Metastatic melanoma in the eye and orbit.

OBJECTIVE: Presentation of a large series of patients with metastatic melanoma involving the eye and orbit. DESIGN: Retrospective clinical study. PARTICIPANTS: Thirteen cases of metastatic melanoma involving intraocular tissues, 6 cases of metastatic melanoma in the orbit, and 1 case of metastatic melanoma involving both the eye and the orbit, treated in Lausanne between 1986 and 2002, were identified from the computer files of the ocular oncology and orbitopalpebral surgery units of Jules Gonin Hospital. METHODS: The analysis is based on the demographic data concerning these patients, clinical data concerning the tumor, the treatment applied, the outcome, and the follow-up. MAIN OUTCOME MEASURES: Clinical presentation of intraocular and orbital metastasis, interval between primary tumor and ocular metastasis, survival of the patients, evaluation of various therapeutic protocols. RESULTS: Intraocular metastases (14 cases, 15 eyes) were situated in the choroid in 11 cases (isolated lesion, 6 cases; multiple lesions, 3 cases; diffuse involvement, 2 cases), in the iris and ciliary body in 2 cases, and in the retina and vitreous in 2 other cases. The primary tumor was a cutaneous melanoma in 8 cases, a melanoma of the contralateral eye in 3 cases, a mucosal melanoma in 1 case, and was unknown in 2 cases. The mean interval between the diagnosis of ocular metastases and the patient's death was 8.8 months (range, 1-48 months). The primary tumor in the 7 cases of orbital metastases was a cutaneous melanoma in 5 cases, a uveal melanoma in the contralateral eye in 1 case, and was unknown in 1 case. The mean interval between the diagnosis of orbital metastases and death was 19.7 months (range, 5-48 months). The patients were treated by various protocols. The best results, in terms of both local tumor control and preservation of visual function, were obtained with circumscribed proton beam radiotherapy or external beam irradiation, depending on the site and extent of the tumor. CONCLUSIONS: Metastatic melanomas to the eye and orbit are rare and generally occur in patients with disseminated metastases during the terminal stages of the disease, with a short life expectancy. Treatment is palliative and, among the various possible treatment options, circumscribed proton beam radiotherapy or global photon beam radiotherapy, at relatively high irradiation doses, seems to achieve the most favorable results.

Management of microphthalmos and anophthalmos: prosthetic experience.

INTRODUCTION: Congenital microphthalmos and anophthalmos are rare. The reduced eyeball size, or its absence, prevents the normal development of the orbit. This lack of development has functional, physical and psychological repercussions. The authors report their experience of prosthetic treatment for microphthalmos and anophthalmos. PATIENTS AND METHODS: Our study is about 11 children with 13 cases of microphthalmos and 2 of anophthalmos. These cases have been clinically observed between 1998 and 2002. A detailed history, ophthalmological and complete paediatric examination and a prosthetic treatment were undertaken for all of them. RESULTS: 4 children had bilateral involvement. 8 children had other ocular or general malformations. No prenatal infections during pregnancy were detected (rubella, cytomegalovirus). One treatment of clinical anophthalmos was interrupted on the parents' request (slow and bad evolution with complete retraction of the orbital socket after a conjunctivitis). For 9 children, the evolution was good with growth of the orbital cavity and a satisfactory cosmetic result. However, hypoplasia was observed on the lateral orbital wall for severe microphthalmos. One child presenting a bilateral microphthalmos received a cosmetic scleral shell with optical correction. CONCLUSIONS: Management of anophthalmos is difficult and unrewarding. But the use of cosmetic scleral shells in all cases of microphthalmos, even severe ones, is useful and effective.

Orbital rhabdomyosarcoma: Clinicopathologic correlation, management and follow-up in two newborns. A preliminary report.

Rhabdomyosarcoma (RMS) is the most common primary malignant tumor of the orbit in childhood. The average age of onset is around seven years. In the present paper, the authors present the clinical and radiological findings in two newborns with histopathologically confirmed orbital rhabdomyosarcoma. A review of the literature was also carried out. In both children, the first sign leading to a visit was a unilateral proptosis, without symptoms or any other sign. Both underwent a CT-scan and an MRI. Confirmation of the histology was made by an open sky biopsy. The children were first treated by chemotherapy but had incomplete resolution of the tumor after more than 11 months for the first case and 9 months for the second. Since the parents of both children refused exenteration, radiotherapy had to be resorted to because of the incomplete response to chemotherapy. Our therapeutic approach and the outcome of these two cases are discussed.