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題名 | Phakomatosis Pigmentovascularis Type IIb with Patent Umbilical Vein and Inferior Vena Cava Hypoplasia=色素血管性母斑症IIb型合併開放性臍靜脈以及下腔靜脈發育不全 |
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作者 | 羅珮瑜; 宗天一; Lo, Pei-yu; Tzung, Tien-yi; |
期刊 | 中華皮膚科醫學雜誌 |
出版日期 | 20020900 |
卷期 | 20:3 2002.09[民91.09] |
頁次 | 頁241-247 |
分類號 | 415.74 |
語文 | eng |
關鍵詞 | 色素血管性母斑症; 開放性臍靜脈; 下腔靜脈; Phakomatosis pigmentovascularis; Patent umbilical vein; Inferior vena cava; |
中文摘要 | 色素血管性母斑症(PPV)的特癥是同時出現微血管畸形和黑色素病灶,在此,我們報告一PPV IIb型同時存在開放性臍靜脈的病例,在以往文獻中未曾被描述過。一位二十歲男性,合併太田母斑,異常蒙古斑與廣泛酒色斑;他左眼失明,並密輕度智障及癲癇病史,符合Sturge-Weber症候群,腦部電腦淫層顯示左腦鈣化及萎縮。另一方面,他右下肢較為肥大,並有反覆性潰瘍,而且腹壁有靜脈曲張。兩腿血管攝影呈現血管瘤與血管畸形,合乎Klippel-Trenaunay-Weber徵候群的描述。進一步的檢查發現此病人存在開放性臍靜脈以及下腔靜脈發育不全。 |
英文摘要 | Phakomatosis pigmentovascularis (PPV) is characterized by coexistence of capillary malformation and various melanocytic lesions. Herein, we reported a case of PPV type IIb with patent umbilical vein and, to our best knowledge, this condition has never been described before. This 20-year-old male had nevus of Ota, aberrant Mongolian spots and extensive port-wine stain. He suffered form Sturge-Weber syndrome with left eye blindness, mild mental retardation and epilepsy. Brain computed tomography demonstrated left cerebral calcification and atrophy. In addition, he had Klippel-Trenaunay-Weber syndrome, manifested as hypertrophy of the right lower leg with repeated ulcers and abdominal varicose vein. Angiography of bilateral lower limbs revealed angiomas and vascular malformations. Further studies showed patent umbilical vein and hypoplasia of inferior vena cava. |
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