title: 12-year retrospective study of Sturge-Weber syndrome and literature review.
abstract: OBJECTIVE
  To review the clinical manifestations and neuroimaging features of patients with Sturge-Weber syndrome (SWS) treated at Srinagarind Hospital over a 12-year period.
  
  
  METHODS
  A retrospective study of sixteen patients with SWS (9 males and 7 females) was conducted. The medical records, photographs, and neuroimaging studies were reviewed
  
  
  RESULTS
  All patients had port-wine stain (PWS) involving the eyelid. Bilateral cutaneous lesions were revealed in four patients (25%). Glaucoma was the main ocular disease being diagnosed in 11 eyes of nine patients (56.25%); four eyes were finally blind. The cyclodestructive procedure and/or surgical treatment was required in four eyes. Other ocular abnormalities were refractive error dilated episcleral vessels, corneal abnormalities, tortuous retinal vessels, choroidal hemangioma, amblyopia, and strabismus. Twelve patients (75%) had neurological impairment including seizure, hemiparesis, headache, and delayed development. However the most common neurological manifestation was epilepsy (75%), which could be controlled with antiepileptic drugs. Neurological imaging was performed in the majority of cases (14 patients). Intracranial abnormalities were demonstrated in 11 patients (78.57%). These included cerebral atrophy (81.82%), cerebral calcification (54.55%), leptomeningeal angioma (27.27%), and enlarged choroidal plexus (27.27%). The ocular complication and intracranial abnormalities were usually ipsilateral to the PWS. One patient with unilateral PWS, however had bilateral intracranial lesion.
  
  
  CONCLUSIONS
  Port-wine stains, glaucoma, and seizure were the most common clinical features of Sturge-Weber syndrome detected in the present study. Complete ophthalmic and neurological evaluation should be performed at the time ofdiagnosis. Multidisciplinary team management as well as lifelong follow-up is needed.
authors.forename: Suthasinee
authors.initials: S
authors.lastname: Sinawat
authors.forename: Narong
authors.initials: N
authors.lastname: Auvichayapat
authors.forename: Paradee
authors.initials: P
authors.lastname: Auvichayapat
authors.forename: Yosanan
authors.initials: Y
authors.lastname: Yospaiboon
authors.forename: Supat
authors.initials: S
authors.lastname: Sinawat
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000453:epidemiology / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005901:Glaucoma / Q000453:epidemiology / Q000209:etiology; D006391:Hemangioma / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D059906:Neuroimaging / Q000379:methods*; D010291:Paresis / Q000453:epidemiology / Q000209:etiology; D019339:Port-Wine Stain / Q000209:etiology*; D012189:Retrospective Studies; D012640:Seizures / Q000453:epidemiology / Q000209:etiology; D013341:Sturge-Weber Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D055815:Young Adult