Autosomal Recessive Bestrophinopathy: Clinical Features, Natural History, and Genetic Findings in Preparation for Clinical Trials.

PURPOSE: To investigate the clinical course, genetic findings, and phenotypic spectrum of autosomal recessive bestrophinopathy (ARB) in a large cohort of children and adults. DESIGN: Retrospective case series. PARTICIPANTS: Patients with a detailed clinical phenotype consistent with ARB, biallelic likely disease-causing sequence variants in the BEST1 gene, or both identified at a single tertiary referral center. METHODS: Review of case notes, retinal imaging (color fundus photography, fundus autofluorescence, OCT), electrophysiologic assessment, and molecular genetic testing. MAIN OUTCOME MEASURES: Visual acuity (VA), retinal imaging, and electrophysiologic changes over time. RESULTS: Fifty-six eyes of 28 unrelated patients were included. Compound heterozygous variants were detected in most patients (19/27), with 6 alleles recurring in apparently unrelated individuals, the most common of which was c.422G→A, p.(Arg141His; n = 4 patients). Mean presenting VA was 0.52 ± 0.36 logarithm of the minimum angle of resolution (logMAR), and final VA was 0.81 ± 0.75 logMAR (P = 0.06). The mean rate of change in VA was 0.05 ± 0.13 logMAR/year. A significant change in VA was detected in patients with a follow-up of 5 years or more (n = 18) compared with patients with a follow-up of 5 years or less (n = 10; P = 0.001). Presence of subretinal fluid and vitelliform material were early findings in most patients, and this did not change substantially over time. A reduction in central retinal thickness was detected in most eyes (80.4%) over the course of follow-up. Many patients (10/26) showed evidence of generalized rod and cone system dysfunction. These patients were older (P < 0.001) and had worse VA (P = 0.02) than those with normal full-field electroretinography results. CONCLUSIONS: Although patients with ARB are presumed to have no functioning bestrophin channels, significant phenotypic heterogeneity is evident. The clinical course is characterized by a progressive loss of vision with a slow rate of decline, providing a wide therapeutic window for anticipated future treatment strategies.

ACUTE IDIOPATHIC MACULOPATHY: A Proposed Disease Staging Based on Multimodal Imaging.

PURPOSE: To describe the clinical course and the multimodal imaging of acute idiopathic maculopathy. METHODS: Medical records and multimodal imaging including color fundus photography, optical coherence tomography, and fundus autofluorescence were retrospectively reviewed. Recognition of the fundus autofluorescence patterns and their relationship with the disease duration, best-corrected visual acuity, and optical coherence tomography features represented the main outcome measures. RESULTS: Seventeen eyes of 16 patients (7 women; mean age 29.9 years) with a mean follow-up of 23.9 months were included. The mean best-corrected visual acuity at presentation was 0.63 ± 0.54 logarithm of the minimum angle of resolution (Snellen equivalent, 20/85). All but one patient had the best-corrected visual acuity recovery to 20/20. Four sequential patterns of fundus autofluorescence corresponding to 4 proposed stages of disease were observed. Patterns 1 (central hypoautofluorescence with surrounding hyperautofluorescence) and 2 (stippled hyperautofluorescence and hypoautofluorescence) were found at presentation. Patterns 3 (central hyperautofluorescence surrounded by hypoautofluorescence) and 4 (hypoautofluorescence) were observed during the disease course and/or at the last follow-up visit. Duration of the disease was significantly different between patterns at baseline and last visit. Pattern 1 significantly related to the presence of subretinal detachment (Fisher's exact test; P =0.003) on optical coherence tomography in comparison with Pattern 2. Pattern 4 showed unique homogeneously decreased autofluorescence with corresponding attenuation of retinal pigment epithelium and restored outer retinal layers on optical coherence tomography. CONCLUSION: A sequential disease staging based on multimodal imaging for acute idiopathic maculopathy is proposed. The recognition of the observed imaging patterns may help clinicians in the correct diagnosis and patient counseling.

THE EXPANDING CLINICAL SPECTRUM OF CHOROIDAL EXCAVATION IN MACULAR DYSTROPHIES.

PURPOSE: To assess the prevalence and the clinical course of focal choroidal excavation (FCE) in patients affected by macular dystrophies. METHODS: Prospective case series. All the patients underwent a complete ophthalmologic examination, including best-corrected visual acuity and spectral domain optical coherence tomography. The presence of choroidal neovascularization (CNV) was assessed on the basis of the leakage detected on fluorescein angiography. RESULTS: A total of 162 eyes from 81 patients with macular dystrophy were included in the study. FCE was diagnosed in seven eyes (4.3% of the eyes), including four eyes with Best vitelliform dystrophy, two eyes with pattern dystrophy associated with pseudoxanthoma elasticum, and one case of Stargardt disease. In eyes with FCE and macular dystrophy, the mean best-corrected visual acuity was 0.4 ± 0.1 logarithm of the minimum angle of resolution (approximately corresponding to 20/50 Snellen equivalent) at baseline and was stable to 0.41 ± 0.1 logarithm of the minimum angle of resolution (approximately corresponding to 20/50 Snellen equivalent) at the final visit. In four of these seven eyes, FCE was associated with a subfoveal CNV. The CNV was managed with one intravitreal anti-vascular endothelial growth factor injection, achieving the complete anatomical stabilization of the CNV and recovery of the best-corrected visual acuity. CONCLUSION: Focal choroidal excavation can be infrequently encountered in patients with macular dystrophies. The presence of CNV may complicate FCE in these patients, and anti-vascular endothelial growth factor seems to be an effective treatment with no progression of FCE over time.

Short-Term Retinal Sensitivity and Metamorphopsia Changes following Half-Fluence Photodynamic Therapy in Central Serous Chorioretinopathy.

PURPOSE: The aim of this study was to investigate the short-term retinal sensitivity and metamorphopsia changes after half-fluence photodynamic therapy (PDT) in patients with central serous chorioretinopathy (CSC). METHODS: Patients with acute CSC and evidence of angiographic leakage and subretinal fluid on optical coherence tomography (OCT) were enrolled. Before, and 1 week (W-1) and 2 months (M-2) after PDT, all patients underwent best-corrected visual acuity, enhanced depth imaging OCT, microperimetry (MP) and preferential hyperacuity perimetry (PHP). We considered 'affected' the Early Treatment of Diabetic Retinopathy Study (ETDRS) sectors showing a mean retinal thickness greater than 350 µm on the baseline OCT thickness map. MP retinal sensitivity, PHP 'score' and retinal thickness were measured in each ETDRS 'affected sector'. Functional correlation between MP and PHP was also investigated. RESULTS: Twelve eyes of 12 patients were included. MP and PHP correlated with each other at baseline (p = 0.04) but not at W-1 (p = 0.12) and M-2 (p = 0.05). Mean MP retinal sensitivity slightly improved from 12.9 ± 5.0 dB at baseline to 14.5 ± 4.7 dB at M-2 (p = 0.2). The PHP score changed from 0.33 at baseline to 0.32 at M-2 (p = 0.3). In the ETDRS 'affected sectors', MP retinal sensitivity improved significantly from 12.9 ± 5.0 dB at baseline to 14.5 ± 4.7 dB at M-2 (p = 0.03), whereas the PHP score did not show significant changes over time. CONCLUSION: Half-fluence PDT achieved short-term improvement in the sensitivity of the retinal 'affected sectors' but did not influence metamorphopsia as measured on PHP in patients with acute CSC.

Stereotactic radiotherapy for polypoidal choroidal vasculopathy: a pilot study.

PURPOSE: To evaluate low-voltage X-ray stereotactic radiotherapy (SRT) delivered in conjunction with intravitreal ranibizumab for the treatment of active macular polypoidal choroidal vasculopathy (PCV). METHODS: At baseline, all eyes received an intravitreal injection of ranibizumab, followed by 16-Gy X-ray SRT to the macula. Further ranibizumab injections were given pro re nata. The primary outcome measure was regression of the polyps assessed by indocyanine green angiography. Secondary outcome measures were best-corrected visual acuity (BCVA) and central foveal thickness (CFT) changes on optical coherence tomography. Local or systemic adverse events were evaluated as well. RESULTS: We examined 12 eyes of 12 patients with PCV. At month 12, an angiographic regression of the polyps was observed in 10 of the 12 eyes. The mean BCVA improved by 7.6 letters: from 65.08 ± 11.4 to 72.7 ± 14.75 letters on the Early Treatment Diabetic Retinopathy Study (ETDRS) chart. The mean CFT decreased from 372.3 ± 79.6 to 215.9 ± 57.9 µm (p < 0.01). No local or systemic adverse events were reported. CONCLUSIONS: The preliminary data support the safety of low-voltage X-ray SRT for the treatment of macular PCV and show polyp closure, reduction in CFT and improvement in the mean BCVA. Additional research is warranted to confirm the efficacy and longer-term safety of this therapy in this population.

Impact of intravitreal dexamethasone implant (Ozurdex) on macular morphology and function.

PURPOSE: To investigate the impact of intravitreal dexamethasone implant (Ozurdex) on macular morphology and function in eyes with macular edema secondary to central retinal vein occlusion. METHODS: Twelve treatment-naive patients with decreased visual acuity because of central retinal vein occlusion-related macular edema were enrolled in this prospective uncontrolled study. Patients were treated with intravitreal Ozurdex and followed up at 1 month and 3 months for the evaluation of morphologic and functional outcomes, by means of best-corrected visual acuity, microperimetry, multifocal electroretinography, and customized high-resolution enhanced depth imaging spectral-domain optical coherence tomography scans. RESULTS: Twelve eyes of 12 patients (10 men, 2 women; mean age 56.2 ± 13.0 years) were included for analysis. At 1 month, mean best-corrected visual acuity, retinal sensitivity (microperimetry), multifocal electroretinography parameters, central macular thickness, and specific neurosensorial retinal measurements improved significantly. We found a significant negative correlation between retinal sensitivity and central macular thickness at 1 month and 3 months (r = -0.831, P = 0.001; r = -0.881, P = 0.001; respectively). Moreover, retinal sensitivity was negatively related to both outer and inner retinal thickness in all four intervals from the fovea. From baseline to Month 1, change in outer retinal thickness was positively related to multifocal electroretinography N1R1 amplitude change (r = 0.698, P = 0.012), whereas change in central macular thickness was negatively related to multifocal electroretinography P1R1 amplitude change (r = -0.701, P = 0.011). At 3 months, improvement of mean retinal sensitivity and central macular thickness slightly decreased. CONCLUSION: In eyes with macular edema secondary to central retinal vein occlusion, intravitreal dexamethasone provides functional benefits that correlate well with ultrastructural macular changes.

Multi-Attribute Decision Making: Parametric Optimization and Modeling of the FDM Manufacturing Process Using PLA/Wood Biocomposites.

The low carbon footprint, biodegradability, interesting mechanical properties, and relatively low price are considered some of the reasons for the increased interest in polylactic acid-based (PLA-based) filaments supplied with natural fillers. However, it is essential to recognize that incorporating natural fillers into virgin PLA significantly impacts the printability of the resulting blends. The complex inter-relationship between process, structure, and properties in the context of fused deposition modeling (FDM)-manufactured biocomposites is still not fully understood, which thus often results in decreased reliability of this technology in the context of biocomposites, decreased accuracy, and the increased presence of defects in the manufactured biocomposite samples. In light of these considerations, this study aims to identify the optimal processing parameters for the FDM manufacturing process involving wood-filled PLA biocomposites. This study presents an optimization approach consisting of Grey Relational Analysis in conjunction with the Taguchi orthogonal array. The optimization process has identified the combination of a scanning speed of 70 mm/s, a layer height of 0.1 mm, and a printing temperature of 220 °C as the most optimal, resulting in the highly satisfactory combination of good dimensional accuracy (Dx = 20.115 mm, Dy = 20.556 mm, and Dz = 20.220 mm) and low presence of voids (1.673%). The experimentally determined Grey Relational Grade of the specimen manufactured with the optimized set of process parameters (0.782) was in good agreement with the predicted value (0. 754), substantiating the validity of the optimization process. Additionally, the research compared the efficacy of optimization between the integrated multiparametric method and the conventional monoparametric strategy. The multiparametric method, which combines Grey Relational Analysis with the Taguchi orthogonal array, exhibited superior performance. Although the monoparametric optimization strategy yielded specimens with favorable values for the targeted properties, the analysis of the remaining characteristics uncovered unsatisfactory results. This highlights the potential drawbacks of relying on a singular optimization approach.

Transient Bacillary Layer Detachment During the Disease Course of Primary Vitreoretinal Lymphoma.

PURPOSE: To report the clinical course and the retinal imaging features of a case of cytology-proven primary vitreoretinal lymphoma (PVRL) presenting with a transient bacillary layer detachment (BALAD) during the disease course. METHODS: Observational case report. RESULTS: A 50 year-old woman was referred to us with a 2-month history of vitritis in both eyes, poorly responding to oral prednisolone. After discontinuation of oral prednisolone, worsening of vitritis and the appearance of multiple creamy-like subretinal infiltrates in the mid-peripheral retina of both eyes, along with the exclusion of common causes of intermediate/posterior uveitis, made us consider PVRL. Aqueous humor sampling detected MYD88 L265P mutation, and subsequent diagnostic pars plana vitrectomy in the left eye yielded a positive cytology for large B cell lymphoma consistent with PVRL. During the disease course, optical coherence tomography of the macula showed a BALAD in the right eye, which resolved during follow-up. CONCLUSION: Our case indicates that BALAD is a possible rare manifestation of PVRL, and this should be considered in the differential diagnosis process in order to avoid diagnostic delays.

Immunomodulatory Treatment Versus Systemic Steroids in Inflammatory Choroidal Neovascularization Secondary to Idiopathic Multifocal Choroiditis.

PURPOSE: To evaluate the influence of immunomodulatory therapy (IMT) on visual and treatment outcomes of inflammatory choroidal neovascularization (iCNV) in patients affected by multifocal choroiditis (MFC), and to compare them to patients treated with steroids as needed. DESIGN: Multicenter retrospective matched cohort study. METHODS: Patients affected by MFC with iCNV were divided into a IMT group and a "steroids as needed" group and matched according to the time between diagnosis and beginning of systemic treatment. Visual acuity (VA), number of anti-vascular endothelial growth factor (VEGF) intravitreal injections, and number of iCNV reactivations during 2 years of follow-up after treatment initiation were compared between the 2 groups. RESULTS: A total of 66 eyes of 58 patients were included, equally divided into the 2 groups. Patients in the IMT group had a lower relative risk (RR) of iCNV reactivation (0.64, P = .04) and of anti-VEGF intravitreal injection retreatment (0.59, P = .02). Relapses of MFC-related inflammation were independently associated with a higher RRs of iCNV reactivation (1.22, P = .003). Final VA was higher in the IMT compared to the steroids as needed group (mean [SD], 69.1 [15.1] vs 77.1 [8.9] letters, P = .01), and IMT was associated with greater VA gains over time (+2.5 letters per year, P = .04). CONCLUSIONS: IMT was associated with better visual and treatment outcomes in MFC complicated by iCNV compared to steroids as needed. The better outcomes of the IMT group and the association between MFC-related inflammation and iCNV reactivations highlight the need for tighter control of inflammation to prevent iCNV relapses and visual loss.

Retinal venous occlusions: diagnosis and choice of treatments.

Retinal vascular occlusive disorders constitute one of the major causes of blindness and impaired vision. There is marked controversy on their pathogeneses, clinical features and particularly their management. Recently, advances in clinical research added antivascular endothelial growth factor, corticosteroids and sustained-release implants to our armamentarium in the management of retinal vein occlusions. The purpose of our paper is to provide an update and a brief review on the current treatment options of retinal vein occlusions.

Optical Coherence Tomography Features of Endogenous Aspergillus Endophthalmitis Secondary to HIV Infection.

PURPOSE: To report imaging findings, including optical coherence tomography (OCT), of a case of endogenous Aspergillus endophthalmitis in a patient with newly diagnosed acquired immunodeficiency syndrome. METHODS: Observational case report. RESULTS: A 38-year-old patient presented with acute painful vision loss in his right eye (RE). Examination of the RE revealed anterior inflammation, vitritis and a fluffy macular infiltrate; OCT showed preretinal hyperreflective aggregates extending into the vitreous cavity with no evidence of subretinal and/or choroidal involvement. Lab tests showed leukopenia with lymphocyte T CD4+ count of 13 cells/µL and positive HIV serology. Vitreous biopsy was positive for Aspergillus niger and diagnosis of endogenous Aspergillus endophthalmitis secondary to HIV infection was made. CONCLUSIONS: OCT findings of this case show that ocular aspergillosis may present with disruption of the inner retinal layers and sparing of the outer retina and choroid, suggesting that Aspergillus may reach the eye through the retinal circulation.

Bilateral intraocular and lung involvements associated with Nasal NK/T-cell lymphoma.

BACKGROUND: To report a rare case of nasal natural killer/T (NK/T) cell lymphoma with bilateral intraocular and lung metastasis and to further describe the clinical features of intraocular manifestations. CASE PRESENTATION: A 54-year-old man presented with a 3-month history of left nasal congestion, and bilateral vision impairment of one week duration. Subsequent maxillary computed tomography (CT) and multiple biopsies confirmed the diagnosis of nasal NK/T-cell lymphoma. EBV-encoded small RNA (EBER) in situ hybridization revealed EBV infection. A comprehensive ophthalmic examination found lymphoma-associated retinopathy and choroidopathy, which presented as bilateral diverse patterns and retinal detachment. In addition, the chest CT showed multiple scattered nodules in both lungs, and soft-tissue mass in the left hilum with mediastinal and axillary lymphadenopathy. The condition of this patient deteriorated rapidly and he died shortly after diagnosis. CONCLUSIONS: The rarity of secondary ocular NK/T-cell lymphoma makes it challenging to identify these tumors early. Both otolaryngologist and ophthalmologists should be aware of ocular involvement and other secondary manifestations of NK/T-cell lymphoma.

Presumed sympathetic ophthalmia following scleral buckling surgery: A case report and review of the literature.

PURPOSE: To report a case of presumed sympathetic ophthalmia (SO) following scleral buckling (SB) surgery and to discuss the possible pathogenesis of this condition by reviewing the current evidence on this subject. METHODS: Case report and narrative review of the literature; our case was imaged with spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA), fundus autofluorescence (FAF), fluorescein angiography (FFA) and indocyanine green angiography (ICGA). RESULTS: A 55-year-old man presented with a macula on rhegmatogenous retinal detachment which was treated with 360° SB surgery, subretinal fluid drain (SRFD), cryopexy and pneumoretinopexy. Due to failure of the primary surgery, a second procedure was performed the day after with the explant of the prior buckle and the implant of a wider circumferential element. At three months from surgery, the patient complained of severe bilateral vision loss. Multimodal imaging revealed bilateral, multi-focal exudative retinal detachments and choroidal swelling. A diagnosis of presumed SO was made and the patient was treated with a combination of steroid and immunosuppressive drugs. The clinical picture completely resolved at postoperative month 12. CONCLUSION: SO may be a rare complication of SB surgery. In our case, early recognition and prompt immunosuppressive treatment achieved good long-term clinical results.

Adaptive Optics of Kyrieleis Plaques in Varicella Zoster Virus-Associated Posterior Uveitis: A Multimodal Imaging Analysis.

Kyrieleis plaques (KP) represent a peculiar type of vasculitis affecting retinal arterial branches in a beaded segmental pattern that can be found in several posterior inflammatory ocular conditions. The nature and precise location of KP is unclear. Adaptive Optics (AO) provides an in vivo visualization of retinal vasculature on a microscopic level, thus permitting a more detailed characterization of KP as compared to traditional imaging techniques. This study aims to report AO imaging of KP in Varicella Zoster virus (VZV)-associated posterior uveitis and to correlate the findings with traditional imaging techniques. Three patients diagnosed with VZV posterior uveitis underwent adaptive optics (AO) imaging and traditional multimodal imaging techniques, including fundus photography, fluorescein angiography, indocyanine green angiography and optical coherence tomography. In all subjects, AO imaging revealed segmental hyporeflectivity confined to the vessel wall, with no evidence of arterial wall disruption or extravascular involvement. In our series, AO findings support the view that KP are localized within the inner arterial wall, possibly at the endothelial level.

Acute-Onset Retinal Conditions Mimicking Acute Optic Neuritis: Overview and Differential Diagnosis.

Acute optic neuritis (AON) is a common cause of sudden visual loss in young patients. Because of the risk of demyelinating disease, patients affected by unilateral or bilateral optic neuritis should be evaluated and treated accordingly. Despite advancements in imaging of the brain and retina, misdiagnosis of AON is not uncommon. Indeed, some acute disorders of the retina have the potential to mimic AON and their prompt diagnosis may avoid unnecessary neurologic investigation, psychological stress to the patient, and delays in treatment. This review describes uncommon retinal disorders presenting with sudden-onset visual loss and absent or subtle funduscopic manifestation that can mimic AON. Multimodal retinal imaging is essential in detecting these conditions and in their differential diagnosis. It behooves neurologists and general ophthalmologists to be aware of these entities and be familiar with multimodal imaging of the retina.

Ocular Phenotypes in Patients With Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis in a Single Center Over 7 Years.

PURPOSE: To investigate the presence and type of ocular abnormalities in patients with hemophagocytic lymphohistiocytosis (HLH). DESIGN: A retrospective cross-sectional study. METHODS: Observational report of ocular findings and their associations with age, sex, underlying disease, and hematologic parameters. HLH was defined according to the 2004 criteria, and the patients were enrolled from March 2013 to December 2021. Analysis began in July 2022 and ended in January 2023. The main outcome measures were ocular abnormalities associated with HLH and their potential risk factors. RESULTS: Of 1525 HLH patients, 341 had ocular examinations, and 133 (133 of 341, 39.00%) had ocular abnormalities. Mean age at presentation was 30.21 ± 14.42 years. The multivariate analysis indicated that old age, autoimmune disorders, decreasing red blood cell count, decreasing platelet count, and increasing fibrinogen level were independent risk factors of ocular involvement in HLH patients. The most common presenting ocular findings were posterior segment abnormalities (66 patients, 49.62%), including retinal and vitreous hemorrhage, serous retinal detachment, cytomegalovirus retinitis, and optic disc swelling. Other HLH-associated ocular abnormalities included ocular surface infection (conjunctivitis, 34 patients, 25.56%; keratitis, 16 patients, 12.03%), subconjunctival hemorrhage (11 patients, 8.27%), chemosis (5 patients, 3.76%), anterior uveitis (11 patients, 8.27%), glucocorticoid-induced glaucoma (5 patients, 3.76%), radiation cataract (1 patient, 0.75%), dacryoadenitis (2 patients, 1.50%), dacryocystitis (1 patients, 0.75%), orbital cellulitis (2 patients, 1.50%), orbital pseudotumor (2 patients, 1.50%), and strabismus (2 patients, 1.50%). CONCLUSIONS: Eye involvement is not uncommon in HLH. Better awareness among both ophthalmologists and hematologists is necessary for prompt diagnosis and institution of appropriate management strategies with potential to save sight and life.

Quantitative Assessment of Choroidal Thickness and Choroidal Vascular Features in Healthy Eyes Based on Image Binarization of EDI-OCT: A Single-Center Cross-Sectional Analysis in Chinese Population.

PURPOSE: To quantify the structural changes in choroidal vessels and to observe choroid microstructural changes in different age and sex groups in a healthy Chinese population. METHODS: Enhanced depth imaging optical coherence tomography (EDI-OCT) was employed to analyze the luminal area, stromal area, total choroidal area, subfoveal choroidal thickness (SFCT), choroidal vascularity index (CVI), large choroidal vessel layer (LCVL), choriocapillaris-medium choroidal vessel layer, and LCVL/SFCT of the choroid in the subfoveal macular area within 1500 µm of the macula. We analyzed the age- and sex-related changes in the subfoveal choroidal structure. RESULTS: A total of 1566 eyes from 1566 healthy individuals were included. The mean age of the participants was 43.62 ± 23.29 years, the mean SFCT of healthy individuals was 269.30 ± 66.43 µm, LCVL/SFCT percentage was 77.21 ± 5.84%, and the mean macular CVI was 68.39 ± 3.15%. CVI was maximum in the 0-10 years group, decreasing with age, and the lowest values occurred in the >80 years group; LCVL/SFCT was the lowest in the 0-10 years group, increasing with age and reaching a maximum in the >80 years group. CVI showed a significant negative correlation with age, and LCVL/SFCT showed a significant positive correlation with age. There was no statistically significant difference between males and females. Interrater and intrarater reliability was less variable with CVI than with SFCT. CONCLUSIONS: The choroidal vascular area and CVI decreased with age in the healthy Chinese population, of which the age-related decrease in vascular components maybe dominated by the decrease in choriocapillaris and medium choroidal vessels. Sex had no effect on CVI. The CVI of healthy populations showed better consistency and reproducibility when compared with SFCT.

Unraveling the Neurological Complexity of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome: A Report of a Challenging Case of a Young Woman and Cutting-Edge Advancements in the Field.

POEMS syndrome-characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes-is an uncommon and complex paraneoplastic disorder encompassing a diverse array of symptoms. Here we report the challenging case of a 34-year-old female who sought medical attention at the emergency department due to distal lower limb weakness. She was breastfeeding her first child at that time. Her condition rapidly deteriorated, making it difficult for her to perform simple tasks independently. Initially, she struggled with activities like jumping or climbing stairs. Eventually, her ability to walk was also compromised. These symptoms underscored the swift evolution of her polyneuropathy. Nerve conduction studies and electromyography confirmed a diagnosis of mixed demyelinating and axonal polyneuropathy. Subsequent investigations, including bone marrow biopsy and immunochemistry testing, revealed a plasma cell disorder characterized by lambda monoclonal gammopathy, along with elevated levels of vascular endothelial growth factor (VEGF > 8000 pg/mL). This pivotal finding led to the diagnosis of POEMS syndrome, prompting the initiation of antineoplastic therapy (daratumumab-lenalidomide-dexamethasone) to manage this condition. An autologous cell transplantation was planned. The rarity of POEMS syndrome and its diverse clinical manifestations often lead to an incorrect or delayed diagnosis. Our case underscores the importance of considering this syndrome in patients presenting with acute or subacute polyneuropathy, even if the patients are young. In conclusion, this case elucidates the diagnostic complexities of POEMS syndrome, emphasizing the integral role of comprehensive multidisciplinary evaluations and the potential influence of increased VEGF as a diagnostic key element and possible therapeutic target.