RT Book, Section
A1 Arzimanoglou, Alexis
A1 Kossoff, Eric
A2 Duchowny, Michael
A2 Cross, J. Helen
A2 Arzimanoglou, Alexis
SR Print(0)
ID 1138411088
T1 Sturge–Weber Syndrome
T2 Pediatric Epilepsy
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071496216
LK accesspediatrics.mhmedical.com/content.aspx?aid=1138411088
RD 2024/04/19
AB Sturge–Weber  syndrome  (SWS)  is  a  nonfamilial  neurocutaneous  disorder  with  a  potentially  progressive  course.  The  syndrome  consists  of  a  nevus  flammeus  (port-wine  stain)  involving  part  of  the  face,  in  most  of  the  patients  all  or  part  of  the  area  supplied  by  the  trigeminal  nerve  (V1  distribution  most  commonly).  In  all  patients  a  venous  angioma  of  the  leptomeninges  and  less  often,  a  choroidal  angioma,  and  ipsilateral  glaucoma  is  present.  The  facial  and  leptomeningeal  angioma  are  usually  ipsilateral,  but  both  can  be  bilateral.  Pial  angiomatosis  more  frequently  occurs  in  the  occipital  region,  but  it  can  be  localized  anywhere  and  can  involve  an  entire  hemisphere  or  even  be  bilateral.  The  extent  of  lesions  that  affect  the  facial  skin,  eyes,  and  central  nervous  system  vary  between  patients,  and  in  some  cases  only  a  single-organ  system  may  be  affected.1  Cases  of  SWS  exist  without  the  facial  port-wine  stain.