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Purpose A 52-year-old woman who had undergone enucleation of the left eye at the age of 7 years for posterior uveal melanoma, had no further problems until 45 years later, when she developed a multifocal malignancy of the ipsilateral orbit. Methods Case report. Results MRI documented the presence of nodular multiple masses in the anophthalmic socket suggesting possible metastatic disease. Hepatic examination and ultrasound evaluation, CT scan of the thorax and bone scintigraphy excluded other sites of metastatic lesions. She underwent excissional of the 3 distinct tumors, 2 inferiorly and one superiorly in the anophthalmic socket. After surgery she received proton beem radiotherapy in the anopthalmic socket for a total of 4.600rads. Histopathology documented malignant melanoma of the orbit characterized by epitheliod cells. Conclusions The long interval between recognition of the primary tumor and enucleation and the appearance of clinically manifest metastatic disease in the ipsilateral orbit is the longest reported in the literature to date. A similar case of metastatic choroidal melanoma to the contralateral orbit 40 years after enucleation has been reported by Coupland (1996).

Summary En Face OCT and OCT Angiography are two innovative diagnostic modalities which were adapted for the diagnosis, therapeutic decision and observation of the evolution following conservative management in ocular oncology. En Face OCT is particulary useful for (1) The definition of the extend of serous retinal detachment and the documentation of its variations (2) The definition of the internal anatomical structure of unpigmented choroidal tumors (3) The definition of the limits of flat and diffused choroidal melanomas in order to define the target volume of the irradiation. Angio-OCT in ocular oncology is mainly used for the definition of retinal vascular lesions observed in radiation induced optic neuropathy and radiation induced maculopathy. This allows a better definition of the therapeutic strategies with anti-VEGF drugs in order to preserve visual function.

Purpose To study the outcome of patients treated for a choroidal hemangioma with proton beam irradiation following unsuccessful PDT and compare the therapeutic results with that of a control group that received proton therapy as a primary treatment. Methods Proton beam irradiation was applied to twelve cases of choroidal hemangioma that had been unsuccessfully treated with PDT (N of sessions: 1–5). Their functional results were compared to those of a control group of 48 cases treated only with proton therapy. The latter were matched (4 to 1) for the duration of symptoms before primary treatment, extent of retinal detachment as well as distance to the macula. Results The mean follow-up following proton beam irradiation therapy was 28 months [range: 6 months-5 years] in the PDT group and 41 months [range: 6 months-10 years] in the control group. In all patients, the retinal detachment and macular edema disappeared following proton therapy. In the study group, final visual acuity was ≤0.1 in 25%, 0.2–0.5 in 25% and ≥0.6% in 50% of cases. Visual acuity improved in 50%, remained stable in 42% and decreased in 8% of cases. In the control group, final visual acuity was ≤0.1 in 4%, 0.2-0.5 in 38% and ≥0.6 in 58% of cases. Visual acuity improved in 65%, remained stable in 29% and decreased in 6% of cases. Conclusions Proton beam irradiation is a valid therapeutic option after inconclusive results following PDT for choroidal hemangioma. Within the limits of this study, therapeutic results were slightly better for those patients that received proton therapy as a primary treatment.

The advanced course of radiotherapy in ophtalmology covers all the technical and medical details of irradiation treatment in occular oncology. Following an historical overview of the evolution of occular radiotherapy all the modern technics of occular radiotherapy are described. The Brachytherapy and Teletherapy with Proton beam of uvual melanomas the irradiation treatment of choroidal hemangiomas, the irradiation treatment of metastatic tumors and lymphomas as well as radiotherapy of pediatric tumors are clearly illustrated.

Neovascular glaucoma is a major complication after proton therapy of uveal melanomas, and is provoked by vasoproliferative agents produced by the tumor or ischemic retina. Risk factors for the presence of an ischemic retina at presentation are largest tumor diameter (LTD) tumor height and the extent of retinal detachment. In a prospective study the efficacy of preventive anti VEGF treatment was estimated following Proton Beam radiotherapy of uveal Melanomas associated with an ischemic retinal detachment. None of the treated cases and 32% of the controlled group developped an neovascular glaucoma. The anti VEGF treatment was completed with and extensive laser photocoagulation of the ischemic retina. Anti VEGF treatment associated to retina photocoagulation appears as valuable therapeutic modality in selected cases of uveal Melanomas following Protom Beam radiotherapy.

Congenital hypertrophy of the Retinal Pigment Epithelium (CHRPE) is a tumour which may be confused with pigmented tumours of the choroid such as nevi or melanomas. These lesions are round or oval shaped, with borders that are well defined or scalloped. They present areas of atrophy which may increase in size. This increase in size of CHRPE is well documented and innocent. However, transformation in adenomas or even adenocarcinomas is also possible. In consequence, a periodical observation for Congenital hypertrophy of the Retinal Pigment Epithelium is generally recommended.

Purpose To present the indications of Anti-VEGF treatment in ocular oncology. Methods Intraocular anti-VEGF injections were used for: • The prevention or the treatment of neovascular glaucoma following irradiation treatment of intraocular tumors (uveal melanomas, choroidal hemangiomas, choroidal metastasis). • The control of cystoids macula edema related to the conservative management of intraocular tumors. • The treatment of secondary retinal detachment related to vascular tumors and pseudo tumors and mainly the Coat’s disease. Results A dramatic decrease of the risk of neovascular glaucoma was obtained with intravitreal anti-vasoproliferative treatment following irradiation treatment of intraocular tumors. The presence of retinal ischemia associated to the secondary exudative retinal detachment is observed with panoramic (150°) fluorescein angiography. The anti-vasoproliferative treatment is delivered during the period preceding the reatachment of the retina which allows a photocoagulation treatment. Anti-vasoproliferative treatment in case of established iris neovascularisation allows a functional success (IOP less than 20mm Hg) in 84% of the cases and anatomical success (reduction of the iris neovascularisation and of diffusion of the dye in fluorescein angiography) in 75% of the cases. Impressive reduction of the secondary retinal detachment is obtained following anti-vasoproliferatives treatment in cases of Coat’s disease allowing the photoablation of the reattached retina. Conclusion Anti-vasoproliferative treatment in ocular oncology is a major contribution for the reduction of side effects induced by irradiation treatment and to maintain useful function in treated eyes.

Purpose Adenocarcinomas of the retinal pigment epithelium (RPE) are rare adult tumors, treated with enucleation or surgical excision. We present a child with an adenocarcinoma of RPE, treated with protontherapy. Methods A 10-year-old girl with loss of vision had a pigmented macular tumor, diagnosed as hamartoma. Five years later, tumor growth led to a transvitreal incisional biopsy. Results Histopathology and immunohistochemistry were compatible with an adenocarcinoma of the RPE. The tumor was irradiated. After 5 years of follow-up, the tumor is under control and the patient presents no distant metastases. Conclusion Adenocarcinomas of the RPE can simulate a combined hamartoma of the retina and RPE. Diagnosis can only be established after biopsy. Protontherapy is a valid therapeutic alternative.

Purpose To present the spectrum of various therapeutic options in the practical management of small melanocytic tumors Methods The therapeutic decision is influenced by: • The cumulative risk factors. • The presence or absence of documented tumor growth. • The localisation and the volume of the tumor. • The personal data of the patient. • The expected functional results following a conservative management. Results The predictions established by the statistical evaluation of the various risk factors, in the majority of the cases are confirmed by the therapeutic outcome. However, an unpredictable evolution which may occur in selected cases has to be taken in account in global therapeutic strategies which are used for the management of small melanocytic tumors. Conclusion Successful management of small melanocytic tumors depends on a correct interpretation of risk factors and careful observation of all individual cases.

Indirect ophthalmoscopy and fundus drawing, fundus photography, standard and 150° ICG, standard and 150° fluorescein angiography, auto fluorescence, OCT, ultrasonography, UBM, MRI and in selected cases intraocular biopsy are the main diagnostic tools used in ocular oncology. The tumors can be documented either by composite standard fundus pictures, or panoramic fundus photographies. Modifications of the pigment epithelium are observed either by standard fluorescein angiography and by standard ICG or by autofluorescence. Hemodynamic modifications of the choroide and the retina are observed with panoramic fluorescein angiography and ICG. Modifications of the retina on the surface of the tumors and modifications of the macula are studied with high resolution OCT. The shape and the height of the tumors are measured with standard 20 or 10 MHz, B mode ultrasonography. The intra tumoral reflectivity and attenuation are studied with A mode ultrasonography. The volume and the shape of tumors located in the anterior segment are observed and studied with non contact 50,35 or 20 MHz high resolution ultrasonography. In selected cases, MRI, cytology and cytogenetics are used in order to define the diagnosis. In small tumors the final diagnosis is often established following a documented tumor growth.

Purpose To present the anti-VEGF treatment in neovascular glaucoma following proton beam irradiation in uveal melanomas. Methods Prospective evaluation on 69 cases of iris neovascularisation and neovascular glaucoma treated with anti-VEGF intraocular injections. Results Iris neovascularisation was classified as pupillary 26%, sectorial 16%, tuffts 15% and diffused 43%. A sectorial iris ischemia related to proton beam irradiation was present in 64% of the cases. Following an observation period of 6 months to 3 years a normalisation of the intraocular pressure (less than 20 mm Hg) was obtained in 84% of the cases. Regression of iris tortuosity was observed in 77% of the cases. Regression of the iris neovascularisation in 68 % of the cases and reduction of diffusion of the dye in 67% of the cases. A remodelling of the iris ischemia was observed in 32% of the cases. Conclusion The intraocular injections of anti-VEGF drugs in selected cases is a promising therapeutic approach for the treatment of iris neovascularisation and neovascular glaucoma related to proton beam irradiation of uveal melanomas.

Purpose: Idiopathic retinal telangiectasis are usually classified as Leber - Coats disease or juxtafoveal telangiectasies. Intermediate and combined forms of these vascular disorders are rare and poorly described and understood. Methods: Clinical presentation of four cases of congenital telangiectasies associated to pigment epithelium disorders, two cases associated with optic disc anomalies, nine cases of multifocal progressive retinal telangiectasies and six cases of junctional congenital retinal telangiectasies. Results: Clinical presentations of four types of poorly described and understood congenital retinal telangiectasies considering fluorescein angiography pattern and therapeutic approach. Conclusions: Intermediate and combined forms of congenital retinal telangiectasies are highly interesting in order to attend a more accurate classification of these disorders based on presumed etiopathogenic mechanisms.

We report on the case of a 59-year-old man with the association of an anterior ischemic optic neuropathy on the left eye and a bilateral anemic retinopathy following an extreme and prolonged post-operative anemia, because the patient had refused blood-transfusion. After one year of follow-up, the visual acuity did improve on the left eye. This association is rare, and only few cases have been reported.

Objectif Etude du spectre de presentation clinique et de la reponse au traitement des metastases iriennes. Materiels et Methodes Analyse retrospective de 30 cas de metastases iriennes (29 patients). Resultats Le registre des tumeurs de l’hopital contient 361 patients atteints de metastases uveales dont 29 (8 %) presentent une localisation irienne. Il s’agit de 7 hommes (24 %) et de 22 femmes (76 %). La tumeur primaire etait un carcinome mammaire dans 15 cas, un carcinome pulmonaire dans 6 cas, un melanome dans 3 cas et divers autres carcinomes dans 5 cas. Le delai entre le traitement de la tumeur primaire et la decouverte des metastases iriennes est variable. Dans 4 cas, le diagnostic des metastases a precede la decouverte de la tumeur primaire. Les metastases iriennes etaient de type modulaires dans 17 cas (57 %) et de type diffus dans 13 cas (43 %). La tension oculaire etait superieure a 22 mm Hg dans 8 cas (27,5 %). Le corps ciliaire etait envahi dans 28 cas (94 %), des metastases choroidiennes dans le meme œil etaient presents dans 22 cas (75 %), dans l’œil controlateral dans 9 cas (31 %). Divers traitements (radiotherapie externe 17 cas, faisceau de protons 7 cas, chimiotherapie 2 cas, enucleation 2 cas) ont ete appliques. La survie des patients varie de 0 a 30 mois (moyenne : 7,6 mois). Aucun cas ne presenta une recidive locale. Discussion Les metastases iriennes sont rares. Elles sont frequemment associees a d’autres metastases uveales et peuvent donner lieu a un glaucome. Ces tumeurs repondent favorablement a la radiotherapie, ce qui permet la conservation du globe oculaire a moyen terme. La survie des patients est toutefois limitee. Conclusion Les metastases iriennes apparaissent dans les stades ultimes d’une metastatisation generalisee et necessite un traitement specifique.

Introduction Control of intraocular pressure fluctuation is of critical importance in glaucomatous patients. We present the first measurement of a novel and minimally invasive method to monitor relative intraocular pressure changes (IOP) on the human eye in-vivo by a wireless soft sensing contact lens. Aims and Methods The principle of measurement is based on the tiny changes in corneo-scleral curvature induced by variations in IOP. The measure is done by a soft silicone Contact Lens Sensor (CLS) with an embedded strain Gauge and allows wireless monitoring of IOP changes via an external antenna. The CLS was tested on five healthy volunteers. A decrease of IOP was induced using oculopression. Measurements of the ocular pulsation were also recorded. Results The filtred CLS signal is well correlated with the Goldmann tonometer. The ocular pulsation is well demonstrated and comparable to the signal obtained by a Dynamic Contour Tonometer. Discussion This is the first report of an in-vivo wireless continuous monitoring of the corneo-scleral deformation induced by the IOP changes and ocular pulsation on five human volunteers. Further clinical test are ongoing to characterize the dependence of the CLS signal amplitude on biometric and biomechanical eye properties. Conclusion This device potentially allows minimally invasive continuous measurement of relative changes in IOP, during prolonged periods, regardless of patient activity. It can thus for example replace 24-hour IOP.

The association of alopecia with a sebaceous verrucose nevus and an epibulbar lipodermoid is a rare condition (8 cases reported in the literature). Only girls suffer from this disease; this suggests a hologynic heredity, as in the case of Aicardi's syndrome. The authors present a new observation of a 7-year-old girl who exhibits all the characteristics of this new syndrome.

4 cases in which type 10 adenovirus was isolated are described in connection with a new form of viral keratoconjunctivitis. The predominant symptoms, after disappearance of the benign-type conjunctivitis signs by the 5th day, are those of edema of the lids, chemosis, multiple corneal erosions and folds in Descemet's membrane, reaching their maxima by the 15th day. The course of the disease is of roughly 30 days.

The authors examined and studied a family of Italian origin presenting with hereditary macular retinoschisis. Two first cousins of the third generation were affected by this disease. The mode of transmission of the pathologic gene is compatible with a recessive sex-linked heredity. The correlation between the severity of the cystic macular lesions and their functional consequences is not necessarily absolute. The lesions, which appear to be present very soon after birth, increase in severity with the years without any noticeable changes in macular function, which remains weak but stable.

In an eye with a proton beam-irradiated malignant melanoma of the ciliary body a second melanoma appeared episclerally on the opposite side of the globe. Microscopic examination did not show any connection to the primary tumor. The different possibilities of pathogenesis (bloodborn metastasis to the same globe, episcleral metastasis by conjunctival lymphatics, surgical dissemination due to the suturing of tantalum marks around the primary tumor) are discussed. The ciliary melanoma has spread over the iris despite the irradiation, probably as a result of insufficient irradiation of its anterior border. The results of histologic examination of the partially necrotic, partially viable irradiated tumor are considered with regard to the conservative tumor therapy administered.

Serious or fatal accidents during fluorescein angiography were assessed by an international survey involving answers from 260 clinics in 30 different countries in respect of 594, 687 angiographies. Incidence of fatal accidents was one case per 49,557 angiographies, and of serious accidents, one case per 18,020 angiographies. The total number of accidents reported was 45 cases, equal to one case per 13,215 angiographies. Age, general health of the patients, and the experience of the different clinics who perform this examination, seem to influence the occurrence and the seriousness of these accidents.

A baby is treated for frontal cutaneous angioma by two sclerosing injections, the second injection giving rise to severe complications and life-long sequelae: total amaurosis of the right eye with eventual bulbar phthisis, sectorial iris necrosis and cataract formation, as well as ugly sunken cutaneous scarring of the right upper eye-lid, forehead and scalp. It is the only case we know of, where such complications occurred after craniofacial angioma treatment by sclerosing injections.