Unusually giant splenic artery and vein aneurysm with arteriovenous fistula with hypersplenism in a nulliparous woman

Abstract

Although splenic artery aneurysm (SAA) is the commonest visceral and third most common intra abdominal aneurysm after aorta and iliac artery, aneurysm of splenic artery along with aneurysm of splenic vein with arteriovenous (a-v) fistula communication between them is a rare entity. Most are <3 cm in diameter. Giant true SAAs are rare and very few lesions >10 cm have been reported. We hereby report a case of an 18 cm x 15 cm size splenic artery and vein aneurysm with a-v fistula in an adult female nulliparous woman who presented with progressively enlarging pulsatile mass in the left upper abdomen with long-standing intractable pancytopenia and splenomegaly. Diagnosis was established by CT (computed tomogram) angiogram and laboratory tests. Laparotomy demonstrated huge well-defined aneurysm of splenic artery and vein with splenic a-v fistula, extending in all except the right lower and inferior quadrants of the abdomen along with splenomegaly. Aneurysmectomy with splenectomy was done.

Contemporary Management of Giant Renal and Visceral Arteriovenous Fistulae

“…Massive hematuria from renal AVFs has been reported, 13 although we did not encounter this in our patients. Longstanding large fistulae with associated venous aneurysms can also present with pain or a mass, 14 as noted in one of our patients. Portal hypertension secondary to a giant splenic AVF has been previously reported as well.…”

Vascular Diseases of the Spleen: A Review

“…It occurs more often in females (80%), especially the multiparous, and its occurrence in men is likely associated to prior surgery or penetrating trauma 61. Only 16% of cases presented without signs of portal hypertension 59. SAVF should be considered in a patient with signs of acute portal hypertension that is not associated with a chronic liver disease 57–63.…”

The role of computed tomography in detecting splenic arteriovenous fistula and concomitant atrial myxoma

“…The estimated risk of rupture depends on the size of the aneurysm. There is an increased risk during pregnancy, but this was not the case for our patient [8]. …”