| A Case of Klippel-Trenaunay Syndrome Combinded with Sturge-Weber Syndrome |
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Mi Young Kim, So Young Lee, Na Yeon Kim, Sun Ju Lee, Won Duck Kim, Sung Min Cho, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi |
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Department of Pediatrics, College of Medicine, Dongguk University, Kyongju, Korea |
| Klippel-Trenaunay 증후군과 Sturge-Weber 증후군이 동반된 1례 |
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김미영, 이소영, 김나영, 이선주, 김원덕, 조성민, 이동석, 김두권, 최성민 |
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동국대학교 의과대학 소아과학교실 |
Correspondence:
Mi Young Kim, Email: just2my@hanmail.net
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| Abstract |
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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. |
| Key Words:
Klippel-Trenaunay syndrome, Sturge-Weber syndrome |
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