275 / 280
mitotically quiescent. VaMs are subclassifed based on
fl
ow
dynamics, as slow-
fl
ow, and fast-
fl
ow VaMs.
30
Slow-
fl
ow vascular malformations
Venous malformation
Venous malformations (VMs) are the most common of all
types of VaM. They present as soft, compressible lesions
that typically in
fi
ltrate multiple tissue planes. Physical ex-
amination generally reveals bluish lesions (
Figs 1
and
6
)
that may enlarge with Valsalva manoeuvre or gravity. There
may be overlying skin involvement. They usually present
during mid to late childhood and become more symptom-
atic as time passes. The lesions vary in size from very small
to extensive involving multiple body parts. The can appear
as sacs
fi
lled with venous blood or as dilated venous
channels with or without communication of systemic veins.
Generally, even when large, VMs tend to be continuous in
nature. They tend to extend within the muscle groups of
extremities, along the nerves and major arteries or veins.
Table 4
Syndromes associated with vascular malformations (VMs).
Syndromes associated with VM
Klippel
e
Trenaunay
Blue rubber bleb nevus
Maffucci syndrome
Syndromes associated with CM
Klippel
e
Trenaunay
Sturge
e
Weber
Syndromes associated with LM
Gorham syndrome
Syndromes associated with AVM
Parkes
e
Weber
Rendu
e
Osler
e
Weber
Bannayan
e
Riley
e
Ruvalcaba syndrome
VM, Venous malformation; LM, Lymphatic malformation; CM, Capillary
malformation; AVM, Arteriovenous malformation.
Figure 12
(a) A 7-year-old female patient with multiple dark, slightly raised,
fi
rm skin lesions on both knees, and over entire body as well. These
are the skin lesions of BRBNS. She has multiple deep VMs on the shoulder and right arm that have been previously percutaneously sclerosed. She
recently reported severe pain around both knees and thighs that prompted MRI. (b
e
c) Coronal T2-weighted images demonstrate multiple small,
lobular T2-bright lesions in the muscle groups and medulla of the bones representing VMs. (d) Ultrasound showing intramuscular VM with
needle accessing it for percutaneous sclerotherapy. (e) Percutaneous venogram of right leg intramuscular VM demonstrating type II drainage
into normal veins (white arrow). (f) Percutaneous venogram of left leg intramuscular VM and infrapatellar VM. Note again the type II VM with
drainage into normal veins (white arrow), and additional type I VM without a draining vein (black arrow).
A. Tekes et al. / Clinical Radiology 69 (2014) 443
e
457
253