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example, midline posterior CMs may be associated with
tethered spinal cord. Facial CMs may be associated with
Sturge
e
Weber syndrome, particularly in the V1 distribution
(
Fig 8
). Patients with V1 distribution CMs should undergo
early neurological and ophthalmological evaluation. Pa-
tients with V2 and V3 involvement are generally not at risk.
Other conditions associated with CMs include Klip-
pel
e
Trenaunay (KT), Parkes
e
Weber syndrome. CMs may be
associated with underlying arteriovenous malformations
(AVMs) as part of the RASA1 mutation.
20
CMs associated with Sturge
e
Weber have a tendency to
become thickened and lobulate with age. Early intervention
with pulsed dye laser to the CM may prevent progression
towards more nodular growth.
35,36
These are very dif
fi
cult
to treat once hypertrophy has occurred and may require
dif
fi
cult and repeated plastic surgical procedures to keep
the enlargement under control. Angiography rarely
demonstrates enough visible hypervascularity to render
embolization an alternative to controlling this growth. In
addition, there may be bony overgrowth that cannot be
controlled. MRI demonstrates the super
fi
cial thickening.
37
Fast-
fl
ow malformations
Arteriovenous malformation
AVMs and arteriovenous
fi
stulas are pulsatile lesions
without a mass and without the capillary transition be-
tween artery and vein, typically with associated bruit or
murmur (
Figs 1
and
9
). They present in early childhood
and grow with the child. They may also undergo periods of
more rapid growth, associated with growth spurts and
puberty as well as occurring after trauma, pregnancy, or
surgery. They can be complicated by arterial steal in
affected extremities. Venous congestion from AVMs can
Figure 14
(a) A 20-year-old man with bluish lesion in left lower
fl
ank since birth. Similar lesions were also noted on the buttocks, right and left
thighs, left wrist, and right forearm (not shown). These were painful when pushed on. His father had similar lesion on his left forearm. Note they
are cutaneous and subcutaneous, raised lesions with some
fi
rmness, yet compressible. (b) Coronal T2 weighted image shows lobular T2-bright
lesion in the subcutaneous fat. (c) Ultrasound during needle access for sclerotherapy. Lesion had
fi
rm borders but extensive venous spaces. No
fl
ow seen on power Doppler (not shown). (d) Percutaneous venogram of lesion during sclerotherapy treatment. Based on MRI only, diagnosis of
glomuvenous malformation is very dif
fi
cult as imaging features overlap with that of a VM. Presence of similar lesions in the patient
’
s father along
with super
fi
cial location and painful nature are very helpful in establishing the diagnosis of a glomuvenous malformation.
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