Paracentral Acute Middle Maculopathy Associated With Cilioretinal Artery Insufficiency Following COVID-19 Vaccination in a Young Patient.

We aim to present a unique case of unilateral paracentral acute middle maculopathy (PAMM) associated with cilioretinal artery insufficiency following the coronavirus disease 2019 (COVID-19) vaccination. A 28-year-old male complained of a sudden blurring of vision in his left eye 40 days after receiving the second dose of COVID-19 immunization. The optical coherence tomography revealed a diffuse paracentral area of hyper-reflective change in the inner plexiform layer and an increase in the inner nuclear layer volume, consistent with PAMM along the course of the cilioretinal artery. PAMM has been connected to an assortment of retinal vasculature anomalies. Considering COVID-19 vaccination, we hypothesize that the immunogenic cascade following vaccination dysregulated coagulation and led to retinal vascular thrombosis. However, the link between COVID-19 vaccination and retinal vascular occlusion disease remains unknown.

An Innovatory Surgical Technique for Submacular Hemorrhage Displacement by Means of a Bioengineering Perspective.

The purpose of this case report is to present a new surgical technique for the treatment of large Subretinal Hemorrhage (SRH) secondary to Age-related Macular Degeneration (AMD). Considering the biomechanics of foam evolution theory, bubble coarsening effect, and gas-liquid biphasic absorption, an SRH due to an AMD case was treated with vitrectomy. The treatment was implemented by subretinal injection of air bubbles combined with rtPA followed by air fluid exchange. The air bubbles helped mess up and remove the blood from the macula area, and no complications occurred. Two weeks postoperatively, there was no sign of hemorrhage and the Central Macular Thickness (CMT) was sharply decreased from 443 µm to 317 µm. At the five-month follow-up, the CMT remained at 267 µm and the patient's visual acuity improved from light perception to 20/70 according to the Snellen chart. The combination of injecting multiple air bubbles and submacular rtPA, followed by air fluid exchange, was able to displace more than (90%) of the subretinal blood just two weeks postoperatively. Our technique is a promising alternative surgical approach for the displacement of SMH due to AMD, with a clear visual and anatomical benefit seen in the early follow-up period.

The P274S Mutation of Lecithin-Cholesterol Acyltransferase (LCAT) and Its Clinical Manifestations in a Large Kindred.

RATIONALE & OBJECTIVE: Lecithin-cholesterol acyltransferase (LCAT) catalyzes the maturation of high-density lipoprotein. Homozygosity for loss-of-function mutations causes familial LCAT deficiency (FLD), characterized by corneal opacities, anemia, and renal involvement. This study sought to characterize kidney biopsy findings and clinical outcomes in a family with FLD. STUDY DESIGN: Prospective observational study. SETTING & PARTICIPANTS: 2 (related) index patients with clinically apparent FLD were initially identified. 110 of 122 family members who consented to genetic analysis were also studied. PREDICTORS: Demographic and laboratory parameters (including lipid profiles and LCAT activity) and full sequence analysis of the LCAT gene. Kidney histologic examination was performed with samples from 6 participants. OUTCOMES: Cardiovascular and renal events during a median follow-up of 12 years. Estimation of annual rate of decline in glomerular filtration rate. ANALYTICAL APPROACH: Analysis of variance, linear regression analysis, and Fine-Gray competing-risk survival analysis. RESULTS: 9 homozygous, 57 heterozygous, and 44 unaffected family members were identified. In all affected individuals, full sequence analysis of the LCAT gene revealed a mutation (c.820C>T) predicted to cause a proline to serine substitution at amino acid 274 (P274S). Homozygosity caused a complete loss of LCAT activity. Kidney biopsy findings demonstrated lipid deposition causing glomerular basement membrane thickening, mesangial expansion, and "foam-cell" infiltration of kidney tissue. Tubular atrophy, glomerular sclerosis, and complement fixation were associated with worse kidney outcomes. Estimated glomerular filtration rate deteriorated among homozygous family members at an average annual rate of 3.56 mL/min/1.73 m2. The incidence of cardiovascular and renal complications was higher among homozygous family members compared with heterozygous and unaffected members. Mild thrombocytopenia was a common finding among homozygous participants. LIMITATIONS: The presence of cardiovascular disease was mainly based on medical history. CONCLUSIONS: The P274S LCAT mutation was found to cause FLD with renal involvement. Tubular atrophy, glomerular sclerosis, and complement fixation were associated with a worse renal prognosis.