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报告1例儿童Maffucci综合征,并对国内外相关病例进行文献回顾。患儿男,9岁。右手中指肿物发现7年。体检发现双手多根手指、掌指骨膨大畸形,右侧肋骨皮下膨出,左右肋骨不对称。左手大鱼际、拇指蓝紫色皮下结节,右胸胁部见一片形状不规则褐色咖啡斑。X片示双手、肋骨多发内生软骨瘤,B超示左手大鱼际及拇指皮下脂肪层血管瘤。诊断:Maffucci综合征。文献回顾发现,Maffucci综合征临床少见,表现为内生软骨瘤病伴发软组织的血管瘤,治疗上没有特异的方法,旨在减轻临床、缓解疼痛和早期发现恶变,定期随访很重要。从该例患者来看,目前未见合并良恶性肿瘤,但由于该综合征恶变易发生在成年或中老年,所以嘱其随访,以防恶变。
Report 1 case of Maffucci syndrome in children, and related literature review at home and abroad. Children male, 9 years old. Right middle finger mass found for 7 years. Physical examination found two hands with multiple fingers, metacarpophalangeal deformity, right subcutaneous bulging ribs, left and right ribs asymmetric. Left-handed grand occasion, the thumb blue-purple subcutaneous nodules, the right chest threatened to see an irregular brown coffee stains. X-ray showed both hands, ribs, multiple endogenous chondroma, B-ultrasound showed left thumb and subcutaneous fat layer hemangioma. Diagnosis: Maffucci syndrome. Literature review found that Maffucci syndrome rare clinical manifestations of endothelium associated with soft tissue hemangioma, there is no specific treatment, aimed at reducing the clinical, pain and early detection of malignant transformation, regular follow-up is very important. From the patient’s point of view, no benign and malignant neoplasm, but because of the syndrome malignant transformation occurs in adults or middle-aged, we urge them to follow-up to prevent malignant transformation.