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involvement of the overlying skin and subcutaneous fat.
These more aggressive imaging features distinguish KHE
from IH, as do the atypical clinical features.
Syndromes associated with haemangiomas
Although the clinical course of the vast majority of
haemangiomas is benign, there are some associated ab-
normalities that should be noted and that may require
further diagnostic evaluation.
Patients with large
segmental facial haemangiomas should be evaluated for
signs and symptoms of PHACES syndrome. PHACES syn-
drome refers to a constellation of
p
osterior fossa brain
malformations,
h
aemangiomas,
a
rterial anomalies,
c
oarc-
tation of the aorta and cardiac defects,
e
ye abnormalities,
and
s
ternal defects.
27
Patients with haemangiomas overlying the lumbosacral
spine can have associated abnormalities, the most common
of which is a tethered spinal cord. MRI should be performed
to exclude this abnormality.
28
Genitourinary anomalies are
possible, although less common.
Airway haemangiomas should be investigated in patients
who have cutaneous cervicofacial haemangiomas distrib-
uted in the chin, anterior neck, lower lip, and pre-auricular
areas (a
“
beard
”
distribution).
29
Vascular malformations
VAs that are present at birth and grow slowly, propor-
tionally to the patient without spontaneous regression are
Figure 9
(a) A 30-year-old woman with a swollen pulsatile mass on the hypothenar eminence of her right hand. View of the dorsal surface of the
patient
’
s right hand compared to the left. Note the enlarged draining veins and relatively bigger size of the right hand. (b) Note the hypothenar
eminence mass on this image of the palmar surface of right hand. (c) Coronal T2-weighted image with fat saturation demonstrates serpiginous
tangle of
fl
ow voids indicating high
fl
ow, in
fi
ltration the hypothenar eminence and the subcutaneous fat. Note absence of associated soft-tissue
mass. (d) MR angiogram demonstrates strong enhancement of the AVM with arterial feeder from the ulnar artery and venous drainage into the
basilic vein. (e) Angiogram demonstrating predominant ulnar feeder (black arrow) to AVM. Note early venous drainage to basilic vein
(arrowhead). Enlargement of the ulnar artery becomes more conspicuous when compared to normal radial artery (white arrow). The draining
vein is also patoulous (arrowhead).
Table 3
Schobinger scale of severity of arteriovenous malformations.
Stage
Stage name
Description
I
Quiescence
Only pink
e
bluish stain and warmth
II
Expansion
Enlarged swelling with pulsation,
thrill, and bruit; veins are tense
and tortuous
III
Destruction
Same as stage II with ulceration,
bleeding, pain, and tissue necrosis
IV
Decompensation Same as stage III with cardiac failure
Modi
fi
ed from reference
37
.
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e
457
251