Table 1 Staging of FAVA
From: Fibro-adipose vascular anomaly (FAVA) - diagnosis, staging and management
Stage | Clinical presentation | Treatment principle |
|---|---|---|
I (Pain stage) | A mass with persistent and severe pain, without significant contracture | Radical resection |
II (Contracture stage) | A mass with pain; Significant contracture with limited joint mobility | Radical resection Possible tendon lengthening and/or transfer Possible neurolysis Stretching exercises |
III (Deformity stage) | A mass with pain; Joint deformity with ankylosis and possible limb length discrepancy | Radical resection if possible Tendon lengthening and/or transfer, tenotomy Neurolysis Joint capsulotomies Oral sirolimus/alpelisib Stretching exercises Possible osteotomy lengthening |