Klippel-trenaunay-weber syndrome with hemimegalencephaly; report of a pediatric case

Hossein Esmailzadeh; Azita Tavassoli; Younes Jahangiri N; Nasibeh Vatankhah

Klippel-trenaunay-weber syndrome with hemimegalencephaly; report of a pediatric case

Iran J Pediatr. 2012 Mar;22(1):147-51.

Authors

Hossein Esmailzadeh¹, Azita Tavassoli, Younes Jahangiri N, Nasibeh Vatankhah

Affiliation

¹ Aliasghar Pediatric Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

PMID: 23056878
PMCID: PMC3448232

Abstract

Background: Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare neurocutaneous syndrome. Hemimegalencephaly (HME) and seizure episodes have been reported previously in a few cases with KTWS.

Case presentation: We report here a 3 day-old girl with partial motor seizures, extensive port-wine staining and mild structural deformities in the feet, and a hemimegalencephaly.

Conclusion: Occurrence of partial motor seizures in addition to bilateral lower extremities extensive port-wine staining is a unique feature seen in our case.

Keywords: Hemimegalencephaly; Klippel-Trenaunay-Weber Syndrome; Neurocutaneous Syndrome.

Publication types

Case Reports