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Ananya Kaginalkar, Vaishali Vasavada and Samaresh Srivastava
This 31YO male presented with recent painful vision loss in his right eye. Vision was 20/200 OD and 20/40 in his normal OS. He was seen 3 years earlier with idiopathic neuroretinitis that resolved following systemic steroids.
Fundus photography shows a large serous detachment extending from the central macula into the temporal midperiphery with multifocal areas of opaque subretinal fluid. OCT scanning shows multiple pockets of elevated subretinal fluid with a bacillary layer detachment (BALAD). B-scan ultrasonography reveals a hyporeflective T-sign.
A diagnosis of posterior scleritis was made, and oral steroids were started. One week later, vision improved to 20/40. The funduscopic, OCT, and B-scan findings dramatically resolved. Oral steroids are being tapered slowly.
Systemic workup suggested sarcoidosis, with an elevated serum ACE level and pulmonary imaging showing ground-glass haziness in the bilateral lower lobes, along with enlarged paratracheal and bilateral hilar lymph nodes. Infectious and autoimmune workup was negative.
Learning Points:
Sarcoidosis, like syphilis, has a myriad of ocular presentations, including anterior, intermediate, and posterior uveitis, vasculitis, papillitis, and choroidal granulomas. Neuroretinitis and scleritis are both uncommon manifestations of sarcoidosis relative to uveitis.
BALAD appears as a unique dome-shaped collection of intraretinal fluid from photoreceptor splitting. These detachments can be observed in an increasing number of conditions, most classically Vogt-Koyanagi-Harada disease (Cicinelli et al, Ophthalmology Retina 2020;4:454-456).
Retin Cases Brief Rep. 2026 Sep 1;20(5):632-641. doi: 10.1097/ICB.0000000000001768.
MacTel2 intraretinal fibrosis Multimodal imaging features: Found in 2% of eyes; Seen in late stage; Likely from Müller, activated glial cells, and RPE cells with vascular remodeling — Retrospective, 7 eyes
Purpose: To explore the clinical and multimodal imaging characteristics of intraretinal fibrosis (IRFib) in macular telangiectasia type-2 (MacTel).
Methods: Macular telangiectasia type-2 eyes with IRFib that had multimodal imaging with color fundus photography, multicolor, blue reflectance, spectral-domain optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography.
Results: Seven eyes of six patients were included. Clinically, all cases had grayish-white fibrosis at macula, with retinal vessel tortuosity in two cases best characterized on multicolor. Two eyes each belonged to Gass and Blodi stages 3 and 4, three eyes were stage 5 MacTel. The mean lesion area at presentation was 1.36 mm 2 ± 0.81 mm 2 (range 0.56-2.99 mm 2 ). The defining optical coherence tomography finding for IRFib was hyper-reflective distortion intraretinally (involving different layers). Other accompanying findings in decreasing order were collapse sign (seven eyes); epiretinal membrane (six eyes); pigment clumping and migration (five eyes); serrated internal limiting membrane and outer-retinal hyper-reflectivity (four eyes); and lamellar macular hole (one eye). On optical coherence tomography angiography, IRFib was associated with right-angled vessels, vascular invasion and distortion of foveal avascular zone, and vessel dilatation and tortuosity in superficial vascular complex; telangiectasia, foveal avascular zone distortion and invasion, vessel drag, capillary looping, and abnormal vascular structures in deep capillary plexus; and nodular tuft or twig-like hyper-reflective vascular lesions in outer-retina choriocapillaris slab.
Conclusion: The incidence of IRFib on multicolor in our MacTel cohort was 1.75% with characteristic findings on optical coherence tomography and optical coherence tomography angiography. They are associated with advanced MacTel and probably implicate Müller cell dysfunction, activated glial cells, and vascular changes during the disease process.
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