Good syndrome and cytomegalovirus retinitis: A literature review.

Good syndrome (GS) is a rare primary immunodeficiency in adults consisting of hypogammaglobulinemia and thymoma that affects both cellular and humoral immunity. It usually appears in patients between the 4th and 6th decade of life and affects both genders equally. Ophthalmological clinical presentation is highly variable; associations with herpetic keratitis, toxoplasmosis, and cytomegalovirus retinitis (CMVR) have been described. GS associated with CMVR is uncommon. Ophthalmologists may be the first to diagnose systemic disease and change the outcome. Only18 cases of CMVR have been described, most of them unilateral with poor visual outcomes. We discuss the clinical features of CMVR in patients with reported GS, pathogenesis, and outline a work-up for diagnosis. CMVR in an apparently healthy patient should encourage the clinician to search for human immunodeficiency virus (HIV) and non-HIV-associated immunosuppression.

GOOD SYNDROME: CYTOMEGALOVIRUS RETINITIS CASE CHALLENGE.

PURPOSE: To describe cytomegalovirus retinitis in a patient with Good syndrome (hypogammaglobulinemia and thymoma), ocular progression despite treatment and fatal outcome. METHODS: A 71-year-old woman with unilateral panuveitis of unknown cause and a history of thymoma resection was referred to the clinic. Visual acuity was 20/100 in her right eye and light perception in her left eye. In slit-lamp examination, the right eye had inferior, fine, pigmented keratic precipitates, 2+ anterior chamber cells, cataract, and 2+ vitreous cells, with no fundus detail and normal ocular ultrasound results. Left eye presented a white cataract, chronic hypotony, and increased choroidal thickness with calcifications. Laboratory evaluations showed normal or negative results for common causes of infection and inflammation. Prednisolone acetate eye drops were started, with improvement of AC inflammation. Cataract surgery was performed, reaching visual acuity of 20/30. Two years later, visual acuity had decreased and 2+ vitritis and retinitis were found. On clinical suspicion of masquerade syndrome, a vitrectomy biopsy was performed; pathologic assessments reported no data on ocular lymphoma. Leukopenia and lymphopenia were found: immunoglobulin levels, CD4 count, and viral load revealed systemic immunosuppression. The aqueous tap was positive for cytomegalovirus. Oral valganciclovir and intravitreal ganciclovir were initiated. RESULTS: In a patient with previous resection of thymoma and hypogammaglobulinemia, final diagnosis was Good syndrome, with cytomegalovirus retinitis being secondary to immunosuppression. Despite treatment, cytomegalovirus retinitis progressed and systemic deterioration resulted in mortal outcome. CONCLUSION: Good syndrome is an extremely rare disease, and association with cytomegalovirus retinitis is uncommon. To the authors' knowledge, only 14 cases exist in the literature.

Risk factors for recurrences and visual impairment in patients with ocular toxoplasmosis: A systematic review and meta-analysis.

BACKGROUND: Ocular toxoplasmosis (OT) is caused by the parasite Toxoplasma gondii. OT is the leading cause of posterior uveitis globally; it is a recurrent disease that may result in visual impairment and blindness. This systematic review and meta-analysis aim to summarize and evaluate the risk factors for recurrences, visual impairment, and blindness described in the literature worldwide. METHODS AND FINDINGS: We performed a systematic literature search in PubMed, Embase, VHL, Cochrane Library, Scopus, and DANS EASY Archive. All studies reporting patients with clinically and serologically confirmed OT presenting any clinical or paraclinical factor influencing recurrences, visual impairment, and blindness were included. Studies presenting secondary data, case reports, and case series were excluded. An initial selection was made by title and abstract, and then the studies were reviewed by full text where the eligible studies were selected. Then, the risk of bias was assessed through validated tools. Data were extracted using a validated extraction format. Qualitative synthesis and quantitative analysis were done. This study was registered on PROSPERO (CRD42022327836). RESULTS: Seventy two studies met the inclusion criteria. Fifty-three were summarized in the qualitative synthesis in three sections: clinical and environmental factors, parasite and host factors, and treatment-related factors. Of the 72 articles, 39 were included in the meta-analysis, of which 14 were conducted in South America, 13 in Europe, four in Asia, three multinational, two in North America and Central America, respectively, and only one in Africa. A total of 4,200 patients with OT were analyzed, mean age ranged from 7.3 to 65.1 year of age, with similar distribution by sex. The frequency of recurrences in patients with OT was 49% (95% CI 40%-58%), being more frequent in the South American population than in Europeans. Additionally, visual impairment was presented in 35% (95% CI 25%-48%) and blindness in 20% (95% CI 13%-30%) of eyes, with a similar predominance in South Americans than in Europeans. On the other hand, having lesions near the macula or adjacent to the optic nerve had an OR of 4.83 (95% CI; 2.72-8.59) for blindness, similar to having more than one recurrence that had an OR of 3.18 (95% CI; 1.59-6.38). Finally, the prophylactic therapy with Trimethoprim/Sulfamethoxazole versus the placebo showed a protective factor of 83% during the first year and 87% in the second year after treatment. CONCLUSION: Our Systematic Review showed that clinical factors such as being older than 40 years, patients with de novo OT lesions or with less than one year after the first episode, macular area involvement, lesions greater than 1 disc diameter, congenital toxoplasmosis, and bilateral compromise had more risk of recurrences. Also, environmental and parasite factors such as precipitations, geographical region where the infection is acquired, and more virulent strains confer greater risk of recurrences. Therefore, patients with the above mentioned clinical, environmental, and parasite factors could benefit from using prophylactic therapy.

Optical Coherence Tomography Angiography in Macular Holes Autologous Retinal Transplant.

In this paper, we compare the post-operative macular microvascular parameters (vascular density and foveal avascular zone) in eyes with refractory macular hole (MH) that underwent pars plana vitrectomy and autologous retinal transplant (ART) with the fellow unoperated eye. We conducted a retrospective case control study of six consecutive patients who underwent pars plana vitrectomy and ART with at least six months of post-operative follow-up. Pre-operatively, all eyes underwent SD-OCT (Spectral Domain Optical Coherence Tomography) examination. Post-operative OCT-A analyses included vascular density (VD) and the foveal avascular zone (FAZ) area. Six patients with a mean age of 63.7 ± 14.3 years were included. The mean follow-up was 24 months (range 6-30 months). The pre-operative BCVA (best-corrected visual acuity) was 0.99 ± 0.46 logMAR and 1.02 ± 0.23 logMAR at the last post-operative visit (p = 1.00). The mean MH diameter was 966 ± 620 µm. VD in the MH group was 28.1 ± 7.3% compared to 20.2 ± 2.9% in the fellow eyes group (p < 0.05). The mean post-operative FAZ area in the MH group was 109.8 ± 114.6 mm2 compared to 41.5 ± 10.4 mm2 in the control group (p < 0.05). In all six eyes, MH closure was obtained. The post-operative visual acuity did not improve after ART. Eyes with a closed MH showed a bigger FAZ with a higher VD compared to the fellow healthy eye.

Combined Ophthalmology and Rheumatology Evaluation in Systemic Autoimmune Diseases with Ocular Involvement: A Third Level Reference Center in Mexico.

AIM: A cross-sectional descriptive study to determine the frequency of ocular manifestations associated with systemic autoimmune diseases in a third-level hospital in Mexico. METHODS: Records from 2014 to 2017 at the Inflammatory Eye Disease Clinic of the Asociación Para Evitar la Cegueraen México were examined by both an ophthalmologist and a rheumatologist on the same day. Diagnosis was achieved from initial ocular manifestations with later systemic assessment. RESULTS: Out of 311 medical records, 276 were included, 75% of the patients were female. Keratoconjunctivitis sicca was the most frequent ocular manifestation (33.3%), followed by anterior uveitis (29.5%), scleritis (23.2%), and peripheral ulcerative keratitis (7.2%). The leading autoimmune diseases were spondyloarthritis (29%), rheumatoid arthritis (28.6%), primary Sjögren's syndrome (10.5%) and granulomatosis with polyangiitis (9.1%). 41.3% of systemic disease diagnoses were made after an initial ocular manifestation. CONCLUSIONS: Inflammatory eye manifestations can imply systemic autoimmune diseases. It is crucial to suspect and confirm this association and provide timely interdisciplinary management.

Kocuria Endophthalmitis: Clinical Spectrum and Long-term Outcomes.

PURPOSE: To expand the knowledge regarding the clinical presentation of Kocuria endophthalmitis. METHODS: We included six cases of Kocuria endophthalmitis between January 2018 and December 2020. From all cases, we collected general demographic data, relevant medical, ophthalmological history, microbiological and antibiotic sensitivity results. All cases had a minimum follow-up of 9 months. All cases were treated with intravitreal moxifloxacin and dexamethasone and pars plana vitrectomy. Undiluted vitreous, corneal, conjunctival, and aqueous samples were cultured and analyzed with the Vitek 2 system. RESULTS: The prevalence of Kocuria endophthalmitis was 6%. (95%CI: 2.26%-12.73%). The mean age at diagnosis was 60.5 ± 15.5 years. All patients had poor visual acuity, severe pain, and severe conjunctival hyperemia at diagnosis. Only one case had a significant improvement in visual acuity at the end of the follow-up. CONCLUSION: Kocuria spp are an emerging cause of endophthalmitis and should be considered as part of the differential diagnosis.