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Left-Sided Portal Hypertension - SASSiT

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Dig Dis Sci (2007) 52:1141–1149 1145<br />

Ultrasonography (US) is often used as a preliminary, noninvasive<br />

test for LSPH. US is the least invasive of all available<br />

tests. The accuracy of US in the diagnosis of SVT, versus in<br />

the diagnosis of portal vein thrombosis, may be limited by the<br />

size and location of the splenic vein [2]. Studies comparing<br />

US with angiography and arterial portography in evaluating<br />

portal vein patency have demonstrated US to be relatively<br />

accurate, with a sensitivity and specificity of 93% and 83%,<br />

respectively [58, 59]. However, US may be less accurate in<br />

assessing splenic vein patency because of the anatomic location<br />

of the splenic vein. In one study, by Alpern et al.,the<br />

direction of flow in the splenic vein was correctly determined<br />

in 9 of 16 patients undergoing duplex US. The study found<br />

that, in some patients with SVT, imaging of collateral vessels<br />

near the splenic hilum could be misinterpreted as the splenic<br />

vein, and that operator experience is paramount [59].<br />

Generally, Doppler ultrasonography is the first imaging<br />

technique used in patients with elevated portal pressure and<br />

is accurate in the assessment of the portal venous system [60,<br />

61]. Noninvasive and relatively inexpensive evaluation can<br />

be achieved with color Doppler US, which shows the portal<br />

vein and provides additional information about velocity<br />

and direction of flow. However, Doppler US is observerdependent<br />

and may be unsuccessful when the acoustic window<br />

is not available for evaluation of the whole portal venous<br />

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

anatomic structure that interests the clinician. Therefore, in<br />

patients who are potential candidates for surgery, a more<br />

exact diagnostic method that covers the whole portal venous<br />

system is required, such as magnetic resonance (MR)<br />

angiography [61].<br />

Recently, endoscopic ultrasound (EUS) has been used to<br />

assess the portal vasculature. This method appears to be a<br />

more accurate test than transabdominal US for evaluating<br />

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

of the most sensitive imaging methods for studying the pancreas,<br />

EUS has resulted in an improved ability to diagnose<br />

pancreatic mass lesions and to assess vascular involvement<br />

in pancreatic malignancies [63, 65]. EUS is superior to both<br />

US and computerized tomography (CT) in diagnosing small<br />

pancreatic lesions and assessing vascular invasion, with an<br />

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

useful in diagnosing chronic pancreatitis, with a sensitivity,<br />

specificity, and accuracy of 80%, 86%, and 84%, respectively<br />

[66]. It should be considered when other diagnostic methods<br />

have failed to confirm SVT as a cause of bleeding gastric or<br />

gastroesophageal varices [66]. It should also be considered<br />

in cases of SVT occurring without a history of chronic pancreatitis,<br />

so that pancreatic carcinoma can be investigated<br />

as a potential cause of SVT. Because SVT is generally associated<br />

with pancreatic pathology, EUS may be an ideal<br />

diagnostic method to evaluate the splenic vasculature and<br />

the pancreatic parenchyma. EUS has also been shown to be<br />

useful and highly sensitive in detecting paraesophageal and<br />

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

and CT, the former was found to be more sensitive in detecting<br />

paraesophageal varices [67]. It has also been reported<br />

that EUS is more sensitive than conventional endoscopy in<br />

detecting gastric varices [70, 71].<br />

Contrast-enhanced CT portography can demonstrate the<br />

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

uses ionizing radiation and requires a large amount of iodinated<br />

contrast material. Unless multidetector CT is used,<br />

CT portography may also suffer from limited longitudinal<br />

coverage because usually CT provides only axial scan mode<br />

[76].<br />

Magnetic resonance imaging (MRI) is an increasingly<br />

valuable tool for the assessment of the portal venous system<br />

and splenic vasculature. MR angiography with gadopentetate<br />

dimeglumine has been shown to be a very promising noninvasive<br />

method for the assessment of the portal venous system<br />

[77–82]. MR angiography appears to be a more accurate diagnostic<br />

procedure than Doppler sonography and CT [60, 72,<br />

83, 84]. In one study, the splenic vein was not evaluated optimally<br />

due to gastrointestinal gas interfering with Doppler<br />

sonography in four patients; however, on MR portograms the<br />

splenic vein was shown to be normal in three patients and<br />

partially thrombosed in one [85]. Contrast-enhanced MR angiography<br />

has also been increasingly used in patients with<br />

portal hypertension for the diagnosis of patency or thrombosis<br />

of the portal venous system [85–87].<br />

It should be mentioned that thrombosis in the proximal<br />

splenic vein may not be shown even by the diagnostic techniques<br />

discussed here, and so sometimes it can be confirmed<br />

only intraoperatively [17, 26].<br />

Treatment<br />

LSPH is one of the rare curable syndromes causing portal<br />

hypertension [88]. To form a consensus on the treatment of<br />

patients with LSPH, the underlying diseases, the presence<br />

and severity of symptoms, and the general condition of the<br />

patients should be considered.<br />

Bleeding (variceal or nonvariceal) is the most common<br />

manifestation of LSPH [5]. Variceal bleeding may be severe<br />

and life-threatening and can originate from esophageal, gastric,<br />

or even colonic varices [27, 56, 89]. Management should<br />

be directed at the splenic side of the portal circulation because<br />

pressure is increased only on that side [5]. Proximal portal<br />

decompressive procedures (any sort of portosystemic shunt)<br />

are hazardous and do not address the disease process, since<br />

these patients have normal portal pressures and generally<br />

normal hepatic function [5]. Conservative therapy including<br />

balloon tamponade, sclerotherapy, vasoconstrictive therapy,<br />

and band ligation may be performed for bleeding control.<br />

Springer

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