Wang Huaijie,Xie Chong,Lin Weilong,et al.Diagnosis and treatment of fibro-adipose vascular anomaly(FAVA)[J].Journal of Clinical Pediatric Surgery,,21():668-674.[doi:10.3760/cma.j.cn101785-202102022-014]
Diagnosis and treatment of fibro-adipose vascular anomaly(FAVA)
- Keywords:
- FAVA; Fibro-Adipose Vascular Anomaly; Hemangioma; Venous Malformation; Contracture; Joint Deformity
- Abstract:
- Objective To explore the diagnostic essentials and therapeutic principle of fibro-adipose vascular anomaly(FAVA).Methods Clinical data were retrospectively reviewed for 13 FAVA children.And the relevant data included gender, age at diagnosis, clinical features, anatomic involvement, previous diagnosis and management, radiological features, management, histopathological findings, rehabilitation and follow-up plan.Results From September 2019 to December 2020, a total of 13 cases of FAVA were diagnosed and treated in our center. Early diagnosis included:venous malformation (n=8), intramyaloid hemangioma (n=2), muscular lymphatic malformation (n=1), arteriovenous malformation (n=1) and cavernous hemangioma (n=1). There were 4 males and 9 females; the age at diagnosis ranged from 1 year and 2 months to 18 years old, with an average age of 10 years. 11 cases of lower extremity lesions, 2 cases of upper extremity. None of the cases involved the trunk. 5 cases were accompanied by hypersensitivity of the skin at the swollen part, and the pain was severe when lightly pressed. 11 cases were associated with reduced social activities, and the patients had a strong sense of inferiority and reduced social interaction with their peers. One case of upper limb patient was relieved of pain after oral administration of sirolimus; the remaining 12 cases underwent surgical treatment, and all of them underwent radical resection, that is, under the premise of protecting important blood vessels and nerves, enlarged resection of localized lesions; diffuse lesions resection of involved lesions The entire muscle and myofascia are affected; if important nerves are involved, incision and release of the epineurium are required; if tendon contracture causes joint mobility impairment, after removal of the entire affected muscle and myofascia, tendon lengthening/transfer is required to Reconstruction function; 12 cases were indwelling wound negative pressure drainage; tendon lengthening/transfer, joint ankylosing correction, plaster immobilization for 1 to 4 weeks. The postoperative follow-up was 1-16 months, and the joint mobility disorder was cured or improved significantly.Conclusion The most distinct features of FAVA include pain, contracture and joint deformity.Based upon unique clinical and radiologic features, it is not difficult to make a definite diagnosis of FAVA.The management strategy for FAVA should be formulated according to staging.With appropriate surgical approaches surgery-based management plan is efficacious for relief of pain, contractures and function improvement.
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Memo
收稿日期:2021-02-16。
基金项目:西安国际医学中心医院院级课题（2021QN016）
通讯作者:郭正团,Email:guozhengtuan@hotmail.com