A Case of Klippel-Trenaunay Syndrome Combinded with Sturge-Weber Syndrome.
- Author:
Mi Young KIM
1
;
So Young LEE
;
Na Yeon KIM
;
Sun Ju LEE
;
Won Duck KIM
;
Sung Min CHO
;
Dong Seok LEE
;
Doo Kwun KIM
;
Sung Min CHOI
Author Information
1. Department of Pediatrics, College of Medicine, Dongguk University, Kyongju, Korea. just2my@hanmail.net
- Publication Type:Case Report
- Keywords:
Klippel-Trenaunay syndrome;
Sturge-Weber syndrome
- MeSH:
Atrophy;
Cerebrum;
Choroid;
Extremities;
Female;
Fistula;
Glaucoma;
Hemangioma;
Humans;
Hypertrophy;
Klippel-Trenaunay-Weber Syndrome*;
Mesoderm;
Neurocutaneous Syndromes;
Nevus;
Parturition;
Port-Wine Stain;
Skin;
Skull;
Sturge-Weber Syndrome*;
Trigeminal Nerve
- From:Journal of the Korean Pediatric Society
2003;46(9):909-912
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata(usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the trigeminal nerve. Homolateral to the skin lesion, there is atrophy and calcification of the cerebral hemisphere. We experienced an unusual 26-months-old female, who had features of both Klippel-Trenaunay syndrome and Sturge-Weber syndrome. She had an extensive nevus flammeus which extended primarily over both sides of her face and the right side of the trunk and extremities, hypertrophy of the right extremity without evidence of arterovenous fistula, right glaucoma, choroidal hemangioma and leptomeningeal hemangioma, which combined Klippel-Trenaunay syndrome and Sturge-Weber syndrome. We reported this rare case with a brief review of some related literatures.
