Experience of diagnosis and treatment of fibro-adipose vascular anomaly
10.3760/cma.j.cn114453-20230406-00073
- VernacularTitle:纤维脂肪性血管性病变的诊断与治疗经验
- Author:
Chong XIE
1
;
Huaijie WANG
;
Zhengtuan GUO
;
Weilong LIN
;
Jinbang ZHOU
;
Weijia YANG
Author Information
1. 西安国际医学中心医院小儿外科与血管瘤科，西安 710000
- Keywords:
Diagnosis;
Treatment;
Fibro-adipose vascular anomaly;
Magnetic resonance imaging;
Surgical procedures, operative
- From:
Chinese Journal of Plastic Surgery
2023;39(11):1175-1182
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the diagnosis, staging, and treatment of fibro-adipose vascular anomaly(FAVA).Methods:The data of the patients with FAVA admitted to Xi’an International Medical Center Hospital between October 2019 and February 2023 were retrospectively reviewed. Ultrasound and magnetic resonance imaging (MRI) were routinely performed. X-ray and CT were performed for patients with unequal length of lower limbs, lesions involving joints, and obvious joint deformities. The treatment plan was made according to the stage: stage Ⅰ (pain stage), open or laparoscopi radical resection of the lesion was performed; stage Ⅱ (contracture stage), radical surgery was performed to remove the lesion, sometimes combined with Achilles tendon lengthening or tenolysis, and rehabilitation training was performed 2 weeks after surgery; stage Ⅲ(deformity stage), comprehensive treatment based on surgical resection was adopted, combined with joint capsule release, Achilles tendon lengthening or dissection, tendon transfer and oral sirolimus (each time 0.08 mg/m 2, twice daily ) before and after the operation. For patients with lesions involving multiple anatomical regions, staged surgery was performed, and in principle, only one anatomical region was operated per time. Patients were followed up for pain, joint activity and recurrence. Results:A total of 42 patients were admitted, including 18 males and 24 females. The onset age was (7.3±5.0) years, but the average age of diagnosis was (12.5±6.0) years. The lower limbs were involved in 38 cases, the upper limbs in 4 cases. There were 17 cases of stage Ⅰ, 17 cases of stage Ⅱ and 8 cases of stage Ⅲ. Only 4 cases had no misdiagnosis experience, and the misdiagnosis rate was 90.5%(38/42). Persistent pain, muscle contractures and joint deformities were the main clinical symptoms of the disease. MRI showed heterogeneous high and low signal intensity on T1-weighted images, and the high signal intensity was the same as that of subcutaneous adipose tissue. T2 fat-suppressed sequences showed stronger heterogeneous hyperintensity. The follow-up time was (14.6±10.8) months. Patients who took sirolimus orally before or after surgery experienced significant relief of pain symptoms. Of the 42 patients, the symptoms of 31 patients were completely relieved after the operation, and 11 patients still had residual pain or joint movement disorder or even deformity after the operation. Sixteen of 17 stage Ⅰ patients were cured, the lesion was further expanded and the pain recurred in 1 case after the operation. Of the 17 stage Ⅱ patients, 15 were cured, and 2 had mild limitation of ankle movement after the operation. Eight stage Ⅲ patients had varying degrees of pain or joint movement disorder after surgery, postoperative oral sirolimus significantly relieved symptoms. All 10 patients with stage Ⅰ and Ⅱ who underwent endoscopic resection were cured.Conclusion:FAVA usually occurred in school-age and adolescent children. Pain, muscle contracture and joint deformity were the characteristic clinical features. Combined with MRI features, the diagnosis can be confirmed. FAVA staging system could guide treatment and predict prognosis. For stage Ⅰ and Ⅱ patients, surgery should be performed as soon as possible and the prognosis was good. For stage Ⅲ patients, surgery-based comprehensive treatment could improve symptoms, but it was difficult to cure them radically. Oral sirolimus was also required after surgery.
