Case report and systematic literature review of a novel etiology of sinistral portal hypertension presenting with UGI bleeding: Left gastric artery pseudoaneurysm compressing the splenic vein treated by embolization of the pseudoaneurysm.

INTRODUCTION: A novel case is reported of upper gastrointestinal (UGI) bleeding from sinistral portal hypertension, caused by a left gastric artery (LGA) pseudoaneurysm (PA) compressing the splenic vein (SV) that was successfully treated with PA embolization. CASE REPORT: A 41-year-old man with previous medical history of recurrent, alcoholic pancreatitis presented with several episodes of hematemesis and abdominal pain for 48 hours. Physical examination revealed a soft abdomen, with no abdominal bruit, no pulsatile abdominal mass, and no stigmata of chronic liver disease. The hemoglobin declined acutely from 12.3 to 9.3 g/dL. Biochemical parameters of liver function and routine coagulation profile were entirely within normal limits. Abdominal CT revealed a 5-cm-wide peripancreatic mass compressing the stomach and constricting the SV. Esophagogastroduodenoscopy showed blood oozing from portal hypertensive gastropathy, small nonbleeding gastric cardial and fundal varices, gastric compression from the extrinsic mass, and no esophageal varices. MRCP and angiography showed that the mass was vascular, arose from the LGA, compressed the mid SV without SV thrombosis, and caused sinistral portal hypertension. At angiography, the PA was angioembolized and occluded. The patient has been asymptomatic with no further bleeding and a stable hemoglobin level during 8 weeks of follow-up. DISCUSSION: Literature review of the 14 reported cases of LGA PA revealed that this report of acute UGI bleeding from sinistral portal hypertension from a LGA PA constricting the SV is novel; one previously reported patient had severe anemia without acute UGI bleeding associated with sinistral portal hypertension from a LGA PA.
CONCLUSION: A patient presented with UGI bleeding from sinistral portal hypertension from a LGA PA compressing the SV that was treated by angiographic obliteration of the PA which relieved the SV compression and arrested the UGI bleeding. Primary therapy for this syndrome should be addressed to obliterate the PA and not the secondarily constricted SV.

KEY POINTS

Previously reported:

GI bleeding from gastric varices or portal hypertensive gastropathy can occur from sinistral (left-sided) portal hypertension, typically secondary to splenic vein injury. Reported etiologies include splenic vein thrombosis, embolism, or compression by various enlarged, adjacent lesions.

What is new here:

A novel case is reported of UGI bleeding from sinistral portal hypertension from a left gastric artery pseudoaneurysm compressing the splenic vein. In this case the UGI bleeding at EGD was from portal hypertensive gastropathy rather than gastric varices, as determined by SRH.

Angiographic embolization of the left gastric artery pseudoaneurysm relieved the splenic vein compression, reversed the sinistral portal hypertension, and arrested the UGI bleeding.

Introduction

Although numerous etiologies of gross gastrointestinal (GI) bleeding from sinistral portal hypertension have been reported (Table 1[), systematic literature review revealed no case of gross GI bleeding from sinistral (left-sided) portal hypertension from splenic vein (SV) compression by a left gastric artery (LGA) pseudoaneurysm (PA). A patient is reported who presented with this novel syndrome from this PA. This syndrome is important to diagnose because therapy for this syndrome should be directed at the PA to eliminate SV compression and reverse the sinistral portal hypertension rather than the secondarily affected SV; this case illustrates this principle by the novel report of angioembolization of this PA to achieve hemostasis.

Table 1

Literature review of reported etiologies of sinistral portal hypertension presenting with gross GI bleeding∗.

Methods

The literature was systematically reviewed using PubMed with the following medical subject headings (MeSH) or keywords: {“left gastric artery”} and {pseudoaneurysm} OR {“sinistral” or “left”} and {“portal hypertension”} and [{“gastrointestinal bleeding”} or {“gastrointestinal hemorrhage”}]. Two authors independently reviewed the literature and decided by consensus which articles to incorporate in this study. Two articles written in French were professionally translated into English.[Table 1 includes only cases of bleeding from PAs of the LGA or its branches and excludes PAs of other arteries arising from the celiac trunk.[ Two cases of bleeding from “gastric arteries” in a study of 104 arterial complications of pancreatitis were excluded because the specific bleeding “gastric” artery and case details were not reported.[ This case report received exemption/approval by the IRB at William Beaumont Hospital, Royal Oak, on July 21, 2016.

Case report

A 41-year-old man with a history of alcoholism for 10 years and sober for the last 3 years, recurrent alcoholic pancreatitis, and no known liver disease, presented with several episodes of hematemesis and abdominal pain for 2 days. Esophagogastroduodenoscopy (EGD), performed 3 years earlier for abdominal pain, had revealed no esophageal varices, gastric varices, portal hypertensive gastropathy, or other GI lesions. Physical examination revealed a blood pressure = 100/60 mm Hg, pulse = 60 beats/min, no jaundice, no stigmata of chronic liver disease, a soft abdomen with mild epigastric tenderness but no rebound tenderness, no abdominal bruit, and no pulsatile abdominal mass. Rectal examination revealed gross melena. Laboratory tests revealed hemoglobin = 12.5 g/dL, platelets = 301,000/mL, INR (international normalized ratio) = 1.0, blood urea nitrogen = 20 mg/dL, and creatinine = 1.1 mg/dL. Serum aspartate aminotransferase = 21 IU/L, alanine aminotransferase = 16 IU/L, total bilirubin = 0.6 mg/dL, alkaline phosphatase = 64 IU/L, albumin = 4.4 gm/dL, and lipase = 32 U/dL. The hemoglobin declined acutely to 9.3 g/dL. Abdomino-pelvic computerized tomography (CT), with intravenous contrast, revealed a 5-cm wide, irregular, pancreatic/peripancreatic mass, compressing both the lesser curvature of the stomach and the SV (Fig. 1A, B), a normal portal vein, and normal liver parenchyma. The SV compression was pathophysiologically significant as indicated by proximal SV dilatation. EGD revealed in the proximal stomach a fine, reticular, pale-white, polygonal, mucosal, network in a snakeskin pattern, and characteristic of portal hypertensive gastropathy that was actively oozing; extensive coffee-ground, blood clots in the stomach; small gastric cardial and fundal varices without stigmata of recent hemorrhage (SRH); and no esophageal varices (Fig. 2). The extrinsic mass produced a large, round bulge extending into the lumen of the proximal gastric body along the lesser curvature (Fig. 2). Magnetic resonance cholangio-pancreatography (MRCP) revealed a 5-cm wide, enhancing, vascular mass likely arising from the LGA and located between the gastric lesser curvature and distal pancreatic body; compressing the stomach; compressing the middle SV; and resulting in large collateral veins draining the SV into the superior mesenteric vein (Fig. 3A, B). Abdominal ultrasound with Doppler studies demonstrated large, turbulent arterial flow into this vascular mass, suggesting a large PA (Fig. 4). Visceral arteriogram showed a 5.3 × 2.2-cm-wide PA supplied by an LGA branch (Fig. 5A), which was embolized and occluded with microcoils (Fig. 5B). Eight weeks later, the patient had a stable hemoglobin level with no further GI bleeding. Abdomino-pelvic CT angiography demonstrated the PA had markedly decreased in diameter, contained numerous microcoils, and had no blood flow (Fig. 1C).

Figure 1

(A) Axial view of abdominal CT, with IV contrast, shows a 5.2 cm pancreatic/peripancreatic mass (labeled∗) compressing the stomach (which has a radiopaque penumbra due to orally ingested contrast) along the lesser curve, and shows that this mass causes a kink (arrow) that partially obstructs the splenic vein and causes sinistral portal hypertension. (B) Coronal view of abdominal CT, with IV contrast, shows a 5.22 cm pancreatic/peripancreatic mass extending superiorly and compressing the stomach, with a radiopaque penumbra due to orally ingested contrast along the lesser curve, and extending inferiorly to the pancreas. (C) CTA 8 weeks after angiographic embolization of left gastric artery demonstrates the pseudoaneurysm has markedly decreased in size, contains metallic coils (producing a metal streak artifact), and has no blood flow. Occlusion of the pseudoaneurysm reverses the splenic vein compression and relieves the sinistral portal hypertension responsible for bleeding from portal hypertensive gastropathy. CT = computerized tomography, CTA = computerized tomographic angiography, IV = intravenous.

Figure 2

Endoscopic retroflexion at esophagogastroduodenoscopy shows a prominent, pale white, reticular, network surrounding individual pink polygons, a characteristic finding of portal hypertensive gastropathy, in the proximal gastric body. This mucosa bulges into the gastric lumen due to extrinsic compression by the pseudoaneurysm. This lesion is actively oozing, as evidenced by stigmata of recent hemorrhage: an intensely erythematous, oozing, lesion on the dependent side of the bulge (arrow) within the area of portal gastropathy. The proximal stomach along the greater curvature is dark because of the presence of dark blood clots within the lumen.

Figure 3

(A, B) Abdominal MRCP shows a 5-cm-wide mass compressing the gastric mid-body, along the lesser curve. The mass is radiolucent before IV contrast administration (A), but markedly enhances after contrast administration (B), a temporal pattern consistent with a vascular mass. The stomach has a moderately radiopaque penumbra from ingested oral contrast. IV = intravenous, MRCP = magnetic resonance cholangio-pancreatography.

Figure 4

Abdominal ultrasound with Doppler studies reveals a strong arterial waveform in tracing on bottom of screen, indicating a vascular, arterial mass. Blue and red Doppler signals within the mass demonstrate turbulent (to-and-fro) blood flow.

Figure 5

Arteriogram during arterial phase after selective injection of contrast into left gastric artery shows pseudoaneurysm filled with injected contrast (A, before embolization); and shows no blood flow (no contrast) within pseudoaneurysm after angiographic coil embolization (B, note coils in pseudoaneurysm and left gastric artery).

Discussion

Comprehensive literature review revealed 14 cases of LGA PA, including the currently reported case (Table 2   [). Twelve patients were male and 1 was female (sex not reported in 1 patient). Twelve patients were adults and 1 was an infant, with a mean age of 46.9 ± 20.4 years old (age not reported in 1 patient). PA etiologies included: recurrent/chronic pancreatitis – 9, blunt abdominal trauma – 2, gastric ulcer penetrating into LGA – 1, recent laparoscopic cholecystectomy – 1, and alcoholic cirrhosis – 1. Nine patients had alcoholic pancreatitis and 1 had alcoholic cirrhosis. Pancreatitis is the most common cause of PAs of this branch or other branches of the celiac axis.[ Pancreatitis causes leakage of pancreatic enzymes that injure the intima and media, the 2 innermost vascular layers, which then merge with pancreatic pseudocysts to create a false cavity communicating with the arterial lumen.[

Table 2

Fourteen reported cases of left gastric artery pseudoaneurysm including the current case: clinical presentation, diagnostic evaluation, pathophysiology, therapy, and outcome.

Clinical presentation in the 14 patients included gross upper gastrointestinal (UGI) bleeding – 9, severe anemia without gross GI bleeding – 2, intrapancreatic (retroperitoneal) hemorrhage – 1, intrahepatic (intraperitoneal) hemorrhage – 1, and abdominal pain – 2 ([Table 2   ]; 1 patient had 2 presentations). Etiologies of gross UGI bleeding in the 9 patients included: PA penetrating through gastric wall into gastric lumen – 5, peptic ulcer eroding into PA in gastric wall – 2, hemosucuss pancreaticus from PA eroding into pancreatic duct – 1, and gastric oozing from portal hypertensive gastropathy with sinistral portal hypertension – 1. PAs commonly present with upper or lower GI bleeding due to the underlying vascular injury.[

Abdominal CT findings of enhancement of a pancreatic pseudocyst or of an adjacent vascular structure are highly suspicious for PAs. However, endoscopic ultrasound (EUS) is superior to CT for diagnosing small PAs.[ PAs usually appear on transabdominal ultrasonography as anechoic, frequently pulsatile lesions, and often contain a cyst within a larger anechoic mass. Color and pulse Doppler sonography further enhance test sensitivity and specificity by demonstrating turbulent blood flow (“to and fro” sign) within the PA. Angiography is the gold standard diagnostic test: it identifies the affected artery, determines local vascular anatomy, and permits interventional therapy.[ This case represents the first case of LGA PA diagnosed by MRCP. Abdominal CT with intravenous contrast showed a pancreatic/peripancreatic mass, initially suspicious for pancreatic adenocarcinoma, but MRCP demonstrated that the mass was vascular, and likely originated from the LGA.

Only 2 of the 14 patients had sinistral portal hypertension. In sinistral portal hypertension, defined as partial portal hypertension without cirrhosis from venous obstruction proximal to the portal vein (typically occurring at the SV), patients commonly bleed from gastric varices or uncommonly from portal hypertensive gastropathy, but do not bleed from esophageal varices because the portal hypertension only affects the stomach. One prior patient had sinistral portal hypertension from LGA PA compression of the SV; but this patient presented with anemia (hemoglobin = 7.1 g/dL) without gross bleeding and had gastric varices identified by EGD which presumably caused the anemia.[ The currently reported patient is the first presenting with gross bleeding from sinistral portal hypertension from SV compression by an LGA PA. This patient had hematemesis from portal hypertensive gastropathy (based on endoscopic SRH), without liver disease, from sinistral portal hypertension.

Among the 9 patients with acute UGI bleeding, angioembolization achieved hemostasis in 5 of 7 cases, surgery was successful in 2 of 3 cases, and dual mode intraoperative angiography and surgery was successful in 1 case. In the current case, hemostasis was achieved by angioembolizing the LGA to relieve SV compression and reverse the sinistral portal hypertension. Angioembolization provides definitive therapy in about 78% of all bleeding visceral PAs, and provides partially successful therapy to stabilize a patient before definitive surgical therapy in many of the remaining cases.[ Rebleeding is the most common complication of angioembolization.[ Alternative therapies include endovascular stents or vascular surgery which is reserved for patients who are appropriate surgical candidates, and who have either failed angioembolization or have other surgical indications, such as pancreatic pseudocyst, pancreatic abscess, or gastric outlet obstruction.[ It is hoped that prompt angioembolization may reduce the previously reported mortality of bleeding from visceral PAs of 60%.[

Splenic vein thrombosis (SVT) also causes GI bleeding associated with pancreatitis from formation of gastric varices due to sinistral portal hypertension.[ SVT should be suspected in patients with pancreatic disease, especially pancreatitis; splenomegaly without generalized portal hypertension or cirrhosis; and isolated gastric varices.[ Notably, this work illustrates different therapies depending upon the etiology: sinistral portal hypertension caused by LGA PA compressing the SV should be treated by obliterating the PA, whereas sinistral portal hypertension caused by SVT should be treated by splenic artery embolization, SV recanalization, or splenectomy.[ The pathophysiology of UGI bleeding for sinistral portal hypertension from LGA is illustrated in Fig. 6.

Figure 6

Flow diagram illustrates the pathophysiology of bleeding from sinistral portal hypertension from splenic vein compression by an adjacent left gastric artery (LGA) pseudoaneurysm from chronic or recurrent acute pancreatitis.

Study limitations include: this represents only 1 case report, the methodology is retrospective, bleeding from leakage from the PA into the gastric lumen or from the small gastric varices cannot be excluded as possible etiologies of the UGI bleeding, and bleeding from portal hypertensive gastropathy, rather than gastric varices, is based primarily on endoscopic SRH. Bleeding from sinistral portal hypertension is usually from gastric varices, but in this reported case was apparently from portal hypertensive gastropathy, as demonstrated by endoscopic SRH. The observed gastric varices were small and lacked SRH, and the bleeding was mild, more consistent with portal hypertensive gastropathy than gastric varices.[ A prospective clinical series is unlikely because this syndrome is rare.

In conclusion, novel findings in this case include: gross gastric bleeding from sinistral portal hypertension secondary to an LGA PA compressing the SV, and hemostasis achieved by occluding the PA to relieve SV compression.

Table 2 (Continued)

Fourteen reported cases of left gastric artery pseudoaneurysm including the current case: clinical presentation, diagnostic evaluation, pathophysiology, therapy, and outcome.

Table 2 (Continued)

Fourteen reported cases of left gastric artery pseudoaneurysm including the current case: clinical presentation, diagnostic evaluation, pathophysiology, therapy, and outcome.

Table 2 (Continued)

Fourteen reported cases of left gastric artery pseudoaneurysm including the current case: clinical presentation, diagnostic evaluation, pathophysiology, therapy, and outcome.