Surgical Management of Severe Venous Malformation of the Right Hand in Maffucci's Syndrome

馬富西症候群(Maffucci's syndrome)是一種罕見，先天性非遺傳性症候群；其特徵是全身多處軟骨瘤(Ollier's disease)及皮下血管畸形。由於中胚層的變性導致其骨骼方面有廣泛性的軟骨瘤；常見部位依次為手部、經／腓骨、足部、股骨、肱骨。血管方面的異常為血管畸型，通常是靜脈型血管畸型，其次才為微血管型及混合型；其常見於手部、足部、手臂、腿、軀幹、頭頸部。對於血管畸型的治療，在過去並無適當的方法被提出。馬富西症候群的病人有高達23%至37%惡性化可能；軟骨肉瘤是最常見的一種。從1881年至今，全世界只有不到二百個報告病例。我們提出一例合併有右手嚴重靜脈畸型及指骨多處軟骨瘤之馬富西症候群。經過連續手術切除後，追蹤半年，病人有滿意的功能及外觀。文獻所及此為台灣之首例。

關鍵字

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並列摘要

Maffucci's syndrome is a rare, congenital non-hereditary syndrome characterized by multiple enchondromas (Ollier's disease) and subcutaneous vascular malformation. The skeleton lesion is diffuse enchondroma which is due to mesodermal dysplasia. The common locations of enchondroma are hand, tibia/fibula, foot, femur and humerus in descending order. The vascular lesion is vascular malformation in which the venous type is most commonly seen, followed by capillary and mixed type. It can be seen in hand, foot, arm, leg, trunk, head/neck and other locations. No definite recommendations for the treatment of vascular malformation was proposed previously. It has been found that Maffucci's syndrome has the potential for malignant change. The overall rate of malignant change ranges from 23% to 37% in which chondrosarcoma is the most common one. Since its first description in 1881, no more than 200 cases have been reported in the world. We present a case of Maffucci's syndrome with severe venous malformation in the right hand and multiple enchondroma in the phalanxes. After sequential excision of venous malformation, the patient has satisfactory hand functions and a positive cosmetic result at the six months' follow-up. In reviewing the available literature, we have determined that this is the first case in Taiwan.

並列關鍵字

Maffucci's syndrome ； multiple enchondroma ； vascular malformation

國際替代計量

Surgical Management of Severe Venous Malformation of the Right Hand in Maffucci's Syndrome

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