Lipede C, Nikkhah D, Ashton R, Murphy G, Barnacle A M, Patel P A, Smith G D, Eastwood D M, Sivakumar B
Department of Plastic Surgery, Great Ormond Street Hospital for Children, London UK.
Department of Radiology, Great Ormond Street Hospital for Children, London UK.
JPRAS Open. 2021 May 19;29:71-81. doi: 10.1016/j.jpra.2021.05.002. eCollection 2021 Sep.
Fibro-adipose vascular anomaly (FAVA) is a discrete type of vascular anomaly. We describe our experience managing FAVA at a tertiary level paediatric hospital and offer a treatment algorithm.
A retrospective review of 27 patients with proven FAVA was undertaken. All patients had undergone MRI and USS evaluation. Patient demographics, presenting concerns, treatment methods, and outcomes were recorded and evaluation with the paediatric outcomes data collection instrument (PODCI) completed a minimum of 12 months after definitive treatment.
Mean age at presentation was 8.9 years (range: 9 m-17.4 y) and mean post-treatment follow-up was 7.4 y (range: 2 y-11.6 y). Twenty of 27 lesions affected the lower limb. Severe neurogenic-type pain was present in 23 cases and contractures across joints in 11 cases. Sclerotherapy with sodium tetradecyl sulphate was used in 11 cases, with no improvement in symptoms. Cryoablation provided pain relief in 3/4 cases, but contracture subsequently increased in one patient and pain recurred in another.Fourteen cases underwent surgery (four surgical excisions alone, 10 in combination with other procedures). Three patients required four further surgical procedures that include one amputation for intractable pain and poor function.PODCI evaluations suggest overall good function, with surgical management and interventional radiology that provide comparable results. Surgery did correct deformity.
If conservative measures or cryoablation fail to achieve symptomatic control, surgical excision should be considered, combined with adjunctive procedures, to correct contractures and balance muscle forces.Relief of pain may compensate for the loss of muscle mass and overall improves function. Multidisciplinary team working is essential.
纤维脂肪血管异常（FAVA）是一种离散型血管异常。我们描述了在一家三级儿科医院处理FAVA的经验，并提供了一种治疗方案。
对27例经证实的FAVA患者进行回顾性研究。所有患者均接受了MRI和超声检查。记录患者的人口统计学资料、就诊时的问题、治疗方法和结果，并在最终治疗后至少12个月使用儿科结局数据收集工具（PODCI）进行评估。
就诊时的平均年龄为8.9岁（范围：9个月至17.4岁），治疗后的平均随访时间为7.4年（范围：2年至11.6年）。27个病变中有20个累及下肢。23例出现严重的神经源性疼痛，11例出现关节挛缩。11例使用十四烷基硫酸钠硬化治疗，症状无改善。冷冻消融在4例中有3例缓解了疼痛，但1例患者随后挛缩加重，另1例疼痛复发。14例接受了手术（4例单纯手术切除，10例联合其他手术）。3例患者需要进一步进行4次手术，其中1例因顽固性疼痛和功能不佳而截肢。PODCI评估显示总体功能良好，手术治疗和介入放射学提供了可比的结果。手术确实纠正了畸形。
如果保守措施或冷冻消融未能实现症状控制，应考虑手术切除，并联合辅助手术，以纠正挛缩和平衡肌肉力量。疼痛缓解可能弥补肌肉量的损失并总体改善功能。多学科团队协作至关重要。
