Table 1.
LM (n=17) |
CLOVES (n=33) |
KTS (n=21) |
FAVA (n=8) |
|
---|---|---|---|---|
Sex (M/F) | (11/6) | (18/15) | (13/18) | (3/5) |
Truncal involvement | 0 | 26 | 2 | 0 |
Limb involvement (LLD/macro/syn) |
4 (0,0,0) |
30 (17/22/6) |
21 (20/14/4) |
8 (0/0/0) |
Cutaneous CM and/or LM | 0 | 28 | 21 | 0 |
LM on imaging (head-neck/trunk/limb) [micro/macro/combination] |
17 (10/3/4) [1/8/8] |
33 (5/13/15) [4/19/10] |
21 (0/0/21) [1/11/9] |
4 |
VM on imaging | 0 | 30 | 20 | 8 |
Complications (INF/GIB/DVT/PVT/Wilms/death) |
(13/0/0/0/0/0) |
(12/7/6/4/1/2) |
(13/8/3/0/0/0) |
none |
Note, LM adjacent to fibro-adipose infiltrated muscle was a frequent histopathologic finding in FAVA. Number of participants with a VM detected by ultrasonography or MRI is indicated. VMs involved the marginal veins or large draining veins in CLOVES and KTS, and were adjacent to the fibro-adipose lesion in FAVA. Complications include infection at site of or originating from a vascular malformation (INF), gastro-intestinal bleed (GIB), deep vein thrombosis ± pulmonary embolism (DVT), portal vein thrombosis (PVT), Wilms tumor (Wilms), and death.