alpha-1 antitrypsin deficiency and splenic artery aneurysm rupture: An association?

OBJECTIVE: Theoretically, patients with alpha l-antitrypsin deficiency may be vulnerable to the development of splenic artery aneurysms, alpha-1 antit rypsin deficiency can induce cirrhosis with portal hypertension, and result ing protease-antiprotease imbalances may exaggerate arterial wall weakness due to proteolysis of arterial structural proteins. A splenic artery aneury sm rupture 7 days after liver transplantation provoked a reassessment of th e incidence of this phenomenon in a liver transplant population. METHODS: Case records from three institutions and the results of a survey s ent to 126 liver transplantation programs in the United Network for Organ S haring database were reviewed. The incidence of splenic artery aneurysm rup ture in the peritransplantation period, etiology of liver disease associate d with this phenomenon, and recommendations regarding management of splenic artery aneurysms was assessed. RESULTS: Twenty-one cases of splenic artery aneurysm rupture were identifie d. alpha-1 antitrypsin deficiency was the most common cause of cirrhosis in the majority of identified patients who presented with splenic artery aneu rysm rupture, which was associated with a mortality rate of 57%. Respondent s to the survey indicated that a preoperative evaluation was warranted if a splenic artery aneurysm was suspected; however, no consensus regarding man agement exists. CONCLUSIONS: The presence and risk of rupture of splenic artery aneurysms m ay be greater in patients with alpha-1 antitrypsin deficiency. If identifie d before rupture, an aggressive approach to diagnosing and treating these a neurysms should be initiated. At present, no consensus exists regarding the management of splenic artery aneurysms. (Am J Gastroenterol 2000;95: 1531- 1534. (C) 2000 by Am. Cell. of Gastroenterology).