Retinal Cases and Brief Reports最新文献

Purpose: To report a successful case of vision restoration and macular reperfusion following branch retinal artery occlusion (BRAO) using pars plana vitrectomy with undermining the artery off the retinal bed.

Methods: This case report involves a 75-year-old patient who was diagnosed immediately with BRAO following cardiac catheterization procedure. An embolus at the superior retinal artery bifurcation was noted. Initial treatments were ineffective, leading to a pars plana vitrectomy and mechanical elevation of the artery, performed six hours post-occlusion.

Results: The surgical intervention successfully dislodged the embolus, resulting in significant visual improvement. Post-operative day 1, 4, and 14 assessments showed visual acuity improvement to counting fingers at 2 meters, 20/40, and 20/20, respectively. Fundoscopic examination and OCT angiography confirmed improved macular perfusion and normalization of the flow signal in the affected artery.

Conclusion: This case highlights the efficacy of pars plana vitrectomy and mechanical elevation of the artery for BRAO where the embolus can be visualized. Prompt identification and referral for surgical intervention in BRAO cases with poor prognosis are crucial for favorable visual outcomes. Further research is needed to assess the efficacy of this surgical technique and other interventions for managing BRAO.

Purpose: To report a case of drusen regression following pars plana vitrectomy with internal limiting membrane peel (ILMP) in a patient with a full-thickness macular hole and dry age-related macular degeneration (AMD).

Methods: A 67-year-old gentleman presented in April 2024 with a full-thickness macular hole in OS and intermediate dry AMD OU. The patient underwent pars plana vitrectomy, ILMP, and an injection of sulfur hexafluoride gas for macular hole repair in OS. The patient's macular changes were monitored through visual acuity, fundus examination and optical coherence tomography (OCT) imaging at baseline and follow-up visits.

Results: The macular hole was closed and there was early stability of the macular drusen in OU. At a five-month follow-up in September 2024, he reported improved vision from 20/100 baseline to 20/40. Fundus examination as well as OCT imaging demonstrated a significant reduction of macular drusen in the operated eye, which was not observed in the fellow eye.

Conclusion: This case demonstrates successful surgical management of a full-thickness macular hole in a patient with concomitant drusen regression. The regression of drusen was limited to the operated eye, which suggests that surgical interventions may influence the course of AMD in certain patients.

Purpose: To describe a rare complication in a patient with extensive macular atrophy with pseudodrusen-like appearance (EMAP), suggesting immune dysregulation in advanced stages of the disease.

Methods: Case Report. Multimodal imaging -including true-color fundus photography, blue autofluorescence, high-resolution optical coherence tomography (Hi-Res OCT), swept-source OCT angiography, and dye-based angiography- was used to evaluate retinal alterations.

Results: A 53-year-old woman with stage 3 EMAP presented with moderately hyperreflective subretinal material co-localizing with a large rupture of the Bruch's membrane (BrM) in the right eye. Multimodal imaging ruled out macular neovascularization, suggesting a diagnosis of punctate inner choroidopathy (PIC)-like reaction. Treatment with oral steroids led to complete regression of the subretinal inflammatory lesion.

Conclusions: This case highlights a novel inflammatory complication in EMAP. Our findings emphasize a potential role of immune dysregulation in late-stage EMAP and underscore the value of multimodal imaging in the management and follow-up of these patients.

Purpose: To report a rare case of Multicentric Castleman's Disease presenting with bilateral panuveitis.

Methods: Case report.

Results: A 65-years-old caucasian man presented with progressive blurred vision in both eyes for two weeks, along with weight loss, polyarthralgias and reduced muscle strength persisting for about a year. Examination revealed bilateral panuveitis after intraocular lymphoma being initially considered. CT scans showed multiple lymph node enlargements suggestive of lymphoproliferative disorder. Excisional biopsy of an axillary lymph node confirmed the diagnosis of plasmacytic type Castleman's disease. Treatment was started with topical dexamethasone and tropicamide for anterior chamber inflammation, followed by oral prednisolone. After definitive diagnosis, treatment with siltuximab was initiated, which led to significant improvement in panuveitis and systemic symptoms. The patient became off corticosteroids and continued on siltuximab with stable visual acuity and low-grade vitritis.

Conclusion: To the best of our knowledge we described the first case in which the diagnosis of Multicentric Castleman's Disease was established through the investigation of bilateral panuveitis, which was successfully managed with corticosteroids and siltuximab.

Purpose: This study examines the multimodal imaging (MMI) findings in two cases of unilateral Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis (SNIFR), including detailed findings from the unaffected fellow eye.

Methods: Macular spectral domain optical coherence tomography (OCT) and 3x3 mm optical coherence tomography angiography (OCTA), microperimetry, full-field electroretinography (ff-ERG) for both the affected and the fellow eye were reviewed.

Results: The MMI findings were consistent across the two cases (71-year-old female and 60-year-old female). OCT imaging of the affected eyes revealed cystoid spaces between the outer nuclear layer (ONL) and the outer plexiform layer (OPL), with the vascular network remaining intact, indicating no alterations in vessel flow. Microperimetry showed slightly lower pointwise sensitivity in both eyes compared to a normative database.

Conclusion: Although SNIFR typically presents as a unilateral condition, our findings suggest that patients may have an inherent structural predisposition to macular schisis, resulting in subtle functional impairments. We hypothesize that the structural damage may involve the macular Müller cells. These observations highlight the importance of further studies to understand SNIFR's pathophysiology and inform therapeutic approaches.

Purpose: To describe a case of stellate multiform amelanotic choroidopathy (SMACH) with focal hyperfluorescence on indocyanine green angiography (ICGA).

Methods: Case report.

Results: A 19-year-old Caucasian woman was seen for an asymptomatic choroidal lesion. Visual acuity was 20/20. Fundus examination revealed an irregular, tan-colored lesion with overlying speckled hyperpigmentation in the macula of the left eye. Multimodal imaging demonstrated a thickened choroidal lesion with stellate, radially oriented projections most visible on near-infrared reflectance, red-free photography, en-face OCT, and ICGA. ICGA demonstrated early hypofluorescence outlining the borders of the lesion and a late, pinpoint area of hyperfluorescence with faint staining. The patient's fundus examination remained stable over 4 years of follow-up.

Conclusion: SMACH may present with focal choroidal hyperpermeability on ICGA while lacking typical features of central serous chorioretinopathy (CSCR). This finding expands the clinical spectrum of SMACH.

Purpose: The purpose of the study was to describe abnormal vitreoretinal findings during macular hole repair in Alport syndrome.

Methods: A case report of preoperative, intraoperative, and postoperative findings related to macular hole surgery in a patient with Alport syndrome was discussed.

Results: A 50-year-old woman with Alport syndrome was found to have bilateral full-thickness macular holes. Surgery was recommended for her left eye given recent onset of vision loss and smaller hole size (313 μ m). Intraoperatively, the vitreous was found to have fine fibrillar strands and to be abnormally adherent to the retinal surface. There was little to no internal limiting membrane present. Vitrectomy was performed with posterior cortical hyaloid peeling and 15% C3F8 gas tamponade. Two months after surgery, the macular hole was successfully closed.

Conclusion: Abnormal vitreous composition and adherence to the retinal surface may contribute to macular hole formation in patients with Alport syndrome. Standard surgical approaches including internal limiting membrane peeling may not be feasible, although thorough removal of the posterior cortical hyaloid may be sufficient to achieve macular hole closure.

Purpose: To describe a case of perfluoro-n-octane (PFO) retention, migration into the anterior chamber, and the subsequent formation of iris nodules after the repair of a giant retinal tear.

Methods: Patient data were collected by a manual chart review. All patient information was deidentified.

Results: Slit-lamp photographs demonstrate nodules on the iris surface and retained PFO in the anterior chamber. Ultrasound biomicroscopy shows multifocal hyperreflective signals on the iris surface, corresponding to the retained PFO within the iris.

Conclusion: Retention and migration of PFO to the anterior chamber after retinal detachment repair can lead to formation of nodules on the surface of the iris.

Purpose: The purpose of the study is to show the excellent anatomical result of the inverted flap-assisted technique with platelet-rich plasma under air for retinitis pigmentosa complicated with macular hole.

Methods: A 32-year-old woman, previously diagnosed with retinitis pigmentosa, was referred to our department complaining of decreased central vision in her left eye for 4 weeks. At the time of presentation, the optical coherence tomography and fundoscopy examination showed the presence of a macular hole of 620 μ m in diameter. The closure of the macular hole was observed after the surgical procedure, but endophthalmitis occurred 5 days, postoperatively.

Results: In our presented case, the cover flap-assisted technique with platelet-rich plasma under air for retinitis pigmentosa complicated with macular hole resulted in excellent anatomical outcomes with unremarkable visual recovery.

Conclusion: The physiopathology of full-thickness macular holes in retinitis pigmentosa patients remains still not fully elucidated. Pars plana vitrectomy with the adjunct of highly concentrated pure platelet-rich plasma have shown successful results.

Purpose: Best vitelliform macular dystrophy is an inherited macular dystrophy associated with over 250 pathogenic variants of the Bestrophin-1 ( BEST1 ) gene. Although several types of lesions of best vitelliform macular dystrophy are well-described, reports of phenotypic variations associated with rare genetic variants are limited.

Methods: This was a retrospective case series performed in 2021 at a tertiary eye care center.

Patients: Three members of one family referred to a tertiary eye care clinic for evaluation of their autosomal dominant macular dystrophy.

Results: Study subjects presented with atypical findings of peripheral schisis-like lesions and atrophy with abnormal electroretinogram in addition to typical macular lesions found in best vitelliform macular dystrophy. Genetic analyses identified a heterozygous BEST1 c.227T>A, p.(Ile76Asn) pathogenic variant in all three subjects.

Conclusion: This study represents the first report of the phenotype associated with the c.227T>A, p.(Ile76Asn) BEST1 variant, which-while mentioned twice in the literature-has not been previously described. The phenotype is unique, comprising features of typical best vitelliform macular dystrophy with electroretinogram and peripheral findings, suggestive of a panretinal dysfunction.