Palladium-103 plaque radiation therapy for retinal angioma

Reply: Mechanisms of post-radiation optic atrophy with neuroretinal rim thinning

Von Hippel-Lindau disease (VHL) is an autosomal dominant tumour syndrome caused by germline mutations of the VHL tumour suppressor gene located on chromosome 3p25-26. In VHL tumours may occur in 14 different target organs, including the eye. Retinal angiomas are considered the first manifestation of VHL disease in 43% of cases, and the cumulative probability of developing a retinal angioma in one or both eyes rises during each decade of life, reaching 80% for patients over 80 years old. Since 1976 patients with VHL at the University Hospital of Utrecht and their at-risk relatives have been screened periodically by a multidisciplinary team. Long-term follow-up ophthalmological data were analysed with special attention to natural course and results of treatment. In addition, we looked for a genotype-phenotype correlation. Retinal angiomas were found in all families. In one large family with a missense mutation (V170D) of the VHL gene, in which the complete spectrum of visceral- and central nervous system (CNS) features of VHL is present, macular, parapapillary, optic disc and ora serrata angiomas were also found. In general, however, a clear-cut genotype-phenotype correlation could not be found. Only early detection and treatment of peripheral retinal angiomas can be expected to decrease the percentage of patients with decreased visual acuity. Therefore, early detection and treatment of these tumours is of paramount importance. Ophthalmological screening of patients and persons at risk should start as early as possible. In patients with apparently sporadic retinal angiomas it is advisable to perform germline DNA analysis, since the risk of developing VHL is high, especially if the angiomas are bilateral, or unilateral and multifocal, if the patient is young, or if there is a family history suggestive of VHL.

Palladium-103 Radiation Therapy for Small Choroidal Melanoma

Regression Patterns of Iris Melanoma after Palladium-103 (103Pd) Plaque Brachytherapy

Quantitative OCT Angiography Evaluation of Peripapillary Retinal Circulation after Plaque Brachytherapy

Purpose Plaque radiotherapy is the first choice of treatment for choroidal melanomas. The standard practice is to position the plaque centrically in relation to the tumour, with a 2mm physical safety margin in all directions. The author has developed techniques for administering brachytherapy with plaques positioned eccentrically, with their posterior edge aligned with the posterior tumour margin. This allows a higher apex dose to be given while reducing the radiation delivered to optic nerve and fovea. Methods The tumour is localized by transillumination and its anterior margin marked on the sclera with a pen. The 'plaque‐tumour difference' measurement is then marked on the sclera at the point where the anterior plaque margin should be. A transparent plaque template is sutured to the sclera. The position of the plaque is checked with a right‐angled fibre‐optic transilluminator, which is passed through a perforation near the posterior edge of the template. If the template is positioned correctly, then with binocular indirect ophthalmoscopy one should see the 'sunset sign', which occurs when the transilluminator is exactly at the posterior tumour margin. Results Eccentric ruthenium plaque positioning has been practised in Liverpool for over a decade. The results have been published. Local tumour control rates have been good (partly because of case selection). Radiation maculopathy and optic neuropathy have been rare if the tumour has not extended within 1 mm of these structures. The main cause of visual loss has been maculopathy, caused by exudation from the irradiated tumour. Methods for treating such exudation are described. Conclusion Plaque radiotherapy is effective for choroidal melanomas up to 5 mm in thickness. It achieves good local tumour control with preservation of vision in most cases.

Pseudo-Foster Kennedy syndrome due to idiopathic intracranial hypertension

Radiation-Induced Optic Neuropathy: Observation versus Intravitreal Treatment: Can Visual Acuity Be Maintained by Intravitreal Treatment?

This study aimed to evaluate treatment effects of intravitreal gas for macular hole with localized retinal detachment (RD) in highly myopic eyes and to examine how the vitreomacular relationship and other factors may influence treatment outcomes. Twenty highly myopic patients with macular holes and localized posterior RD, treated initially with intravitreal injection of C(3) F(8) , were prospectively studied. Recurrent disease was treated with repeated gas injection or vitrectomy according to the extent of detachment. Length of follow-up was ≥ 12 months. Vitreomacular conditions were determined before and within 2 months after gas injection by standard optical coherence tomography. Demographics, refractive errors, axial length, the extent of chorioretinal (CR) atrophy and visual acuity were recorded. The success rate after primary gas injection was 70%. The remaining 30% of patients achieved anatomic success after further treatment, including vitrectomy with gas or silicone oil tamponade and/or scleral buckling. All cases achieved anatomic success after a mean of 1.4 surgeries. Univariate analysis showed that the patterns of the posterior vitreoretinal relationship did not differ significantly between those successfully treated with gas only (group 1) and those requiring vitrectomy (group 2) (p = 1.000). Logistic regression showed no statistically significant differences in any characteristics between groups 1 and 2. Intravitreal gas tamponade alone may achieve anatomic success in more than two-thirds of highly myopic patients with macular holes and localized RD. Patients with different clinical characteristics and vitreoretinal relationships seem to have similar opportunities to achieve reattachment through this relatively non-invasive surgery.

Palladium-103 plaque radiotherapy for choroidal melanoma: Results of a 7-year study

Lack of Radiation Maculopathy After Palladium-103 Plaque Radiotherapy for Iris Melanoma

Introduction Uveal melanoma has a relatively low incidence. Transpupillary thermotherapy (TTT), hypofractioned stereotactic radiotherapy (RT), stereotactic radiosurgery, plaque brachytherapy, charged particle radiation therapy, local tumor resection, enucleation, and exantation are applied in the treatment. Methods The importance given to radiotherapy has increased to get more satisfactory results while treating the patient. However, it is the treatment of radiation retinopathy and optic neuropathy from complications. Results Radiation retinopathy and optic neuropathy are the most important complications related to radiotherapy in the treatment of uveal melanoma. In recent years, many studies have been performed on the treatment of radiation retinopathy and optic neuropathy. Conclusion The consecutive use of triamcinolone in combination with anti-VEGF supports that it may be a future therapeutic agent in the treatment of complications.

The authors review the histopathologic findings in the eyes of a patient with multiple retinal angiomas and von Hippel-Lindau disease, who underwent treatment with argon laser photocoagulation with follow-up of more than 20 years. The patient was studied ophthalmoscopically and by fluorescein angiography before and after argon laser photocoagulation of retinal angiomas. The eyes were obtained postmortem, and the central portion of the right eye, including the macula and optic nerve head, was sectioned serially for light microscopy. The pupil-optic nerve segment of the left eye was step-sectioned serially for light microscopy. Histopathologic study of the right eye disclosed mild cystoid macular edema and focal areas of exudation in the midperiphery possibly secondary to irradiation of the head. A 1.5 x 0.3-mm area of residual angioma was present in the nasal peripapillary retina. Superotemporally, four chorioretinal scars were present in one photocoagulated area. These scars were composed of dense fibrous tissue with vascularization and variable retinal pigment epithelium hyperplasia. Large, nonangiomatous vessels within each of the scars were continuous with other retinal vessels. Inferotemporally, two chorioretinal scars were present in one photocoagulated area. Histopathologically, these scars were similar to the superotemporal scars, except that no patent retinal vessels traversed the inferotemporal scars. Neovascularization of the retina was associated with one superotemporal and one inferotemporal scar. No residual angiomatous tissue was present in the supero- or inferotemporal areas. Histopathologic examination of the left eye disclosed extensive vitreous organization and periretinal fibrovascular proliferation, extensive gliosis of the retina, and a 4.5 x 2-mm schisis cavity filled with fibrinous exudate. Three angiomas with variable fibrosis were present in the left eye. Despite a poor clinical course in one eye treated with xenon arc photocoagulation, trans-scleral diathermy, and argon laser photocoagulation, a patient with von Hippel-Lindau disease and multiple retinal angiomas retained good vision in the other eye after successful treatment with argon laser photocoagulation with follow-up of more than 20 years. The amount of regression of angiomatous tissue after photocoagulation varied from lesion to lesion (complete in some; minimal in others). The authors conclude that argon laser photocoagulation of early lesions is effective in ablating smaller ( < or = 3-disc diameter) retinal angiomas.

Early Peripapillary and Macular Microvascular Changes Following Ruthenium-106 Plaque Brachytherapy For Uveal Melanomas.

Radiation optic neuropathy after megavoltage external-beam irradiation: analysis of time-dose factors