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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.

A. Tekes et al. / Clinical Radiology 69 (2014) 443

e

457

255