Table 2 Clinical characteristics of FAVA cohort
From: Fibro-adipose vascular anomaly (FAVA) - diagnosis, staging and management
No | Gender | Age At Onset (y) | Age At Referral (y) | Symptoms | Locations | Affected Muscles | Interventions Before Referral | Stage | Interventions At Authors’ Institution | Clinical Change After Intervention(s) |
|---|---|---|---|---|---|---|---|---|---|---|
1 | M | 1 | 11 | Mass and severe pain Loss of ankle plantar flexion Muscle atrophy | Right leg and foot | Extensor digitorum longus Extensor hallucis longus | Sclerotherapy | III | Radical resection | No pain Loss of ankle plantar flexion Improving atrophy |
2 | F | 3 | 11 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Left leg | Soleus | Partial resection Sclerotherapy | II | Radical resection Stretching exercises | No pain Near normal range of ankle motion Improving atrophy |
3 | F | 7 | 13 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Right leg | Lateral gastrocnemius Soleus | Sclerotherapy Endovascular embolization | II | Radical resection | No pain Near normal range of ankle motion Improving atrophy |
4 | F | 1 | 9 | Mass and severe pain Contracture of knee Muscle atrophy | Left thigh | Biceps femoris Vastus lateralis Vastus intermedius | Sclerotherapy Oral sirolimus | III | Radical resection Relaxation of the knee Stretching exercises | No pain Near normal range of knee motion Re-walking Improving atrophy |
5 | F | 2 | 9 | Mass and severe pain Loss of ankle dorsiflexion Muscle atrophy | Left leg | Tibialis posterior Flexor hallucis longus Flexor digitorum longus Soleus | Sclerotherapy Oral sirolimus | II | Radical resection Achilles tendon lengthening Relaxation of ankle capsule Stretching exercises | No pain Normal range of ankle motion Improving atrophy |
6 | F | 3 | 15 | Mass and severe pain Loss of ankle dorsiflexion Pes cavus Contracture of knee Muscle atrophy | Right thigh, leg and foot | Soleus Gastrocnemius Biceps femoris Flexor digitorum longus Flexor digitorum brevis | Sclerotherapy Oral sirolimus | III | Staged resection Oral alpelisib | Mild pain Increased range of ankle motion Improving atrophy |
7 | M | 8 | 9 | Mass and pain | Left leg | Fascia muscularis | None | I | Radical resection | Normal |
8 | F | 15 | 17 | Mass and severe pain Limited forearm pronation | Left forearm | Supinator | Sclerotherapy Oral sirolimus Partial resection | II | Radical resection | No pain Loss of elbow motion after sclerotherapy |
9 | F | 3 mo | 1 | Mass and severe pain | Left leg | Medial gastrocnemius Soleus | None | I | Radical resection | Normal |
10 | M | 12 | 18 | Mass and pain Contracture of wrist | Right forearm | Extensor carpi ulnaris | Sclerotherapy | III | Radical resection | Normal |
11 | F | 5 | 10 | Mass and severe pain Limited plantar flexion of ankle | Left leg | Tibialis anterior Extensor digitorum longus | Sclerotherapy Oral sirolimus | II | Radical resection Tendon transfer | Normal |
12 | M | 8 | 13 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Right leg | Soleus | Sclerotherapy Oral sirolimus | II | Radical resection Achilles tendon lengthening | No pain Near normal range of ankle motion Improving atrophy |
13 | F | 7 | 14 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Right leg | Lateral gastrocnemius | Sclerotherapy Endovascular embolization | II | Radical resection Achilles tendon lengthening | No pain Near normal range of ankle motion Improving atrophy |
14 | F | 9 | 9 | Mass and severe pain Limited ankle dorsiflexion | Left leg | Lateral gastrocnemius | Sclerotherapy | II | Radical resection | Normal |
15 | M | 18 | 23 | Mass and severe pain | Right thigh | Rectus femoris | Sclerotherapy | I | Radical resection | Normal |
16 | F | 17 | 18 | Mass and severe pain Limited plantar flexion of ankle | Right leg | Extensor digitorum longus | Sclerotherapy | II | Radical resection | Normal |
17 | F | 9 | 10 | Mass and severe pain Limited ankle dorsiflexion | Right leg | Medial gastrocnemius | Sclerotherapy | II | Radical resection (endoscopic) | Normal |
18 | M | 9 | 11 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Left leg | Medial gastrocnemius | Sclerotherapy | II | Radical resection Achilles tendon lengthening (endoscopic) | No pain Near normal range of ankle motion Improving atrophy |
19 | F | 11 | 20 | Mass and severe pain Limited ankle dorsiflexion | Left leg | Soleus | Sclerotherapy | II | Radical resection | Normal |
20 | F | 2 | 14 | Mass and severe pain Loss of ankle dorsiflexion Pes cavus Muscle atrophy | Right leg | Gastrocnemius Soleus | Sclerotherapy | III | Radical resection Achilles tenotomy Relaxation of ankle capsule Stretching exercises | No pain Loss of ankle plantar flexion Re-walking Improving atrophy |
21 | F | 5 | 11 | Mass and severe pain | Left thigh | Vastus medialis | Sclerotherapy | II | Radical resection | Normal |
22 | F | 17 | 22 | Mass and severe pain | Left thigh | Adductor magnus | Sclerotherapy | II | Radical resection | Normal |
23 | M | 20 | 33 | Mass and severe pain | Right thigh | Semimembranosus Semitendinosus | Sclerotherapy | II | Radical resection | Normal |
24 | F | 10 | 11 | Mass and severe pain Limited ankle dorsiflexion | Left leg | Soleus | None | II | Radical resection | Normal |
25 | F | 9 | 11 | Mass and severe pain Contracture of knee Limited ankle dorsiflexion Muscle atrophy | Right thigh and leg | Gracilis Saphenous fascia of leg Soleus | Sclerotherapy | II | Radical resection Stretching exercises | No pain Normal range of knee motion Near normal range of ankle motion Improving atrophy |
26 | F | 6 | 11 | Mass and severe pain Limited ankle dorsiflexion | Left leg | Lateral gastrocnemius | None | II | Radical resection (endoscopic) | No pain Near normal range of ankle motion |
27 | M | 7 | 11 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Left leg | Lateral gastrocnemius | Sclerotherapy | II | Radical resection (endoscopic) | No pain Near normal range of ankle motion |
28 | F | 1 | 4 | Mass and severe pain Limited ankle dorsiflexion Muscle atrophy | Left leg | Gastrocnemius | Sclerotherapy | II | Radical resection Achilles tendon lengthening Stretching exercises | No pain Near normal range of ankle motion Improving atrophy |
29 | M | 3 | 10 | Mass and severe pain | Right leg | Peronaeus longus | Sclerotherapy | I | Radical resection | Normal |
30 | F | 5 | 23 | Mass and severe pain Loss of ankle dorsiflexion Pes cavus Muscle atrophy | Right leg | Posterior muscle group | Sclerotherapy | III | Oral sirolimus Stretching exercises | Mild pain No change of the range of ankle motion Improving atrophy |
31 | F | 5 | 13 | Mass and severe pain Contracture of knee Loss of ankle dorsiflexion Muscle atrophy | Right thigh and leg | Biceps femoris Posterior muscle group of leg | Sclerotherapy | III | Oral sirolimus Stretching exercises | Mild pain Increased range of knee motion No change of the range of ankle motion |
32 | F | 8 | 16 | Mass and severe pain Loss of ankle dorsiflexion Pes cavus Muscle atrophy | Right leg | Posterior muscle group | Sclerotherapy Partial resection | III | Oral sirolimus Stretching exercises | Mild pain No change of the range of ankle motion Improving atrophy |