Clinical Conundrum: An Inflammatory AAA…A Cautionary Tale

Abstract

Introduction: Inflammatory AAA account for 5–10% of all AAA. Although the pathogenesis of inflammatory AAA appears to involve an immune response localized to the vessel wall, the aetiology of the inflammatory reaction is unknown. Chronic periaortitis is a spectrum of diseases including inflammatory AAA. In most cases chronic periaortitis is idiopathic; other causes are drugs, retroperitoneal injury, infection and malignancy. Case Description: A previously fit and active 66yo man was admitted under medics with blurring of vision and a suspected TIA. During admission he c/o left flank pain with CT showing a 4.3cm inflammatory AAA, left hydronephrosis and right ureteric stone. He required bilateral JJ stents and percutaneous nephrostomy due to persistent left hydronephrosis. Autoimmune screen and temporal artery biopsy were negative. Subsequently he developed bilateral leg swelling and duplex scan showed DVT. Then he developed RUQ pain and obstructive jaundice. Investigations showed gall stones, intrahepatic biliary dilatation, and subsequently CBD dilatation. Initially Unsuccessful, subsequent ERCP and PTC relieved the obstruction but resulted in AKI secondary to CIN and sepsis. Tumour markers were elevated with CA19.9 of 2779. The patient was transferred to ICU and developed multi organ failure and unfortunately passed away. PM revealed a very rare case of signet ring carcinoma of the CBD. Discussion: Causes of inflammatory AAA are multifactorial. This case highlights the need for caution before repair, as a rapidly progressive and aggressive disease course may indicate a malignant aetiology.