Axillary Artery Aneurysm in Tuberous Sclerosis—a Report of Two Cases

Abstract

A three month old male child with known tuberous sclerosis presented with ischemia affecting the tips of the fourth and fifth fingers of the left hand. A pulsatile axillary mass was felt, but with no clinically palpable pulse at the wrist. The hands were symmetrical in size but the left hand was cold, with delayed capillary filling at the pulp of the medial two fingers. A colour duplex scan and a selective left subclavian angiography were performed. These demonstrated a smooth stenosis of the axillary artery followed by an aneurysm of the proximal brachial artery. (Fig. 1). The brachial artery was occluded in the region of the antecubital fossa. Collaterals reformed the proximal few centimetres of the radial and the ulnar artery, which were distally occluded in the mid forearm. The distal forearm and the hand were supplied by collaterals, but no radial, ulnar or palmar arteries could be identified. Exploration of the brachial artery and its bifurcation was performed because of clinical deterioration. The arterial wall was markedly thickened causing narrowing of the lumen. There was weak, non-pulsatile forward flow, with very little back bleeding. In the absence of any distal run off, no arterial bypass was feasible. The aneurysm was left undisturbed because there was no risk of distal embolization. Histology of the brachial artery revealed almost complete occlusion of the lumen by fibroelastic intimal hyperplasia. Five months after the operation, there was mummification and dry gangrene of the medial four digits, requiring amputation at the level of metacarpophalangeal joints.