"En face" OCT in Small Pigmented Choroidal Tumors.

Background "En face" optical coherence tomography (OCT) is an imaging technique with optic sections parallel to the retinal pigment epithelium (RPE). This study aims to define the use of this technique in the evaluation of small pigmented choroidal tumors. Patients and Methods Investigation of 27 small pigmented choroidal tumors with "en face" OCT. Following manual segmentation, the optic sections performed with a spectral domain OCT at 30, 80 and 130 µ under the RPE were selected for morphological and statistical analysis. Results Internal tumor reflectance is variable (hyper-, iso-, or hypo-reflective), with a uniform or a bull's eye appearance. The reflectance becomes progressively lower towards the centre of the tumor. The margins of the pigmented tumors appear similar or less extensive than ophthalmoscopically. Peritumoral vascularization appears distinct from the 80 µ section downwards. Conclusions On "en face" OCT, small pigmented choroidal tumors present with a uniform or bull's eye internal reflectance, and are of variable intensity relative to the surrounding choroid. Their margins can appear more restrained than those on colour fundus photography.

Pitfalls in colour photography of choroidal tumours.

Colour imaging of fundus tumours has been transformed by the development of digital and confocal scanning laser photography. These advances provide numerous benefits, such as panoramic images, increased contrast, non-contact wide-angle imaging, non-mydriatic photography, and simultaneous angiography. False tumour colour representation can, however, cause serious diagnostic errors. Large choroidal tumours can be totally invisible on angiography. Pseudogrowth can occur because of artefacts caused by different methods of fundus illumination, movement of reference blood vessels, and flattening of Bruch's membrane and sclera when tumour regression occurs. Awareness of these pitfalls should prevent the clinician from misdiagnosing tumours and wrongfully concluding that a tumour has grown.

Scleritis after proton therapy in uveal melanoma.

BACKGROUND: Sclera is a very radioresistant tissue and scleritis after proton therapy has not been described so far. HISTORY AND SIGNS: Four female patients, aged between 31 and 74 years, were treated with proton therapy for uveal melanoma (height range: 2.2 - 3.5 mm), located in the macula, the superior equator and 2 in the ciliary body. All patients had a history of a previous or active inflammatory disease and developed scleritis after radiotherapy. THERAPY AND OUTCOME: Two patients had infectious scleritis and were treated with adequate antibiotic therapy. After systemic corticotherapy, 3 patients recovered completely; the remaining patient was managed with additional immunosuppressive treatment as well as a conjunctival and scleral graft, but has not become pain free yet. CONCLUSION: Scleritis is a possible complication after proton therapy, probably on an ischemic basis, where there is a predisposing factor such as inflammatory systemic disease.

Epibulbar osseous choristoma: a clinicopathological case series and review of the literature.

BACKGROUND: Choristomas are benign, congenital tumours composed of normal tissue in an abnormal location. Osseous choristomas represent the rarest form of epibulbar choristomas, with now 65 cases reported in the literature. We did a retrospective clinicopathological study of all patients with epibulbar osseous choristoma observed at our institution since 1982 and updated the last review of the literature. HISTORY AND SIGNS: Three Caucasian male patients, aged between 3 months and 11 years, were identified. All osseous choristomas were located under the superotemporal bulbar conjunctiva of the right eye. THERAPY AND OUTCOME: All lesions were managed with surgical excision. Histopathology revealed the presence of lamellar bone in all cases, one of which was associated with a dermolipoma. CONCLUSIONS: We report a small rare case series of 3 epibulbar osseous choristomas and did a review of the literature. In one patient, the osteoma was associated with a dermolipoma, corresponding to the fourth reported complex choristoma of this type, in an otherwise normal eye, in the literature.

Effective temporary analgesia for severe painful blind eye.

There are various methods of providing pain relief for painful blind eyes. We wish to recommend this effective method of providing temporary analgesia in patients suffering from a severe painful blind eye before undergoing enucleation.

[Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma]. / Escleritis nodular posterior gigante compatible con sarcoidosis ocular simulando un melanoma de la coroides.

CASE REPORT: A 30-year-old man was referred to our ocular oncology service with a diagnosis of amelanotic choroidal melanoma of the left eye. The following tests were performed: ophthalmoscopy, fluorescein angiography, indocyanine green angiography, ultrasonography, magnetic resonance imaging and biopsy. DISCUSSION: The diagnosis of giant nodular posterior scleritis, as suggested by ultrasonography, was confirmed by biopsy. A comprehensive medical evaluation was performed, but no etiology was found. The histology revealed a granuloma compatible with ocular sarcoidosis. A rapid response was obtained by systemic steroid administration (1 mg/kg). Sarcoidosis continues to be a challenge in diagnosis. It is important to distinguish nodular posterior scleritis from choroidal melanoma.

Escleritis nodular posterior gigante compatible con sarcoidosis ocular simulando un melanoma de la coroides / Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma

Caso clínico: Un paciente de 30 años fue enviado a nuestro servicio oncológico de referencia con el diagnóstico de melanoma amelanótico de la coroides del ojo izquierdo. Se hicieron los siguientes exámenes: oftalmoscopía, angiografía fluoresceínica, angiografía con verde de indocianina, ecografía, resonancia magnética y biopsia. Discusión: El diagnóstico de escleritis nodular posterior gigante basado en la ecografía se confirmó mediante una biopsia. Se realizó un examen médico completo buscándose el posible agente causal; no se encontró la etiología. La histología reveló un granuloma que era compatible con una sarcoidosis ocular. Mediante la administración de esteroides por vía oral (1 mg/kg), se obtuvo una evolución rápidamente favorable del cuadro. El diagnóstico de esta afección sigue siendo un desafío. Es importante diferenciar una escleritis posterior nodular de un melanoma de la coroides

Case report: A 30-year-old man was referred to our ocular oncology service with a diagnosis of amelanotic choroidal melanoma of the left eye. The following tests were performed: ophthalmoscopy, fluorescein angiography, indocyanine green angiography, ultrasonography, magnetic resonance imaging and biopsy. Discussion: The diagnosis of giant nodular posterior scleritis, as suggested by ultrasonography, was confirmed by biopsy. A comprehensive medical evaluation was performed, but no etiology was found. The histology revealed a granuloma compatible with ocular sarcoidosis. A rapid response was obtained by systemic steroid administration (1 mg/kg). Sarcoidosis continues to be a challenge in diagnosis. It is important to distinguish nodular posterior scleritis from choroidal melanoma

Subretinal choroidal neovascularization associated with choroidal nevus.

PURPOSE: Evaluation of a large series of choroidal nevi inducing the formation of a neovascular membrane in order to more clearly define the clinical presentation and to evaluate the efficacy of various treatment options. METHOD: Retrospective study of 22 clinical cases. RESULTS: All nevi were situated in the posterior choroid. They had a mean diameter of 3.8 mm and a mean thickness of 1.4 mm. Neovascular membranes were classic in all cases, extrafoveal in 13 cases (59%), and subfoveal in 9 cases (41%). A serous retinal detachment was present in every case, hemorrhages were present in 13 cases (59%), and lipid deposits were present in 16 cases (73%). All extrafoveal neovascular membranes were successfully treated by thermal laser photocoagulation. Initial visual acuity was 0.1 in three cases, 0.2-0.4 in five cases, 0.5-0.8 in four cases, and 1.0 or more in two cases. Final visual acuity was 0.1 in one case, 0.2-0.4 in one case, 0.5-0.8 in four cases, and 1.0 or more in seven cases. Five subfoveal neovascular membranes were treated either by thermal laser, photodynamic therapy, or irradiation. No treatment was applied in four cases and in one of these cases, spontaneous resolution of the neovascular membrane was observed. No growth of the pigmented tumor was observed with a mean follow-up of 4.8 years. CONCLUSIONS: Proliferation of a neovascular membrane on the surface of a pigmented choroidal tumor is a rare complication and is considered to be a relative indicator of a benign nature of the lesion. In the authors' experience, neovascular membranes are extrafoveal in more than half of cases and are accessible to laser photocoagulation. In contrast, the various modalities used to treat subfoveal neovascular membrane were ineffective and functional prognosis was unfavorable in these cases.

[Choroidal melanoma in neurofibromatosis type 2: description of a case]. / Description d'un mélanome de l'uvée dans le cadre d'une neurofibromatose de type 2.

To the best of our knowledge this is the first description of a choroidal melanoma with documented growth in neurofibromatosis type 2 (NF2). A 20-year-old patient with NF2 presenting deafness due to bilateral acoustic neurinomas and unilateral amaurosis due to a meningioma of the optic nerve developed a pigmented parapapillary choroidal tumor. Despite signs indicating the diagnosis of a melanoma, periodic observation was chosen in order to postpone functional amputation following optic nerve irradiation. The tumor growth was slow during the 5 years that followed, and once progression became rapid, the tumor was treated by accelerated proton beam radiotherapy. One year later, visual acuity diminished due to actinic optic neuropathy and was stabilized at 0.3 for the 2 following years. The tumor presented objective signs of regression, and no sign of metastatic disease was observed. The therapeutic approach in this case provided local control of the tumor while preserving useful visual function.

Maximizing local tumor control and survival after proton beam radiotherapy of uveal melanoma.

PURPOSE: This study reports local tumor control and survival after proton beam radiotherapy (PBRT) of uveal melanoma. It identifies the risk factors for local tumor-control failure and for ocular tumor-related death. It presents the improvements implemented to increase the rate of local tumor control, and compares the survival rate of patients with locally controlled tumors to those of patients who had to receive a second treatment. PATIENTS AND METHODS: We have treated 2,435 uveal melanomas with PBRT between March 1984 and December 1998. Data were analyzed as of September 1999. Patients' age ranged from 9 to 89 years; there were 1,188 men and 1,247 women. The largest tumor diameter ranged from 4 to 26 mm, and tumor thickness from 0.9 to 15.6 mm. Median follow-up time was 40 months. RESULTS: Local tumor control probability at 5 years was improved from 90.6 +/- 1.7% for patients treated before 1988, to 96.3 +/- 0.6% for patients treated between 1989 and 1993, and became 98.9 +/- 0.6% for patients treated after 1993. Among 2,435 treated patients, 73 (3%) had to receive a second treatment because of tumor regrowth. Cause-specific survival at 10 years was calculated to 72.6 +/- 1.9% for patients with controlled tumors compared to 47.5 +/- 6.5% for those with recurrent tumors. CONCLUSION: Reduced safety margins, large ciliary body tumors, eyelids within the treatment field, inadequate positioning of tantalum clips, and male gender were identified to be the main factors impairing local tumor control. The improvement of local tumor control rate after 1993 is attributed to changes implemented in the treatment procedure. Our data strongly support that the rate of death by metastases is influenced by local tumor control failure: improvement of the local tumor control rate results in a better survival rate.

Indocyanine green angiographic findings in choroidal hemangiomas: A study of 75 cases.

Indocyanine green (ICG) angiography is a new diagnostic modality that was suggested, in small series, to provide a typical angiographic pattern in cases of choroidal hemangioma. Our study, through an exceptionally large series of 75 patients, assessed in a prospective way whether a typical ICG pattern of choroidal hemangioma exists and what would be its possible variations. The most constant feature is the sequence of the different ICG angiographic phases. The arterial phase demonstrates the filling of intratumoral vessels on a hypofluorescent tumoral background. During the venous phase, the hemangioma reaches a stage of maximal ICG-A fluorescence, with superimposed hyper- and hypofluorescent spots. Sturge-Weber cases have also extratumoral hyperfluorescent spots. The late phase shows a hypofluorescent lesion with residual hyperfluorescent caverns and a well-delineated, but complex border structure.

Adenocarcinoma of the nonpigmented ciliary body epithelium: report of a rare case.

Acquired tumours of the nonpigmented ciliary body epithelium are rare. We present herein a case of low-grade adenocarcinoma in order to stress the problems related to the clinical diagnosis. The tumour in our case was circumscribed, localised on the ciliary body, and nonpigmented. The height of the tumour, measured with ultrasound biomicroscopy, was 3.5 mm. The internal reflectivity of the tumor was homogeneous with low attenuation. The tumour was surgically excised with an uneventful iridocyclectomy. Both clinical presentation and preoperative examinations allowed to circumscribe the correct diagnosis which could be confirmed only with histology.