Arteriovenous malformation D18.0

Rare, congenital malformation of arterial and venous vessels with formation of arteriovenous anastomoses, a consecutive hypertensive varicosis. In principle, AVM can occur in any organ (e.g. cerebral, pulmonary, spinal, musculature) and will cause different symptoms depending on its location. The skin can be directly or indirectly involved. Not uncommon are capillary malformations (naevus flammeus) with/without varicosis, or an isolated varicosis without capillary skin involvement in combination with arterioverous shunts of the subcutis or muscles. AVMs are mostly a therapeutic indication due to their hemodynamic effects and progression tendency (no tumor occurrence, the name hemangioma is not justified).

Stage I (clinical latency): Detection of AV shunts by Doppler sonography; skin lesions (nevus flammeus) may be present.

Stage II: Always detect AV shunts by Doppler sonography; skin lesions (flammeous nevus) with large caliber veins.

Stage III: Destructive tissue changes on the skin, in the subcutis or the musculature.

Stage IV (stage of decompensation): Venous hypertension with increased cardiac load (increased shunt volume). Signs of advanced venous insufficiency of the skin.

Lower leg, complete leg, almost always hemiplegic; hands, face, skin involvement (nevus flammeus, varicosis) is not obligatory.

Possible development of skeletal changes, restricted movement, pain dependent on movement.

Radiation refractory, if necessary surgery or catheter-based embolization. The decisive factor is the elimination of the so-called nidus, which is the site of the short-circuit connection between the artery and the often aneurysmatically dilated drainage vein. The sole embolization of the incoming artery without reaching the actual nidus should be avoided. Absolute 96% alcohol with a coiling of the nidus has proven to be the most effective therapy.

1. Kutzner H (2003) Vascular tumors of the skin. In: Kerl H et al (Ed.) Histopathology of the skin. Springer Publishing House, Berlin Heidelberg New York, S. 763
2. Yang CH, Ohara K (2002) Acquired digital ateriovenous malformation: a report of three cases annd study with epiluminescence microscopy. Br J Dermatol 147: 1007-1009