Venous Vascular Malformation

venous vascular malformation

Coronal T2W MRI of the head and neck of a 20 year-old male. There are areas of high signal with a trans-spatial distribution: base of tongue; the tonsillar region; the masticator space; the subcutaneous tissues of the neck; and the deep neck spaces including the larynx and upper trachea. This appearance is characteristic of vascular malformation. There are areas of low signal within some of the vascular spaces, consistent with phleboliths.

Vascular malformations are classified according to Mulliken & Glowacki, 1982. They may be low-flow (venous, capillary, lymphatic, or mixed) or high-flow (arteriovenous) malformations. Vascular malformations are non-proliferative congenital abnormalities. They should be differentiated from capillary haemangioma of infancy, a common proliferative lesion. Vascular malformations (especially mixed type) are associated with Klippel-Trenaunay, Osler-Weber-Rendu, Maffucci, blue-rubber-bleb naevus, and other syndromes. Frequently there is associated tissue hypertrophy, although atrophy may also occur. Lesions are typically trans-spatial, involving subcutaneous, muscle, joint, bone and organ compartments.

Clinical presentation of venous malformation may be with cosmetic deformity, pain, haemorrhage, thrombosis, loss of function, or compression of vital structures. Management of these complex lesions is best undertaken in a multidisiplinary team environment, in specialised vascular birthmark clinics. Treatment may be with surgical excision or sclerotherapy. Surgical excision is often complicated by recurrence, as complete excision is rarely possible. Sclerotherapy with ethanol or foamed surfactants has been shown to be efficacious in reducing the symptoms and complications, and the complication rate is low. Complications of sclerotherapy include skin ulceration, sclerosant embolisation and nerve palsy.

References:
1. Mulliken JB & Glowacki J. Hemangiomas and Vascular Malformations in Infants and Children: A Classification Based on Endothelial Characteristics. Plast. Reconst. Surg. 1982; 69(3):412-420
2. Burrows PE. Percutaneous Treatment of Low Flow Vascular Malformations. J Vasc Interv Radiol. 2004; 15:431-445

Credit: Dr Laughlin Dawes