Enjolras Odile, Chapot René, Merland Jean Jacques
Vascular Anomalies clinics, Paris, France.
J Pediatr Orthop B. 2004 Nov;13(6):349-57. doi: 10.1097/01202412-200411000-00001.
Growth of the limb in a child can be impaired, with the coexistence of a vascular malformation. In these vascular bone syndromes, altered growth is manifest as overgrowth or hypotrophy. The vascular malformation is usually complex and gets progressively worse with time. The two types of vascular anomalies in limbs, fast-flow and slow-flow, can be associated with limb length discrepancies. The fast-flow vascular malformations together with arteriovenous fistulae are part of Parkes Weber syndrome, characterized by congenital red cutaneous staining, hypertrophy in girth and increasing of limb length, lymphedema, increasing skin alterations due to a distal vascular steal, and pain, all of which develop during childhood. Treatment is generally conservative. An affected lower extremity can be complicated by pelvic tilting and scoliosis because leg length discrepancy may reach 10 cm. To avoid such a course, stapling epiphysiodesis of the knee cartilages is often performed, but this orthopedic procedure may augment the worsening of the arterial venous malformation in the limb. Therefore, less aggressive orthopedic management is preferable. Slow-flow vascular anomalies associated with limb growth alteration include (1) a diffuse capillary malformation (port-wine stain) with congenital hypertrophy of the involved extremity which is non-progressive; (2) purely venous malformations invading skin, muscles and joints, with pain, functional impairment, a chronic localized intravascular coagulopathy requiring distinctive management, and usually a slight undergrowth of the affected extremity and progressing amyotrophy; (3) the triad of a port-wine stain, anomalous veins and overgrowth of the limb, often known as Klippel-Trenaunay syndrome, which requires orthopedic management to decide the optimal timing for epiphysiodesis (i.e. when leg length discrepancy is >2.5 cm). Varicose veins are sometimes surgically removed after ultrasonographic and Doppler evaluation has confirmed a normal deep venous system. Capillary malformations can be effectively treated with pulsed dye laser, but results are usually poor in distal extremities.
儿童肢体生长可能会受到损害，并伴有血管畸形。在这些血管性骨综合征中，生长改变表现为过度生长或发育不全。血管畸形通常较为复杂，且会随时间逐渐加重。肢体的两种血管异常，即高流量和低流量异常，可能与肢体长度差异有关。高流量血管畸形与动静脉瘘一起，是帕克思·韦伯综合征的一部分，其特征为先天性红色皮肤染色、周径增粗和肢体长度增加、淋巴水肿、由于远端血管盗血导致的皮肤改变加重以及疼痛，所有这些症状均在儿童期出现。治疗通常采用保守方法。由于下肢长度差异可能达到10厘米，患侧下肢可能会并发骨盆倾斜和脊柱侧弯。为避免这种情况，常对膝关节软骨进行骨骺固定术，但这种骨科手术可能会加剧肢体动静脉畸形的恶化。因此，采用不太激进的骨科治疗方法更为可取。与肢体生长改变相关的低流量血管异常包括：（1）弥漫性毛细血管畸形（葡萄酒色斑），伴有受累肢体先天性肥大，且病情无进展；（2）单纯性静脉畸形侵犯皮肤、肌肉和关节，伴有疼痛、功能障碍、一种需要特殊处理的慢性局部血管内凝血病，且通常患侧肢体轻度发育不全并伴有进行性肌萎缩；（3）葡萄酒色斑、异常静脉和肢体过度生长三联征，通常称为克-特综合征，需要进行骨科治疗以确定骨骺固定术的最佳时机（即当下肢长度差异>2.5厘米时）。在超声和多普勒评估证实深静脉系统正常后，有时会手术切除静脉曲张。毛细血管畸形可用脉冲染料激光有效治疗，但在肢体远端效果通常较差。
