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1 General aspects (0)   1.1 Epidemiology (3)   1.2 Population genetics (0)   1.3 Pathogenesis (4)   1.4 Quality of life (8)   1.5 Glaucomas as cause of blindness (6)   1.6 Prevention and screening (7) 2 Anatomical structures in glaucoma (0)   2.1 Conjunctiva (4)   2.2 Cornea (14)   2.3 Sclera (10)   2.4 Anterior chamber angle (3)   2.5 Meshwork (0)     2.5.1 Trabecular meshwork (12)     2.5.2 Schlemms canal (4)     2.5.3 Scleral outlet channels (0)   2.6 Aqueous humor dynamics (1)     2.6.1 Production (0)     2.6.2 Outflow (1)       2.6.2.1 Trabecular meshwork (0)       2.6.2.2 Uveoscleral (0)     2.6.3 Compostion (3)   2.7 Episcleral veins and venous pressure (0)   2.8 Iris (2)   2.9 Ciliary body (1)   2.10 Lens (1)   2.11 Vitreous body (0)   2.12 Choroid, peripapillary choroid, peripapillary atrophy (6)   2.13 Retina and retinal nerve fibre layer (19)   2.14 Optic disc (19)   2.15 Optic nerve (0)   2.16 Chiasma and retrochiasmal central nervous system (8)   2.17 Stem cells (5)   2.20 Other (0) 3 Laboratory methods (0)   3.1 Microscopy (2)   3.2 Electron microscopy (1)   3.3 Immunohistochemistry (1)   3.4 Molecular genetics (0)     3.4.1 Linkage studies (0)     3.4.2 Gene studies (19)   3.5 Molecular biology incl. SiRNA (20)   3.6 Cellular biology (26)   3.7 Biochemistry (9)   3.8 Pharmacology (9)   3.9 Pathophysiology (10)   3.10 Immunobiology (5)   3.11 Pharmacogenetics (0)   3.12 Proteomics (2)   3.13 In vivo imaging (0)     3.13.1 Laser Scanning (0)       3.13.1.1 Confocal Scanning Laser Ophthalmoscopy (0)       3.13.1.2 Confocal Scanning Laser Polarimetry (0)     3.13.2 Optical Coherence Tomography (0)       3.13.2.1 Anterior Segment (0)       3.13.2.2 Posterior Segment (0)     3.13.3 RGC Imaging (3)     3.13.4 Other (0)   3.20 Other (0) 4 Tissue culture of ocular cells (0) 5 Experimental glaucoma; animal models (0)   5.1 Rodent (16)   5.2 Primates (2)   5.3 Other (11) 6 Clinical examination methods (0)   6.1 Intraocular pressure measurement; factors affecting IOP (0)     6.1.1 Devices, techniques (9)     6.1.2 Fluctuation, circadian rhythms (5)     6.1.3 Factors affecting IOP (8)   6.2 Tonography, aqueous flow measurement (see also 2.6) (0)   6.3 Biomicroscopy (slitlamp) (0)     6.3.1 Anterior segment (0)     6.3.2 Posterior segment (1)   6.4 Gonioscopy (1)   6.5 Ophthalmoscopy (0)   6.6 Visual field examination and other visual function tests (0)     6.6.1 Conventional manual (0)     6.6.2 Automated (21)     6.6.3 Special methods (e.g. color, contrast, SWAP etc.) (7)   6.7 Electro-ophthalmodiagnosis (5)   6.8 Photography (0)     6.8.1 Anterior segment (2)     6.8.2 Posterior segment (4)   6.9 Computerized image analysis (0)     6.9.1 Laser scanning (0)       6.9.1.1 Confocal Scanning Laser Ophthalmoscopy (4)       6.9.1.2 Confocal Scanning Laser Polarimetry (2)     6.9.2 Optical coherence tomography (0)       6.9.2.1 Anterior (10)       6.9.2.2 Posterior (38)     6.9.5 Other (5)   6.10 Fluorescein (ICG) angiography (0)     6.10.1 Anterior segment (1)     6.10.2 Posterior segment (0)   6.11 Bloodflow measurements (16)   6.12 Ultrasonography and ultrasound biomicroscopy (2)   6.13 Provocative tests (3)   6.19 Telemedicine (0)   6.20 Progression (12)   6.30 Other (6) 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(0)     9.4.1 Steroid-induced glaucoma (6)     9.4.2 Glaucomas associated with disorders of the cornea, conjunctiva, sclera (0)       9.4.2.1 Iridocorneal endothelial syndrome (ICE, incl. irisatrophy) (2)       9.4.2.2 Posterior polymorphous dystrophy (0)       9.4.2.3 Fuchs' endothelial dystrophy (0)       9.4.2.5 Other (0)     9.4.3 Glaucomas associated with disorders of the iris and ciliary body (0)       9.4.3.1 Pigmentary glaucoma (2)       9.4.3.5 Other (1)     9.4.4 Glaucomas associated with disorders of the lens (0)       9.4.4.1 Exfoliation syndrome (7)       9.4.4.2 Glaucomas associated with cataracts (1)       9.4.4.3 Glaucomas associated with lens dislocation (0)       9.4.4.5 Other (3)     9.4.5 Glaucomas associated with disorders of the retina, choroid and vitreous (0)       9.4.5.1 Neovascular glaucoma (0)       9.4.5.5 Other (4)     9.4.6 Glaucomas associated with inflammation, uveitis (7)     9.4.7 Glaucomas associated with ocular trauma (3)     9.4.8 Glaucomas associated with intraocular tumors (3)     9.4.9 Glaucomas associated with elevated episcleral venous pressure (0)       9.4.10 Glaucomas associated with hemorrhage (1)     9.4.11 Glaucomas following intraocular surgery (0)       9.4.11.1 Ciliary block (malignant) glaucoma (1)       9.4.11.2 Glaucomas in aphakia and pseudophakia (6)       9.4.11.3 Epithelial, fibrous, and endothelial proliferation (0)       9.4.11.4 Glaucomas associated with corneal surgery (3)       9.4.11.5 Glaucomas associated with vitreoretinal surgery (1)     9.4.15 Glaucoma in relation to systemic disease (20)     9.4.20 Other (5) 10 Differential diagnosis e.g. anterior and posterior ischemic optic neuropathy (4) 11 Medical treatment (0)   11.1 General management, indication (1)   11.2 Cholinergic drugs (3)   11.3 Adrenergic drugs (0)     11.3.1 Epinephrine (0)     11.3.2 Thymoxamine, dapiprazole (0)     11.3.3 Apraclonidine, brimonidine (3)     11.3.4 Betablocker (4)     11.3.5 Other (0)   11.4 Prostaglandins 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    12.8.1 Without tube implant (12)     12.8.2 With tube implant or other drainage devices (28)     12.8.3 Non-perforating (3)     12.8.4 Using laser (0)     12.8.5 Other (10)     12.8.10 Woundhealing antifibrosis (14)     12.8.11 Complications, endophthalmitis (11)   12.9 Trabeculotomy, goniotomy (4)   12.10 Cyclodestruction (3)   12.11 Cyclodialysis (1)   12.12 Cataract extraction (0)     12.12.1 Intracapsular (0)     12.12.2 Extracapsular (0)     12.12.3 Phacoemulsification (5)   12.14 Combined cataract extraction and glaucoma surgery (0)     12.14.1 Intracapsular (0)     12.14.2 Extracapsular (0)     12.14.3 Phacoemulsification (4)   12.15 Capsulotomy (0)   12.16 Vitrectomy (2)   12.17 Anesthesia (0)   12.20 Other (3) 13 Therapeutic prognosis and outcome (0)   13.1 Prognostic factors (0)   13.2 Outcome (0)     13.2.1 IOP (1)     13.2.2 Visual field (0)       13.2.2.1 Progression (0)       13.2.2.2 Improvement (0)     13.2.3 Other (0) 14 Costing studies; pharmacoeconomics (3) 15 Miscellaneous (28)

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Abstract #71352 Published in IGR 18-3

Sturge-Weber Syndrome: A Review

Higueros E; Roe E; Granell E; Baselga E
Actas dermo-sifiliograficas 2017; 108: 407-417

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Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder caused by a somatic activating mutation in GNAQ; it affects 1 in every 20,000 to 50,000 newborns. It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. Seizures are the most common neurological manifestation and typically present in the first months of life. Glaucoma may be present at birth or develop later. Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Standard treatment for Sturge-Weber syndrome includes laser treatment for the Port-wine stain, anticonvulsants, and medical or surgical treatment for the glaucoma. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma.

Servicio de Dermatología, Hospital Santa Creu i Sant Pau, Barcelona, España.

Full article

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Classification:

9.4.20 Other (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)

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