Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice.
Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice.
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DOI：
10.1016/j.jpra.2021.05.002
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发表时间：
2021-09
期刊：
影响因子：
1.4
通讯作者：
Sivakumar B
中科院分区：
文献类型：
--
作者：
Lipede C;Nikkhah D;Ashton R;Murphy G;Barnacle AM;Patel PA;Smith GD;Eastwood DM;Sivakumar B
纤维脂肪血管异常（FAVA）是一种离散类型的血管异常。我们描述了我们在三级儿科医院管理 FAVA 的经验，并提供了治疗算法。对 27 名经证实患有 FAVA 的患者进行了回顾性审查。所有患者均接受了 MRI 和 USS 评估。记录患者人口统计数据、提出的问题、治疗方法和结果，并在最终治疗后至少 12 个月内使用儿科结果数据收集工具 (PODCI) 完成评估。就诊时的平均年龄为 8.9 岁（范围：9 m-17.4 岁），治疗后平均随访时间为 7.4 岁（范围：2 岁-11.6 岁）。 27 个病变中有 20 个影响下肢。 23 例出现严重神经源性疼痛，11 例出现关节挛缩。 11例采用十四烷基硫酸钠硬化疗法，症状无改善。冷冻消融术缓解了 3/4 例患者的疼痛，但随后一名患者的挛缩加剧，另一名患者的疼痛再次出现。 14 例接受了手术（4 例单独手术切除，10 例联合其他手术）。三名患者需要接受四次进一步的外科手术，其中包括一次因顽固性疼痛和功能不良而截肢。 PODCI 评估表明总体功能良好，手术治疗和介入放射学可提供可比较的结果。手术确实矫正了畸形。如果保守措施或冷冻消融无法实现症状控制，应考虑手术切除，结合辅助手术，以纠正挛缩和平衡肌肉力量。疼痛的缓解可以补偿肌肉质量的损失并整体改善功能。多学科团队合作至关重要。
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.
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