Taiwan Journal of Ophthalmology最新文献

Advances in retinoblastoma (RB) therapy have led to significantly improved ocular preservation rates, consequently limiting access to histologic and genomic information traditionally obtained from enucleated eyes. Moreover, genomic information from enucleated specimens often represents heavily pretreated, refractory disease. The introduction of aqueous humor (AH) biopsy marks a significant milestone in ocular oncology, offering in vivo, real-time tumoral genomic data that can be collected at diagnosis and repeatedly throughout treatment. This liquid biopsy has detected RB1 gene mutations and deletions and identified potential clinical biomarkers, such as amplification of the 6p chromosomal region and the MYCN gene. In addition, monitoring trends in tumor fraction provides dynamic insights into disease progression. While further research is required to validate these findings in larger populations and uncover new biomarkers, the scientific community has shown growing interest in the clinical potential of this novel approach. AH biopsy is a safe and reproducible procedure that supports precision oncology by providing tumor-specific genomic data for each patient and eye.

Purpose: The purpose of this review is to demonstrate the safety and procedures involved in the use of intensity-modulated radiation therapy (IMRT) and three-dimensional (3D)-conformal techniques for the management of diffuse choroidal hemangioma (DCH) and to compare dosimetric outcomes of these techniques to simulated episcleral plaque brachytherapy in the same cohort of 10 patients (11 eyes) with DCH.

Materials and methods: A single institutional retrospective review of patients with Sturge-Weber syndrome associated DCH managed with IMRT (20 Gy in 10 fractions). Those same patients were planned for simulated brachytherapy and simulated doses to the fovea, disc, and lens were collected. The dose to organs at risk (critical visual and surrounding structures) was compared to determine potential long-term risks.

Results: Ten patients (11 eyes) examined in this review were treated between 2005 and 2023. The use of IMRT and 3D allowed for effective coverage of the planning target volume (PTV) with low doses to critical visual and surrounding structures. Doses to the fovea, disc, and lens using external beam radiotherapy (EBRT) were uniform ranging from 1784 to 2151 cGy, 1695-2204 cGy, and 260-1579 cGy, respectively. Doses to the fovea, disc, and lens using episcleral plaque brachytherapy had ranged from 270 to 12270 cGy, 20623829 cGy, and 270-618 cGy, respectively.

Conclusion: IMRT and 3D-conformal technique used to deliver 20 Gy in 10 fractions provides a uniform safe plan in patients with DCHs with reasonable sparing of the lens, fovea, and optic disc. Simulated brachytherapy yielded variable radiation exposure to critical visual and surrounding structures with potential risk of vision-threatening toxicity. Use of EBRT or brachytherapy should be considered on a case-by-case basis driven by estimated radiation dosimetric parameters.

To elucidate the mechanisms underlying multilayered retinal hemorrhage following anterior chamber paracentesis and to provide a comprehensive overview of the clinical features, risk factors, and prognosis for this complication. We conducted a systematic review in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A comprehensive literature search was carried out up to March 1, 2024, across the PubMed/MEDLINE, Cochrane Library, and EMBASE databases. Studies reporting the cases of retinal hemorrhage following anterior chamber paracentesis were included. In addition, we present three cases of multilayered retinal hemorrhage that developed postparacentesis, with underlying diagnoses of uveitic glaucoma, neovascular glaucoma, and endophthalmitis. We reviewed the literature on seven patients, in addition to three cases from our institution, who developed retinal hemorrhage following anterior chamber paracentesis. Uveitis was the most prevalent diagnosis, accounting for four of the cases. Other diagnoses included neovascular glaucoma, macular branch arterial occlusion, and primary open-angle glaucoma. Fundus examinations revealed preretinal hemorrhage in six patients, superficial hemorrhage in three, blot hemorrhage in nine, and Roth spots in seven. The overall prognosis was favorable, with none of the patients requiring additional interventions for ocular decompression retinopathy (ODR). The reliability of this systematic review is constrained by the limited number of available studies and the inherent biases in case reports. Nevertheless, evidence suggests that anterior chamber paracentesis can cause ODR. ODR can mimic central retinal vein occlusion in fundus images, making accurate diagnosis essential, as the prognosis and therapeutic approaches for these two conditions can differ significantly.

Purpose: To report outcomes in patients treated with low-dose lens-sparing intensity-modulated radiation therapy (IMRT) for diffuse choroidal hemangioma (DCH) associated subretinal (subfoveal) fluid (SRF).

Material and methods: Retrospective interventional case series. 10 patients (11 eyes) treated with IMRT for diffuse choroidal hemangioma-associated SRF. Resolution of subretinal fluid, tumor regression, and best-corrected visual acuity (BCVA).

Results: The mean age was 9.9 years (range, 0.3-32), with 8 (73%) having exudative retinal detachment in the right eye. The rationale for treatment was the presence of SRF (11 eyes, 100%). At baseline, the maximal height of the tumor was 3.4 mm (range, 1.7-5.3). The mean BCVA in the affected eye was 40 ETDRS letters (range, 5-75). All eyes were treated with IMRT (lens-sparing) at a dose of 20 Gy in 10 fractions. Patients were followed up for an average duration of 7 years (range 6-120 months), with 9 (82%) followed up for more than 2 years. Resolution of SRF was noted in all 11 (100%) eyes, with 6 (55%) eyes showing resolution within 3 months. Tumor response was observed in all eyes 11 (100%) (reduction or stabilization of tumor height), with the mean posttreatment height of 2.0 mm. BCVA improved or remained stable in 10 (91%). Radiation-related complications (cataracts, radiation retinopathy, radiation optic neuropathy) were not observed in any of the treated patients.

Conclusions: Low-dose lens-sparing IMRT is a highly effective treatment for diffuse choroidal hemangioma. IMRT resolves subretinal fluid, induces tumor regression, and preserves visual acuity in vast majority of cases.

Orbital venous-lymphatic malformations (VLMs) are complex vascular anomalies that pose significant challenges to ophthalmologists due to their diverse clinical manifestations and the difficulties inherent in their therapeutic management, particularly given their proximity to critical orbital structures. This narrative review synthesizes the current knowledge on the classification, pathology, molecular mechanisms, imaging characteristics, and therapeutic strategies for VLMs. Historically misclassified as orbital lymphangiomas, VLMs are now recognized as complex malformations comprising interconnected venous and lymphatic components, with the potential for significant complications, including proptosis, visual impairment, and esthetic disturbances. Recent molecular insights have revealed critical pathogenetic mechanisms, particularly mutations in the PIK3CA gene that activate the phosphatidylinositol 3-kinase (PI3K)/AKT/mammalian target of rapamycin transduction network, alongside the upregulation of vascular endothelial growth factor (VEGF)-mediated signaling. These aberrations not only illuminate the disease etiology but also present promising therapeutic targets. Contemporary management strategies emphasize a multidisciplinary approach, with sclerotherapy emerging as a particularly promising intervention. Sclerosing agents such as bleomycin and pingyangmycin typically yield effective treatment outcomes with relatively favorable safety profiles. Surgical excision is reserved for accessible lesions but often requires adjunctive therapies due to the infiltrative nature of VLMs. Emerging treatments targeting molecular pathways, including PI3K inhibitors and anti-VEGF therapies, show promise in refractory cases. As our understanding deepens, clinicians can now offer more personalized interventions that consider factors such as lesion location, extent, architecture, and hemodynamic characteristics, thereby minimizing morbidity and optimizing clinical and aesthetic outcomes.

Radiation retinopathy is a common complication of radiation treatment, a standard treatment modality for selected uveal melanoma patients. Although radiation offers high levels of ocular control, radiation complications can preclude useful visual function. An unmet need exists for new approaches to the management of uveal melanoma treatment to avoid such complications. In this review, we explore several important trials underway which have the possibility to change the conventional management approach.

The introduction of immune checkpoint inhibitors (ICIs) into clinical medicine has resulted in more robust response rates for various malignancies, including metastatic and locally advanced periocular and ocular tumors. Their increased utility constitutes a pivotal shift from invasive surgical treatments allowing patients to proceed with eye preserving therapies while also achieving local and metastatic tumor control. The aim of this review article is to provide a summary and updates on the current clinical utility of ICIs for conjunctival and periocular melanoma and squamous cell carcinoma, periocular Merkel cell carcinoma and periocular basal cell carcinoma resistant to BRAF inhibitors. A literature search was conducted on PubMed of programmed cell death protein 1 (nivolumab, pembrolizumab), PD-L1 (atezolizumab, avelumab, durvalumab), and CTLA-4 inhibitors (ipilimumab, tremelimumab) along with previously noted conjunctival and periocular tumors. While this article references several large clinical trials for cutaneous tumors, most of the ocular data are limited to case reports and series. Our overall review presents promising results with the usage of ICI for patients, noting an increased overall survival rate, clinical control of local and metastatic disease and decreased surgical morbidity, while avoiding orbital exenteration. These improvements have not come without considerations for adverse immune-related side effects and clinicians needs to be judicious is deciding between the overall efficacy and side effects.

Purpose: New treatments for advanced retinoblastoma (RB) have offered alternatives to primary enucleation. We assessed the impact of these therapies on the indications for enucleation and the histopathological findings in enucleated eyes with RB.

Materials and methods: Eyes of all patients who underwent enucleation for RB at a single institution between January 2005 and August 2021 were included. Data collected retrospectively included demographics, clinical and pathologic staging, pathologic findings, and management. Statistical analysis included Kendall's τ_b, Pearson χ ², and Cramér's V.

Results: There were 254 eyes from 252 patients with information available for review. Annual enucleations decreased between 2005 and 2008 at a rate of 4.2 enucleations/year, increased from 2008 to 2013 at a rate of 2.7 enucleations/year and decreased from 2013 to 2019 at a rate of 1.5 enucleations/year, reflecting changes in RB therapies. When compared to earlier years, the eyes enucleated in recent years were more likely to be enucleated for patient symptoms (P < 0.001) and insufficient view (P = 0.019), were more likely to have prior treatment (P < 0.001), had lower tumor stage (P = 0.010) and grade (P = 0.006), contained no viable tumor (P < 0.001), and were phthisical (P = 0.003). Five of 252 patients (2%) developed metastases; one of these patients had no viable tumor in a previously treated enucleated eye.

Conclusion: Therapeutic innovations shifted the management of RB from primary enucleation in favor of eye salvage. Enucleated eyes show less viable tumor and disease severity but more intraocular degeneration, emphasizing the importance of skilled pathologic interpretation.