Unilateral Pulmonary Fibrosis Due to Absence of Right Pulmonary Artery.

Pulmonary fibrosis is typically a bilateral, progressive interstitial lung disease that is often idiopathic but can be associated with risk factors such as advanced age, environmental exposure, and drug toxicity. The pathophysiology is incompletely understood but involves transforming growth factor. The treatment choices for idiopathic disease include medical therapy that manipulates epigenetic pathways and lung transplantation. Here we present a 30-year-old female with no identifiable risk factors who developed unilateral pulmonary fibrosis. Clinical investigation eventually revealed a congenitally absent right pulmonary artery which was presumed to cause her unilateral disease. In contrast to idiopathic pulmonary fibrosis, treatment options for pulmonary fibrosis due to unilateral absence of a pulmonary artery include ipsilateral pulmonary vasculature embolization and/or pneumonectomy if disease is unmanageable without therapy.

Endovascular therapy of traumatic vascular lesions of the head and neck.

Pseudoaneurysm and fistula formation are well-documented complications of arterial vascular injury and may be associated with significant morbidity and mortality. The purpose of this manuscript is to review the presentation and therapy of patients with traumatic vascular injuries of the head and neck. Eight patients were admitted to a Level 1 Trauma Center and diagnostic angiography of the carotid artery and vertebral circulation was performed. The mechanisms of injury included motor vehicle accident, gunshot wound, stab wound and aggravated assault. Cause of trauma, vascular lesion, endovascular therapy and outcome were analyzed retrospectively. The angiographic findings, clinical presentation and hospital course were reviewed. There were eight patients, seven males and one female, aged 17-65. Four patients (50%) had multiple lesions; four had pseudoaneurysms, two with fistula formation and two with active arterial hemorrhage. A total of 17 lesions were embolized using coils. Polyvinyl Alcohol (PVA), Gelfoam or a combination. Two of the 17 lesions received stents. Six of the eight patients remained clinically improved or stable at varying follow-up intervals. One of the four patients who presented with penetrating trauma and neurological deficits had resolution of right hemiplegia at the 8th month follow-up. One of the four patients who sustained blunt trauma and carotid-cavernous fistula presented with a new pseudoaneurysm at the 2-month post-embolization follow-up. The evolution of diagnostic neuroangiographic techniques provides opportunities for endovascular therapy of traumatic vascular lesions of the head and neck that are minimally invasive, attractive options in selected cases.