{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T07:50:25Z","timestamp":1791359425306,"version":"4.3.1"},"reference-count":12,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"10","license":[{"start":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T00:00:00Z","timestamp":1790640000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,10]]},"abstract":"\n Objectives:<\/jats:title>\n To evaluate the patterns of visual field (VF) defects in patients with Sturge\u2013Weber syndrome (SWS) and phakomatosis pigmentovascularis (PPV) using standard automated perimetry.<\/jats:p>\n <\/jats:sec>\n \n Methods:<\/jats:title>\n This retrospective study analyzed the clinical and VF data of patients with SWS or PPV undergoing treatment for glaucoma and had VF test data over a 27-year period. VF defects were classified as glaucomatous, neurological, combined, or unclassifiable. Statistical analyses were performed to determine factors associated with type of field defects.<\/jats:p>\n <\/jats:sec>\n \n Results:<\/jats:title>\n \n A total of 86 patients (172 eyes) were included, with 62 (72%) having SWS and 24 (28%) PPV. Glaucoma was present in 49.2% of SWS eyes and 91.6% of PPV eyes. Reliable VF tests were more common in SWS than in PPV (\n P<\/jats:italic>\n < 0.001). Glaucomatous defects were observed in 35.5% of eyes, 7.7% had neurological defects, and 4.1% had combined defects. Advanced field loss was the most common glaucomatous defect (54%). Eyes treated with external beam radiotherapy for diffuse choroidal hemangioma developed localized field defects mimicking glaucoma and homonymous hemianopia.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions:<\/jats:title>\n Glaucoma remains the primary cause of VF loss in SWS and PPV, with a significant proportion of patients demonstrating advanced glaucomatous damage. Accurate interpretation of VF results is crucial for differentiating glaucomatous progression from other ocular and neurological causes in these rare phakomatoses.<\/jats:p>\n <\/jats:sec>","DOI":"10.4103\/ijo.ijo_1518_26","type":"journal-article","created":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T14:03:54Z","timestamp":1790690634000},"page":"1525-1530","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Visual field loss in Sturge\u2013Weber syndrome and phakomatosis pigmentovascularis: Implications for glaucoma assessment"],"prefix":"10.4103","volume":"74","author":[{"given":"Tanvi","family":"Choudhary","sequence":"first","affiliation":[{"name":"Kalathur Venugopal Reddy Glaucoma Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India"},{"name":"Standard Chartered Eye Care Education, L V Prasad Eye Institute, Hyderabad, Telangana, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sirisha","family":"Senthil","sequence":"additional","affiliation":[{"name":"Kalathur Venugopal Reddy Glaucoma Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,9,29]]},"reference":[{"key":"R1-20261007","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1080\/13816810.2020.1731834","article-title":"Incidence of Sturge-Weber syndrome and associated ocular involvement in Olmsted County, Minnesota, United States","volume":"41","author":"Rihani","year":"2020","journal-title":"Ophthalmic Genet"},{"key":"R2-20261007","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/S0161-6420(97)30346-7","article-title":"Glaucoma in phakomatosis pigmentovascularis","volume":"104","author":"Teekhasaenee","year":"1997","journal-title":"Ophthalmology"},{"key":"R3-20261007","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1111\/aos.13074","article-title":"Visual outcome in Sturge-Weber syndrome: A systematic review and Dutch multicentre cohort","volume":"94","author":"Koenraads","year":"2016","journal-title":"Acta Ophthalmol"},{"key":"R4-20261007","first-page":"6","article-title":"Prevalence and causes of visual impairment in patients seen at Nkhensani Hospital Eye Clinic, South Africa","volume":"7","author":"Maake","year":"2015","journal-title":"Afr J Prm Health Care Fam Med"},{"key":"R5-20261007","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1177\/0883073812451326","article-title":"Sturge-weber syndrome: Clinical spectrum, disease course, and outcome of 30 patients","volume":"28","author":"Jagtap","year":"2013","journal-title":"J Child Neurol"},{"key":"R6-20261007","first-page":"180","article-title":"Focal venous hypertension as a pathophysiologic mechanism for tissue hypertrophy, port-wine stains, the Sturge-Weber syndrome, and related disorders: Proof of concept with novel hypothesis for underlying etiological cause (an American Ophthalmological Society thesis)","volume":"111","author":"Parsa","year":"2013","journal-title":"Trans Am Ophthalmol Soc"},{"key":"R7-20261007","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.jaapos.2009.04.007","article-title":"Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome","volume":"13","author":"Sharan","year":"2009","journal-title":"J Am Assoc Pediatr Ophthalmol Strabismus"},{"key":"R8-20261007","first-page":"171","article-title":"Hemangioma of the choroid","volume":"57","author":"Maclean","year":"1959","journal-title":"Trans Am Ophthalmol Soc"},{"key":"R9-20261007","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.4103\/ijo.IJO_2036_19","article-title":"Circumscribed choroidal hemangioma: An overview of clinical manifestation, diagnosis and management","volume":"67","author":"Sen","year":"2019","journal-title":"Indian J Ophthalmol"},{"key":"R10-20261007","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1016\/S0161-6420(90)32483-1","article-title":"Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome","volume":"97","author":"Iwach","year":"1990","journal-title":"Ophthalmology"},{"key":"R11-20261007","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1016\/j.jaapos.2012.05.007","article-title":"Visual acuity outcomes in pediatric glaucomas","volume":"16","author":"Khitri","year":"2012","journal-title":"J AAPOS"},{"key":"R12-20261007","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.ogla.2021.06.003","article-title":"Outcomes of management of glaucoma in phacomatosis pigmentovascularis over the last three decades: A single-center experience","volume":"5","author":"Mandal","year":"2022","journal-title":"Ophthalmol Glaucoma"}],"container-title":["Indian Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/IJO.IJO_1518_26","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T07:00:24Z","timestamp":1791356424000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/IJO.IJO_1518_26"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,29]]},"references-count":12,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/ijo.ijo_1518_26","relation":{},"ISSN":["0301-4738","1998-3689"],"issn-type":[{"value":"0301-4738","type":"print"},{"value":"1998-3689","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,29]]},"assertion":[{"value":"2026-05-28","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-31","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}