Redondo Pedro, Bastarrika Gorka, Aguado Leyre, Martínez-Cuesta Antonio, Sierra Alejandro, Cabrera Juan, Alonso-Burgos Alberto
Department of Dermatology, Unit of Vascular Malformations, University Clinic of Navarra, Pamplona, Spain.
J Am Acad Dermatol. 2009 Oct;61(4):621-8. doi: 10.1016/j.jaad.2009.04.027. Epub 2009 Jul 3.
Klippel-Trénaunay syndrome (KTS) is a capillary-lymphatic-venous malformation associated with soft tissue and skeletal hypertrophy of one or more limbs. Deep venous system (DVS) anomalies are reported to be present in 8% to 18% of patients with KTS; approximately 25% of patients with KTS have hand or foot malformations.
We sought to assess whether the presence of hand or foot malformations in KTS is a predictor of DVS anomalies.
Retrospective data were collected from 51 consecutive patients with KTS seen in a university hospital between January 2000 and February 2008. Patients with possible Proteus syndrome were not included. The presence and patency of the DVS was studied using conventional venography, multidetector computed tomography, or fast 3-dimensional magnetic resonance imaging venography.
Seventeen hand or foot malformations were present in 9 patients, consisting of: toe macrodactyly in 5 patients (two bilateral and one with plantar expansion); toe microdactyly in one patient; finger macrodactyly in one patient; finger macrodactyly and ectrodactyly in one patient; syndactyly in 4 patients; and clinodactyly with camptodactyly of the hand of one patient with lower limb KTS. Eleven patients had DVS anomalies (one with aplasia of entire DVS; one with duplication of the superficial femoral vein; 7 with hypoplasia of femoral vein; and 7 with aplasia of the popliteal vein). All patients with hand or foot malformations also had DVS anomalies (P < .001).
Small sample size was a limitation.
The presence of hand or foot malformations in KTS may predict the presence of DVS anomalies.
克-特综合征（KTS）是一种与一个或多个肢体的软组织和骨骼肥大相关的毛细血管-淋巴管-静脉畸形。据报道，8%至18%的KTS患者存在深静脉系统（DVS）异常；约25%的KTS患者有手部或足部畸形。
我们试图评估KTS患者手部或足部畸形的存在是否是DVS异常的预测指标。
收集了2000年1月至2008年2月在一家大学医院就诊的51例连续KTS患者的回顾性数据。排除可能患有变形综合征的患者。使用传统静脉造影、多排螺旋计算机断层扫描或快速三维磁共振成像静脉造影研究DVS的存在和通畅情况。
9例患者存在17处手部或足部畸形，包括：5例患者有趾巨趾症（2例双侧，1例伴有足底扩张）；1例患者有趾小趾症；1例患者有指巨趾症；1例患者有指巨趾症并缺指畸形；4例患者有并指畸形；1例下肢KTS患者有手部的小指畸形并屈曲挛缩。11例患者存在DVS异常（1例整个DVS发育不全；1例股浅静脉重复；7例股静脉发育不全；7例腘静脉发育不全）。所有有手部或足部畸形的患者也都有DVS异常（P <.001）。
样本量小是一个局限性。
KTS患者手部或足部畸形的存在可能预示着DVS异常的存在。
