Spontaneous rupture of a left subclavian artery pseudoaneurysm in a child

A 14-year-old boy with history of generalized joint hypermobility and ongoing genetics work-up for connective tissue disorder presented to our hospital for one day of acute left-sided chest pain and shortness of breath after feeling a “pop” in his chest while trying to put on a jacket. He was tachycardic and had absent breath sounds in the left lobe. A chest X-ray showed a large left pleural effusion and a subsequent CT chest demonstrated a large left hemothorax with a psuedoaneurysm of the left subclavian artery (LSCA) at the base of the vertebral artery. Angiography confirmed the presence of a LSCA pseudoaneurysm that measured 15 by 8mm (Figure 1A video/Panel A). A 5mm by 3 cm Cordis OptaPro balloon was then placed in the LSCA just distal to the vertebral artery and proximal angiogram demonstrating that the bleed could be occluded without obstructing flow to the vertebral artery. Subsequently, the patient underwent successful placement of a 6 mm Gore VBX stent (Figure 1B video/Panel B) via positioning with an Amplatz Extra Stiff wire in the distal LSCA without obstruction to flow to the vertebral artery. At follow-up, the patient is doing well and was recently confirmed to have PLOD1-related kyphoscoliotic Ehlers-Danlos syndrome (1902+1G>T variant). Causes of subclavian artery pseudoaneurysms include connective-tissue disorders, congenital defects and infections.