Biomaterial Database

Ocular manifestations of Sturge-Weber syndrome. 2019

Marlee Silverstein, and Jonathan Salvin

Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.

OBJECTIVE Sturge-Weber syndrome (SWS) is a rare, congenital disease which frequently involves the eye. It is important that ophthalmologists recognize this syndrome and are aware of its range of ocular manifestations. The aim of this article is to present our understanding of the pathogenesis and clinical manifestations of this syndrome and provide updated information on the treatment of SWS glaucoma and choroidal hemangioma. RESULTS SWS glaucoma usually fails medical management. Surgical options include angle procedures, filtering procedures, device placement, and combination procedures. Combination procedures have become popular in this population due to the single procedure failure rate of angle surgery and the complications associated with device implantation. Choroidal hemangioma is best treated by photodynamic therapy. CONCLUSIONS Lifelong monitoring for ocular complications related to SWS is essential. There is a need for consensus guidelines on care and surveillance of patients with SWS to provide the best care for these patients.

UI	MeSH Term	Description	Entries
D010778	Photochemotherapy	Therapy using oral or topical photosensitizing agents with subsequent exposure to light.	Blue Light Photodynamic Therapy,Photodynamic Therapy,Red Light PDT,Red Light Photodynamic Therapy,Therapy, Photodynamic,Light PDT, Red,PDT, Red Light,Photochemotherapies,Photodynamic Therapies,Therapies, Photodynamic
D002830	Choroid Neoplasms	Tumors of the choroid; most common intraocular tumors are malignant melanomas of the choroid. These usually occur after puberty and increase in incidence with advancing age. Most malignant melanomas of the uveal tract develop from benign melanomas (nevi).	Choroidal Neoplasms,Choroid Neoplasm,Choroidal Neoplasm,Neoplasm, Choroid,Neoplasm, Choroidal,Neoplasms, Choroid,Neoplasms, Choroidal
D005901	Glaucoma	An ocular disease, occurring in many forms, having as its primary characteristics an unstable or a sustained increase in the intraocular pressure which the eye cannot withstand without damage to its structure or impairment of its function. The consequences of the increased pressure may be manifested in a variety of symptoms, depending upon type and severity, such as excavation of the optic disk, hardness of the eyeball, corneal anesthesia, reduced visual acuity, seeing of colored halos around lights, disturbed dark adaptation, visual field defects, and headaches. (Dictionary of Visual Science, 4th ed)	Glaucomas
D006391	Hemangioma	A vascular anomaly due to proliferation of BLOOD VESSELS that forms a tumor-like mass. The common types involve CAPILLARIES and VEINS. It can occur anywhere in the body but is most frequently noticed in the SKIN and SUBCUTANEOUS TISSUE. (from Stedman, 27th ed, 2000)	Angioma,Chorioangioma,Hemangioma, Histiocytoid,Hemangioma, Intramuscular,Chorangioma,Chorangiomas,Chorioangiomas,Hemangiomas,Hemangiomas, Histiocytoid,Hemangiomas, Intramuscular,Histiocytoid Hemangioma,Histiocytoid Hemangiomas,Intramuscular Hemangioma,Intramuscular Hemangiomas
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013341	Sturge-Weber Syndrome	A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects.	Neuroretinoangiomatosis,Phakomatosis, Sturge-Weber,Angiomatosis Oculoorbital-Thalamic Syndrome,Encephalofacial Hemangiomatosis Syndrome,Meningo-Oculo-Facial Angiomatosis,Meningofacial Angiomatosis-Cerebral Calcification Syndrome,Parkes Weber Syndrome,Parkes-Weber Syndrome,Sturge Disease,Sturge Syndrome,Sturge's Syndrome,Sturge-Kalischer-Weber Syndrome,Sturge-Weber-Dimitri Syndrome,Sturge-Weber-Krabbe Syndrome,Angiomatosis, Meningo-Oculo-Facial,Hemangiomatosis Syndrome, Encephalofacial,Meningo Oculo Facial Angiomatosis,Phakomatosis, Sturge Weber,Sturge Kalischer Weber Syndrome,Sturge Weber Dimitri Syndrome,Sturge Weber Krabbe Syndrome,Sturge Weber Syndrome,Sturge-Weber Phakomatosis,Syndrome, Encephalofacial Hemangiomatosis,Syndrome, Parkes Weber,Syndrome, Parkes-Weber,Syndrome, Sturge,Syndrome, Sturge's,Syndrome, Sturge-Kalischer-Weber,Syndrome, Sturge-Weber,Syndrome, Sturge-Weber-Dimitri,Syndrome, Sturge-Weber-Krabbe
D018463	Filtering Surgery	A surgical procedure used in treatment of glaucoma in which an opening is created through which aqueous fluid may pass from the anterior chamber into a sac created beneath the conjunctiva, thus lowering the pressure within the eye. (Hoffman, Pocket Glossary of Ophthalmologic Terminology, 1989)	Filtration Surgery,Surgery, Filtration,Surgery, Filtering
D019339	Port-Wine Stain	A vascular malformation of developmental origin characterized pathologically by ectasia of superficial dermal capillaries, and clinically by persistent macular erythema. In the past, port wine stains have frequently been termed capillary hemangiomas, which they are not; unfortunately this confusing practice persists: HEMANGIOMA, CAPILLARY is neoplastic, a port-wine stain is non-neoplastic. Port-wine stains vary in color from fairly pale pink to deep red or purple and in size from a few millimeters to many centimeters in diameter. The face is the most frequently affected site and they are most often unilateral. (From Rook et al., Textbook of Dermatology, 5th ed, p483)	Nevus Flammeus,Port Wine Stain,Port-Wine Stains,Stain, Port-Wine,Stains, Port-Wine