PEM Source

Your source for all things Pediatric Emergency Medicine

1

Question: Congenital condition

(Click the link to comment and to vote – voting not working through email, sorry!)

A 2 year old child has a flat lesion of the entire left forehead and upper eyelid the color of light red wine that has been present since birth. The family has presented to the ED because they are concerned that the child has right sided weakness.

 

sturge-weber-1

https://mindfulmema.wordpress.com/tag/sturge-weber/

Which of the following is the child NOT at increased risk for?

 

DermNeuroSyndrome

pemsou5_wp • September 18, 2018


Previous Post

Next Post

Comments

  1. Kelly September 18, 2018 - 9:31 pm Reply

    B) Intussusception. The child’s facial lesion as described is a port-wine stain, or facial capillary malformation in the V1 and V2 distribution. Approximately 10% of patients with port wine stain in this distribution will have Sturge-Weber syndrome. Common complications of Sturge-Weber syndrome, which includes leptomeningeal vascular malformation, include: seizures (80%), intellectual disability (50%) and behavioral problems, hemiparesis and hemiatrophy on the contralateral side, glaucoma, visual field defects, and other ophthalmologic findings, and growth hormone deficiency. Associated intestinal vascular malformations increasing risk of intussusception are not a feature.

Leave a Reply

Your email address will not be published / Required fields are marked *