Dalmonte Pietro, Granata Claudio, Fulcheri Ezio, Vercellino Nadia, Gregorio Sandro, Magnano Gianmichele
Cardiovascular Department, Giannina Gaslini Children's Hospital, Genova, Italy.
J Pediatr Orthop. 2012 Jun;32(4):394-8. doi: 10.1097/BPO.0b013e31824b29ef.
Intra-articular venous malformations (IAVM) of the knee represent a rare group of low-flow vascular malformations, mainly reported in the literature as synovial hemangiomas, usually with an onset in early childhood. The main symptoms and signs are knee pain, swelling, and hemarthrosis. These lesions are slowly progressive and can lead to chronic synovitis and joint damage. Confusing nomenclature and classification of these lesions have often led to misdiagnosis and inappropriate treatment. We report our experience in the treatment of 14 consecutive patients affected by knee IAVM.
We carried out a retrospective study based on the review of the medical records and diagnostic imaging of the patients admitted to our department from October 1999 to June 2009, and discharged with the final diagnosis of IAVM of the knee.
We observed 14 consecutive patients (8 boys, 6 girls); the median age at symptom onset was 5 years (range, 2.5 to 13 y). Magnetic resonance imaging was diagnostic in all cases. Surgical resection associated with a wide synoviectomy was the therapeutic procedure of choice in all cases. Within 6 months after surgery and physiotherapy, all the patients were symptom free with a full or at least acceptable knee range of motion. CD34 staining carried out in the last 7 cases of our series showed labeling of the endothelium of the thick-walled vessels, whereas the endothelium of the thin-walled vascular spaces was not stained. This finding of unstained vascular spaces suggests that a component of lymphatic vessels mixed with dysplastic blood vessels may be frequently present in these abnormalities.
Magnetic resonance imaging plays a pivotal role in identifying the lesion. Surgical excision is always indicated and should be performed as early as possible to avoid lesion progression and to reduce the risk of chondral degeneration. According to the microscopic features of our resected specimens, we suggest that these lesions of the knee should be more properly named as IAVM instead of hemangiomas.
Level IV.
膝关节内静脉畸形（IAVM）是一种罕见的低流量血管畸形，在文献中主要报道为滑膜血管瘤，通常在儿童早期发病。主要症状和体征为膝关节疼痛、肿胀和关节积血。这些病变进展缓慢，可导致慢性滑膜炎和关节损伤。这些病变令人困惑的命名和分类常常导致误诊和不恰当的治疗。我们报告了连续治疗14例膝关节IAVM患者的经验。
我们进行了一项回顾性研究，基于对1999年10月至2009年6月在我科住院并最终诊断为膝关节IAVM的患者的病历和诊断影像进行回顾。
我们观察了连续14例患者（8例男孩，6例女孩）；症状出现的中位年龄为5岁（范围2.5至13岁）。所有病例磁共振成像均具有诊断价值。手术切除联合广泛滑膜切除术是所有病例的首选治疗方法。在手术后6个月内及进行物理治疗后，所有患者均无症状，膝关节活动范围完全或至少可接受。在我们系列的最后7例病例中进行的CD34染色显示厚壁血管内皮有标记，而薄壁血管腔的内皮未染色。血管腔未染色这一发现表明，这些异常中可能经常存在淋巴管成分与发育异常的血管混合的情况。
磁共振成像在识别病变方面起关键作用。总是需要进行手术切除，并且应尽早进行，以避免病变进展并降低软骨退变的风险。根据我们切除标本的微观特征，我们建议将这些膝关节病变更恰当地命名为IAVM而非血管瘤。
四级
