下肢先天性静脉畸形骨肥大综合征的诊断及外科治疗

Diagnosis and Surgical Treatment of the Klippel-Trenaunay Syndrome

下肢先天性静脉畸形骨肥大综合征（KTS）11例，8例经手术治疗获得较好的临床效果，3例由于深静脉缺如或发育不良仅作局部探查手术，采取保守疗法，其效果不佳。KTS的主要临床表现为患肢骨和软组织增生（增粗11例，增长4例）、浅静脉曲张（10例）和血管瘤（11例）。深静脉顺行性造影显示深静脉不通8例、缺如2例、扩张1例，深静脉逆行性造影显示深静脉瓣膜功能不全和正常各2例。作者认为，只有在深静脉高压得到缓解后，才能施行曲张浅静脉的结扎、剥脱术。

Eleven patients with the Klippel-Trenaunay syndrome (KTS) have been sur gically reported. Good clinical results have been achieved in eight cases after operation. The results were poor in three cases because of the absence or hypoplasia of the deep veins. Main clinical findings of KTS were hypertrophy of the soft tissues and bones (enlargement in 11 cases, lengthening in 4 cases), varicosity (10 cases) and hemangioma (11 cases) of the af fected limbs. Ascending venography showed deep venous atresia in eight cases, absence of deep veins in two cases and dilatation in one case. Descending venography revealed valvularincompetence and competence of the deep veins in two cases respectively. In our opinion, it is not until the deep venous hypertension is relieved that the ligation and stripping of varicosities should be Derformed.