Isolated Systemic Arterial Supply to Normal Lung with Aneurysm Formation: A Rare Entity with An Even Rarer Complication

Patient: Male, 61-year-old Final Diagnosis: Isolated systemic arterial supply to normal lung Symptoms: Asymptomatic Medication:— Clinical Procedure: — Specialty: Radiology OBJECTIVE: Rare disease BACKGROUND: Isolated systemic arterial supply to normal (unsequestered) lung (ISSNL) without associated pulmonary malformation is rare, and lies towards the milder end of the spectrum of congenital lung abnormalities. Aneurysmal dilatation of the anomalous artery is an infrequent complication, with only 5 published cases thus far. CASE REPORT: We present the case of a 61-year-old man whose screening chest radiograph showed a retrocardiac mass. Further evaluation with axial imaging demonstrated an ISSNL, complicated by aneurysmal dilatation. The genesis of this condition has been postulated to be due to persistence of primitive aortic branches to the developing lung bud. Initially reported in 1777, this entity is now more accurately classified within the spectrum of pulmonary and bronchovascular abnormalities, with refinement of the latter. The origin of an aberrant artery from the aorta implies that a higher-pressure systemic circulation is being shunted into a lower-pressure pulmonary circulation. While these supplying arteries are known to be large, aneurysmal dilation is exceptionally rare. Here, we review the cases published in the literature and present a case of our own. We aim to describe its pathogenesis, and touch on the classification systems and management. CONCLUSIONS: ISSNL is usually first suspected on a screening chest radiograph, as many patients are asymptomatic. Based on contrast-enhanced axial imaging, the diagnosis can be established non-invasively. Definitive management includes surgical and endovascular techniques.