Klippel-Trenaunay Syndrome: Characteristic Manifestations on CT and MRI

克利伯全納諾症候群(KTS)之電腦斷層與磁振造影影像表徵

陳欣琪(Hsin-Chi Chen) ； 李瑤華(Yiu-Wah Li) ； 徐劍耀(Jane Chien-Yao Hsu) ； 蘇誠道(Cheng-Tau Su) ； 黃秀芬(Show-Fan Huang)

《中華放射線醫學雜誌》 21卷2期 (1996/04) Pp. 87-92

摘要

克利伯全納諾症候群(Klippel-Trenaunay syndrome)又稱作血管畸形骨肥大綜合症(Angio-osteo-hypertrophy syndrome)，是一種先天性血管生成病變，我們在此分析五個病例之電腦斷層與核磁共振影像表徵，並與其血管攝影及病理所見相互配合。這五個病人均有腰部及下肢的軟組織肥大和瀰漫性血管瘤。第一個病人尚併有巨趾及瀰漫性後腹膜腔淋巴管瘤，造成壓迫性水腎；而第二與第五個病人一出生即可見血液凝固困難及血管瘤漫延至外生殖器；第三個病人因懷孕期間迅速腫大疼痛的血管瘤而就診；第四個病人因合併出現動靜脈畸形而歸類於克利伯全納諾威勃症候群(Klippel-Trenaunay-Weber syndrome)。藉由電腦斷層與核磁共振檢查可以清楚的區分出有良好顯影劑加強的血管瘤和不被顯影劑加強的淋巴管瘤，且在核磁共振影像上也可觀察到血管瘤的特性：在以梯度回波(Gradient recalled-echo)為主和以T2為主之影像中，有極高的訊號；且腫瘤內包含一些蟲樣彎曲的血管腔和血流陰影(flow voids)。相對地；淋巴管瘤則有其典型的多重隔膜表現，而在以梯度回波為主和以T2為主之影像中，其訊號稍低於血管瘤。值得注意的是，以梯度回波為主之影像，對偵測表淺靜脈瘤及深部血管暢通與否有較好的證實。我們比較這五個病人不同的電腦斷層與核磁共振影像表徵，希望藉此經驗以求提供更完整的資料，早期預防可能發生之併發症。

關鍵字

血管瘤 ； 四肢，電腦斷層 ； 四肢，磁振造影 ； 克利伯全納諾症候群 ； 淋巴管瘤

並列摘要

Klippel-Trenaunay syndrome (KTS) is a rare, sporadic, congenital vascular anomaly. Of the 5 cases of KTS (3 females, 2 males; aged 2 days to 20 years, mean=8.8 years old) presented, all had soft tissue hypertrophy and hemangioma at the flank and thigh, as demonstrated by computed tomography (CT) and magnetic resonance imaging (MRI). Case 1 had macrodactyly of both feet and prominent retroperitoneal lymphangioma to induce hydronephrosis. For Case 2 and 5, there were external genitalia involvement and clinical clotting difficulty during birth. Progressive swelling of the hemangioma over the right lower extremity was noted during pregnancy in Case 3. Case 4 manifested as limb lymphangiohemangioma with arteriovenous malformations, compatible with Klippel-Trenaunay-Weber syndrome (KTWS). CT and MRI can clearly differentiate between coexisting hemangioma and lymphangioma. On CT scan, the former presented as a well-enhanced heterogeneous soft tissue mass; however, the latter manifested as a non-enhanced infiltrating mass. On MR images, hemangioma had very high signal intensity (SI) on gradient recalled-echo (GRE) and T2-weighted images (T2WI) with characteristic worm-like vascular channels, flow voids and sometimes multiple fluid-fluid levels. On the other hand, the multiseptated lymphangioma appeared as less hyperintense on GRE and T2WI. Also, GRE images were helpful in better demonstration of superficial varicosities and deep vessels patency. The CT and MRI features of these five cases were compared and correlated with angiographic and pathological findings.

並列關鍵字

Angioma ； Extremities, CT ； Extremities, MR ； Klippel-Trenaunay syndrome ； Lymphangioma