{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T14:22:08Z","timestamp":1788186128953,"version":"build-2803163510"},"reference-count":52,"publisher":"American Academy of Pediatrics (AAP)","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,9,1]]},"abstract":"<jats:p>Sturge-Weber syndrome (SWS) is a neurocutaneous disorder that classically presents with a triad of vascular anomalies affecting the skin, eyes, and brain. Previously, the trigeminal nerve distribution of a port-wine birthmark (PWB) of the face was used to identify risk of SWS. However, recent evidence has demonstrated that PWBs are vascular, not neurologic, in embryologic origin, and facial PWBs at highest risk for the brain involvement of SWS involve the forehead location. Furthermore, a PWB involving the upper or lower eyelid carries a risk of glaucoma, which requires lifelong monitoring. The gold standard of treatment for PWB is the pulsed dye laser, which has many advantages when started as early as possible in infancy. In this review, we discuss the locations of facial PWBs at risk for neurologic and ophthalmologic complications, the differential diagnosis of facial vascular birthmarks, recommendations for patient referral(s) when needed, and the advantages of early laser therapy when desired for the PWB. We also provide additional resources for pediatricians to support patients and their families.<\/jats:p>","DOI":"10.1542\/pir.2021-005437","type":"journal-article","created":{"date-parts":[[2022,8,31]],"date-time":"2022-08-31T23:03:16Z","timestamp":1661986996000},"page":"507-516","source":"Crossref","is-referenced-by-count":30,"title":["Port-wine Birthmarks: Update on Diagnosis, Risk Assessment for Sturge-Weber Syndrome, and Management"],"prefix":"10.1542","volume":"43","author":[{"given":"Anna","family":"Poliner","sequence":"first","affiliation":[{"name":"*Departments of Internal Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esteban","family":"Fernandez Faith","sequence":"additional","affiliation":[{"name":"\u2020Division of Dermatology, Department of Pediatrics, Nationwide Children\u2019s Hospital and The Ohio State University College of Medicine, Columbus, OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Blieden","sequence":"additional","affiliation":[{"name":"\u2021Ophthalmology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristen M.","family":"Kelly","sequence":"additional","affiliation":[{"name":"\u00a7Department of Dermatology, University of California, Irvine, CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denise","family":"Metry","sequence":"additional","affiliation":[{"name":"\u00b6Department of Dermatology, Texas Children\u2019s Hospital, Houston, TX"},{"name":"**Dermatology, Baylor College of Medicine, Houston, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2022,9,1]]},"reference":[{"issue":"1","key":"2022090103025151000_B1","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1111\/pde.14392","article-title":"The anatomic distribution of isolated and syndrome-associated port-wine stain","volume":"38","author":"Elias","year":"2021","journal-title":"Pediatr Dermatol"},{"issue":"21","key":"2022090103025151000_B2","doi-asserted-by":"crossref","first-page":"1971","DOI":"10.1056\/NEJMoa1213507","article-title":"Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ","volume":"368","author":"Shirley","year":"2013","journal-title":"N Engl J Med"},{"issue":"4","key":"2022090103025151000_B3","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1016\/j.jid.2017.10.033","article-title":"Analyzing the genetic spectrum of vascular anomalies with overgrowth via cancer genomics","volume":"138","author":"Siegel","year":"2018","journal-title":"J Invest Dermatol"},{"issue":"10","key":"2022090103025151000_B4","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1097\/DAD.0000000000000785","article-title":"Activation of PKC\u03b1 and PI3K kinases in hypertrophic and nodular port wine stain lesions","volume":"39","author":"Yin","year":"2017","journal-title":"Am J Dermatopathol"},{"issue":"4","key":"2022090103025151000_B5","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1111\/bjd.13203","article-title":"New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk","volume":"171","author":"Waelchli","year":"2014","journal-title":"Br J Dermatol"},{"issue":"4","key":"2022090103025151000_B6","doi-asserted-by":"crossref","first-page":"1110","DOI":"10.1016\/j.jaad.2020.05.017","article-title":"Forehead location and large segmental pattern of facial port-wine stains predict risk of Sturge-Weber syndrome","volume":"83","author":"Boos","year":"2020","journal-title":"J Am Acad Dermatol"},{"issue":"1","key":"2022090103025151000_B7","doi-asserted-by":"crossref","first-page":"105","DOI":"10.4103\/0976-3147.143215","article-title":"Diagnostic dilemma: Sturge-Weber syndrome, without facial nevus","volume":"6","author":"Zanzmera","year":"2015","journal-title":"J Neurosci Rural Pract"},{"issue":"2","key":"2022090103025151000_B8","first-page":"215","article-title":"Sturge-Weber disease without facial nevus","volume":"33","author":"Maiuri","year":"1989","journal-title":"J Neurosurg Sci"},{"issue":"1","key":"2022090103025151000_B9","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1684\/j.1950-6945.2008.tb00254.x","article-title":"Sturge-Weber syndrome, without a facial port-wine stain, with epilepsy onset in the fifth decade","volume":"10","author":"Kim","year":"2008","journal-title":"Epileptic Disord"},{"issue":"3","key":"2022090103025151000_B10","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.jaad.2014.11.009","article-title":"A prospective study of risk for Sturge-Weber syndrome in children with upper facial port-wine stain","volume":"72","author":"Dutkiewicz","year":"2015","journal-title":"J Am Acad Dermatol"},{"issue":"8","key":"2022090103025151000_B11","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1001\/archderm.142.8.994","article-title":"Bilateral facial capillary malformation associated with eye and brain abnormalities","volume":"142","author":"Mazereeuw-Hautier","year":"2006","journal-title":"Arch Dermatol"},{"issue":"3","key":"2022090103025151000_B12","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1159\/000235546","article-title":"Trigeminal dermatome distribution in patients with glaucoma and facial port wine stain","volume":"219","author":"Mehta","year":"2009","journal-title":"Dermatology"},{"key":"2022090103025151000_B13","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/j.jpeds.2017.05.053","article-title":"Size of facial port-wine birthmark may predict neurologic outcome in Sturge-Weber syndrome","volume":"188","author":"Dymerska","year":"2017","journal-title":"J Pediatr"},{"key":"2022090103025151000_B14","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.ajo.2020.08.003","article-title":"Facial port-wine stain phenotypes associated with glaucoma risk in neonates","volume":"220","author":"Ha","year":"2020","journal-title":"Am J Ophthalmol"},{"issue":"3","key":"2022090103025151000_B15","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1053\/seiz.2001.0629","article-title":"Prophylactic antiepileptic treatment in Sturge-Weber disease","volume":"11","author":"Ville","year":"2002","journal-title":"Seizure"},{"issue":"6","key":"2022090103025151000_B16","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1177\/0883073810388646","article-title":"Survey of aspirin use in Sturge-Weber syndrome","volume":"26","author":"Bay","year":"2011","journal-title":"J Child Neurol"},{"issue":"2","key":"2022090103025151000_B17","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1177\/0883073812463607","article-title":"Aspirin use in Sturge-Weber syndrome: side effects and clinical outcomes","volume":"28","author":"Lance","year":"2013","journal-title":"J Child Neurol"},{"issue":"2","key":"2022090103025151000_B18","doi-asserted-by":"crossref","first-page":"276","DOI":"10.3174\/ajnr.A1350","article-title":"A spectrum of unusual neuroimaging findings in patients with suspected Sturge-Weber syndrome","volume":"30","author":"Adams","year":"2009","journal-title":"AJNR Am J Neuroradiol"},{"issue":"12","key":"2022090103025151000_B19","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1177\/0009922816677806","article-title":"Feed and wrap MRI technique in infants","volume":"56","author":"Antonov","year":"2017","journal-title":"Clin Pediatr (Phila)"},{"key":"2022090103025151000_B20","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.ejpn.2021.07.005","article-title":"Neurological presentations and cognitive outcome in Sturge-Weber syndrome","volume":"34","author":"Powell","year":"2021","journal-title":"Eur J Paediatr Neurol"},{"key":"2022090103025151000_B21","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.pediatrneurol.2018.04.005","article-title":"A multidisciplinary consensus for clinical care and research needs for Sturge-Weber syndrome","volume":"84","author":"De la Torre","year":"2018","journal-title":"Pediatr Neurol"},{"issue":"4","key":"2022090103025151000_B22","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/j.jaad.2016.10.039","article-title":"Quality of life in adults with facial port-wine stains","volume":"76","author":"Hagen","year":"2017","journal-title":"J Am Acad Dermatol"},{"issue":"2","key":"2022090103025151000_B23","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1097\/IIO.0b013e3181693582","article-title":"Surgical management of glaucoma with the Sturge Weber syndrome","volume":"48","author":"Patrianakos","year":"2008","journal-title":"Int Ophthalmol Clin"},{"issue":"2","key":"2022090103025151000_B24","doi-asserted-by":"crossref","first-page":"134","DOI":"10.5301\/ejo.5000510","article-title":"The effect of oral propranolol on intraocular pressure in infants with Sturge-Weber syndrome glaucoma","volume":"25","author":"Wygnanski-Jaffe","year":"2015","journal-title":"Eur J Ophthalmol"},{"key":"2022090103025151000_B25","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.jpeds.2016.07.054","article-title":"PHACE syndrome: consensus-derived diagnosis and care recommendations","volume":"178","author":"Garzon","year":"2016","journal-title":"J Pediatr"},{"issue":"1","key":"2022090103025151000_B26","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.jaad.2016.02.1167","article-title":"Prognosis and response to laser treatment of early-onset hypertrophic port-wine stains (PWS)","volume":"75","author":"Passeron","year":"2016","journal-title":"J Am Acad Dermatol"},{"issue":"3","key":"2022090103025151000_B27","doi-asserted-by":"crossref","first-page":"360","DOI":"10.4103\/0976-237X.118345","article-title":"Sturge-Weber syndrome with pyogenic granuloma","volume":"4","author":"Mopagar","year":"2013","journal-title":"Contemp Clin Dent"},{"issue":"6","key":"2022090103025151000_B28","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1016\/j.jaad.2012.05.027","article-title":"Hypertrophy in port-wine stains: prevalence and patient characteristics in a large patient cohort","volume":"67","author":"van Drooge","year":"2012","journal-title":"J Am Acad Dermatol"},{"key":"2022090103025151000_B29","doi-asserted-by":"crossref","first-page":"bcr2014207663","DOI":"10.1136\/bcr-2014-207663","article-title":"Sturge-Weber syndrome: oral and extra-oral manifestations","volume":"2015","author":"Tripathi","year":"2015","journal-title":"BMJ Case Rep"},{"issue":"2","key":"2022090103025151000_B30","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/pde.14503","article-title":"Light-based treatment of pediatric port-wine birthmarks","volume":"38","author":"Tran","year":"2021","journal-title":"Pediatr Dermatol"},{"issue":"7","key":"2022090103025151000_B31","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1002\/lsm.20529","article-title":"Efficacy of early treatment of facial port wine stains in newborns: a review of 49 cases","volume":"39","author":"Chapas","year":"2007","journal-title":"Lasers Surg Med"},{"issue":"6","key":"2022090103025151000_B32","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/j.clindermatol.2015.09.010","article-title":"Laser therapy in dermatology: kids are not just little people","volume":"33","author":"Shahriari","year":"2015","journal-title":"Clin Dermatol"},{"issue":"1","key":"2022090103025151000_B33","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1001\/jamadermatol.2020.4226","article-title":"Consensus statement for the management and treatment of port-wine birthmarks in Sturge-Weber syndrome","volume":"157","author":"Sabeti","year":"2021","journal-title":"JAMA Dermatol"},{"issue":"4596","key":"2022090103025151000_B34","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1126\/science.6836297","article-title":"Selective photothermolysis: precise microsurgery by selective absorption of pulsed radiation","volume":"220","author":"Anderson","year":"1983","journal-title":"Science"},{"issue":"3","key":"2022090103025151000_B35","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1007\/s10103-014-1695-0","article-title":"Can we predict the outcome of 595-nm wavelength pulsed dye laser therapy on capillary vascular malformations from the first beginning: a pilot study of efficacy co-related factors in 686 Chinese patients","volume":"30","author":"Liu","year":"2015","journal-title":"Lasers Med Sci"},{"issue":"4","key":"2022090103025151000_B36","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/0190-9622(95)90346-1","article-title":"Adverse effects associated with the 577- and 585-nanometer pulsed dye laser in the treatment of cutaneous vascular lesions: a study of 500 patients","volume":"32","author":"Levine","year":"1995","journal-title":"J Am Acad Dermatol"},{"issue":"6","key":"2022090103025151000_B37","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1016\/0190-9622(90)70348-L","article-title":"Treatment of port-wine stains during childhood with the flashlamp-pumped pulsed dye laser","volume":"23","author":"Reyes","year":"1990","journal-title":"J Am Acad Dermatol"},{"issue":"6","key":"2022090103025151000_B38","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1111\/j.1524-4725.1995.tb00256.x","article-title":"Repetitive pulsed dye laser treatments improve persistent port-wine stains","volume":"21","author":"Kauvar","year":"1995","journal-title":"Dermatol Surg"},{"issue":"5","key":"2022090103025151000_B39","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1046\/j.1365-2133.1998.02219.x","article-title":"Facial port wine stains in childhood: prediction of the rate of improvement as a function of the age of the patient, size and location of the port wine stain and the number of treatments with the pulsed dye (585 nm) laser","volume":"138","author":"Nguyen","year":"1998","journal-title":"Br J Dermatol"},{"issue":"2","key":"2022090103025151000_B40","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1001\/archderm.1993.01680230066007","article-title":"Anatomical differences of port-wine stains in response to treatment with the pulsed dye laser","volume":"129","author":"Renfro","year":"1993","journal-title":"Arch Dermatol"},{"issue":"5","key":"2022090103025151000_B41","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1016\/j.jaad.2011.11.964","article-title":"Investigation into optimal treatment intervals of facial port-wine stains using the pulsed dye laser","volume":"67","author":"Anolik","year":"2012","journal-title":"J Am Acad Dermatol"},{"issue":"11","key":"2022090103025151000_B42","doi-asserted-by":"crossref","first-page":"1174","DOI":"10.1001\/archotol.1996.01890230022005","article-title":"Port-wine stains: an assessment of 5 years of treatment","volume":"122","author":"Orten","year":"1996","journal-title":"Arch Otolaryngol Head Neck Surg"},{"issue":"3","key":"2022090103025151000_B43","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1016\/0190-9622(91)70075-D","article-title":"Flashlamp-pumped pulsed dye laser for port-wine stains in infancy: earlier versus later treatment","volume":"24","author":"Ashinoff","year":"1991","journal-title":"J Am Acad Dermatol"},{"issue":"7","key":"2022090103025151000_B44","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1056\/NEJM198902163200702","article-title":"Treatment of children with port-wine stains using the flashlamp-pulsed tunable dye laser","volume":"320","author":"Tan","year":"1989","journal-title":"N Engl J Med"},{"issue":"10","key":"2022090103025151000_B45","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1001\/archpedi.1995.02170230096014","article-title":"Initial lesion size as a predictive factor in determining the response of port-wine stains in children treated with the pulsed dye laser","volume":"149","author":"Morelli","year":"1995","journal-title":"Arch Pediatr Adolesc Med"},{"issue":"4","key":"2022090103025151000_B46","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1001\/jamadermatol.2018.5249","article-title":"Pulsed dye laser treatment of port-wine stains in infancy without the need for general anesthesia","volume":"155","author":"Jeon","year":"2019","journal-title":"JAMA Dermatol"},{"key":"2022090103025151000_B47","unstructured":"FDA. FDA Drug Safety Communication: FDA approves label changes for use of general anesthetic and sedation drugs in young children. Published April 2017. Available at: https:\/\/www.fda.gov\/drugs\/drug-safety-and-availability\/fda-drug-safety-communication- fda-approves-label-changes-use-general-anesthetic-and-sedation-drugs"},{"issue":"4","key":"2022090103025151000_B48","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1213\/ANE.0000000000002833","article-title":"SmartTots update regarding anesthetic neurotoxicity in the developing brain","volume":"126","author":"Orser","year":"2018","journal-title":"Anesth Analg"},{"issue":"8","key":"2022090103025151000_B49","article-title":"Evaluating the effects of general anesthesia on sleep in children undergoing elective surgery: an observational case-control study","volume":"41","author":"Selvadurai","year":"2018","journal-title":"Sleep (Basel)"},{"issue":"1","key":"2022090103025151000_B50","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1002\/lsm.22532","article-title":"A randomized side-by-side study comparing alexandrite laser at different pulse durations for port wine stains","volume":"49","author":"Carlsen","year":"2017","journal-title":"Lasers Surg Med"},{"issue":"6","key":"2022090103025151000_B51","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1097\/DSS.0000000000000723","article-title":"Assessment of efficacy of the 595-nm pulsed dye laser in the treatment of facial port-wine stains in indian patients","volume":"42","author":"Khandpur","year":"2016","journal-title":"Dermatol Surg"},{"issue":"2","key":"2022090103025151000_B52","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1093\/jpepsy\/jss106","article-title":"Stigmatization predicts psychological adjustment and quality of life in children and adolescents with a facial difference","volume":"38","author":"Masnari","year":"2013","journal-title":"J Pediatr Psychol"}],"container-title":["Pediatrics In Review"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatricsinreview\/article-pdf\/43\/9\/507\/1357302\/pedsinreview.2021005437.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatricsinreview\/article-pdf\/43\/9\/507\/1357302\/pedsinreview.2021005437.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T21:36:17Z","timestamp":1727904977000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatricsinreview\/article\/43\/9\/507\/188938\/Port-wine-Birthmarks-Update-on-Diagnosis-Risk"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,1]]},"references-count":52,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2022,9,1]]},"published-print":{"date-parts":[[2022,9,1]]}},"URL":"https:\/\/doi.org\/10.1542\/pir.2021-005437","relation":{},"ISSN":["0191-9601","1526-3347"],"issn-type":[{"value":"0191-9601","type":"print"},{"value":"1526-3347","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,9,1]]}}}