Optical coherence tomography angiography macular biomarkers of peripheral retinal ischemia in diabetic macular edema: secondary endpoints from the clinical study "FOVEA".

PURPOSE: To investigate the relationship between the macular values of fractal dimension (FD) and lacunarity (LAC) on optical coherence tomography angiography (OCTA) images and the presence of peripheral retina non-perfusion areas (NPAs) on fluorescein angiography (FA) in patients with treatment-naïve diabetic macular edema (DME). METHODS: Fifty patients with treatment-naïve DME underwent a full ophthalmic examination, including best-corrected visual acuity measurement, FA, spectral-domain optical coherence tomography, and OCTA. Specifically, FA was performed to detect the presence of retinal NPAs, whereas fractal OCTA analysis was used to determine macular FD and LAC values at the level of the superficial and deep capillary plexus (SCP and DCP). FA montage frames of the posterior pole and peripheral retina, as well as macular OCTA slabs of the SCP and DCP, were obtained. RESULTS: Thirty (60%) eyes with FA evidence of peripheral retinal NPAs in at least one quadrant showed significantly lower FD and higher LAC in both SCP and DCP, when compared with eyes presenting a well-perfused peripheral retina. Furthermore, macular FD and LAC values were found to be significantly associated with the extent of retinal NPAs. CONCLUSIONS: Macular FD and LAC of both SCP and DCP seem to be strongly associated with the extent of peripheral retinal NPAs, thus suggesting that may be useful predictive biomarkers of peripheral ischemia in treatment-naïve DME eyes.

Hyperbaric oxygen therapy in malignant otitis externa: a retrospective analysis.

PURPOSE: Malignant otitis externa (MOE) is a rare form of invasive osteomyelitis of the external ear canal. It is typically caused by Pseudomonas aeruginosa in immunocompromised patients. The diagnosis is clinical, and the initial treatment involves systemic antibiotics or antifungal therapy. Surgery is usually only considered when medical treatment has failed. Although hyperbaric oxygen therapy (HBOT) is recommended for refractory osteomyelitis, there are no specific guidelines for MOE. METHODS: This is a retrospective study that evaluates clinical data, treatment, and results obtained in patients diagnosed with MOE treated with HBOT at the Pedro Hispano Hospital between 2007 and 2022. RESULTS: During the study period, fifteen patients diagnosed with MOE were admitted for treatment with HBOT. All patients received antibiotic and/or antifungal therapy, and three required surgical intervention before starting HBOT. The pathology was successfully managed on all patients. CONCLUSIONS: HBOT may be an effective adjuvant treatment option in patients with MOE but it lacks robust scientific evidence. However, its therapeutic value should not be underestimated due to the good results and few adverse effects reported in recent retrospective studies and case reports.

Stereotactic anatomy of the third ventricle.

PURPOSE: Endoscopic third ventriculostomy (ETV) is a surgical procedure that can lead to complications and requires detailed preoperative planning. This study aimed to provide a more accurate understanding of the anatomy of the third ventricle and the location of important structures to improve the safety and success of ETV. METHODS: We measured the stereotactic coordinates of six points of interest relative to a predefined stereotactic reference point in 23 cadaver brain hemi-sections, 200 normal brain magnetic resonance imaging (MRI) scans, and 24 hydrocephalic brain MRI scans. The measurements were statistically analyzed, and comparisons were made. RESULTS: We found some statistically significant differences between genders in MRIs from healthy subjects. We also found statistically significant differences between MRIs from healthy subjects and both cadaver brains and MRIs with hydrocephalus, though their magnitude is very small and not clinically relevant. Some stereotactic points were more posteriorly and inferiorly located in cadaver brains, particularly the infundibular recess and the basilar artery. It was found that all stereotactic points studied were more posteriorly located in brains with hydrocephalus. CONCLUSION: The study describes periventricular structures in cadaver brains and MRI scans from healthy and hydrocephalic subjects, which can guide neurosurgeons in planning surgical approaches to the third ventricle. Overall, the study contributes to understanding ETV and provides insights for improving its safety and efficacy. The findings also support that practicing on cadaveric brains can still provide valuable information and is valid for study and training of neurosurgeons unfamiliar with the ETV technique.

POLYPOIDAL CHOROIDAL NEOVASCULARIZATION VERSUS TYPE 1 CHOROIDAL NEOVASCULARIZATION IN AGE-RELATED MACULAR DEGENERATION: A Fractal Analysis Study.

PURPOSE: To compare quantitative optical coherence tomography angiography parameters between polypoidal choroidal neovascularizations (PCNVs) and Type 1 choroidal neovascularizations (CNVs) in patients with age-related macular degeneration. METHODS: PCNV and Type 1 CNV lesions were retrospectively recruited in a cohort of patients with age-related macular degeneration. All the patients underwent a comprehensive ophthalmic evaluation, including best-corrected visual acuity, fluorescein and indocyanine green angiography, structural optical coherence tomography (OCT), and optical coherence tomography angiography.Vascular perfusion density, fractal dimension, and lacunarity were computed by means of fractal analysis of neovascular en face optical coherence tomography angiography slabs. RESULTS: Sixty-eight eyes were included in the analysis. Of them, 35 of 68 eyes (51.5%) had PCNV and 33 of 68 (48.5%) had Type 1 CNV. Patients with PCNV were significantly younger (P = 0.0003) and had a higher best-corrected visual acuity (P < 0.0001). The mean vascular perfusion density was 0.83 ± 0.11% in PCNVs and 0.46 ± 0.10% in Type 1 CNVs (P < 0.0001). The mean fractal dimension was 1.44 ± 0.1 in PCNVs and 1.45 ± 0.09 in Type 1 CNVs (P = 0.86) while the mean lacunarity was 2.46 ± 1.03 in PCNVs and 1.86 ± 0.52 in Type 1 CNVs (P = 0.006). CONCLUSION: PCNVs resulted to be more heterogeneous and characterized by higher vascular perfusion density and lacunarity values than Type 1 CNVs. These interesting findings seem to support the idea that PCNVs and Type 1 CNVs are two separate clinical entities. However, future studies based on optical coherence tomography angiography fractal analysis, but also involving other relevant parameters such as demographics, presentation, morphology on multimodal imaging, and response to treatment, are necessary before drawing any definitive conclusions on whether PCNV is a specific clinical entity or a neovascular age-related macular degeneration variant.

Quantitative Optical Coherence Tomography Angiography Biomarkers for Alport Syndrome.

PURPOSE: The aim of this study was to evaluate microvascular abnormalities of patients with Alport syndrome (AS) using optical coherence tomography angiography (OCT-A) quantitative biomarkers. METHODS: This was cross sectional, prospective evaluation of consecutive patients with AS and healthy subjects. AS diagnosis was performed by the genetic test. All participants underwent a retinal vasculature evaluation by spectral-domain optical coherence tomography (SD-OCT) and OCT-A of the macula. Quantitative analysis included whole vascular density, foveal avascular zone area, fractal dimension (FD), and lacunarity (LAC). RESULTS: Ninety-four eyes were included in this study, 45 eyes from patients with AS and 49 eyes from healthy subjects. The pathogenic mutation in the COL4A5 gene on the chromosome X was found in 14 patients; the pathogenic autosomal recessive mutations in the COL4A3 gene were found in 9 patients. Quantitative evaluation demonstrated a significant difference between AS and healthy subjects on LAC of the superficial capillary plexus and deep capillary plexus (DCP) (p < 0.001 and p < 0.001, respectively) and on FD in the DCP (p < 0.001). CONCLUSION: The DCP Alport patients have a higher vessel nonuniformity than DCP of healthy subjects. We hypothesize that endothelial cell lesion in the setting of low resistance at the DCP circuit could lead to long-term structural disorganization.

QUANTITATIVE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES OF INACTIVE MACULAR NEOVASCULARIZATION IN AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To compare quantitative optical coherence tomography angiography parameters between treatment-naïve quiescent macular neovascularizations (MNVs) and previously treated nonexudative Type 1 MNVs, in patients with age-related macular degeneration. METHODS: The eyes included in the study were analyzed by fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. According to their medical history and multimodal imaging evaluation, Type 1 MNVs were divided into 2 groups: 1) treatment-naïve quiescent MNVs; 2) previously treated nonexudative Type 1 MNVs. Quantitative optical coherence tomography angiography parameters, including perfusion density (PD), fractal dimension (FD), and lacunarity (LAC) were calculated. Receiver operating characteristic curves, showing the ability of PD, FD, and LAC to discriminate between the two MNV groups, were built. RESULTS: Twenty-two eyes with treatment-naïve quiescent MNVs and 20 eyes with MNVs previously treated nonexudative Type 1 MNVs were analyzed. Mean FD and LAC were statistically different between the two study groups (P < 0.05). Lacunarity showed the best discrimination ability, followed by FD and PD (area under curve = 0.83, 0.78, 0.62, respectively). CONCLUSION: Results suggest that FD and LAC may be useful optical coherence tomography angiography biomarkers to objectively discriminate inactive MNVs with different prognosis, such as treatment-naïve quiescent MNVs and previously treated nonexudative Type 1 MNVs, in age-related macular degeneration patients.

IMPLICATIONS OF THE MORPHOLOGIC PATTERNS OF TYPE 1 MACULAR NEOVASCULARIZATION ON MACULAR ATROPHY GROWTH ON PATIENTS UNDER ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR TREATMENT.

PURPOSE: To evaluate the correspondence between macular atrophy (MA) progression and Type 1 macular neovascularization morphology during long-term anti-vascular endothelial growth factor treatment for exudative neovascular age-related macular degeneration. METHODS: Retrospective review of consecutive patients with complete retinal pigment epithelium and outer retina atrophy overlying or in the proximity of macular neovascularization. The assessment of MA was based on spectral domain optical coherence tomography, en-face near infra-red imaging and fundus autofluorescence. Macular neovascularization blood flow morphology was evaluated by swept-source optical coherence tomography-angiography. Qualitative features were categorized per ETDRS sector as: immature, mature; and hypermature pattern. An automatic analysis was designed in MATLAB coding language to compute MA per ETDRS. Measurements were compared between the baseline and the last follow-up visit. RESULTS: Twenty eyes from 20 patients were included; the mean age was 85.4 (8.3) years. The median follow-up was 1.85 (1.0-2.4) years and the median anti-vascular endothelial growth factor injection rate during follow-up was 4.0 (2.0-5.0) injections/year. During follow-up, sectors with persistence of an immature blood flow pattern had a lower MA growth rate than sectors with mature macular neovascularization flow patterns (P = 0.001). CONCLUSION: The presence of an immature blood flow pattern on optical coherence tomography-angiography is associated with a lower progression rate of MA.

PREDICTIVE ACTIVATION BIOMARKERS OF TREATMENT-NAIVE ASYMPTOMATIC CHOROIDAL NEOVASCULARIZATION IN AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To assess the long-term evolution of treatment-naive quiescent choroidal neovascularization (CNV), in age-related macular degeneration (AMD), to identify predictive activation biomarkers. METHODS: Patients with quiescent CNV underwent a comprehensive ophthalmological examination, including fluorescein and indocyanine green angiographies, structural optical coherence tomography (OCT), and OCT angiography. Qualitative and quantitative analyses of structural OCT and OCT angiography images were performed during the study period. At the last follow-up evaluation, the enrolled eyes were divided into two groups: eyes with quiescent CNV converting to exudative AMD (eAMD) and those not progressing to eAMD. RESULTS: Sixty-eight eyes of 68 patients were enrolled in the study. Mean follow-up duration was 40 ± 28 months using multimodal imaging and 22 ± 13 months using OCT angiography. On structural OCT, quiescent CNV not converting to eAMD showed a preferential growth of the pigment epithelium detachment greatest linear diameter (P = 0.009), whereas the eAMD group presented a preferential growth of the pigment epithelium detachment maximal height (P < 0.0001) during the study period. Quantitative analysis of choriocapillaris OCT angiograms confirmed the CNV area growth during follow-up (from 4.18 ± 4.77 mm at baseline to 5.10 ± 5.06 mm at the last follow-up visit; P = 0.02). CONCLUSION: A close follow-up is recommended to early identify predictive activation biomarkers of treatment-naive quiescent CNV.

Morphology and Navigational Landmarks of the Cranio-orbital Foramen in a Portuguese Population.

PURPOSE: The cranio-orbital and accessory foramina are located in the lateral wall of the orbit and adjacent to the superior orbital fissure. In the literature, there is a lack of consistency concerning the location and morphology of these foramina in different populations. Therefore, considering its clinical importance during orbital surgeries, it was the authors' aim to determine the incidence, location, and number of cranio-orbital and accessory foramina in a Portuguese population and compare the findings with data from other studies. METHODS: A total of 310 orbits from 155 dry skulls from the collection of the Museum of Anatomy of the Faculty of Medicine of Porto were studied. The characterization of both cranio-orbital and accessory cranio-orbital foramina was performed. RESULTS: The cranio-orbital foramen was present in at least 1 orbit per skull in 58.17% with a median diameter of 0.60 ± 0.33 mm. No relation was found between the presence of this foramen and the gender of the individuals or a tendency for laterality. However, it was found that the presence of 1 or more accessory foramina was related to higher diameters of the cranio-orbital foramen. CONCLUSIONS: There might be a relationship between the localization, diameter, and communication of foramina with the cranial fossae. Foramina located on the greater wing of the sphenoid bone presented a larger diameter and communicated with the middle cranial fossa. High diameters of the main vessel in the cranio-orbital foramen may imply more developed branches and collateral irrigation of the orbital structures using 1 or more accessory foramina. Surgeons should be aware of the location of the cranio-orbital foramen to reduce potential sources of hemorrhage during orbital dissections.

Outer Retina and Choroidal Thickness in Intermediate Age-Related Macular Degeneration: Reticular Pseudodrusen Findings.

PURPOSE: To evaluate outer retina and choroidal thickness in subjects with intermediate age-related macular degeneration (iAMD) and to describe associations with the presence of reticular pseudodrusen (RPD). METHODS: This was a retrospective, cross-sectional analysis of 157 consecutive eyes (specifically: 62 eyes classified as having RPD and 95 eyes with drusen ≥125 µm). Only cases with digital color fundus photographs, red-free, and infrared, obtained and graded according to the Age-Related Eye Disease Study to define iAMD, were used for this study. Outer retina and choroidal thickness were manually segmented and quantified at 12 locations in the horizontal meridian. RESULTS: RPD appeared to be associated with thinning of the outer layers even after adjustment for gender and age. The presence of RPD in iAMD decreased with increase of choroidal thickness (total odds ratio [OR] 0.991, 95% confidence interval [CI] 0.985-0.996; nasal OR 0.992, 95% CI 0.986-0.997), with increased thickness of the myoid zone of the photoreceptors (total OR 0.812, 95% CI 0.688-0.958; nasal OR 0.863, 95% CI 0.755-0.987) and with increased thickness of the outer segment of the photoreceptors (total OR 0.850, 95% CI 0.731-0.989; nasal OR 0.857, 95% CI 0.736-0.989). CONCLUSIONS: The greatest differences between eyes with and without RPD are found at the level of the choroidal thickness and at the level of the photoreceptors.

Isolated corneal involvement by Lichen Planus - multi-modal evaluation.

PURPOSE: To describe a case of ocular Lichen Planus, successfully managed using a multimodal evaulation, including Anterior Segment OCT (AS-OCT). OBSERVATIONS: A female patient in her forties with a history of cutaneous Lichen Planus presents with blurred vision and burning eye sensation. Anterior segment evaluation revealed bilateral punctate keratitis, stromal haze and subepithelial pigmented dots. AS-OCT was pivotal for diagnosis, showing anterior stromal hyperreflective dots. A diagnosis of ocular Lichen Planus was estabilished and the patient was treated with topical hydrocortisone, with complete subsidence of the complaints. CONCLUSIONS AND IMPORTANCE: Ocular Lichen Planus can present as isolated corneal involvement independent from severe cicatrizing conjunctivitis. Appropriate and timely treatment can prevent irreversible ocular surface disease. Ophthalmologists should be aware of Lichenoid Tissue Reaction (LTR) disorders, especially in patients with relentless blepharitis and/or ocular surface disease.

Intravenous Human Immunoglobulin in the Treatment of BK Virus Nephropathy in Kidney Transplant: A Case Report.

The BK virus infection is common in the immunocompetent population and is asymptomatic in the majority of cases. However, in renal transplant patients, reactivation and replication can occur, leading to the development of BK virus-associated nephropathy (BKVN), which is associated with renal injury and graft loss. The objective of this case report was to demonstrate a case of BKVN that showed a good response to the use of human immunoglobulin. A 37-year-old man who underwent a second transplant received rabbit-derived antithymocyte human immunoglobulin at a dose of 6 mg/kg intravenously as induction immunosuppressive therapy, and maintenance therapy with tacrolimus, prednisone, and mycophenolate sodium (MFS). At 3 months post-transplant, he presented sustained BK virus viremia (70,000-100,000 copies/mL), leading to a reduction in the dose of MFS and tacrolimus. A biopsy diagnosed BKVN class 2/B2, and viremia increased to over 1 million copies/mL at 22 months, prompting the discontinuation of tacrolimus without response. Intravenous human immunoglobulin (IVIG) was administered at 2 g/kg at 22 months and again at 33 months, with viremia peaking at 2 million copies 3 months later. However, it steadily declined to 5500 copies/mL at 52 months post-transplant. Currently, the only proven therapy for BKVN is the reduction of immunosuppression. However, in patients who do not respond, IVIG is considered as an option, with good results demonstrated in case reports, as shown here. Nevertheless, the data are based on case reports or case series, and the development of controlled clinical trials is necessary for confirmation of the efficacy.

Corrigendum to "WCN24-1675 ACUTE NECROTIZING ULCERATIVE GENGIVITIS IN KIDNEY TRANSPLANTATION: A CASE REPORT" [Kidney International Reports Volume 9, Issue 4, Supplement, April 2024, Page S473].

[This corrects the article DOI: 10.1016/j.ekir.2024.02.994.].

Clinical Implications of Alternating Hypointense Bands on OCT Angiography in Retinal Vascular Occlusive Disease.

PURPOSE: To demonstrate the relationship between alternating hypointense signal bands on OCT angiography (OCTA), real-time fluorescein angiography (FA), and structural OCT findings in patients with retinal vascular occlusive disease (RVOD). DESIGN: Retrospective, consecutive case series. SUBJECTS: Consecutive patients with a clinical diagnosis of acute RVOD and alternating bands of hypointense OCTA flow signal on en face projections. METHODS: Complete ophthalmic examination and multimodal imaging, including color fundus photography, real-time FA, spectral-domain OCT, and OCTA performed with different instruments having different scan speeds and acquisition protocols. MAIN OUTCOME MEASURES: The primary outcomes were: hypointense OCTA band characteristics (number, width, orientation, and location), OCTA acquisition characteristics (speed and scan direction), and FA findings including delayed arteriovenous (AV) transit and pulsatile flow. Secondary outcomes were: structural OCT changes including retinal fluid, paracentral acute middle maculopathy (PAMM) lesion, and a prominent middle limiting membrane (p-MLM) sign. RESULTS: OCT angiography hypointense bands were detected in the superficial and deep vascular plexuses in 9 eyes of 9 patients with either partial central retinal vein occlusion (RVO) or nonischemic RVO. When obtained on the same device, hypointense bands were thinner and more numerous at lower (100 kHz) scan speeds compared with higher (200 kHz) scan speeds. Band orientation was parallel to the OCTA scan direction, and their extent correlated with the area of delayed AV transit on FA. Structural OCT showed multiple PAMM lesions in 78% of cases and a p-MLM sign centered in the fovea in 44% of cases. CONCLUSIONS: OCT hypointense bands are a novel biomarker in RVOD indicating delayed AV transit and pulsatile filling without the need for dye angiography. Structural OCT often shows PAMM in these eyes and, less commonly, a p-MLM sign. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Tracheal metallic hybrid stent removal via tracheostomy: an unconventional approach.

Increasing use of tracheal expandable metallic hybrid stents will lead to common encounters with these devices in emergency airway management. The presence of these stents qualifies any patient as a challenge when an emergency tracheostomy is needed. We report an unorthodox technique of tracheostomy with concomitant removal of tracheal stent, without any major complications. Although the combined approach with bronchoscopy and tracheostomy has been reported in similar cases, we present a safe procedure when rigid bronchoscopy is not available.

Mutational spectrum and deep phenotyping in Pseudoxanthoma Elasticum: Findings from a Portuguese cohort.

INTRODUCTION: Pseudoxanthoma Elasticum (PXE) is a rare autosomal recessive disorder originated by disease-causing variants in ABCC6 gene. The purpose of this study was to characterize the genetic landscape, phenotypic spectrum and genotype-phenotype correlations in a Portuguese cohort of PXE patients. METHODS: Multicentric cross-sectional study conducted in patients with a clinical and genetic diagnosis of PXE. Patients were identified using the IRD-PT registry (www.retina.com.pt). Genotypes were classified into 3 groups: (1) two truncating variants, (2) two non-truncating variants, or (3) mixed variants. Deep phenotyping comprised a comprehensive ophthalmologic and systemic evaluation using the updated Phenodex Score (PS). RESULTS: Twenty-seven patients (23 families) were included. Sixteen different ABCC6 variants were identified, 7 of which are novel. The most prevalent variant was the nonsense variant c.3421C > T p.(Arg1141*) with an allele frequency of 18.5%. All patients exhibited ocular manifestations. Cutaneous manifestations were present in most patients (88.9%, n = 24/27). A PS score > E2 was strongly associated with worse visual acuity (B = -29.02; p = 0.001). No association was found between genotypic groups and cutaneous, vascular or cardiac manifestations. CONCLUSIONS: This study describes the genetic spectrum of patients with PXE for the first time in a Portuguese cohort. A total of 16 different variants in ABCC6 were found (7 of which are novel), thus highlighting the genotypic heterogeneity associated with this condition and expanding its mutational spectrum. Still, no major genotype-phenotype associations could be established.

SCLERAL EXCAVATION AT OBLIQUE MUSCLE INSERTIONS: IMAGING CHARACTERISTICS AND CORRELATION WITH SCLERAL PLAQUES AND SCLEROCHOROIDAL CALCIFICATION.

PURPOSE: To compare the imaging features of lesions showing hyporeflective posterior scleral excavation found near the insertions of the oblique extraocular muscles to the features and the natural course of Cogan scleral plaques. METHODS: Multimodal imaging with color fundus photography, spectral-domain optical coherence tomography (OCT), swept-source optical coherence tomography, and B-scan ultrasonography. RESULTS: A 71-year-old man and an 89-year-old man presented with ring-shaped hypopigmented lesions measuring between 200 µm and 300 µm transversally, and located along the superior vascular arcade and temporal to the fovea. All lesions were identified near the insertion of oblique muscles, with one observed in the temporal macula, and two found along the superotemporal arcades. Enhanced depth imaging-optical coherence tomography showed hyporeflective boat-shaped areas of scleral excavation with reduced choroidal thickness along their margins. B-scan ultrasonography showed the lesions to be intensely reflective with varying degrees of posterior shadowing. CONCLUSION: To our knowledge, this is the first report of excavated hyporeflective scleral lesions found near the oblique muscle insertions. Imaging and clinical data support the diagnosis of a posterior form of Cogan scleral plaque and are consistent with the natural course of this entity.