{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T02:23:50Z","timestamp":1774405430942,"version":"3.50.1"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,7,18]],"date-time":"2018-07-18T00:00:00Z","timestamp":1531872000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>There is a lack of consensus regarding how best to screen children with facial port\u2010wine stains for Sturge\u2010Weber syndrome. Many favor brain magnetic resonance imaging, and adjunctive electroencephalography is increasingly used. However, the sensitivity, specificity, and negative and positive predictive value of magnetic resonance imaging and electroencephalography and whether screening improves seizure recognition is unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective review of children with high\u2010risk port\u2010wine stains presenting consecutively to the outpatient laser clinic of a tertiary pediatric hospital between December 2015 and November 2016 was undertaken. Primary outcome measures were yield, accuracy, age of and protocols for screening magnetic resonance imaging and electroencephalography, type of and age at presenting seizure, and percentage referred to neurology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 126 patients with facial port\u2010wine stains, 25.4% (32\/126) were at high risk of Sturge\u2010Weber syndrome (hemifacial, median, and forehead PWS phenotypes); 43.7% of these (14\/32) underwent screening magnetic resonance imaging. Sturge\u2010Weber syndrome was detected in 7.1% (1\/14). Magnetic resonance imaging had false\u2010negative results in 23.1% (3\/13) of those screened. Screening magnetic resonance imaging had sensitivity of 25%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 76.9% for the detection of Sturge\u2010Weber syndrome (hemifacial, median and forehead PWS phenotypes). Only one\u2010third of those with false\u2010negative magnetic resonance imaging were referred to neurology. Mean age of first seizure in those with false\u2010negative screening magnetic resonance imaging was 28\u00a0months, vs 14\u00a0months in those not screened. Abnormal electroencephalographic signs were detected in the two infants who underwent presymptomatic electroencephalography.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Findings from this small cohort of individuals with port\u2010wine stains that put them at high risk of Sturge\u2010Weber syndrome suggest that children with positive screening magnetic resonance imaging will almost certainly develop Sturge\u2010Weber syndrome but that negative screening magnetic resonance imaging cannot exclude Sturge\u2010Weber syndrome (in up to 23.1% of cases). False\u2010negative magnetic resonance imaging may delay seizure recognition. Seizure education, monitoring, and consideration of adjunctive electroencephalography are important irrespective of magnetic resonance imaging findings.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pde.13598","type":"journal-article","created":{"date-parts":[[2018,7,19]],"date-time":"2018-07-19T06:52:34Z","timestamp":1531983154000},"page":"575-581","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Retrospective review of screening for Sturge\u2010Weber syndrome with brain magnetic resonance imaging and electroencephalography in infants with high\u2010risk port\u2010wine stains"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1604-0547","authenticated-orcid":false,"given":"Michaela","family":"Zallmann","sequence":"first","affiliation":[{"name":"Department of Dermatology Eastern Health Monash University Melbourne Vic. Australia"},{"name":"Murdoch Children's Research Institute Melbourne Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark T.","family":"Mackay","sequence":"additional","affiliation":[{"name":"Murdoch Children's Research Institute Melbourne Vic. Australia"},{"name":"Royal Children's Hospital Melbourne Vic. Australia"},{"name":"Department of Pediatrics University of Melbourne Melbourne Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard J.","family":"Leventer","sequence":"additional","affiliation":[{"name":"Murdoch Children's Research Institute Melbourne Vic. Australia"},{"name":"Royal Children's Hospital Melbourne Vic. Australia"},{"name":"Department of Pediatrics University of Melbourne Melbourne Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Ditchfield","sequence":"additional","affiliation":[{"name":"Monash Health Monash University Clayton Vic. Australia"},{"name":"Monash Health Diagnostic Imaging Clayton Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip S.","family":"Bekhor","sequence":"additional","affiliation":[{"name":"Royal Children's Hospital Melbourne Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John C.","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Dermatology Eastern Health Monash University Melbourne Vic. Australia"},{"name":"Murdoch Children's Research Institute Melbourne Vic. Australia"},{"name":"Royal Children's Hospital Melbourne Vic. Australia"},{"name":"Department of Pediatrics University of Melbourne Melbourne Vic. Australia"},{"name":"Monash Health Monash University Clayton Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,7,18]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.11.009"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13203"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213507"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073812451326"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100008878"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320570110"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2013.10.003"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.04169.x"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2011.01720.x"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2011.01485.x"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1089\/lrb.2007.1016"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-004-1333-2"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/NRL.0b013e318220c5b6"},{"key":"e_1_2_5_15_1","unstructured":"OdaciE.Face embryology. Medscape.2013. (online).https:\/\/emedicine.medscape.com\/article\/844962-overviewAccessed January 8 2017."},{"key":"e_1_2_5_16_1","first-page":"719","article-title":"Contrast\u2010enhanced fluid\u2010attenuated inversion recovery imaging for leptomeningeal disease in children","volume":"24","author":"Griffiths PD","year":"2003","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.21435"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/ACO.0b013e328304115b"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1213\/01.ane.0000268712.00756.dd"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1213\/ANE.0b013e318213be52"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/pde.13304"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-013-0253-6"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2009.06.005"},{"key":"e_1_2_5_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2014.01.023"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpde.13598","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.13598","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T06:11:06Z","timestamp":1695017466000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.13598"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,18]]},"references-count":23,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2018,9]]}},"alternative-id":["10.1111\/pde.13598"],"URL":"https:\/\/doi.org\/10.1111\/pde.13598","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,7,18]]},"assertion":[{"value":"2018-07-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}