RESUMEN
PURPOSE: To report clinical and imaging features of optic nerve and retinal involvement in a patient with mucopolysaccharidosis (MPS) type II B. METHODS: A 27-year-old man, diagnosed with MPS type II B and undergoing enzymatic substitution therapy for the past 19 years, was referred to the retina service. An ophthalmological evaluation, which included multimodal imaging, was conducted to investigate potential retinal and optic disc involvement. RESULTS: The eye examination revealed a pigmentary retinopathy with a predominant loss of the outer retinal loss, primarily in the parafoveal and perifoveal regions. Notably, multimodal imaging identified macular edema without any signs of leakage, implying an association between macular edema and retinal neurodegeneration. Additionally, both eyes exhibited an optic disc with blurred margins. CONCLUSION: We herein describe the multimodal imaging findings of retinal and optic disc involvement in a patient with MPS type II B. This report describes for the first-time the presence of macular edema without leakage alongside photoreceptor damage and optic disc swelling.
Asunto(s)
Angiografía con Fluoresceína , Edema Macular , Mucopolisacaridosis II , Disco Óptico , Papiledema , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Masculino , Adulto , Edema Macular/diagnóstico , Edema Macular/etiología , Papiledema/diagnóstico , Papiledema/etiología , Angiografía con Fluoresceína/métodos , Disco Óptico/patología , Disco Óptico/diagnóstico por imagen , Mucopolisacaridosis II/complicaciones , Mucopolisacaridosis II/diagnóstico , Mucopolisacaridosis II/tratamiento farmacológico , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/complicaciones , Imagen Multimodal , Fondo de OjoRESUMEN
PURPOSE: To report the use of intravitreal triamcinolone to treat macular edema associated with isolated perifoveal exudative vascular anomalous complex (PVAC) and resembling lesion (PVAC-RL). METHODS: In this case series, three diabetic patients (3 eyes) with PVAC-RLs and one healthy patient (1 eye) with PVAC lesion associated with cystic spaces underwent three intravitreal injections of aflibercept before switching to one intravitreal triamcinolone injection in each patient. RESULTS: Macular edema improved from 297.5 ± 8.10 µm, at baseline, to 269.2 ± 8.89 µm, after triamcinolone; whereas visual acuity improved from 20/38 to 20/26 (ETDRS). CONCLUSION: PVAC and PVAC-RL are rare and often misdiagnosed lesions that may be associated with decreased vision. Our outcomes suggest that intravitreal injection of triamcinolone may be an effective and affordable treatment for PVAC and PVAC-RL with intraretinal fluid.
Asunto(s)
Retinopatía Diabética , Edema Macular , Humanos , Triamcinolona , Edema Macular/diagnóstico , Edema Macular/tratamiento farmacológico , Edema Macular/etiología , Retinopatía Diabética/diagnóstico , Retinopatía Diabética/tratamiento farmacológico , Glucocorticoides , Inyecciones Intravítreas , Triamcinolona AcetonidaRESUMEN
PURPOSE: To evaluate the follow up and treatment outcome of central serous chorioretinopathy (CSCR) based on the new multimodal imaging-based classification and identify the predictors for anatomic and visual outcome. METHODS: Retrospective, multicentric study on 95 eyes diagnosed with CSCR and a follow up of at least 12 months were included. Eyes with macular neovascularization, atypical CSCR or any other disease were excluded. RESULTS: At the baseline, observation was advised to 70% eyes with simple CSCR whereas photodynamic therapy (PDT) was performed in 49% eyes with complex CSCR. Over the follow up, decrease in CMT was significantly higher in simple CSCR as compared to complex CSCR (P = 0.008) and the recurrences were significantly more in eyes with lower CMT at baseline (P = 0.0002). Median time of resolution of SRF was 3 months and 6 months in simple and complex CSCR respectively (P = 0.09). For the 12 months follow up, the median fluid free period was greater (P = 0.03) while number of interventions performed was lesser in eyes with simple CSCR as compared to complex CSCR (P = 0.006). Multiple regression analysis showed baseline best corrected visual acuity (BCVA) and baseline persistent SRF to be significantly predictive of BCVA and persistent SRF at 12 months (P < 0.0001, 0.04) respectively. CONCLUSIONS: Complex CSCR more often required PDT, was associated with shorter fluid free interval and longer time for SRF resolution. Baseline BCVA and persistent SRF were predictive of final visual and anatomical outcome. The new multimodal imaging based classification is helpful in establishing objective criteria for planning treatment approaches for CSCR.