A large solitary congenital arteriovenous fistula presenting as lymphoedema

Abstract

A SOLITARY congenital arteriovenous fistula of approximately 5 mm. external diameter and 5 mm.i n length between the left subclavian artery and the triple junction of the left subclavian, left internal jugular, and left innominatc vcins, forms the subject of this report. CASE REPORTA female infant, aged 8 months presented with a history of sudden onset of oedema of the left upper limb starting at the age of 3 months. The child was apparently normal in every way before that. Her mother stated: 'Her limb was perfectly all right when I dressed her in On the basis of the clinical picture a confident diagnosis of an arteriovenous fistula between the left subclavian artery and the left subclavian vein was made and arteriography was deemed not necessary.AT OPERATION.-At operation on 19 Feb. approach was made through an inverted L-shaped incision 2 s 2 in. with its angle over the suprasternal notch. The sternum was split to the level of the 2nd interspace, then outwards into this space, and the left strap muscles and sternomastoid were divided. This allowed the clavicle and two upper ribs to be bent outwards, thus giving excellent exposure of the whole neck root.A very large varicosity exactly at the triple junction of left subclavian, left internal jugular, and left innominate the morning, but when I bathed her in the evening her left hand was red and swollen'-the swelling remained the same size until the operation. There had been no pregnancy illnesses.ON EXAMINATION.-This showed a healthy wellnourished infant. There was solid oedema of the whole upper limb and slightly of the left side of the neck and face, but most noticeable in the hand and forearm where there was a tendency to peau d'orange. Dilated veins were seen in the upper arm. There was no pulsation or obvious increase of temperature. Blood-pressure in the right arm was I I S /~S . A loud 'machinery' murmur was present similar in all respects to that of a persistent ductus arteriosus except that it was audible with maximal intensity just over the left sternoclavicular joint. A wellmarked thrill corresponded in position with the murmur. Limbs were equal in length. Chest radiograph showed grade one cardiac enlargement on 24 Jan., 1963, and a repeat examination on 16 Feb. showed no change. veins was now visible with the superior intercostal vein entering its elongated lower end. A11 veins connected with this varicosity were quite small (Fig. I).On division of the superior intercostal vein, it was possible to rotate the varicosity upwards and to display the fistula leading from the subclavian artery (Fig. 2 ) .Well-marked pre-fistula dilatation of the subclavian artery was present. All other arteries were quite small.With the arterial end of the fistula controlled by a Pott's clamp, the venous end of the fistula was first tied off and then transfixed with a No. I silk suture. The fistula was now divided and the arterial end doubly stitched with a continuous s/o silk suture. The elongated lower part of the varicosity was excised and the defect also sutured with S/...