HMME-PDTで治療されたSturge-Weber症候群を合併したKlippel-Trenaunay症候群の1例

A rare case of Klippel-Trenaunay syndrome in association with Sturge-Weber syndrome treated by HMME-PDT.

• Lian Liu
• Erlong Li
• Dan Hao
• Xian Jiang

抄録

30歳の男性患者は、生まれた時から赤色の斑紋を呈し、その後紫色の斑紋を呈し、顔面、乳房、下肢、陰嚢、陰茎にまで及んでいた。また，右下肢は左下肢に比べて長く，太い。X線検査では左大腿骨と脛骨が反対側の大腿骨よりも長くなっていた。三次元CTでは右下肢に高密度の血管網が認められた．頭部MRIでは右脳室が拡大し，右後頭葉と髄膜が血管奇形の石灰化のために強化されていることが示された．胸部X線撮影と腹部CTは正常であった．5年前に両眼の緑内障の手術歴があった。発作や脳卒中様エピソードなどの既往はなかった。身体所見や画像検査の結果、男性はクリッペル-トレナウネ症候群（KTS）と重なるSturge-Weber症候群（SWS）と診断された。

A 30-year-old male patient presented with red patches since birth, and then turned to purple patches, involving his face, breast, lower extremities, scrotum as well as penis. Additionally, the right lower limb was longer and thicker than the left lower limb. X-ray examination illustrated the left femur and tibia were longer than the opposite ones. High density vascular network was shown on the right lower extremity by three-dimensional CT angiography. Enhanced MRI of head indicated that right ventricle was enlarged, the right occipital lobe and meningeal membrane were enhanced for the calcification of vascular malformations. The chest radiography and abdominal CT were normal. He had a history of surgery for glaucoma on both eyes 5 years ago. There was no history of seizure, stroke-like episodes, etc. According to the physical examinations and imaging studies, the man was considered to be diagnosed as Sturge-Weber syndrome (SWS) overlapping with Klippel-Trenaunay syndrome (KTS).

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