Timothy J. Sullivan

Sturge-Weber syndrome (SWS) is a rare neuro-oculocutaneous disorder. The ocular component manifests as glaucoma and vascular malformations of the conjunctiva, episclera, choroid, and retina. The authors reviewed the records of and recalled for examination 51 consecutive patients with a diagnosis of SWS seen at The Hospital for Sick Children, Toronto, Ontario, Canada, from 1980 to 1991. Of the 51 patients, 36 (71%) had glaucoma, 26 experienced onset before 24 months of age and the remainder after 66 months of age, 35 (69%) had conjunctival or episcleral hemangiomas, and 28 (55%) had choroidal hemangiomas, which were bilateral in 12 patients. Other ocular manifestations (retinal vascular tortuosity, iris heterochromia, retinal detachment, and strabismus) also occurred. Thirty-four of 51 (67%) glaucomatous eyes had a final visual acuity of 20/40 or better, or central, steady, and maintained fixation in each eye.

PURPOSE: To review the clinical features, treatment, outcome and survival of metastatic tumors of the orbit. MATERIALS AND METHODS: Retrospective, non-comparative, chart review of 80 patients with orbital metastasis treated in four tertiary orbital centres in Australia. RESULTS: The study included 80 patients of which, 44 were male with a mean age of 60 years. Orbital involvement commonly presented late in a multisystemic disease; however, the orbit was the first presentation in 15% of the cases. Diplopia (48%), pain (42%), and visual loss (30%) were the commonest symptoms at presentation; whereas proptosis (63%), strabismus (62%), and visual loss (41%) were the most frequent clinical signs. Computed tomography commonly showed a solid enhancing mass (42 cases) located within the orbital fat (43%), or enlarging an extraocular muscle (28%). Breast carcinoma (29%), melanoma (20%), and prostatic cancer (13%) were the most frequent histological types. Treatment was often multi-disciplinary and modalities included radiotherapy, chemotherapy, hormone therapy, surgery, and immunotherapy. Survival was limited to 1.5 years after diagnosis independent of the histological type, with 29% of patients alive after 17 months follow-up. CONCLUSIONS: A high index of suspicion and appropriate intervention with histological diagnosis can help in the management and quality of life in patients with metastatic orbital disease. Overall survival is limited and we encountered statistical limitations proving differences in the survival based on the sub-type of primary tumour involved. Metastatic orbital melanoma presented a higher incidence when compared with previous studies, probably due to the increase frequency of skin found in the Australian population.