To describe the sequential multimodal imaging features of an isolated necrotic macular hole secondary to Toxoplasma retinochoroiditis.
A 23-year-old male was referred for surgical management of an idiopathic macular hole following sudden decrease in vision in his right eye. Right eye examination showed best-corrected visual acuity of 20/200, mild anterior segment inflammation, and a full thickness non operculated macular hole (MH) with ill-defined ragged margins and surrounding strip of pallid edema. Further multimodal imaging including optical coherence tomography (OCT), fundus autofluorescence (FAF), fluorescein angiography (FFA), and OCT-angiography confirmed the atypical configuration and inflammatory nature of MH. Serological tests showed elevated level of Toxoplasma gondii-specific antibodies. A diagnosis of necrotic isolated full-thickness MH secondary to toxoplasma retinochoroiditis was made. Patient was treated medically with anti-toxoplasma medication for 6 months. Sequential multimodal imaging highlighted the healing process of necrotic MH with vision improving to 20/80 at 6 months after presentation.
Conclusion and importance
A high level of suspicion and multimodal imaging plays an important role in accurate etiological diagnosis and management of atypical macular hole as in our case. Sequential multimodal imaging may provide an insight into the pathogenesis and healing pattern of such lesion.
Ocular toxoplasmosis, caused by the parasite Toxoplasma gondii, is the most common cause of infectious posterior uveitis in immunocompetent individuals worldwide. , The typical and most common presentation of ocular toxoplasmosis is recurrent episodes of unilateral focal necrotising retinochoroiditis accompanied by vitritis, frequently associated with an adjacent pigmented retinochoroidal scar. , Toxoplasma retinochoroiditis is frequently associated with the vitreoretinal complications such as epiretinal membrane, cystoid macular edema, vitreoretinal traction, choroidal neovascularization and retinal detachment. However, formation of macular hole secondary to toxoplasma retinochoroiditis has been rarely reported in literature.
We report a case of toxoplasma retinochoroiditis associated with macular hole and the process of healing following treatment with multimodal imaging.
A 23-year-old male was referred to our vitreoretinal services for surgical management of an idiopathic macular hole in his right eye. There was a history of sudden decrease in vision in the right eye for three weeks. There was no history of ocular trauma or any previous ocular complaints.
On examination, his best-corrected visual acuity (BCVA) was 20/200 in the right eye and 20/20 in the left eye. A slit-lamp examination of the right eye revealed 1+ cells and 1+ flare in anterior chamber, and 1+ cells in anterior vitreous face. Fundus examination revealed a full thickness non-operculated macular hole (MH) with ill-defined ragged margins and surrounding strip of pallid edema [ Fig. 1 A]. Rest of the fundus including periphery was normal. There was no evidence of posterior hyaloid detachment. The left eye examination was normal. Optical coherence tomography (OCT) of the right eye showed a full thickness MH with a loosely overhanging intact internal limiting membrane (ILM) and hyper-reflective material deposit at the base [ Fig. 1 B]. Due to the absence of classical configuration of MH in the OCT along with the presence of anterior segment inflammation and vitritis in a young patient without any history of ocular trauma, we suspected an inflammatory etiology of MH.
Fundus Autofluorescence (FAF) of the right eye showed a central hyper-autofluorescence corresponding to the area of clinically observed MH and a surrounding halo of hypo-autofluorescence corresponding to the retinal edema, which in turn was encompassed by another ring of hyper-autofluorescence. The outer ring of hyper-autofluorescence suggested that the inflammatory pathology extended well beyond the clinically observed lesion [ Fig. 1 C]. Fluorescence angiography (FFA) showed central blocked fluorescence caused by the central necrotic deposit, enlargement of foveal avascular zone in early phase, and leakage due to perifoveal edema in the subsequent phases resulting in multiple hyper-fluorescent satellite lesions suggestive of inflammatory activity [ Fig. 1 D and E]. OCT-angiography (OCTA) of the right eye revealed an enlargement of foveal avascular zone (FAZ) with the areas of capillary dropouts at the level of superficial and deep capillary plexus suggesting an ischemic insult [ Fig. 1 F and G]. OCTA image at choriocapillaris layer showed low flow suggesting sub foveal choroidal hypoperfusion [ Fig. 1 H].
The inflammatory work-up included routine blood tests and serological tests for Toxoplasma gondii (T. gondii), Human immunodeficiency virus and Treponema pallidum . The serology results were positive for T. gondii-specific immunoglobulin M antibody, done by ELISA kit, (2.7 S/Co, cut off value 1.3 S/Co) and immunoglobulin G antibody (8.5 S/Co, cut off value 1.2 S/Co) and the titres were elevated. Serological tests for Human immunodeficiency virus and Treponema pallidum were negative and all other tests including CBC and CD4 count (912 cells/mm 3 ) were normal. Therefore, a diagnosis of necrotic isolated full-thickness MH secondary to toxoplasma retinochoroiditis was made in the right eye. Patient was started on oral Prednisolone (40mg/day) along with empirical oral treatment with anti-toxoplasmosis drugs -sulphamethoxazole (800 mg)/trimethoprim (160 mg) twice a day. Oral steroid was gradually tapered off, but anti-toxoplasmosis drugs were continued for 6 months. Serial OCT showed gradual disappearance of the necrotic debris, regeneration of photoreceptors, appearance and disappearance of intraretinal cystoid spaces, gradual reduction in the size of the MH and finally its conversion to residual outer lamellar hole at 6 months [ Fig. 2 ]. Sequential autofluorescence images during follow-up visits correlated with changes of reducing inflammation with healing and scarring [ Fig. 3 ]. During serial follow-ups, the intraocular inflammation subsided gradually with a final BCVA of 20/80 at 6 months after presentation. No surgical intervention was considered.