveitis, is a rare bilateral granulomatous panuveitis that occurs after a penetrating injury to an eye. Following injury to an eye caused by either surgery or accident, a variable period of time passes before a sight-threatening inflammation develops in both eyes. The injured eye is known as the exciting eye and the fellow eye as the sympathizing eye.1
The various surgical procedures implicated for sympathetic ophthalmia are cataract extraction (particularly when complicated), iris surgery (iridectomy), retinal detachment repair, vitrectomy, paracentesis, cyclodialysis, and keratectomy.2 The risk of sympathetic ophthalmia increases when these surgical procedures are accompanied or followed by additional operations, particularly in the posterior segment of the eye.3 Advances in eye care and especially in surgical techniques have dramatically decreased the incidence of sympathetic ophthalmia in the past century. The incidence of post-vitrectomy sympathetic ophthalmia has been estimated at 0.01%.4
Sympathetic ophthalmia uncommonly occurs after 3 months, but it may occur years after the traumatic event.2 The typical symptoms are photophobia, loss of accommodation, or watering, and signs include bilateral low grade persistent granulomatous uveitis and Dalen–Fuchs nodules in the retina, although these are not pathognomic of sympathetic ophthalmia.1 However, our case differed in the following ways. The child developed sympathetic ophthalmia several years after the last surgical intervention in the exciting eye, he presented with a sudden decrease in vision, and there was only mild retrolental cells and flare in the sympathizing eye with no signs of anterior segment inflammation in either eye. Finally, there was serous retinal detachment at the macula on fundus examination. All of these factors made the diagnosis of sympathetic ophthalmia difficult in our case, but we began treatment on the basis of clinical suspicion and were able to salvage the eye.
Enucleation of the exciting eye having no visual potential helps to prevent the recurrence of sympathetic ophthalmia by decreasing the antigen load causing inflammation repeatedly. Histopathology of the enucleated eye also helped in confirming the diagnosis of sympathetic ophthalmia in our case.
A variant of sympathetic ophthalmia with predominantly posterior segment signs and minimal anterior chamber reaction has been described by McPherson et al.5 In a retrospective study by Gupta et al.,6 36 of 40 patients had presented with predominantly posterior segment involvement.
The current case highlights that sympathetic ophthalmia may have an atypical clinical presentation or this may be an early stage of the classic presentation. A high clinical suspicion may be required for a timely diagnosis and management to salvage the vision in the sympathizing eye, which at many times is the only seeing eye of the patient.
- Duke-Elder S, Perkins ES. Diseases of the uveal tract. In: Duke Elder S, ed. System of Ophthalmology, vol. 9. London: Henry Kimpton; 1966:558–559.
- Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic ophthalmia: a clinicopathologic review of 105 cases (1913–1978). Ophthalmology. 1980;87:109–121.
- Goto H, Rao NA. Sympathetic ophthalmia and Vogt-Koyanagi-Harada syndrome. Int Ophthalmol Clin. 1990;30:279–285. doi:10.1097/00004397-199030040-00014 [CrossRef]
- Gass JD. Sympathetic ophthalmia following vitrectomy. Am J Ophthalmol. 1982;93:552–558.
- McPherson SD Jr, Dalton HT. Posterior form sympathetic ophthalmia. Trans Am Ophthalmol Soc. 1975;73:251–263.
- Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a single centre long-term study of 40 patients from North India. Eye. 2008;22:1459–1464. doi:10.1038/sj.eye.6702927 [CrossRef]