Retinal vein and artery occlusion as the first manifestation of primary antiphospholipid syndrome in a pediatric patient / Oclusão arterial e venosa como primeira manifestação da síndrome antifosfolipide primária em criança

ABSTRACT Antiphospholipid syndrome is an acquired autoimmune disease characterized by hypercoagulability associated with recurrent venous and arterial thromboembolism in the presence of antiphospholipid antibodies. Herein, we report a case of rapid sequential retinal vein and artery occlusion as the first manifestation of a primary antiphospholipid syndrome triggered by an acute Mycoplasma infection in a previously healthy 11-year-old patient. On day 1, ophthalmoscopy revealed a central retinal vein occlusion. The patient developed temporal branch retinal artery occlusion the next day. On day 3, a central retinal artery occlusion was observed. Serum lupus anticoagulant, immunoglobulin (Ig) G anticardiolipin, IgG anti-β2-glycoprotein 1 antibody, and Mycoplasma pneumoniae IgM antibody levels were increased. Thus, retinal vascular occlusions can be the first manifestation of primary antiphospholipid syndrome. Although it may not improve visual prognosis, prompt diagnosis and treatment are essential to avoid further significant morbidity.

RESUMO A síndrome antifosfolipide é uma doença autoimune adquirida caracterizada por hipercoagulabilidade associada a tromboembolismo venoso e arterial recorrente na presença de anticorpos antifosfolipídicos. Aqui, relatamos um caso clínico de oclusão sequencial de veia e artéria da retina como primeira manifestação de uma síndrome antifosfolipíde primária desen­cadeada por uma infeção aguda por Mycoplasma num paciente de 11 anos previamente saudável. No primeiro dia, a oftalmoscopia revelou uma oclusão da veia central da retina. No dia seguinte, o paciente desenvolveu uma oclusão do ramo temporal da artéria central da retina. No terceiro dia, uma oclusão da artéria central da retina foi diagnosticada. Os níveis de anticoagulante lúpico sérico, anticorpos IgG anticardiolipina e IgG anti-β2-glicoproteína 1 e anticorpos IgM para Mycoplasma pneumoniae estavam aumentados. As oclusões vasculares retinianas podem ser a primeira manifestação da síndrome antifosfolipíde primária. Apesar do prognóstico visual ser reservado, o seu diagnóstico e o tratamento imediatos são essenciais para evitar outras morbilidades associadas.

Retinal vein and artery occlusion as the first manifestation of primary antiphospholipid syndrome in a pediatric patient.

Antiphospholipid syndrome is an acquired autoimmune disease characterized by hypercoagulability associated with recurrent venous and arterial thromboembolism in the presence of antiphospholipid antibodies. Herein, we report a case of rapid sequential retinal vein and artery occlusion as the first manifestation of a primary antiphospholipid syndrome triggered by an acute Mycoplasma infection in a previously healthy 11-year-old patient. On day 1, ophthalmoscopy revealed a central retinal vein occlusion. The patient developed temporal branch retinal artery occlusion the next day. On day 3, a central retinal artery occlusion was observed. Serum lupus anticoagulant, immunoglobulin (Ig) G anticardiolipin, IgG anti-ß2-glycoprotein 1 antibody, and Mycoplasma pneumoniae IgM antibody levels were increased. Thus, retinal vascular occlusions can be the first manifestation of primary antiphospholipid syndrome. Although it may not improve visual prognosis, prompt diagnosis and treatment are essential to avoid further significant morbidity.

Visual and refractive outcomes following secondary intraocular lens implantation.

PURPOSE: To analyze the visual and refractive results after secondary IOL implantation using different surgical techniques - iris-claw aphakic IOL through a corneal incision or scleral tunnel, and 3-piece IOL into the ciliary sulcus. PATIENTS AND METHODS: Retrospective study including patients that were submitted to secondary IOL implantation from January 2017 to December 2019 at the Department of Ophthalmology of Hospital de Braga, Portugal. We collected demographic data (age, surgical indication, comorbidities, surgical technique, IOL implanted, and intra and postoperative complications) and visual and refractive data [preoperative and 3-month, 6-month, and 12-month postoperative corrected-distance visual acuity (CDVA), spherical equivalent (SE), manifest cylinder, and intraocular pressure]. RESULTS: 128 eyes from 123 patients were included. The most frequent surgical indications were IOL subluxation/luxation (62.5%) and intraoperative posterior capsular rupture (23.4%). CDVA improved from 1.26 ± 0.51 to 0.47 ± 0.49 logMar (p < .001). CDVA was significantly better in the 3-piece into the ciliary sulcus IOL group than both other groups. The final SE was -0.68 ± 0.94 diopters. The mean manifest refractive cylinder remained stable until the 6th month after the surgery (p = .454) and improved in the last 6 months of follow-up (p = .015). In the postoperative period, the cylinder was higher in the corneal incision iris-claw aphakic IOL group and lower in the 3-piece IOL into the ciliary sulcus group (p < 0,05). The corneal incision iris-claw aphakic IOL group presented the most postoperative complications. CONCLUSION: All techniques showed satisfying results. The 3-piece IOL into the ciliary sulcus group showed the best visual and refractive performance, followed by the scleral tunnel iris-claw IOL group and the corneal incision iris-claw IOL group.

Intraocular Lens Position and Anterior Chamber Parameters Evaluation After Nd:YAG Laser Posterior Capsulotomy for Posterior Capsular Opacification Using Anterior Segment Swept-Source Optical Coherence Tomography.

PURPOSE: To evaluate the effect of neodymium-doped yttrium aluminum garnet (Nd:YAG) laser capsulotomy on intraocular lens (IOL) position and anterior segment parameters with a new swept-source anterior segment optical coherence tomography (AS-OCT) device (Anterion®, Heidelberg Engineering GmbH). PATIENTS AND METHODS: A total of 50 eyes from 50 consecutive patients were included. All patients had visually significant posterior capsular opacification (PCO) after uneventful phacoemulsification surgery with manual capsulorhexis and single-piece C-loop acrylic IOL implantation (AcrySof® SA60AT) and were treated with Nd:YAG laser. Anterior segment images were captured with Anterion® in non-dilated conditions before and one month after the procedure. In the "Metrics App", we collected data of the anterior chamber angle (ACA) 3 and 9 o'clock, the anterior chamber depth (ACD), the anterior chamber volume (ACV), and the central corneal thickness (CCT). We also collected demographic and clinical data [age, gender, months from surgery to Nd: YAG capsulotomy, pre- and post-capsulotomy corrected distance visual acuity (CDVA) and spherical equivalent, and axial length]. RESULTS: We did not find a statistically significant difference in ACD after the Nd:YAG capsulotomy (3.96 ± 0.55 vs 3.97 ± 0.55 mm, p = 0.10). In 28 patients, we noted a backward movement of the IOL; in the remaining 22, the IOL moved forward or did not change at all. We did not observe significant changes in ACA at the 3 and 9 o'clock reference points, ACV, or CCT. There was an improvement in CDVA after the procedure (0.37 ± 0.21 vs 0.12 ± 0.27 logMAR, p = 0.015), without statistically significant differences in SE (-0.15 ± 0.84 vs -0.25 ± 0,93, p = 0.42). Axial length was not correlated with ACD variations after the procedure (p = 0.67). CONCLUSION: Our results show that Nd:YAG laser capsulotomy does not change the axial position of a single-piece C-loop acrylic IOL inside the capsular bag, as well as other anterior chamber parameters.

Macular edema secondary to retinal vein occlusion in a real-life setting: a multicenter, nationwide, 3-year follow-up study.

PURPOSE: To evaluate the presence of macular edema secondary to retinal vein occlusion (RVO)-both central retinal vein occlusion (CRVO) and branch retinal vein occlusion (BRVO)-3 years after diagnosis in patients who underwent intravitreal therapy and to identify potential prognostic factors and biomarkers of persistent macular edema. METHODS: National multicenter, observational, exploratory, retrospective cohort study of 104 consecutive patients with macular edema secondary to RVO diagnosed from January 2014 to December 2015 with minimum 3-year follow-up time. Data analyzed included best-corrected visual acuity (BCVA), clinical and demographic data, and spectral domain optical coherence tomography parameters. RESULTS: At final observation, median baseline central retinal thickness significantly improved from baseline 538 to 290 µm (p < 0.001) and complete macular edema resolution was achieved in 51.0% of patients (56.3% and 42.5% in BRVO and CRVO patients, respectively). BCVA also improved (p < 0.01). Logistic regression analysis revealed a relationship between recurrence of macular edema and disorganization of retinal inner layers (DRIL) at baseline (odds ratio = 2.88; p = 0.013). CONCLUSION: Good long-term anatomical and functional outcomes are achieved with intravitreal treatments in RVO patients. Anatomical success and visual gains seen in the first year were maintained throughout the entire follow-up, though DRIL is a major risk factor for recurrence.

Five-year long-term outcomes of intrastromal corneal ring segment implantation using the manual technique for keratoconus management.

PURPOSE: To evaluate the long-term effectiveness, safety, and stability of Ferrara-type intrastromal corneal ring segments (ICRS) by manual surgery implantation in patients with keratoconus. SETTING: Ophthalmology Department, Hospital de Braga, Braga, Portugal. DESIGN: Retrospective cohort study. METHODS: This study included 124 eyes that had ICRS implantation using the manual technique with a follow-up of 5 years. Uncorrected distance visual acuity (UDVA) and corrected distance visual acuity (CDVA), subjective refraction, keratometry, aberrometry, and pachymetry maps were evaluated preoperatively and at 6 months, 1 year, and 5 years postoperatively. A secondary analysis of all variables was performed comparing 2 subgroups of patients: a group younger than 30 years and a group of 30 years or older. RESULTS: At 5 years, both mean UDVA and CDVA improved significantly (P < .0001) from a preoperative value of 0.91 ± 0.36 to 0.46 ± 0.32 logMAR and 0.40 ± 0.27 to 0.22 ± 0.20 logMAR, respectively. Spherical equivalent, refractive cylinder, and all topography values significantly decreased postoperatively (P < .0001). No regression was observed in any visual or topographic parameter during the entire follow-up. Regarding the subgroup analysis, both younger and older patients demonstrated similar and stable results from the preoperative to the 5-year visit, except for minimum pachymetry value change over time. CONCLUSIONS: Ferrara-type ICRS implantation significantly improved visual acuity, refractive error, and topographic values; the improvement was stable throughout a 5-year follow-up period. This study confirms that ICRS implantation surgery for keratoconus is a stable procedure in a long-term follow-up, regardless of the preoperative patient's age.

Bilateral papilledema and abducens nerve palsy following cerebral venous sinus thrombosis due to Gradenigo's syndrome in a pediatric patient.

PURPOSE: To report a case of bilateral papilledema and abducens nerve palsy following cerebral venous sinus thrombosis in a 9-year-old female with undiagnosed Gradenigo's Syndrome. OBSERVATIONS: The patient presented to our Emergency Room with a unilateral left 6th nerve palsy, left eye relative afferent pupillary defect, and bilateral papilledema. She underwent cranial magnetic resonance imaging with gadolinium contrast and magnetic resonance venography, which diagnosed a left mastoiditis, left sigmoid sinus and jugular vein thrombosis. The patient underwent urgent mastoidectomy and myringotomy with tube placement and was admitted for adequate treatment with intravenous antibiotics and anticoagulants. CONCLUSIONS AND IMPORTANCE: Gradenigo's syndrome is a rare complication of otitis media, and even rarer is cerebral venous thrombosis. This potentially life-threatening situation requires immediate surgical and antibiotic therapy. Ocular symptoms can be the initial signs of this illness.