Upper gastrointestinal bleeding with duodenal varix: A case report

Abstract

BACKGROUND

Ectopic varices present diagnostic and management challenges when encountered unexpectedly in clinical practice. Given their potential for fatal outcomes, with mortality rates reaching 40%, it is essential to discuss their clinical manifestations as well as current management guidelines.

CASE SUMMARY

We report the case of a 56-year-old male patient with a history of liver transplantation and segmentectomy for hepatocellular carcinoma with underlying liver cirrhosis and chronic hepatitis B virus infection. He had previously undergone gastrojejunostomy for a perforated duodenal ulcer. The patient presented to the emergency department with hematochezia that began two days prior to his visit, shortly after discharge following recovery from upper gastrointestinal bleeding caused by an anastomotic ulcer at the gastrojejunostomy site. Initial esophagogastroduodenoscopy showed a healing anastomotic ulcer without active bleeding but revealed several hyperemic spots suggesting angiodysplastic changes near the anastomosis. Argon plasma coagulation was performed for cauterization of these microvascular changes. During admission, recurrent bleeding occurred, and abdominal computed tomography revealed portal vein (PV) and superior mesenteric vein (SMV) thrombosis, as well as a varix in the third portion of the duodenum. The patient successfully achieved hemostasis through a combination of embolization and surgical PV-SMV bypass.

CONCLUSION

A systematic approach is essential for diagnosing and managing ectopic varices. Further, evidence-based studies are needed to improve outcomes.

Key Words: Upper gastrointestinal bleeding; Duodenal varix; Ectopic varix; Portal hypertension; Case report

Core Tip: Ectopic varices occur at uncommon sites outside the typical locations of esophageal or gastric varices, making their assessment and management particularly challenging. We report the case of a 56-year-old male with a history of liver transplantation and segmentectomy for hepatocellular carcinoma with underlying liver cirrhosis and hepatitis B virus infection. Successful hemostasis was achieved through a combination of embolization and surgical portal vein and superior mesenteric vein bypass.

INTRODUCTION

Ectopic varices occur at uncommon sites outside the typical locations of esophageal or gastric varices, making their assessment and management particularly challenging. Portal hypertension leads to the formation of portosystemic collaterals and varices in unusual locations beyond the gastroesophageal region. Ectopic varices account for 1%-5% of all variceal bleeding and are associated with severe gastrointestinal hemorrhage and high mortality rates[1,2]. Unexpected varices may occur in various sites, most commonly in the duodenum, which represents 17%-40% of all ectopic cases[3]. Other possible sites include the jejunum, ileum, colon, parastomal regions, biliary tract, genitourinary organs such as the urinary bladder and vagina, and the retroperitoneum, where they are occasionally encountered in clinical practice[3].

Ectopic varices develop secondary to portal hypertension, most often caused by cirrhosis, extrahepatic portal vein obstruction, Budd-Chiari syndrome, portal vein thrombosis, or post-surgical shunts. These ectopic collaterals form connections between portal tributaries and systemic veins and are classified according to their location, based on the feeding and draining veins involved. Specifically, duodenal varices receive blood from the superior or inferior pancreaticoduodenal veins and drain into the gonadal, inferior vena cava, or capsular veins[1,4,5]. Jejunal and ileal varices are supplied by branches of the superior mesenteric vein and drain into retroperitoneal veins[1,6]. Colonic varices are fed by the superior or inferior mesenteric veins and drain into the iliac veins[1,7].

Clinically, ectopic varices often present with massive or intermittent bleeding, manifesting as melena, hematochezia, or hematemesis. The mortality rate associated with bleeding from ectopic varices is approximately 40% and the rebleeding rate ranges from 20%-40% unless portal hypertension is adequately controlled[1].

CASE PRESENTATION

Chief complaints

A 56-year-old male visited the gastroenterology outpatient clinic with complaints of melena, requesting further evaluation of his condition and laboratory testing.

History of present illness

Approximately one month prior to this visit, the patient had been discharged following recovery from upper gastrointestinal bleeding caused by an anastomotic ulcer at the gastrojejunostomy site, which had been treated with therapeutic endoscopic management.

History of past illness

The 56-year-old male had a past medical history of liver transplantation and segmentectomy with cholecystectomy for hepatocellular carcinoma on a background of liver cirrhosis and chronic hepatitis B virus infection, performed in 2016. He had also undergone gastrojejunostomy for a perforated duodenal ulcer in 2009. Since after cholecystectomy in 2016, he had recurrently undergone multiple common bile duct stone removal by percutaneous transhepatic cholangioscopy with percutaneous transhepatic biliary drainage (PTBD) owing to post-surgical anastomotic stricture and deformity causing limited conventional endoscopic approach with endoscopic retrograde cholangiopancreatography (ERCP). During his admission one month prior to the current visit, initial esophagogastroduodenoscopy (EGD) revealed a large amount of blood clots in the gastric lumen, suggesting active bleeding. An active ulcer at the anastomosis site with visible vessel exposure (Forrest IIb) was identified and treated with argon plasma coagulation (APC) followed by clipping to achieve hemostasis (Figure 1A).

Figure 1 Endoscopic examination. A: Anastomosis site active ulcer with Forrest IIb was controlled with argon plasma coagulation and clipping; B: Argon plasma coagulation was performed for cauterization of angiodysplastic change nearby anastomosis site; C: Vascular cauterization was performed at broad vascular change with hyperemia on afferent loop.

Personal and family history

The patient had no relevant family history.

Physical examination

The patient presented with hemodynamic and respiratory stability. Physical examination revealed a PTBD tube in place, without abdominal tenderness. On the day of his presentation to the emergency department, the patient appeared pale and lethargic. His vital signs, including body temperature, blood pressure, heart rate, and oxygen saturation, were within normal limits. Physical examination revealed no abnormalities in the abdominal, cardiovascular, pulmonary, or skin systems, except for anemic conjunctiva.

Laboratory examinations

Initial laboratory results showed (Table 1): White blood cell (WBC) count 4.9 × 10⁹/L, hemoglobin (Hb) 6.1 g/dL, and platelet count 275 × 10⁹/L. Serum aspartate aminotransferase (AST), total bilirubin, blood urea nitrogen (BUN), and creatinine were 19 U/L, 0.39 mg/dL, 23.6 mg/dL, and 0.96 mg/dL, respectively. Coagulation profile had prothrombin time 12.1 seconds with international normalized ratio (INR) 1.1. Consequent laboratory findings after recurrent bleeding just before evaluation with enteroscope presented on hospital day 20 (Table 1): WBC count 4.3 × 10⁹/L, Hb 7.6 g/dL, and platelet count 243 × 10⁹/L. Serum AST, total bilirubin, BUN, and creatinine were 22 U/L, 0.51 mg/dL, 10.4 mg/dL, and 0.74 mg/dL. Coagulation profile demonstrated prothrombin time 12.6 seconds and INR 1.2.

Table 1 Laboratory findings during hospitalization.

Parameter	Reference range	Initial presentation (hospital day 1)	Recurrent bleeding (hospital day 20)
Complete blood count
Hemoglobin (g/dL)	13.0-17.0	6.1	7.6
Hematocrit (%)	39-52	20.6	22.8
White blood cells (× 109/L)	4.0-10.0	4.9	4.3
Platelets (× 109/L)	150-400	275	243
Renal function
Blood urea nitrogen (mg/dL)	8-23	23.6	10.4
Creatinine (mg/dL)	0.6-1.2	0.96	0.74
Estimated GFR (mL/minute/1.73 m2)	> 60	93	106
Electrolytes
Sodium (mmol/L)	135-145	138	140
Potassium (mmol/L)	3.5-5.1	4.4	4.0
Chloride (mmol/L)	98-107	105	107
Liver function tests
Aspartate aminotransferase (U/L)	0-40	19	22
Alanine aminotransferase (U/L)	0-40	11	25
Alkaline phosphatase (U/L)	40-129	117	118
Total bilirubin (mg/dL)	0.2-1.2	0.39	0.51
Albumin (g/dL)	3.5-5.0	3.6	2.9
Coagulation profile
Prothrombin time (second)	11-14	12.1	12.6
INR	0.8-1.2	1.1	1.2

INR: International normalized ratio.

Imaging examinations

Initial EGD after admission revealed a healing anastomotic ulcer without evidence of active bleeding. However, several hyperemic spots suggestive of angiodysplastic changes were observed near the anastomosis site, and APC was performed for cauterization of these microvascular lesions (Figure 1B). After recurrent bleeding during the admission period, follow-up esophagogastroduodenoscopy was performed using an enteroscope to assess the afferent and efferent loops. Broad vascular changes with hyperemia were observed in the afferent loop, and APC was applied for vascular cauterization (Figure 1C). In addition, abdominal computed tomography (CT) revealed thrombosis of the portal vein (PV) and superior mesenteric vein (SMV), as well as a duodenal varix located in the third portion of the duodenum (Figure 2). Despite these interventions, the patient experienced refractory bleeding and hemodynamic instability. Angiography was performed but showed no definite arterial bleeding focus on the superior mesenteric artery, celiac, or inferior mesenteric artery arteriograms. The following day, the patient again developed hemodynamic instability requiring high-dose inotropes, accompanied by hematochezia. Endoscopic evaluation with both diagnostic and therapeutic intent was performed at the afferent loop, where blood oozing from a vascular lesion was treated with clipping. However, there was no improvement in clinical status, and a repeat angiography was performed for embolization.

Figure 2 Abdominal computed tomography. A: On the coronal view, there was portal vein (orange arrow) and superior mesenteric vein thrombus as well as duodenal varix of 3^rd portion (yellow arrow); B: On the axial view, there was duodenal varix at the 3^rd portion (yellow arrow).

FINAL DIAGNOSIS

During the second angiography, the SMV venogram demonstrated distal SMV occlusion and the presence of a duodenal varix.

TREATMENT

Due to the location of the inflow vein above the SMV occlusion, vascular access was limited. Therefore, superselective catheterization was achieved using a microcatheter, followed by embolization with 33% glue and four microcoils at the draining vein of the duodenal varix, resulting in successful hemostasis (Figure 3). After embolization, terlipressin was administered for pharmacological management, and scheduled surgical intervention with PV-SMV bypass was performed. During intra-abdominal venous bypass surgery, upstream thrombosis and severe stricture were noted during distal SMV dissection. The PV sidewall was exposed, and side-to-side anastomosis was performed between both sides using a cadaveric iliac vein graft (Figure 4). With a total of 50 days hospital stay including 16 days in intensive care unit, he was discharged on apixaban for prophylactic anticoagulation management.

Figure 3 Interventional radiology. Embolization with 33% glue and 4 micro-coils at draining vein of duodenal varix for hemostasis.

Figure 4 Intra-abdominal vein bypass surgery. There was upstream thrombosis and severe stricture and portal vein side wall was exposed for side-to-side anastomosis both side with cadaveric iliac vein graft (arrow).

OUTCOME AND FOLLOW-UP

After PV-SMV bypass surgery and discharge, the patient clinically presented no signs of recurrent gastrointestinal bleeding. Postoperative endoscopic and radiologic evaluations were performed to assess the duodenal varix, which had been the primary cause of his clinical presentation. Follow-up CT imaging demonstrated improvement of the duodenal varix. Endoscopic examination performed three months after surgery and discharge showed normal findings at the anastomosis site, with no evidence of recurrent bleeding from either the afferent or efferent loop.

DISCUSSION

The ectopic location of varices is the most significant factor complicating their assessment and management, particularly when they present in unexpected sites. This case illustrates the diagnostic challenges posed by ectopic varices, which manifested as acute, severe, intermittent bleeding despite negative findings on upper endoscopic evaluation. Therefore, a diagnostic approach based on a comprehensive assessment of vascular integrity, rather than focusing solely on localized findings, is essential, especially in patients with comorbidities related to portal hypertension.

Ectopic varices are most commonly found in the duodenum but can also occur in the small bowel (jejunum and ileum), colon, parastomal sites, biliary tract, and genitourinary system[3]. Norton et al[2] reported that duodenal varices were the most common type (17%) among 169 patients with ectopic variceal bleeding, followed by varices in the jejunum and ileum (17%), colon (14%), and rectum (8%). However, data from a national survey in Japan showed differing results, with rectal varices being the most common (44.5%), followed by duodenal varices (32.9%)[8].

From a pathophysiological perspective, ectopic varices develop as a secondary consequence of portal hypertension caused by cirrhosis, extrahepatic portal vein obstruction, Budd-Chiari syndrome, portal vein thrombosis, or post-surgical shunts.

Clinically, they may present with massive or intermittent bleeding, manifesting as melena, hematochezia, or hematemesis. In some cases, they appear as obscure gastrointestinal bleeding following negative endoscopic findings.

Current therapeutic guidelines recommend individualized management strategies based on the location, bleeding activity, anatomy, and portal pressure of the ectopic varices[9]. Endoscopic therapies, including band ligation, cyanoacrylate injection, endoscopic clipping, or endoscopic ultrasound (EUS)-guided coil and glue injection, are currently available options[10-12]. For duodenal varices, EUS-guided coil and glue injection may be considered[11], while rectal varices can be treated with band ligation or sclerotherapy[13]. However, these procedures carry risks of perforation, glue embolism, and recurrence due to persistent collateral circulation.

Independency from radiation exposure as well as minimal invasiveness promotes wide spread introduction of EUS guided therapy. Since the case patient has undergone hemodynamic instability with progression of active bleeding, application of EUS guided therapy was out of consideration. Real-time manipulation with high contrast resolution enables various treatment approach in therapeutic endoscopic field including variceal therapy. EUS guided vascular therapy allows real-time visualization of variceal structure to perform minimally invasive targeted treatment with effective injection of sclerotic agents[14]. International multi-center study with propensity-matched analysis demonstrated EUS-guided coil and glue injection as safe technique with better efficacy and lower re-bleeding rates compared to the conventional endoscopic glue injection[15]. Also an observational, retrospective study presented cost-effectiveness of EUS-guided coils plus cyanoacrylate compared to cyanoacrylate injection[16]. In addition, case reports of successful hemostasis with parastomal variceal bleeding as well as Dieulafoy lesion support real-world clinical evidence of its effectiveness and safety outcome[17,18].

Interventional radiologic procedures including balloon-occluded retrograde transvenous obliteration, plug-assisted retrograde transvenous obliteration (PARTO), balloon-occluded antegrade transvenous obliteration, transhepatic embolization using microcoils and sclerosants, and transjugular intrahepatic portosystemic shunt, offer additional therapeutic options (Table 2)[5,19-22].

Table 2 Selected reports on management of ectopic varices.

Ref.	Varix site	Modality	Intervention	Outcome
Kim et al[22], 2020	Duodenum	Interventional radiology	Percutaneous trans-splenic variceal obliteration	Alternative option for balloon-occluded retrograde transvenous obliteration
Lee et al[5], 2023	Duodenum	Interventional radiology	PARTO	Successful PARTO for an isolated duodenal varix
Hau et al[24], 2014	Duodenum	Surgery	Collateral caval shunt	Surgical shunt controlled bleeding in non-cirrhotic portal hypertension
Solanki et al[23], 2021	Jejunum	Surgery	Segmental bowel resection	Effective hemostasis on localized, refractory ectopic varices

PARTO: Plug-assisted retrograde transvenous obliteration.

Surgical management, including segmental resection or shunt creation, is generally reserved for localized or refractory ectopic varices (Table 2)[23,24].

Pharmacological management includes the use of nonselective β-blockers such as propranolol and carvedilol to lower portal pressure, as well as octreotide or terlipressin to reduce splanchnic blood flow and variceal pressure[9,25].

Recent case reports have described both endoscopic and radiologic interventions for duodenal varices. Hamilton et al[26] successfully performed endoscopic band ligation, while Lee et al[5] reported a case treated with PARTO without complications.

Therefore, it is noteworthy that our case represents a remarkable outcome achieved through a combined interventional radiology and surgical approach for the management of duodenal ectopic variceal bleeding.

CONCLUSION

In conclusion, a comprehensive and systematic approach is essential for both the diagnosis and therapeutic decision-making in cases of ectopic varices. Given their potential for obscurity and high mortality, further evidence-based clinical studies are needed to guide optimal assessment and management strategies.