Alcoholic liver cirrhosis complicated by duodenal and small intestinal variceal rupture recurrent bleeding: a case report of unusual ectopic varices

Introduction

Ectopic varicose veins are varicose veins occurring in the digestive tract except the esophagus and the fundus of the stomach (1). Ectopic varicose veins include duodenal varicose veins, jejunal varicose veins, ileum varicose veins, small intestine varicose veins, colon varicose veins, rectal varicose veins, biliary tract varicose veins, peritoneal varicose veins, anastomotic varicose veins, etc. (2). There are two main causes of portal hypertension in cirrhosis and non-cirrhotic portal hypertension. Ectopic varicose veins bleeding accounts for 1–5% of all variceal bleeding. Compared with esophageal and gastric variceal bleeding, ectopic varicose veins bleeding is less common in patients with portal hypertension, but the risk of ectopic variceal bleeding increases by four times, and the mortality rate can be as high as 40%. In addition, ectopic variceal bleeding is usually large and life-threatening (3-5). Ectopic varicose veins can occur simultaneously in multiple sites. Treatment should address portal hypertension from the root cause. Merely performing simple devascularization may aggravate the degree of varicose veins in other sites. We present this case in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-24-278/rc).

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. This article does not involve any identifiable personal information. Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.

A 58-year-old male was admitted to The Second Hospital of Hebei Medical University (2023.5.30) with a history of intermittent hematemesis for over 10 years and melena for 10 days (2023.5.20) (Figure 1). The patient had been diagnosed with “alcoholic cirrhosis” more than a decade ago. Ten days prior to admission, he experienced hematemesis, melena, palpitations, dizziness, and transient loss of consciousness following the ingestion of “ibuprofen” for toothache and consumption of biscuits. On admission, physical examination revealed an anemic appearance, with pale conjunctivae and lips. The abdomen was soft, without tenderness, rebound tenderness, or muscle guarding. No tenderness was noted in the hepatic region. Laboratory tests showed the following results: albumin 30.5 g/L, hemoglobin 62 g/L, and platelet count 69×10⁹/L. Emergency gastroscopy demonstrated mild esophageal varices and duodenal varices with local mucosal injury and red thrombi, which were suspected to be the source of bleeding (Figure 2). The patient underwent endoscopic sclerotherapy targeting the duodenal varices. Emergency colonoscopy revealed the presence of hemorrhagic fluid and blood clots. Despite daily supportive care, including blood transfusions, following the sclerotherapy for duodenal varices, the patient continued to experience intermittent hematochezia, and his hemoglobin levels progressively decreased.

Figure 1 A timeline figure.

Figure 2 Under endoscopy, a blue vein at the descending junction of the duodenal bulb was tortuously dilated, mucosal injury and red thrombus were locally visible, and no obvious bleeding was observed. Sclerotherapy was performed later.

This clinical course raised the suspicion of ongoing bleeding from the small intestine. This case highlights the complexity of managing gastrointestinal bleeding in patients with cirrhosis and portal hypertension. The presence of both esophageal and duodenal varices underscores the need for a comprehensive and individualized approach to treatment. The persistence of bleeding despite endoscopic intervention and supportive care suggests the possibility of additional bleeding sources, such as from the small intestine, which may require further diagnostic and therapeutic interventions.

Abdominal computed tomography (CT) imaging revealed the presence of ectopic varicose veins (Figure 3A,3B). Given the diagnosis, transjugular intrahepatic portosystemic shunt (TIPS) was considered as a potential intervention to reduce portal pressure. However, the patient was found to have cavernous transformation of the portal vein, poor vascular conditions, and multiple portal vein thromboses (Figure 3C,3D). These findings posed significant challenges, as surgical intervention carried the risk of exacerbating thrombosis. Moreover, the presence of portal vein thrombosis limited the availability of suitable target vessels for TIPS placement.

Figure 3 Imaging data. (A,B) Ectopic varicose veins on enhanced CT (red arrows). (C,D) Cavernous transformation of the portal vein with portal vein thrombosis on abdominal enhanced CT (blue arrows). CT, computed tomography.

To further localize the source of bleeding, enteroscopy was performed, which demonstrated jejunal varicose veins but no clear bleeding focus (Figure 4). Despite these efforts, the patient’s hematochezia remained uncontrolled, and his hemoglobin levels continued to decline, indicating ongoing active bleeding.

Figure 4 Varicose jejunum veins were seen in the enteroscopy, but no definite bleeding site was seen.

Follow-up gastroscopy after sclerotherapy showed improvement in the varices at the previously treated site (Figure 5). Capsule endoscopy was subsequently performed, revealing red—stained intestinal fluid in the region of the jejunal varices (2023.5.30–2023.6.9). Given the persistence of bleeding and the findings on imaging, the possibility of bleeding from colonic varices was considered. Consequently, the patient was transferred to the Department of Gastrointestinal Surgery for preparation of laparoscopic exploration to further investigate and manage the source of bleeding.

Figure 5 The gastroscope was reexamined, and the varicose veins in the original treatment site were better than before.

Surgical findings and management (2025.6.15)

During the exploratory laparoscopy, approximately 45 cm distal to the ligament of Treitz, the small intestine was found to be adhered and fixed in the splenic fossa. The patient had a history of subtotal gastrectomy and splenectomy due to previous esophagogastric variceal bleeding. The adhesions were likely related to his prior surgical interventions. After carefully loosening the adhesions, clusters of varicose veins were observed within the intestinal wall and the corresponding mesentery. No abnormalities were noted in other intestinal segments, leading to the conclusion that the patient’s bleeding originated from small intestinal varices.

Subsequently, the affected mesentery and intestinal segments were meticulously addressed. The small intestine was resected 10 cm from both the proximal and distal ends of the varicose vein cluster. The surgical procedure was completed smoothly, and the resected specimen was sent for pathological examination. Histopathological analysis of the resected specimen revealed chronic inflammation of the small intestinal mucosa. The mesentery exhibited numerous vessels with varying lumen sizes and wall thicknesses. Some vessels showed dilatation and congestion with evidence of bleeding, while others contained organized thrombi (Figure 6). These findings further confirmed that the patient’s persistent bleeding was due to small intestinal varices secondary to portal hypertension.

Figure 6 Intraoperative and pathological findings showing varicose veins. The blue arrow indicates ectopic varicose veins. Pathological staining was HE staining with a magnification of 40× objective. HE, hematoxylin and eosin.

After surgery: bleeding once improved, stool turned yellow, but not for a long time, intermittent black stool accompanied by aggravated anemia again. Endoscopic examination was performed again: after treatment with hardener for duodenal varicose veins, varicose veins at the original treatment site were tortuous and dilated again, accompanied by bleeding (Figure 7A). After sealing the vessels with titanium clips, tissue glue was injected (Figure 7B). After the patient’s stool turned yellow, the patient resumed a normal diet, recovered, and was discharged from the hospital. Later telephone follow-up: the patient did not appear black stool again, laboratory tests and others are normal.

Figure 7 Changes in duodenal varices after surgery. (A) The duodenal varices are thicker than before. (B) After the vessels were closed with titanium clips, tissue glue was injected.

Discussion

Small intestine varicose veins are a common type in ectopic varicose veins. Varicose veins in this patient were considered to be related to the reduction of the portal shunt after esophagogastric subfundotomy. Although esophagogastric variceal bleeding is temporarily controlled after cardio-subtotal gastrectomy, the natural course of portal hypertension was not blocked, and new varicose veins would still form. Duodenal varicose veins are the most bleeding ectopic varicose veins. The jejunum, located below the Trez ligament, is also the natural access to varicose veins. The patient developed duodenal and jejunal varicose veins at the same time. Moreover, after surgical resection of the jejunal varicose veins, the duodenal varicose veins were more advanced. In clinical practice, due to the special location of small intestine, it is difficult to detect by general gastroscopy and colonoscopy, especially because the amount of bleeding is relatively small, which is often ignored by us. When repeated electronic gastroscopy and colonoscopy failed to find a clear bleeding site in clinical practice, the small intestine should be examined, and the preferred examination methods include abdominal enhanced CT, enteroscopy, capsule endoscopy, etc. Most hemorrhagic jejunal varicose veins and ileal varicose veins are usually found in abdominal surgery, and the condition is more serious due to the difficulty in early diagnosis. The literature has also noted that the triad of portal hypertension, hematochezia without hematemesis, and previous abdominal surgery is characteristic of small intestinal varices (6). And with the presence of larger esophageal varices, ascites, portal gastropathy, or colon disease, and the increase in the Child-Pugh classification, the likelihood of small intestinal varices increases (7-9). Our case has a history of portal hypertension due to alcoholic cirrhosis, splenectomy, and cardiac-subtotal gastrectomy. The patient was admitted because of both duodenal and jejunal varices.

In recent years, more and more attention has been paid to the study of the risk factors of ectopic varicose veins due to the high rate of bleeding and mortality. In a study conducted by Watanabe et al., a total of 137 patients with ectopic varices were included, 94.8% of the patients with rectal varices had a history of esophageal varices, and 87.0% of them had received previous treatment for esophageal varices. It was concluded that esophageal varices treatment was a risk factor for the occurrence of rectal varices (10). Through the review and analysis of previous relevant studies, He et al. proposed that portal gastropathy, eradicated esophageal varices, and a history of abdominal and pelvic surgery might be possible risk factors for ectopic varices (11). Research on the risk factors of ectopic varices is particularly important for guiding the early detection of high-risk groups, timely and effective prevention, and treatment. However, no relevant consensus has been formed so far, and further research is needed (12).

For ectopic varicose veins, we commonly use digestive endoscopy, which is the most intuitive and preferred examination method for diagnosis, and endoscopic treatment can also be performed. Enhanced CT scan is more accurate to identify ectopic varicose veins. In our case, the presence of ectopic varicose veins was found on enhanced CT scan. In addition, there are ultrasound, mesenteric angiography, magnetic resonance, etc., but there are some limitations.

In terms of treatment, duodenal varices are usually treated with endoscopic variceal ligation (EVL) or sclerosing agent/tissue glue injection. Relevant studies also show that there is no significant difference in hemostasis rate, rebleeding rate, mortality rate, and adverse event rate between the EVL group and the endoscopic injection sclerotherapy (EIS) group, indicating that the efficacy and safety of the two endoscopic techniques in the treatment of ectopic varicose veins bleeding are comparable (13). EUS-guided ligation or tissue glue/hardener can also be used to treat ectopic varicose veins bleeding. Studies have shown that endoscopic ultrasound (EUS)-guided spring coil injection with or without tissue glue injection is a good choice in the treatment of ectopic varicose veins bleeding when traditional treatment methods fail or there are contraindications (14). Of course, for refractory empty and ileal variceal bleeding, due to its special location, endoscopic treatment is difficult, and surgical treatment is often required. When the first gastroscopy was performed in this case, it was found that the varicose veins of the duodenum were treated with sclerosis, but the patient still had intermittent hematochezia and a continuous decline in hemoglobin, and then, it was determined whether there were other bleeding sites. The enteroscopy and capsule endoscopy were improved again, and it was found that the patient was suspected to have varicose veins in other parts of the small intestine. After the intestinal segment of small intestinal varices was surgically removed, the patient’s condition improved temporarily, but later black stools recurred and hemoglobin decreased again. The cause of portal hypertension was not solved, leading to the recurrence of duodenal varices rupture and bleeding. Relevant literature indicates that the current endoscopic tissue glue injection treatment for duodenal varices is relatively safe and effective. It can be used as a first-line treatment, but there are risks of pulmonary embolism, hilar splenic vein embolism, and cerebrovascular accidents in clinical practice. Routine gastroscopy can only reach the descending part of the duodenum, so varicose veins below the descending part of the duodenum are not suitable for EVL (15). In recent years, TIPS and other portal shunt procedures have also been used for the treatment of portal hypertension complicated with ectopic varicose veins (16-18). We also initially considered using TIPS to reduce portal pressure in the patient to control bleeding from ruptured ectopic varices, but this was not performed successfully because of the patient’s portal vein thrombosis. Due to the limited clinical data, the specific efficacy needs to be further studied. Clinically, for patients with multi-site varicose vein rupture and hemorrhage, we should adopt different treatment plans according to the characteristics of different varicose vein sites, and should actively conduct multidisciplinary joint diagnosis and treatment, especially small intestine varicose veins, which are difficult to detect early, usually need to be combined with imaging and colonoscopy to assist diagnosis, and often need to be treated with surgery.

Outlook

Ectopic varicose vein is an important complication of portal hypertension. Due to the small number of clinical cases, the relevant research is not perfect at present. However, due to its high mortality after rupture and bleeding, and the difficulty of early detection, timely and effective treatment cannot be taken, more and more people begin to pay attention to ectopic varicose veins. A multidisciplinary approach is essential for the short - and long-term preventive management of ectopic varicose veins. Clinical awareness of the possible presence of ectopic varicose veins is essential for early detection and management in patients with gastrointestinal bleeding whose source is uncertain. There is also no standard treatment for external cephalic version (ECV). Endoscopic treatment should be individualized according to the ECV of different sites.

Conclusions

Ectopic varicose vein is an important complication of portal hypertension. In clinical practice, the risk factors of ectopic varicose veins should be studied, and effective primary prevention should be carried out for high-risk populations.

Acknowledgments

Thanks for the support of the Department of Gastroenterology and The Second Hospital of Hebei Medical University. The authors are also grateful to teachers and friends for their valuable assistance.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-278/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-278/prf

Funding: This study was supported by the Hebei Natural Science Foundation (No. H2023206912).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-278/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. This article does not involve any identifiable personal information. Written informed consent for publication of this case report and accompanying images was not obtained from the patient or the relatives after all possible attempts were made.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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