Left-Sided Portal Hypertension - SASSiT

Dig Dis Sci (2007) 52:1141–1149 

DOI 10.1007/s10620-006-9307-x 

REVIEW ARTICLE 

Left-Sided Portal Hypertension 

Seyfettin Köklü · Şahin Çoban · Osman Yüksel · 

Mehmet Arhan 

Received: 11 December 2005 / Accepted: 6 March 2006 / Published online: 24 March 2007 

C○ Springer Science+Business Media, Inc. 2007 

Abstract Left-sided portal hypertension is a rare clinical 

syndrome which may lead to bleeding from isolated gastric 

varices. Pancreatic disease is the most common etiology. 

Left-sided portal hypertension should be considered in the 

presence of gastrointestinal bleeding with normal liver function 

and unexplained splenomegaly. It may be difficult to 

diagnose this entity both endoscopically and radiologically. 

While splenectomy is the treatment of choice for cases complicated 

by variceal bleeding, there is no consensus on the 

treatment of asymptomatic patients. The prognosis of left- 

S. Köklü 

Department of Gastroenterology, Ankara Education and Research 

Hospital, 

Ankara, Turkey 

Ş. Çoban 

Department of Gastroenterology, Ankara University School of 

Medicine, 


O. Yüksel 

Department of Gastroenterology, Ankara Numune Hospital, 


M. Arhan 

Department of Gastroenterology, Ankara Oncology Hospital, 


S. Köklü () 

Karargahtepe mahallesi, Kumrulu sokak, 

18/1, 06300, Keçiören, Ankara, Turkey 

e-mail: gskoklu@yahoo.com 

sided portal hypertension mainly depends on the underlying 

etiology. 

Keywords Left-sided portal hypertension . Splenic vein . 

Thrombosis . Obstruction . Splenectomy . Gastrointestinal 

bleeding 

Incidence 

Left-sided portal hypertension (LSPH) is a localized form 

of portal hypertension that usually occurs as a result of isolated 

obstruction of the splenic vein [1, 2]. Obstruction of 

the proximal part of the portal vein together with the splenic 

vein may also be a rare complication in LSPH. The incidence 

of LSPH has increased over the past three decades 

due to increased awareness of the entity and advances in diagnostic 

approaches. Since most patients are asymptomatic 

and experience no complications, its exact incidence is unknown. 

However, it accounts for less than 5% of all patients 

with portal hypertension [3]. Diagnostic difficulties and lack 

of inclusion in the differential diagnosis may partly explain 

this low figure. Sutton et al. reviewed the English literature 

between 1900 and 1968 and reported 54 cases [4]. Madsen 

et al. reviewed the English literature from 1969 to 1984 and 

found 209 cases [3]. Since then, case reports and several case 

series have been added. To date, approximately 450 cases of 

LSPH have been reported in all. Most of the studies in the 

literature comprise a limited number of patients and are usually 

retrospective [3, 5–9]. In a prospective study performed 

by Bernades et al., 266 patients with chronic pancreatitis 

(one of the most common causes of LSPH) were screened 

with ultrasonography, and angiography or computed tomography 

was used to confirm venous obstruction [10]. In 

that study, the overall incidence of occlusion of one of the 

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1142 Dig Dis Sci (2007) 52:1141–1149 

major splanchnic veins was 13%, with the splenic vein being 

occluded in 8% of patients, the portal vein in 4%, and the 

superior mesenteric vein in 1% [10]. 

Synonyms 

LSPH has also been referred to as segmental [11], sinistral 

[7], regional [4], localized [12], compartmental [13], lineal 

[14], or splenoportal hypertension [15]. 

Anatomy 

The splenic vein is a large and nontortuous vessel formed 

by five or six tributaries from the spleen. It lies inferior to 

the splenic artery and, after leaving the splenic hilus, runs 

behind the tail and the body of the pancreas. It is approximately 

0.5 cm in diameter and 12 cm long. The splenic vein 

crosses anterior to the left kidney, being separated from the 

left sympathetic trunk and crus by the left renal vessels and 

from the abdominal aorta by the superior mesenteric artery 

and left renal vein [16]. The tributaries of the splenic vein 

include the short gastric, left gastroepiploic, pancreatic, and 

inferior mesenteric veins. Behind the neck of the pancreas 

the splenic vein joins the superior mesenteric vein to form the 

portal vein. Since the splenic vein is contiguous with the 

pancreas throughout that organ’s entire length, pancreatic 

disorders contribute the main etiology, and any significant 

pancreatic pathology may be complicated with venous obstruction 

(Fig. 1). There is also close approximation between 

the splenic vein and the neighboring pancreatolienal lymph 

nodes. Therefore, even retroperitoneal diseases may contribute 

to splenic vein occlusion [17–21]. 

Fig. 1 Illustration of splenic venous thrombosis and fundal varices. 

CV, coronary veins; GEV, gastroepiploic vein; PV, portal vein; SV, 

splenic vein; SMV, superior mesenteric vein 

Pathophysiology 

Blood flow through the splenic vein may be blocked secondary 

to either thrombosis formation or neighboring mass 

effect. Splenic vein occlusion results in venous hypertension 

in collateral pathways that carry splenic arterial blood to 

the superior mesenteric and portal veins including the short 

gastric, coronary, and gastroepiploic veins and the veins located 

in the upper half of the stomach. Following obstruction, 

splenic blood typically drains through the short gastric veins 

to the stomach. In the gastric wall veins of the fundus, blood 

flow and pressure increase and submucosal structures consequently 

dilate, producing gastric varices. Eventual decompression 

into the portal system occurs through the coronary 

and epiploic veins. The coronary vein drains to different parts 

of the portal system (directly to the portal vein, to the junction 

of the splenic and portal veins, and to the splenic vein). 

When the coronary vein drains distal to the obstruction in 

the splenic vein, esophageal varices may occur alone or in 

combination with gastric varices [2, 7, 22]. However, due to 

several anatomic variations, obstruction of the splenic vein 

may not always result in portal hypertension or formation of 

varices. 

Etiology 

The main cause of LSPH is splenic vein thrombosis (SVT). 

Rare causes of LSPH include compression of the splenic 

vein by other organs, edema, enlarged lymphadenopathies, 

and splenic artery aneurysm. There is a strong association 

between pancreatic disorders and SVT because of the splenic 

vein’s location. Because the splenic vein is posterior to the 

pancreas and in direct contact with it, any type of pancreatic 

disease is likely to involve the splenic vein [3, 8, 16]. 

Acute and chronic pancreatitis and pancreas neoplasms are 

the most common causes of SVT [3, 8, 10, 16, 23–26]. In 

an early report in 1970, Sutton et al. found that 35% of their 

cases of isolated SVT were caused by tumors and only 17% 

by pancreatitis [4]. More recent reviews have found acute 

or chronic pancreatitis to be the probable cause of isolated 

SVT in the majority of cases [2]. In a study by Moosa et al., 

pancreatitis—diagnosed with biopsy or operation—was the 

etiology in 87 (60%) of 144 cases, while pancreas malignancy 

was detected in only 13 (9%) of the patients [16]. 

The reason for this difference may be due to increases in the 

incidence of pancreatitis and in diagnostic activities, as well 

as to improvements in diagnostic procedures [27]. 

Single episodes of acute pancreatitis may lead to SVT, 

and the risk of SVT does not correlate with the severity of 

pancreatitis. Also, SVT may occur silently, as a complication 

of mild pancreatitis [7, 8]. 

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Dig Dis Sci (2007) 52:1141–1149 1143 

Table 1 

Etiologies of splenic vein obstruction 

1. Pancreatic diseases 

a. Pancreatitis 

Acute pancreatitis [3, 16, 23, 34] 

Chronic pancreatitis [3, 8, 10, 16, 23, 28] 

Familial pancreatitis [16, 29] 

Traumatic pancreatitis [13] 

b. Pancreas malignancies 

Adenocarcinoma [3, 5, 24] 

Islet cell carcinoma [30–32] 

Cystadenoma [5, 33] 

Pancreatic lymphoma [16] 

c. Other pancreatic causes 

Pancreatic pseudocysts [3, 5, 23, 26, 35] 

Pancreatic abscess [1] 

Pancreatic divisum [23] 

Pancreatic transplantation [16] 

Congenital cyst [36] 

Pancreatic pseudotumor [37] 

2. Nonpancreatic disorders 

a. Surgical procedures 

Umbilical vein catheterization [38] 

Partial gastrectomy [39] 

Distal splenorenal shunt [16] 

Splenectomy [2, 16] 

Selective venous catheterization [16] 

b. Metastatic carcinoma 

Lymphoma [3, 16] 

Oat cell carcinoma [5] 

Retroperitoneal liposarcoma [16] 

Renal cancer [3, 40] 

Gastric cancer [22] 

Colon cancer [3, 22] 

c. Miscellaneous 

Retroperitoneal fibrosis [12] 

Splenic artery aneurysms [16] 

Gastric ulcer [1] 

Hepatoportal sclerosis [22] 

Hereditary thrombocytemia [41] 

Myeloproliferative disorders [41] 

Protein S deficiency [22] 

Systemic lupus erythematosus [22] 

Renal abscess [17] 

Tuberculous adenitis [42] 

Retroperitoneal abscess [17, 21] 

Benign renal cysts [1] 

Various disorders other than pancreatic diseases can cause 

isolated SVT. However, they are rare and have a wide spectrum 

in terms of their mechanisms of splenic vein obstruction 

(Table 1) [1–5, 8, 10, 12, 13, 16, 17, 21–42]. 

Presenting signs/symptoms 

The major clinical consequences of portal hypertension in 

general are the formation of gastroesophageal varices, ascites, 

and splenomegaly. 

Patients with portal hypertension frequently form varices 

[43]. When only a segment of the portal venous bed is obstructed, 

varices develop only in areas that decompress the 

corresponding segment. For example, segmental portal hypertension 

within the splenic vein (SVT) is associated with 

the formation of isolated gastric varices in the fundus of the 

stomach. 

Most commonly, LSPH is asymptomatic and is found 

incidentally on investigation. In symptomatic cases, the first 

clinical manifestation of LSPH is generally acute or chronic 

GI bleeding from ruptured esophageal or gastric varices, 

and rarely from colonic varices [44]. Usually the bleeding is 

serious. Patients may also present with chronic anemia due 

to portal hypertensive gastropathy. Gastrointestinal bleeding 

is the presenting symptom in 45% [16] to 72% [3] of patients 

with LSPH. The number of patients that bleed varies from 

series to series (Table 2) [5, 10, 16, 22, 44, 45]. Based on 

prospective studies, it appears that most patients with SVT do 

not bleed, suggesting that adequate low-pressure collateral 

flow develops without the formation of varices. 

Splenomegaly is a hallmark of long-standing portal hypertension 

and is frequently seen in patients with LSPH. The 

degree of splenomegaly in patients with presinusoidal portal 

hypertension including isolated SVT is often greater than 

in those with cirrhosis [46]. The mechanisms are not fully 

understood but are related to increased venous congestion 

and splenic arterial flow. Although up to 71% of patients 

have splenomegaly, few patients suffer from splenic pain 

and develop leukopenia or thrombocytopenia [3]. 

Abdominal pain without bleeding can be caused in different 

patients by a variety of conditions, such as chronic 

pancreatitis, pseudocyst, carcinoma, and splenomegaly. It 

may be the presenting symptom in 25%–38% of patients 

[3, 16, 22]. 

Patients with LSPH typically do not have significant ascites 

unless they develop acute dilutional hypoalbuminemia 

during fluid resuscitation for a variceal bleed or have associated 

cirrhosis. Therefore, development of ascites is a rare 

presenting manifestation in LSPH [47]. 

Diagnosis 

The diagnosis of LSPH is based on clinical, biochemical, and 

radiological evaluation. Ultrasonography may show normal 

liver architecture. When the diagnosis is in doubt, a liver 

biopsy may be performed to rule out cirrhosis. 

Esophageal varices can be seen both radiologically and 

endoscopically. In contrast, gastric varices are often difficult 

to diagnose by either technique. On barium contrast studies, 

gastric varices appear as thick and tortuous mucosal folds, 

filling defects or distorted mucosal configurations anywhere 

along the greater curvature toward the cardia [48]. Although 

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1144 Dig Dis Sci (2007) 52:1141–1149 

Table 2 The origin of acute 

gastrointestinal bleeding in 

patients with left-sided portal 

hypertension (LSPH) 

a For prospective studies. 

b Review of the literature. 

c Prospective studies. 

Number of Acute GI Mean 

patients with bleeding due Esophageal Gastric Combined follow-up 

Reference LSPH to varices varices varices varices time (mo) a 

78 

2 

2 20 

Moosa et al., 73 51 4 38 31 

1985 [16] b 

Warshaw 8 2 8 

et al., 1987 

[45] 

Bradley 11 2 6 

[44] c 

et al., 1987 

Evans et al., 12 10 10 

1990 [5] 

Bernades 22 1 100 

[10] c 

et al., 1992 

Sakorafas 34 6 12 

et al., 2000 

[7] 

Heider et al., 53 2 11 30 

2004 [23] 

Köklü et al., 24 6 21 

2005 [22] c 

most patients with gastric varices have thickened mucosal 

folds in the fundus of the stomach, the abnormality is rarely 

specific for the disorder [49]. These findings can be misinterpreted 

as gastric carcinoma, lymphoma, multiple polyps, 

or Menetrier’s disease [50, 51]. The reported accuracy of 

barium contrast studies in patients with proved gastric varices 

has ranged from 14% to 74% [52, 53]. Currently, bariumcontrast 

examinations are not frequently used because newer 

diagnostic techniques are preferred. 

Upper gastrointestinal endoscopy can be used to detect 

the presence of gastric varices. The endoscopic appearance 

of gastric varices varies from clear-cut variceal veins to irregularities 

or nodularities in the rugae [54]. If isolated gastric 

varices are seen on endoscopy, LSPH should be considered, 

and advanced diagnostic studies should be performed 

to clarify the diagnosis. As endoscopists have become more 

competent, the accuracy of visualizing gastric varices has 

increased. In an early series, less than half of the patients 

with gastric varices found on splenoportography were diagnosed 

correctly by endoscopy [49]. In a more recent study, 

gastric varices have been accurately diagnosed and localized 

in nearly 90% of cases [55]. A careful examination should 

be made to find gastric varices in every patient with gastrointestinal 

bleeding of obscure origin, particularly if it is 

accompanied by splenomegaly. 

Two techniques of angiography are available for diagnosing 

LSPH. Splenoportography was popularized by Leger 

in 1951 as a method to visualize the portal venous system, 

but that procedure is rarely used today because it is invasive 

and has potential morbidity, particularly in patients with portal 

hypertension [11]. Arteriography is the preferred method 

for diagnosis and is a safer and more controlled technique 

in patients with a prolonged prothrombin coagulation time 

and elevated portal or systemic pressures [56]. Angiography 

outlines the location of obstruction and the route of decompression. 

Catheterization of the celiac axis provides access to 

surrounding arterial structures, including abnormal hepatic 

and pancreatic neoplastic vasculature. Subsequently, venous 

phase studies can be performed [16]. The portal system is 

generally shown clearly; however, arterographic studies often 

fail to demonstrate collateral veins that can be seen on 

splenoportography [1]. 

Venous phase angiography accurately shows both the location 

of the splenic vein obstruction and collateral flow. 

The diagnosis of SVT is confirmed on angiography when 

the splenic vein fails to opacify on the venous phase of the 

splenic artery injecton. Often venous collaterals in the splenic 

hilum and dilated gastroepiploic and short gastric veins are 

also seen [26]. 

Intra-arterial digital substraction angiography (DSA) reliably 

shows the portal venous system, collateral circulation, 

shunt location and postoperative changes. The major advantage 

of intra-arterial DSA is the smaller amount of contrast 

medium injected, so that local and systemic side effects 

are rare [57]. Other well-known advantages of intra-arterial 

DSA are quicker execution, less injury to arteries offered 

by smaller-caliber catheters, and low cost. The major disadvantage 

of intra-arterial DSA is that the field size of the 

intensifier is limited to 6–9 in. 

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Dig Dis Sci (2007) 52:1141–1149 1145 

Ultrasonography (US) is often used as a preliminary, noninvasive 

test for LSPH. US is the least invasive of all available 

tests. The accuracy of US in the diagnosis of SVT, versus in 

the diagnosis of portal vein thrombosis, may be limited by the 

size and location of the splenic vein [2]. Studies comparing 

US with angiography and arterial portography in evaluating 

portal vein patency have demonstrated US to be relatively 

accurate, with a sensitivity and specificity of 93% and 83%, 

respectively [58, 59]. However, US may be less accurate in 

assessing splenic vein patency because of the anatomic location 

of the splenic vein. In one study, by Alpern et al.,the 

direction of flow in the splenic vein was correctly determined 

in 9 of 16 patients undergoing duplex US. The study found 

that, in some patients with SVT, imaging of collateral vessels 

near the splenic hilum could be misinterpreted as the splenic 

vein, and that operator experience is paramount [59]. 

Generally, Doppler ultrasonography is the first imaging 

technique used in patients with elevated portal pressure and 

is accurate in the assessment of the portal venous system [60, 

61]. Noninvasive and relatively inexpensive evaluation can 

be achieved with color Doppler US, which shows the portal 

vein and provides additional information about velocity 

and direction of flow. However, Doppler US is observerdependent 

and may be unsuccessful when the acoustic window 

is not available for evaluation of the whole portal venous 

system [60–62]. In addition, US does not show the overall 

anatomic structure that interests the clinician. Therefore, in 

patients who are potential candidates for surgery, a more 

exact diagnostic method that covers the whole portal venous 

system is required, such as magnetic resonance (MR) 

angiography [61]. 

Recently, endoscopic ultrasound (EUS) has been used to 

assess the portal vasculature. This method appears to be a 

more accurate test than transabdominal US for evaluating 

patency of the splenic vein in some reports [63, 64]. As one 

of the most sensitive imaging methods for studying the pancreas, 

EUS has resulted in an improved ability to diagnose 

pancreatic mass lesions and to assess vascular involvement 

in pancreatic malignancies [63, 65]. EUS is superior to both 

US and computerized tomography (CT) in diagnosing small 

pancreatic lesions and assessing vascular invasion, with an 

accuracy of 94% and 87%, respectively [63, 65]. EUS is also 

useful in diagnosing chronic pancreatitis, with a sensitivity, 

specificity, and accuracy of 80%, 86%, and 84%, respectively 

[66]. It should be considered when other diagnostic methods 

have failed to confirm SVT as a cause of bleeding gastric or 

gastroesophageal varices [66]. It should also be considered 

in cases of SVT occurring without a history of chronic pancreatitis, 

so that pancreatic carcinoma can be investigated 

as a potential cause of SVT. Because SVT is generally associated 

with pancreatic pathology, EUS may be an ideal 

diagnostic method to evaluate the splenic vasculature and 

the pancreatic parenchyma. EUS has also been shown to be 

useful and highly sensitive in detecting paraesophageal and 

gastric varices [67–69, 90–92]. In a study comparing EUS 

and CT, the former was found to be more sensitive in detecting 

paraesophageal varices [67]. It has also been reported 

that EUS is more sensitive than conventional endoscopy in 

detecting gastric varices [70, 71]. 

Contrast-enhanced CT portography can demonstrate the 

portal venous system in a short time [72–75]. However, it 

uses ionizing radiation and requires a large amount of iodinated 

contrast material. Unless multidetector CT is used, 

CT portography may also suffer from limited longitudinal 

coverage because usually CT provides only axial scan mode 

[76]. 

Magnetic resonance imaging (MRI) is an increasingly 

valuable tool for the assessment of the portal venous system 

and splenic vasculature. MR angiography with gadopentetate 

dimeglumine has been shown to be a very promising noninvasive 

method for the assessment of the portal venous system 

[77–82]. MR angiography appears to be a more accurate diagnostic 

procedure than Doppler sonography and CT [60, 72, 

83, 84]. In one study, the splenic vein was not evaluated optimally 

due to gastrointestinal gas interfering with Doppler 

sonography in four patients; however, on MR portograms the 

splenic vein was shown to be normal in three patients and 

partially thrombosed in one [85]. Contrast-enhanced MR angiography 

has also been increasingly used in patients with 

portal hypertension for the diagnosis of patency or thrombosis 

of the portal venous system [85–87]. 

It should be mentioned that thrombosis in the proximal 

splenic vein may not be shown even by the diagnostic techniques 

discussed here, and so sometimes it can be confirmed 

only intraoperatively [17, 26]. 

Treatment 

LSPH is one of the rare curable syndromes causing portal 

hypertension [88]. To form a consensus on the treatment of 

patients with LSPH, the underlying diseases, the presence 

and severity of symptoms, and the general condition of the 

patients should be considered. 

Bleeding (variceal or nonvariceal) is the most common 

manifestation of LSPH [5]. Variceal bleeding may be severe 

and life-threatening and can originate from esophageal, gastric, 

or even colonic varices [27, 56, 89]. Management should 

be directed at the splenic side of the portal circulation because 

pressure is increased only on that side [5]. Proximal portal 

decompressive procedures (any sort of portosystemic shunt) 

are hazardous and do not address the disease process, since 

these patients have normal portal pressures and generally 

normal hepatic function [5]. Conservative therapy including 

balloon tamponade, sclerotherapy, vasoconstrictive therapy, 

and band ligation may be performed for bleeding control. 

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1146 Dig Dis Sci (2007) 52:1141–1149 

Unfortunately, these treatment methods have often failed 

[1, 2, 5]. 

Sclerotherapy is effective in patients with esophageal 

varices, but not in those with isolated varices in the fundus or 

gastric body [2, 90]. Recurrent bleeding occurs in more than 

half of patients who undergo endoscopic sclerosis of fundal 

varices [2]. However, current approaches in sclerotherapy 

have shown hopeful results. Some studies have demonstrated 

that endoscopic injection of cyanoacrylate is an effective and 

safe treatment for gastric variceal bleeding and obliteration 

of gastric varices, especially for the localized types [91–94]. 

Compared with the other sclerotherapy agents, cyanoacrylate 

provides better hemostasis and is associated with less bleeding 

recurrence and mortality [93, 94]. However, this method 

may have life-threatening complications such as hemorrhage 

and peripheral embolic events (pulmonary embolism, cerebral 

infarction, or portal vein embolism). At present, the risk 

of peripheral embolic events limits its use in spite of currently 

available cyanoacrylate analogues [91, 95–97]. 

A patient with active bleeding unresponsive to conservative 

management should be operated on quickly [1, 8]. 

Splenectomy is the treatment of choice [98]. Removal of 

the spleen decreases the venous outflow through the collateral 

circulation and decompresses the associated varices to 

prevent further hemorrhage [16, 27]. In a large splenectomy 

series that included patients with isolated SVT, Moossa et al. 

reviewed the English literature between 1969 and 1984 and 

reported splenectomy in 79 of 144 cases [16]. Postoperative 

mortality was 8% and deaths were due to causes other than 

bleeding. None of the 73 patients had recurrent bleeding after 

splenectomy during the mean follow-up period of 11 months. 

In another study, reported by Evans et al., splenectomy was 

performed as a primary therapy in 10 patients. Hemorrhage 

was effectively controlled in all but one of these patients [5]. 

Patients with LSPH and no history of bleeding are a problem 

in terms of the risk of future bleeding [8]. Recent studies 

have focused on investigations of optimal management 

for patients with asymptomatic SVT [23]. In a retrospective 

study of pancreatitis-induced splenic vein thrombosis, 53 

cases were followed up for a median period of 34 months; 

of these, 2 needed splenectomy due to variceal bleeding 

and 3 had hemorrhage due to nonvariceal sources. Because 

of the low incidence of gastric variceal hemorrhage [4%] 

and the absence of mortality related to variceal hemorrhage, 

the authors suggested that splenectomy not be performed 

routinely for these patients [23]. In a retrospective study 

done by Sakorafas et al., none of the eight patients with 

symptomatic LSPH who underwent splenectomy had recurrent 

gastrointestinal bleeding. Splenectomy was performed 

for three of the five asymptomatic patients with esophageal 

or gastric varices and none of those five patients had gastrointestinal 

hemorrhage during follow-up. Twelve of the 21 

asymptomatic patients without esophageal or gastric varices 

underwent splenectomy. None of those had bleeding; however, 

one of the remaining nine patients had bleeding during 

follow-up [7]. In our own experience, 6 of 24 patients with 

LSPH presented with gastrointestinal bleeding on admission. 

Urgent surgery was needed for only one patient who had a severe 

variceal hemorrhage. Other patients admitted with gastrointestinal 

bleeding did not need invasive treatment. None 

of our patients had recurrent bleeding during follow-up [22]. 

Finally, there is not enough evidence supporting prophylactic 

splenectomy in the treatment of asymptomatic patients. 

Nonetheless, if abdominal surgery is performed for other 

purposes in a patient with known asymptomatic varices, a 

splenectomy may be preferred [2, 7]. 

Transcatheter splenic artery embolization has been suggested 

by Jones and associates to produce a “nonsurgical 

splenectomy” [99]. It is performed by transcatheter deposition 

of gianturco coils, autologous clot, or absorbable gelatin 

sponge [6]. Embolization of the splenic artery by selective 

catheterization has been tried with varying success and 

has not become the preferred approach [27]. However, transcatheter 

embolization as a preoperative adjunct to splenectomy 

for hypersplenism has been advocated. Perhaps its role 

in LSPH should be as the planned first stage of a two-step 

therapeutic plan, embolization followed by splenectomy [5, 

6, 100]. Adams et al. showed that splenic artery occlusion 

during the splenectomy procedure diminished intraoperative 

blood loss [6]. However, splenectomy can be performed 

with an acceptable blood loss without splenic artery inflow 

control in many patients. Nevertheless, patients with LSPH 

who require operation for complications of chronic pancreatitis 

may benefit from preoperative placement of the splenic 

artery balloon catheter [5]. Splenic infarction and abscess 

formation may occur after the embolization procedure [99]. 

Considering these issues, embolization without splenectomy 

should be restricted to patients for whom surgery would be 

highly risky [5, 16]. 

Reconstruction of the splenic vein may be an alternative 

therapy in selected patients who have peripheral thrombosis 

of the splenic vein [101]. However, there are not enough data 

to evaluate the utility of that procedure. 

Prognosis 

The prognosis of LSPH mainly depends on the underlying 

disease. The occurrence or recurrence rate of gastrointestinal 

bleeding cannot be estimated. Since nearly half of the 

patients have an underlying malignancy, especially pancreas 

adenocarcinoma, most of those patients have a shorter life 

expectancy and the incidence of gastrointestinal bleeding 

is very low in that short period. In our recent prospective 

study, all but 1 of the 11 patients with malignancy complicated 

with LSPH died within a year, and 1 patient had a 

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Dig Dis Sci (2007) 52:1141–1149 1147 

gastrointestinal hemorrhage before death [22]. None of the 

patients with nonmalignant disorders (three and eight patients 

with and without splenectomy, respectively) had bleeding 

during a mean period of 30 months. Similarly, in another 

prospective study none of the 23 patients with splenoportal 

vein thrombosis and LSPH (10 without splenectomy and 13 

with splenectomy) had digestive tract bleeding during the 

30-month follow-up period after diagnosis [10]. 

Conclusion 

LSPH is a clinical syndrome that usually occurs as a result 

of isolated SVT, which in turn arises from various etiologies, 

mainly pancreatic diseases. LSPH should be considered in 

the presence of gastrointestinal bleeding with normal liver 

function tests and unexplained splenomegaly. It may be difficult 

to diagnose this entity both endoscopically and radiologically. 

However, noninvasive techniques including CT 

and MR angiography are making the diagnosis easier. Endoscopic 

ultrasonography is sensitive in determining isolated 

gastric varices and should be considered in highly suspect 

patients. Treatment should be directed to the underlying diseases, 

and while splenectomy is the treatment of choice for 

cases complicated by variceal bleeding, there is no consensus 

on the treatment of asymptomatic patients. Recurrent 

hemorrhage is not usual and the prognosis mainly depends 

on the underlying etiology. 

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