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Maffucci 综合征临床上罕见,并有内脏海绵状血管瘤者更为罕见,最近我院收治1例,报告如下。病历摘要患者女,13岁。生后3个月发现左手较右手大。左手拇、食、中指及无名指的指间关节掌侧横纹有黄豆大小之软组织硬结。5～6岁时逐渐出现上述诸指明显畸形。12岁时左手除小指正常外,其余各指皆粗长,指间关节呈节段梭形增粗,末节指上翘。左足(足母)趾、右手食指同样改变。家族无类似疾病,父母非近亲结婚。检查:发育欠佳,比同龄儿童矮小,面色萎黄,走路踱行。左上腹可触及巨大肿块,位于肋缘下8cm,边缘(?)圆,质地硬,无压痛。左手除小指正常外,均较正常粗4～5倍,长3～4cm,中指最长约11.5cm。
Maffucci syndrome is rare in clinical practice, and visceral cavernous hemangioma is even rarer. Recently, 1 case was treated in our hospital. The report is as follows. Medical record summary Female patient, 13 years old. Three months after birth, the left hand was found to be larger than the right hand. The palmar lateral stripes of the interphalangeal joints of the left thumb, the food, the middle finger, and the ring finger have soy-sized soft tissue induration. Obvious deformities of the above-mentioned fingers gradually appear when 5 to 6 years old. At the age of 12 years, except for the normality of the little finger, the left hand was thick and long, and the interphalangeal joint showed a segmental spindle-shaped thickening, and the distal segment was upturned. The left foot (foot mother) toe and right index finger also changed. There is no similar disease in the family. Parents are not married to a close relative. Inspection: Poor development, shorter than the same age children, pale complexion, walking limping. A large mass can be reached in the left upper abdomen, 8 cm below the costal margin, rounded (?), hard texture, and no tenderness. With the addition of the little finger, the left hand was 4 to 5 times thicker than normal, 3 to 4 cm long, and the longest was about 11.5 cm.