The vascular tumors of the retina and choroid comprise a diverse group of congenital and acquired lesions. The major vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, retinal vasoproliferative tumor, and racemose hemangiomatosis of the retina or Wyburn–Mason syndrome. Choroidal vascular tumors include circumscribed choroidal hemangioma and diffuse choroidal hemangioma. While classified as benign, visual symptoms secondary to exudative retinal detachment and a variety of other mechanisms are common and are a major source of long-term visual disability. While many therapeutic modalities exist, treatment of symptomatic cases can be challenging. Of particular importance, many of the vascular tumors of the retina and choroid have significant associations with systemic disease. As ocular symptoms are often the most common presenting disease manifestation, the ophthalmologist plays an important role in accurate and early diagnosis. The ability to initiate prompt screening and treatment in appropriate cases is critical. In the following article, the key clinical and diagnostic features of the major retinal and choroidal vascular tumors, their systemic associations, and the literature pertaining to the most currently available treatment strategies are reviewed.
Purpose: To review the spectrum of imaging modalities commonly used in clinical practice for the diagnosis and management of uveal melanoma. Materials and Methods: Review of literature. Results: Various forms of ophthalmic imaging are useful in the characterisation and treatment planning of uveal melanoma. These include digital photography, ultrasonography, angiography and optical coherence tomography. Digital photography is useful for documenting the clinical findings for future comparison and response to treatment. Ultrasonography provides information on tumour biometry, local extension and tumour composition (internal reflectivity). Angiography can be used to assess the degree of tumour vascularity. Newer forms of imaging, including optical coherence tomography (OCT), have excellent resolution and provide detail on surface features. Conclusion: The accuracy of clinical diagnosis of uveal melanoma exceeds 99 % when using currently available imaging modalities. There are advantages and disadvantages to each modality depending on tumour location, size, surface characteristics and internal structure. Ancillary techniques such as computed tomography, magnetic resonance imaging and positron emission tomography may be useful in atypical lesions or in the surveillance for metastatic disease.Uveal melanoma is the most common primary intraocular malignancy in adults and comprises approximately 85 % of all ocular melanomas. 1
The combination of both pigmented and non-pigmented lesions occurring in the same individual with grouped CHRPE is rare. It is important to distinguish grouped CHRPE lesions from the POFLs associated with familial cancer syndromes.
Spondylometaphyseal dysplasia with cone-rod dystrophy is a rare congenital disorder of unknown inheritance pattern and pathophysiolgy. The ocular manifestations appear to stabilize in early adolescence whereas the skeletal abnormalities are progressive with age.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.