GE Port J Gastroenterol.

2015;22(6):231---233

www.elsevier.pt/ge

EDITORIAL

Left-Sided Portal Hypertension: A Clinical Challenge

Pedro Pereira a,b,∗ , Armando Peixoto a,b

a
Gastroenterology Department, Centro Hospitalar de São João, Porto, Portugal
b
Faculty of Medicine, University of Porto, Porto, Portugal

Left-sided portal hypertension (LSPH), also known as in portal hypertension or formation of varices. It is believed
segmental, regional, localized, compartmental, lineal, that similar pathophysiological mechanisms may occur in
splenoportal, or sinistral hypertension is a rare, but life cases of superior mesenteric vein obstruction, although in
threatening cause of upper gastrointestinal bleeding. It usu- this case the risk of ectopic varices involving the proximal
ally occurs as a result of isolated obstruction of the splenic small bowel might be more common.
vein. The incidence of LSPH has increased over the past A recent study confirmed the past presumptive knowl-
three decades due to increased awareness of the entity and edge that most common pathologies resulting in splenic
advances in diagnostic approaches. Since most patients are vein thrombosis or obstruction and leading to LSPH are
asymptomatic and experience no complications, its exact chronic pancreatitis, pancreatic pseudocysts and pancre-
incidence is unknown. However, it accounts for less than 5% atic neoplasms.4 In particular, the presence of pseudocyst
of all patients with portal hypertension.1 To date, less than in patients with chronic pancreatitis might be associ-
500 cases of LSPH have been reported in all. Most of the stud- ated with significantly higher incidence of splenic vein
ies in the literature comprise a limited number of patients thrombosis.5 These account for about 18% of the LSPH cases
and are usually retrospective.2,3 Due to its low incidence, it include benign neoplasms, adenocarcinoma and functioning
is likely that most cases of sinistral hypertension are initially and non-functioning neuroendocrine tumors.6,7 Iatrogenic
misdiagnosed as a generalized portal hypertension. splenic vein injury after liver transplantation, infiltration
Blood flow through the splenic vein may be blocked sec- by colonic tumor, spontaneous splenic vein thrombosis and
ondary to either thrombosis formation or neighboring mass perirenal abscess have also been linked with LSPH, but these
effect.1 Following obstruction, splenic blood typically drains etiological factors are rare.1
through the short gastric veins to the stomach. In the gastric The diagnosis of sinistral portal hypertension should be
wall veins of the fundus, blood flow and pressure increase considered in all those with upper gastrointestinal bleed-
and submucosal structures consequently dilate, producing ing associated with splenomegaly and normal liver function
gastric varices. Fortunately, due to several anatomic varia- tests. Most commonly, however, LSPH is asymptomatic and
tions, obstruction of the splenic vein may not always result is found incidentally on investigation. In up to 73% of the
symptomatic cases,2 the first clinical manifestation of LSPH,
is generally acute (often massive) or chronic upper gas-
DOI of original article: trointestinal bleeding from ruptured esophageal or gastric
http://dx.doi.org/10.1016/j.jpge.2015.09.006 varices, and rarely from colonic varices.8 Up to 71% of
∗ Corresponding author. patients have splenomegaly, few patients suffer from splenic
E-mail address: pedro.pedroreispereira@gmail.com (P. Pereira). pain and develop leukopenia or thrombocytopenia.2 As the

http://dx.doi.org/10.1016/j.jpge.2015.10.001
2341-4545/© 2015 Sociedade Portuguesa de Gastrenterologia. Published by Elsevier España, S.L.U. This is an open access article under the
CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
232 P. Pereira, A. Peixoto

majority of patients does not present with cirrhosis, devel- Management of asymptomatic patients is more controversial
opment of ascites is rare unless they develop acute dilutional than the symptomatic ones: splenectomy has been sug-
hypoalbuminemia for any reason.1 gested as a prophylactic measure by some while others
While diagnosis is mainly clinical and often made by have not shown any significant benefit of this procedure
exclusion of systemic portal hypertension, diagnostic imag- in survival. However, more evidence suggests that watchful
ing plays an important role in confirming the diagnosis in waiting as acceptable course of management in asymp-
the majority of cases.9 Angiography of splenic vein remains tomatic individuals.9
the gold standard in diagnosing sinistral portal hyperten- Excluding deaths from gastrointestinal bleeding, the
sion, although it is less used today by the availability of prognosis of LSPH mainly depends on the underlying disease,
less invasive methods. Transabdominal ultrasonography (US) with those with pancreatic malignancies having the worst
with Doppler is often the initial imaging modality utilized, outcomes. As such, the evidence LSPH, although benign in
but it is more helpful in excluding presence of systemic most cases, can sometimes be the first signal of a sinister
portal hypertension and its primary etiologies such as liver entity, like the pancreatic cancer.
cirrhosis. The accuracy of trans-abdominal US is limited In this issue of GE Portuguese Journal of Gastroenterol-
in detecting splenic or superior mesenteric veins throm- ogy, Fernandes et al17 report the clinical presentation,
bosis, which are smaller and more subtle than those of etiologies and outcome in a cohort of 22 patients with LSH.
portal veins.10 Recently, endoscopic ultrasound (EUS) has This retrospective analysis highlights some particular issues
been used to assess the portal vasculature. This method regarding this difficult to diagnose and manage entity. Sim-
appears to be a more accurate test than transabdominal ilarly to reported series, most patients (n = 14) experience
US for evaluating patency of the splenic vein.11 EUS should no symptoms or presented non-specific abdominal pain, and
be considered when other diagnostic methods have failed LSH was found incidentally on investigation. This raises
to confirm splenic vein thrombosis as a cause of bleeding the question about the value of screening asymptomatic
gastric or gastroesophageal varices, especially in patients patients for the presence of LSPH. Bernardes et al. search
with previous history of pancreatitis.1 It should also be for splenoportal venous obstruction in a medical-surgical
considered in cases occurring without previous history of prospective series of 266 patients with chronic pancreatitis
chronic pancreatitis, so that pancreatic carcinoma can be who were followed up a mean time of 8.2 years18 Spleno-
investigated as a potential cause. In recent years contrast portal venous obstruction was found in 35 patients (13.2%)
enhanced CT scan and magnetic resonance angiography are but was symptomatic in only two. Development of varices in
gaining popularity due to faster acquisition of images with LSPH occurs only when formation of adequate low-pressure
low invasiveness.9 However, the sensitivity and specificity collateral do not develop, so diagnostic test should evalu-
for detection of minor alterations of splenic vasculature are ate not only splenic vein obstruction but also the presence
still modest. of varices. In this context, EUS may be the ideal diagnostic
Management of this condition traditionally involves modality since it can be used to assess portal vasculature,
removal of the primary cause if possible, which can be patency of splenic vein and to diagnose gastric varices.
combined with splenectomy. Symptomatic patients with In this cohort of patients EUS was used in nine patients
variceal bleeding may be severe and life threatening. Cur- with confirmation of gastrointestinal varices in all of them.
rent guidelines recommend endoscopic therapy as first-line Moreover, the authors find that the principal etiology of
treatment for bleeding gastric varices. Endoscopic thera- LSPH was pancreatic pathology (chronic pancreatitis, acute
peutic options for gastric variceal bleeding include band pancreatitis and pancreatic tumours), situations in which
ligation, cyanoacrylate (CYA) injection, and thrombin. The EUS has demonstrated superior accuracy to transabdominal
greatest evidence exists for CYA injection, which is rec- US or abdominal CT. Another controversial issue raised by
ommended as first-line endoscopic therapies in both the this study is the management of LSPH, namely the treat-
American Association for the Study of Liver Diseases guide- ment of varices and the need to initiate anticoagulation
lines and by the Baveno V consensus.12,13 CYA injection therapy. In this regard, we agree with the authors that anti-
is also indicated as the first-line endoscopic treatment of coagulation therapy should be instituted after treatment
ectopic small bowel varices.14 Main complications include of varices, since it’s difficult to determinate the poten-
pulmonary or portal vein embolism. Recently, Bhat et al tial risk of bleeding from gastric varices. CYA obliteration
reported the six-year experience of EUS-guided treatment is the recommend approach to control upper gastrointesti-
of gastric fundal varices with combined injection of coils nal bleeding, however treatment of asymptomatic patients
and CYA in 152 patients.15 Treatment was technically suc- with gastric varices is not consensual since this treatment is
cessful in the majority (99%), with re-bleeding in only 3% associated with risk of embolic events.
during long-term follow-up. The injection of CYA with coils This pertinent study brings to the forefront a condition
under EUS guidance appears to increase the efficacy, and not as uncommon as previously thought, but whose approach
may reduce the risk of CYA embolization. remains based on small cases series and clinical cases and
A patient with active bleeding unresponsive to conser- whose consequences can be catastrophic.
vative management should be submitted to an emergent
splenectomy, which decreases the venous outflow through References
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