Multiple Splenic Artery Aneurysms: A Rare Cause of Extrahepatic Portal Hypertension and Massive Splenomegaly

Abstract

A 39-year-old female presented with one year history of dragging abdominal discomfort and fatigability of six months duration. She was non-alcoholic and had no other medical illness. General physical examination was unremarkable and abdominal palpation suggested splenomegaly of 10 cms below left coastal margin, which was firm and nontender. There was no ascites or hepatomegaly. Examination of other systems was unremarkable.Investigations revealed normal complete blood counts, urine analysis, liver and renal function tests and peripheral smear study. Investigation reports are shown in [Table/ Fig-1]. Ultrasonography (US) with color doppler of abdomen revealed splenomegaly, normal liver echotexture and cavernous transformation of portal vein with multiple spleno-renal collaterals. ECG and echocardiography was normal. Upper gastro-intestinal endoscopy showed grade 1-2 esophageal varices with portal hypertensive gastropathy. Multidetector Computed tomography (MDCT) abdomen revealed multiple aneurysms in the distal half of splenic artery, the largest one measuring (5.1cmx5.8cmx4.4cms) with dilated and tortuous course of the proximal splenic artery with multiple collaterals along the course of splenic and portal vein b,. Wall calcification and organized thrombus was seen in few of the aneurysms at the splenic hilum. DisCussionSplenic artery aneurysms (SAA) are the most common visceral aneurysms, accounting for up-to 60% of cases [1,2]. Splenic artery is the third most common site for intra-abdominal aneurysm after aorta and iliac artery [2]. It has an incidence of 0.01-0.2%, females being more commonly affected (4:1). SAA presenting as extrahepatic portal hypertension is rare [1,3]. Here we report a patient with multiple splenic artery aneurysms presenting as extrahepatic portal hypertension and massive splenomegaly. SAA are very rare with a varied prevalence of 0.01%-10.4% [4]. SAAs are associated with several conditions, including pregnancy, degenerative atherosclerosis, portal hypertension, medial fibrodysplasia, arteritis, collagen vascular disease, α 1-antitrypsin deficiency, and pancreatitis [2]. Hormonal changes during pregnancy, increased blood volume and cardiac output causing portal congestion are the proposed mechanisms of SAA in pregnancy internal medicine section [5]. SAA are usually single, isolated and <3cms in size. It is usually located in the distal part of splenic artery. Larger aneurysms like in our patient are seldom reported [1,3,6]. The aneurysm generally develops on the main splenic artery commonly on its distal third or on intrasplenic branches. Multiple Splenic Artery Aneurysms: A Rare Cause of Extrahepatic Portal Hypertension and Massive Splenomegaly aBstRaCtA 39-year-old nulliparous female was admitted with massive splenomegaly. Computed tomography of abdomen revealed multiple aneurysms in the distal half of the splenic artery. Splenic artery aneurysms are rare in nulliparous women and most cases are reported in females with a past history of pregnancy. Splenic artery aneurysms, though very...

Prevalence and predictive factors for development of splenic artery aneurysms in cirrhosis

“…In adult with cirrhosis, its incidence is reported between 7 % and 17 % [6,9]. It has been reported that SAA are more frequent in females compared to males [9,13,14] and adults compared to children in liver transplant patients [9]. We did not identify any literature that comprehensively evaluated the predictive factor for development of SAA in cirrhosis.…”

“…The pathogenesis of SAA is unknown. Pregnancy, degenerative atherosclerosis, portal hypertension, medial fibrodysplasia, arteritis, collagen vascular disease, alfa-1 antitrypsin deficiency, and pancreatitis are associated with increased incidence of SAA [9,13,15,16]. Several authors reported an increased elastase content of the aneurysm, with a subsequent decrease in the elastin content of the vessel wall.…”

“…But in some cases with SAA, recurrent left upper quadrant pain [10,12,[17][18][19], spontaneous rupture with intraperitoneal hemorrhage [2,4,6,8,19,20], extrahepatic portal hypertension associated with massive splenomegaly [13], nontender pulsatile lump in left upper abdomen [1] and SAA associated with spontaneous splenorenal shunt in noncirrhotic liver [21] have been reported. In our cases with SAA, we did not record any symptoms and signs can be related to SAA.…”

A Rare Quadruple Association: Fibromuscular Dysplasia, Giant Splenic Artery Aneurysm, Extrahepatic Portal Hypertension, and Hypersplenism

“…It shows a 4:1 female preponderance, especially affecting the young reproductive age group. 5 , 6 , 10 The common causes of SAA include pregnancy, cirrhotic portal hypertension, trauma, and polyarteritis nodosa. 6 , 10 Fibromuscular dysplasia is a very rare cause of SAA.…”

“… 4 – 6 Splenic artery aneurysm can cause compression of the splenic veins, thus causing venous stasis and thrombosis. 5 , 6 This can lead to extrahepatic portal vein obstruction with consequent portosystemic shunting and enlarged collaterals. Our case showed a slowed portal flow, multiple enlarged lienorenal collaterals, and portal cavernoma, thus confirming EPHT.…”