RESUMEN

The choroid is a thin layer of highly vascular uveal tissue enclosed externally by sclera and internally by neurosensory retinal tissue. The choroid is a "middle layer" ocular tissue with anatomically challenging surgical access. The primary functional role of the choroid is to provide rapid, oxygenated, and nutrient-rich blood flow to both the highly metabolic retinal pigment epithelium and outer retina (i.e. photoreceptors) while simultaneously removing waste products. Historically, incisional choroidal surgery (ICS) has involved tumor biopsy or excision, removal of choroidal neovascular complex or autologous choroidal translocations; however, ICS also holds unique potential for novel and innovative approaches to address macular pathology. Using large-animal surgical studies, researchers have explored ICS with the objective of finding safer and more effective techniques to reduce surgical risks such as bleeding, tissue contraction, and scar tissue formation. We explore the relevant anatomy and embryology, existing surgical techniques, discuss the implications for retinal drug delivery, define ICS guiding principles, and offer a rationale for implementation of ICS into a vitreoretinal surgical practice. We also identify other future challenges and anticipate future innovations that will advance ICS.

RESUMEN

Introduction: This case series aims to present the unusual clinical manifestation of subretinal exudation in patients diagnosed with untreated choroidal melanoma. A total of 886 patients were diagnosed and treated for primary choroidal melanoma between November 2017 and June 2023 at St. Paul's Eye Unit, Royal Liverpool University Hospital, UK. The fundus photographs were screened for lipid exudates by two independent clinical experts. The patients' demographics, clinical manifestations, and imaging features were analysed, whereas the location of exudation was documented with fundus photographs and optical coherence tomography (OCT). The histopathological and genetic results were also analysed in cases with tumour biopsy available. Case Presentations: Eight cases with subretinal exudates were identified (n = 8/886, 0.90%). No gender predilection was noticed (male/female 1:1), whereas the mean age was 51 years (range 39-79). Four patients were asymptomatic at presentation, 2 patients reported reduced visual acuity, and 2 patients presented with photopsia. OCT scans revealed the presence of subretinal fluid and subretinal exudates, while the ultrasound showed medium or low internal reflectivity in 7 out of 8 cases. The biopsy analysis was available in 4 cases, all showing low-risk spindle cell choroidal melanoma with disomy 3. Conclusion: Lipid exudates are an atypical fundoscopic finding in patients with untreated choroidal melanoma. The subretinal location could differentiate them from other retinal vascular conditions and facilitate early diagnosis and intervention. Interestingly, all cases tested cytogenetically were of low metastatic risk; these exudates may, therefore, be a positive clinical prognostic sign.

RESUMEN

A 72-year-old Chinese male presented with unilateral left eye panuveitis, then diagnosed as bilateral T-cell primary vitreoretinal lymphoma (T-PVRL) through chorioretinal biopsy and immunohistochemistry. No CNS nor systemic involvement was found at diagnosis. Despite initiating intravenous and intrathecal chemotherapy and intravitreal methotrexate, the disease eventually spread to the fellow eye with subsequent recurrence and systemic metastasis. To our knowledge, no cases of T-PVRL treated in a silicone-filled eye were reported in the literature. T- PVRL is exceedingly rare, with most PVRL being the malignant B-cell variant. This case highlights the challenges encountered throughout the treatment course of this aggressive entity, including the administration of intravitreal methotrexate in a silicone oil-filled eye. The poor overall survival rate and grim prognosis of T-PVRL are highlighted. Therefore, we recommend prompt tissue biopsy and immediate initiation of systemic chemotherapy and intravitreal methotrexate.

RESUMEN

PURPOSE: To report the clinical course of patients with diagnostic confirmation of choroidal lymphoma by anterior chamber paracentesis and aqueous fluid flow cytometry. METHODS: Single-center case series. RESULTS: Two patients with choroidal thickening were suspected to have choroidal lymphoma based on clinical findings and ultrasonographic evidence of extrascleral extension. In each case, anterior chamber paracentesis was performed due to the observation of the associated anterior chamber reaction. Flow cytometry detected the presence of a clonal B-cell population consistent with non-Hodgkin's lymphoma. In one case, external beam radiation therapy resulted in a complete therapeutic response. More invasive methods of ocular tissue biopsy were avoided. CONCLUSIONS: Definitive diagnosis in suspected cases of choroidal lymphoma remains challenging. Ocular fluid sampling may be a low morbidity and convenient alternative for confirming a suspected diagnosis in cases associated with cellular infiltration of the intraocular fluids.

RESUMEN

PURPOSE: To describe the management of submacular hemorrhage (SMH), a vision threatening complication following transvitreal choroidal biopsy, with intravitreal tissue plasminogen activator (tPA) and pure perfluoropropane (C3F8) gas bubble injection. OBSERVATIONS: A 53 year old female with choroidal melanoma of the left eye underwent iodine-125 plaque brachytherapy placement and 27 gauge transvitreal fine needle aspiration choroidal biopsy for gene expression profiling. On postoperative day 2, large SMH was identified on dilated fundus examination. At the time of plaque brachytherapy removal, intravitreal tPA and pure C3F8 gas bubble injection with post operative positioning was also performed to attempt displacement of SMH. At postoperative month 1 following tPA and gas bubble displacement, the SMH was completely displaced inferotemporally outside of the macula and visual acuity improved from 20/70 at postoperative week 1 to 20/25 at postoperative month 1. CONCLUSIONS AND IMPORTANCE: Subretinal hemorrhage can be a complication of transvitreal choroidal tumor biopsy but early detection and prompt treatment can result in good visual outcomes.

RESUMEN

Rosai- Dorfman disease (RDD) is a rare systemic pseudo-lymphomatous disorder with unknown etiology. No guidelines exist regarding its management and treatment when the disease is progressing. Choroidal involvement in RDD has rarely been reported and has often been misdiagnosed. We describe a case of a 64-year-old male diagnosed with RDD by means of choroidal biopsy, successfully treated with a MEK inhibitor, namely Cobimetinib, and its follow-up over 5 years, with good final anatomical and functional results. This is the first reported case of RDD diagnosed with an intraocular biopsy performed on a non-enucleated globe, thus preserving the integrity and function of the eye. This case emphasizes the need for a choroidal biopsy when the diagnosis is not straightforward and the starting of targeted therapy to retain a good visual function.

Asunto(s)

Azetidinas , Histiocitosis Sinusal , Biopsia , Humanos , Masculino , Persona de Mediana Edad , Piperidinas

RESUMEN

Purpose: To report of a 55-year-old woman suffering from choroidal infiltrates as a first clinical manifestation of T-LGL leukemia. Methods: Retrospective case report. Results: A healthy woman presented with photophobia in both eyes since 1 month. She showed a panuveitis with anterior chamber as well as vitreous cells, creamy-white choroidal lesions in both eyes and a cystoid macular edema in the left eye. Lab testing showed only a moderate lymphocytosis, all other tests, including a pars plana vitrectomy and an anterior chamber tap, were negative. Due to the persistent lymphocytosis, she was referred to the oncologist. A biopsy of the bone marrow revealed T-LGL leukemia. A subsequent biopsy of the choroid showed an infiltration of T-LGL and therefore systemic therapy with cyclophosphamide was started. Conclusions: This is a very rare case describing an involvement of the choroid in T-LGL leukemia.

Asunto(s)

Neoplasias de la Coroides/diagnóstico , Leucemia Linfocítica Granular Grande/diagnóstico , Antineoplásicos Alquilantes/uso terapéutico , Neoplasias de la Coroides/tratamiento farmacológico , Colorantes/administración & dosificación , Ciclofosfamida/uso terapéutico , Femenino , Angiografía con Fluoresceína , Humanos , Verde de Indocianina/administración & dosificación , Leucemia Linfocítica Granular Grande/tratamiento farmacológico , Linfocitosis/diagnóstico , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía de Coherencia Óptica

RESUMEN

PURPOSE: The aim of this paper is to describe a case of relapsed pediatric acute lymphoblastic leukemia (ALL) presenting as a rapidly progressive subretinal infiltrate, as diagnosed by ultrasound-guided fine needle aspiration (FNA). METHODS: We conducted a clinical pathological retrospective chart review. RESULTS: Eleven months after documented remission of T-cell ALL while on maintenance therapy, this 17-year-old patient presented with acute open angle glaucoma in the right eye. B-scan ultrasonography suggested total retinal detachment. Eight weeks later, based on routine cerebrospinal fluid analysis, the patient was diagnosed with central nervous system relapse of T-cell ALL. Repeat B-scan 1 week later showed a new hyperechoic subretinal mass. FNA of the mass confirmed leukemic infiltrate. The involved eye was enucleated, demonstrating leukemic cells throughout the subretinal space, choroid, and the optic nerve. Following hematopoietic stem cell transplant, the patient continues to maintain bone marrow remission 5 months after enucleation without involvement in the opposite eye. CONCLUSION: Retinal detachment in any patient with a history of leukemia should raise the possibility of relapse and may warrant aspiration/biopsy if other means of diagnosing relapse are inconclusive. Subretinal infiltrate may progress rapidly and prompt diagnosis is paramount to tailoring therapy and preserving vision.

RESUMEN

PURPOSE: We hypothesized that the elastic nature of the choroid leads to tissue contraction following a full-thickness, sharp incision. Furthermore, we sought to quantify, measure, and compare tissue contraction in ex vivo porcine globes and human globes of various ages using predetermined variables. METHOD: A full-thickness, ex vivo choroidal incision was performed in either pig (n = 97) or human (n = 30) specimens. Variables included trephine diameter (1.5, 2.0, or 2.5 mm) versus a straight surgical blade, and temperature (1.7 °-4.4° vs. 36.6°F). Central centripetal and surround centrifugal tissue contractions were measured. Mean percentage tissue contraction was assessed as a ratio of trephine diameter to final tissue contraction measured immediately following each incision using a standardized device. RESULTS: For trephination in pig specimens, centripetal contraction ranged from 38% to 50% with a mean of 44%. Centrifugal contraction was approximately 15%. Human choroidal contraction was 39% and 15%, respectively, with a statistically significant inverse relationship to age (R2 = 0.35, P ≤ 0.01). Asymmetric contraction was noted when incisions were closer to choroidal attachment sites to the sclera, such as near vortex ampullae. Linear incisions resulted in contraction that correlated with incision length (R2 = 0.35, P ≤ 0.001). CONCLUSIONS: A full-thickness choroidal incision results in significant tissue contraction. For circular incisions, the centripetal contraction approaches 50% of the original incision size. For linear incisions, the contraction corresponds directly with incision length. In human specimens, there is less contraction with advancing age. TRANSLATIONAL RELEVANCE: Our findings have clinical relevance for choroidal biopsy, traumatic injury, and choroidal translocation surgery.