Enucleation after Stereotactic Radiosurgery in Patients with Uveal Melanoma.

INTRODUCTION: In the past enucleation was the treatment of choice for all the patients with uveal melanoma. Nowadays, we prefer glope-sparing treatment modalities, except for large tumors, tumors with extrascleral extension and painful blind eyes. Most of the patients perform radiotherapy or local resection techniques. In Slovak Republic, the only one possibility is a stereotactic radiotherapy on a linear accelerator LINAC. Nevertheless, enucleation after radiotherapy is necessary for some patients. The causes are postradiation complications, mainly neovascular glaucoma, tumor recurrence, tumor progression or patient´s decision. MATERIAL AND METHODS: The retrospective non-randomised study of 168 eyes of the patients with choroidal or ciliary body melanoma, who performed one-day session stereotactic radiosurgery at the linear accelerator LINAC during period 2007-2016. The data about postradiation complications were analysed based on the medical records of the patients and the data about enucleated eyes based on the histopathological findings. RESULTS: The occurence of enucleation after radiotherapy in our cohort was 17 % (28 patients), with median time period after radiotherapy 21,5 months. The most common cause was neovascular glaucoma (82 %), then tumor progression (14 %) and patient´s decision (4 %). The most common histopathological finding was spindle-cell melanoma. DISCUSSION: Others authors describe similar enucleation rate and causes. The histopathological findings indentified more viable melanoma cells in eyes enucleated for tumor progression in comparison with enucleation for other causes. Enucleation may be more difficult and the occurence of postoperative complications can be higher in the eyes after radiotherapy rather than primary enucleation. CONCLUSION: The management of the patients with uveal melanoma is difficult, and requires the cooperation of ophthalmologist, oncologist, radiation physicist and pathologist. Even if we make effort to preserve the eye, enucleation after radiotherapy is necessary in some patients.

RADIATION MACULOPATHY AFTER ONE-DAY SESSION STEREOTACTIC RADIOSURGERY IN PATIENTS WITH CILIARY BODY AND CHOROIDAL MELANOMA.

INTRODUCTION: Nowadays radiotherapy is the standard treatment care for patients with uveal melanoma. In Slovakia the only one option is a one-day session stereotactic radiosurgery at linear accelerator LINAC. Irradiation of surrounding tissues of the eye and orbit causes radiation complications. One of the most serious and vision-threatening is the radiation retinopathy, which divides into maculopathy and peripheric retinopathy. The clinical signs include microanerysms, teleangiectases, hard exsudates, cotton wool spots and macular edema, neovascularisation, and vitreous hemorrhage. Radiation macular edema can be classified by optical coherence tomography into cystoid or noncystoid edema. On fluorescein angiography macular edema is divided into non-ischemic and ischemic, while the latter means usually irreversible loss of the visual acuity. This paper is focused on risk factors of radiation retinopathy although it strongly influences functional result of radiotherapy and preservation of the visual acuity. PURPOSE: By means of bivariate and multivariate analysis to determine the association of radiation dose and other variables with the development of radiation maculopathy. MATERIAL AND METHODS: The retrospective analysis of 168 patients with ciliary body or choroidal melanoma who performed one-day session stereotactic radiosurgery on a linear accelerator LINAC in a period 2007-2016. RESULTS: The prevalence of the radiation maculopathy was 29% with the median time from the irradiation to maculopathy 16 months. Median radiation dose on the macula was 37 Gy. Variables statistically significantly associated with the maculopathy were: radiation dose (p = 0.0016), postequatorial location of the tumor (p = 0.0271) and better visual acuity before treatment (p = 0.0007). The tumor touch of the macula was strongly associated with the visual acuity in the bivariate analysis (p = 0.0006), thus it could be omitted from the final model. DISCUSSION: The radiation dose on a macula is the key determinant for radiation-induced maculopathy, according to other authors and our study approvingly. Other variables were related to proximity of the tumor to the macula, so the radiation dose on the macula was higher indirectly. Better visual acuity before treatment as a risk factor for maculopathy can be a consequence of: a) earlier diagnostics of tumor with proximity to the posterior pole, b) lower enucleation rate; frequency of the enucleation was 1.6 higher in patients without maculopathy. Naturally, on enucleated eyes maculopathy was not diagnosed. Five patients underwent intravitreal application of the bevacizumab as a treatment of the radiation maculopathy, without improvement of the visual acuity. CONCLUSION: Radiation complications can be vision and eye threatening. Radiation maculopathy is a consequence of higher radiation dose on the macula. The treatment modalities of radiation maculopathy are rather ineffective.

[Orbital Exenteration in Patient with Metastatic Choroidal Melanoma - a Case Report]. / Exenterácia orbity u pacienta s generalizovaným choroidálnym melanómom.

INTRODUCTION: Uveal melanoma is the most common primary intraocular tumour in adults in Caucasians and in 75% is arising from choroid. It threatens not only the patients loss of vision and eye, but also 50% of patients after 5-year interval after therapy die due to distant metastases. The treatment of small and medium-sized melanoma are methods preserving eye globe. Almost half of the total number of patients is still unavoidable enucleation. Considerably rarer is indicated exenteration of an orbit. These tumors metastasize only hematogenous, while the most frequent place of localization of distant metastases is the liver. Generalized disease prognosis is poor, and our current treatment options in this stage are ineffective. MATERIAL AND METHODS: Case report of 59 years old patient with choroidal melanoma stage T4 N1 M1 massively infiltrating the orbit. At the time of diagnosis of the primary tumor distant metastases were present. The patient underwent exenteration of the orbit and systemic chemotherapy. DISCUSSION: Although choroidal melanomas with extrascleral extension and infiltration into the orbit have no better prognosis after exenteration of the orbit, surgery is providing us local tumour control. Good cosmetic effect after this mutilating procedure is offered by individually made prosthesis (epithesis). All patients with uveal melanoma require lifelong dispensation, distant metastases may occur even after many years. In the treatment of generalized disease is available systemic chemotherapy and immunotherapy only palliative. The best effect on survival has complete surgical resection of single metastasis. Uveal melanoma has a different genetic profile as cutaneous melanoma. The biological nature of uveal melanoma seems to be the key to determining risk patients, as well as the development of targeted systemic therapy. CONCLUSION: Treatment of patients with generalized large uveal melanoma with extrascleral extension is difficult. A better understanding of biological interest may be the key to the detection of patients at higher risk of distant metastases formation, and to an effective systemic treatment. KEY WORDS: large uveal melanoma, extrascleral extension, orbital exenteration, the treatment of generalized disease.

[Cataract after lightning injury--case report]. / Katarakta po úraze bleskom.

Lightning injuries are dangerous and often deadly trauma. We present a case of 41 year old woman who suffered an indirect lightning injury, dropping into unconsciousness followed by amnesia. Initial irritation of her left eyeball was followed by subtle changes in retinal pigment epithelium without any drop in visual acuity for a period of three months. Subsequently there began a slow deterioration of visual acuity and progression of cataract causing BCVA to drop to 5/50. Patient underwent uncomplicated cataract phacoemulsification with IOL implantation which resulted in restoring BCVA to 5/5 postoperatively. Such cases are scarce in literature.

[Iris racemose vascular anomalies]. / Cievne anomálie dúhovky racemózneho typu.

Authors present 7 rare iris racemose vascular anomalies that were discovered by biomicroscopy during routine ocular examination. The morphology, clinical features and iris circulation of these cases are documented by iris fluoroangiography. They also report common features, some specific details of arrangement and classification criteria. The iris vascular anomaly appears to be benign stationary condition that has none systemic and ocular associations.

[Retinal and retinal pigment epithelium hamartoma]. / Kombinovaný hamartóm retiny a retinálneho pigmentového epitelu.

Authors present four patients with combined hamartoma of the retina and retinal pigment epithelium who were observed for a period of 10 years. The significance of this entity lies primarily in its resemblance to malignant condition of the retina and choroid and in the vizual reduction. Clinical finding, diagnosis, natural history, histopathology, association with other diseases and therapy are discussed in this report as well.

Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.

OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.