More articles from PEDIATRICS

MR imaging of 139 children presenting with port-wine stain and/or Sturge-Weber syndrome between 1998 and 2017 was evaluated by 2 pediatric neuroradiologists for marrow signal abnormality and pial angioma and other Sturge-Weber syndrome features. Groups were divided into port-wine stain-only (without intracranial Sturge-Weber syndrome features) and Sturge-Weber syndrome (the presence of cerebral pial angioma). In the port-wine stain-only cohort, 78% had ipsilateral bony changes and 17% had no intraosseous changes. In the Sturge-Weber syndrome cohort, 84/99 had associated port-wine stain, 91% had bony changesipsilateral to the port-wine stain or had no bone changes in the absence of port-wine stain, and 77% had bony changes ipsilateral to a cerebral pial angioma. The authors conclude that intraosseous marrow changes are strongly associated with facial port-wine stain. No significant association was found between pial angioma and bone marrow changes.