Surgical Management of Fibroadipose Vascular Anomaly of the Lower Extremities
Surgical Management of Fibroadipose Vascular Anomaly of the Lower Extremities
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DOI：
10.1097/bpo.0000000000001406
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发表时间：
2020-03-01
影响因子：
1.7
通讯作者：
Spencer, Samantha A.
中科院分区：
文献类型：
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作者：
Wang, Kemble K.;Glenn, Rachel L.;Spencer, Samantha A.
背景：纤维脂肪血管畸形是一种新近发现的血管畸形，常累及四肢，多见于儿童时期。患者可能会因疼痛、肿胀、关节痉挛和腿长不等而去看骨科医生。目前还没有既定的治疗方法或治疗模式。我们报告了这种情况下手术切除后的结果。方法：2007-2016年间，采用病历、放射学发现和远程医疗回顾相结合的方法，对所有35名接受了下肢动脉切除的患者进行了回顾性研究。结果：首次发病的平均年龄为12.3±6.8岁。在我们机构确诊后的平均随访时间为66个月(范围：12至161个月)。术后平均随访35个月(6~138个月)。女性比男性受影响更大(71%比29%)。蚕豆最常见的部位是小腿(49%)，其次是大腿(40%)。最常见的受累肌肉是腓肠肌(29%)，其次是股四头肌(26%)。在手术后的最近一次随访中，患者在静息疼痛(63%比29%)、活动疼痛(100%比60%)以及止痛使用(94%比37%)方面都有改善。14名患者(40%)有症状残留疾病或复发，需要进一步治疗。6名患者(17%)需要进一步手术，6名患者(17%)需要进一步介入放射治疗。3名患者(9%)因顽固性疼痛和功能丧失而最终需要截肢。有直接神经受累的病变与最近一次随访时持续的神经病症状有关(P=0.002.0 1)，以及有症状的残留病变和/或需要进一步治疗的复发(P=0.0 1)。17例患者(49%)术前有19个关节痉挛。19个肌挛缩中有18个(95%)在最近一次随访中持续改善。结论：在精挑细选的患者中，手术切除蚕豆可改善症状。然而，有症状的残留病和/或复发并不少见。直接神经参与与更糟糕的结果相关。
Background: Fibroadipose vascular anomaly (FAVA) is a recently-defined vascular malformation often involving the extremities and presenting in childhood. Patients may present to orthopaedic surgeons with pain, swelling, joint contractures, and leg length discrepancy. There is no established therapy or treatment paradigm. We report on outcomes following surgical excision for patients with this condition. Methods: Between 2007 and 2016, all 35 patients that underwent excision of lower-extremity FAVA were retrospectively reviewed using a combination of medical records, radiologic findings, and telemedicine reviews. Results: Mean age at initial presentation was 12.3 +/- 6.8 years. Mean follow-up from time of definitive diagnosis at our institution was 66 months (range: 12 to 161 mo). Mean follow-up after surgery was 35 months (range: 6 to 138 mo). Females were affected more than males (71% vs. 29%). The most common location of FAVA was in the calf (49%), followed by the thigh (40%). The most commonly involved muscle was gastrocnemius (29%), followed by the quadriceps (26%). At latest follow-up after surgery, there was an improvement in the proportion of patients with pain at rest (63% vs. 29%), pain with activity (100% vs. 60%), as well as analgesia use (94% vs. 37%). Fourteen patients (40%) had symptomatic residual disease or recurrence of FAVA requiring further treatment. Six patients (17%) required further surgery and 6 (17%) required further interventional radiologic procedures. Three patients (9%) required eventual amputation for intractable pain and loss of function. Lesions with direct nerve involvement were associated with persistent neuropathic symptoms at latest follow-up (P=0.002) as well as symptomatic residual disease and/or recurrence requiring further treatment (P=0.01). Seventeen patients (49%) had 19 preoperative joint contractures. Eighteen of the 19 contractures (95%) had sustained improvement at latest follow-up. Conclusions: In carefully selected patients, surgical excision of FAVA results in improvement of symptoms. However, symptomatic residual disease and/or recurrence are not uncommon. Direct nerve involvement is associated with a worse outcome.