2015 HSC Section 1 Book of Articles

A. Tekes et al. / Clinical Radiology 69 (2014) 443 e 457

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).

involvement of the overlying skin and subcutaneous fat. These more aggressive imaging features distinguish KHE from IH, as do the atypical clinical features.

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

Syndromes associated with haemangiomas

Vascular malformations

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.

VAs that are present at birth and grow slowly, propor- tionally to the patient without spontaneous regression are

Table 3 Schobinger scale of severity of arteriovenous malformations.

Stage

Stage name Quiescence Expansion

Description

I

Only pink e bluish stain and warmth Enlarged swelling with pulsation, thrill, and bruit; veins are tense and tortuous Same as stage II with ulceration, bleeding, pain, and tissue necrosis

II

III

Destruction

IV

Decompensation Same as stage III with cardiac failure

Modi fi ed from reference 37 .

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