272 / 280
ill-de
fi
ned purpuric mass, often painful; however, presen-
tation may be later in childhood.
24
The destructive/in
fi
l-
trative nature and very rapid growth of the vascular tumour
helps differentiation from IH. Kasabach
e
Merritt phenom-
enon (KMP) can be seen up to 50% of patients. KHE has a
high mortality rate (24%) related to coagulopathy or com-
plications from local tumour in
fi
ltration. The
fi
rm,
indurated lesion has a more invasive appearance and pur-
plish colouration (
Figs 1
and
5
).
These cells form slit-like lumina containing erythrocytes
that resemble Kaposi
’
s sarcoma, thus the name KHE.
25
KHE
appears as a solid mass with ill-de
fi
ned borders and vari-
able echogenicity at US.
26
MRI demonstrates an in
fi
ltrative
pattern with crossing of multiple soft-tissue planes with
Figure 7
(a) A 4-year-old male patient with a large,
fi
rm mass on his right shoulder/chest wall. It was
fi
rst noted soon after birth, and he
underwent surgical debulking at that time. A known LM that recently enlarged in size. (b
e
c) Coronal T2-weighted image with fat saturation
shows a T2 bright multicystic/septate, large mass that only shows enhancement of the cyst walls and septa (c), typical for LMs. Relatively large
size of each cyst quali
fi
es for a macrocystic LM. (d) Ultrasound during percutaneous access demonstrates macrocystic LM. (e) Contrast medium
injection into one of three macrocysts being treated with doxycycline sclerotherapy.
Figure 8
Two patients with Sturge
e
Weber, (a) 23 years-old, and (b) 35 years old, both with extensive CMs of the face. Note the thickening of the
CM especially in b, which can be seen in CMs over time.
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e
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250