Thirty Years of Experience With Ocular Adnexal T-Cell Lymphoma.

PURPOSE: To evaluate the disease characteristics and survival of patients with ocular adnexal T-cell lymphoma. METHODS: A retrospective, observational study of patients with a histopathological diagnosis of T-cell lymphoma of the ocular adnexa seen between 1992 and 2022. Demographic data, clinical presentation, imaging, histology, immunohistochemistry, treatment, and outcomes were reviewed. RESULTS: Fifteen patients were included in the study with the mean age at diagnosis of 50 years old (range 7-85). The most common presenting symptoms were ulcerated eyelid skin lesions (40%) followed by eyelid swelling (13.3%), and lacrimal passageways obstruction (13.3%). The anaplastic large cell lymphoma (33%) and primary cutaneous T-cell lymphoma (33%) were the most diagnosed peripheral T-cell lymphoma subtypes, followed by the nasal type natural killer/T-cell lymphoma (27%) and peripheral T-cell lymphoma not otherwise specified (7%). The most prevalent stage of disease progression was stage I and stage IV (Ann Arbor classification) with seven (47%) patients each. Eight (53%) patients succumbed to the disease process of which three (37.5%) deceased in the first six months and six (75%) within the first year of diagnosis. We identified a strong statistical association between stage and disease-related death ( p = 0.003). CONCLUSIONS: Peripheral T-cell lymphoma occurring in the orbit and ocular adnexa is extremely rare. Advanced stage at diagnosis leads to almost certain death from the disease despite aggressive local and systemic treatment. Early diagnosis improves the chances of survival but can be hindered by this condition's ability to simulate benign inflammatory conditions both clinically and histologically.

A unique case of Paracentral Acute Middle Maculopathy with associated cystoid macular oedema post uncomplicated pterygium surgery.

PURPOSE: To present a unique and unusual case of Paracentral Acute Middle Maculopathy (PAMM) with associated cystoid macular oedema (CMO) following ocular surface surgery in a low risk patient. OBSERVATIONS: This observational case report describes the presentation of a single case of Paracentral Acute Middle Maculopathy following pterygium surgery. The patient harboured no risk factors and underwent uncomplicated pterygium surgery with no perioperative complications. Post-operatively the patient demonstrated marked visual acuity loss to count fingers only to the operated eye. Optical Coherence Tomography demonstrated hyper-reflective band-like lesions affecting the middle layers of the retina, pathognomonic for PAMM. Unusually, cystoid macular oedema was also evident. He was managed on topical and oral therapy, with limited visual recovery. CONCLUSIONS AND IMPORTANCE: We report a rare and unique case of PAMM with associated cystoid macular oedema occurring in a patient deemed low risk following pterygium surgery resulting in significant permanent visual loss. To our knowledge, this is the first reported incidence of PAMM following ocular surface surgery.