Gastrointestinal Hemorrhage from a Duodenal Varix Rupture: a Case Report

Case Report Page 1 of 6

Gastrointestinal hemorrhage from a duodenal varix rupture: a case report

Pieter Dries1, Michiel de Maat1, Bart De Schepper2, Olivier D’Archambeau3, Guy Hubens1, Anthony Beunis1

1Department of Abdominal, Children and Reconstructive Surgery, University Hospital (UZA), Antwerp, Belgium; 2Department of Gastroenterology, Sint Augustinus Hospital, Antwerp, Belgium; 3Department of Radiology, University hospital (UZA), Antwerp, Belgium Correspondence to: Pieter Dries. Department of Abdominal, Children and reconstructive surgery, University hospital (UZA), Antwerp, Belgium. Email: [email protected].

Abstract: A 77-year-old male with background of antithrombin-III deficiency, SMV thrombosis and duodenal ulcer was transferred to our clinic with a sudden onset, russet diarrhoea and a concomitant episode of heavy sweating. Later on, he developed severe hematemesis and haematochezia. Ultimately, he was diagnosed on CT-angiography with a hemorrhage from extensive, isolated duodenal (D3) varices. Emergency surgical ligation was performed after endoscopy could not reveal an active bleeding site and the patient had become hemodynamically unstable. Duodenotomy confirmed three protruding veins, one of which was bleeding. After the ligation, the patient stabilised and further recovery was uneventful. He was discharged from ICU on the third day after surgery and left the hospital seven days later. In a second stage, a selective endovenous embolization of the duodenal varices (DV) with cyanoacrylate (Glubran®, 3×1 mL) was performed. DV are a rare cause of gastro-intestinal bleeding but should be considered in all patients with a history of splanchnic venous thrombosis. Endoscopy plays a pivotal role in the diagnosis and acute management of DV and should be carried out with the possibility of distal DV in mind. We advise that urgent endoscopic interventions are to be carried out by experienced clinicians as this might avoid open surgery. However, surgical ligation remains a valid therapy as endoscopic diagnosis and treatment is not always possible. Guidelines, meta-analyses and high-quality reviews that compare different therapies are still lacking and therefore further research is necessary.

Keywords: Duodenal varices (DV); gastrointestinal tract bleeding; superior mesenteric vein thrombosis; portal hypertension (PHT); case report

Received: 08 June 2020; Accepted: 28 December 2020; Published: 30 December 2020. doi: 10.21037/dmr-20-86 View this article at: http://dx.doi.org/10.21037/dmr-20-86

Introduction natural portosystemic shunts and most patients have portal hypertension (PHT) (intra- or extrahepatic) with liver Duodenal varices (DV) are part of ectopic varices (EcV), cirrhosis accounting for 30% of the patients with DV (5). defined as dilated splanchnic (mesoportal) veins/varicosities and/or portosystemic collaterals in the gastrointestinal tract However, EcV can form in the absence of PHT as described except the gastroesophageal region (1). They account for in abdominal trauma, tumours, vascular anomalies and up to 17% of the EcV and are seldomly seen as prevalence thrombotic disorders (Table 1) (6,7). is reported between 0.2–0.4% in all patients undergoing Bleeding from EcV accounts for 2–5% of all upper endoscopy (2,3). DV are predominantly found in the gastrointestinal tract bleeding (8). The majority of these duodenal bulb and second part of the duodenum as their EcV bleeding is caused by peristomal (26%), duodenal frequency declines further distally (4). EcV function as (17%) and small intestinal (17%) varices (9,10). When

Table 1 Based on Bommana et al. (6) diagnosed eight years prior to presentation, possibly due to Etiology of duodenal varices in world literature a suspected antithrombin-III deficiency (Factor V Leiden

Cirrhosis of the liver and JAK2 mutation analysis both negative) for which he received anticoagulants in the six months following Hepatocellular carcinoma diagnosis. At the time of presentation, he was no longer on Hepatic vein thrombosis (e.g., Budd-Chiari syndrome) anticoagulants neither was the diagnosis of antithrombin- Inflammatory process (pancreatitis, cholangitis) III deficiency confirmed later on. Other relevant history consisted of oesophagitis grade A, duodenal ulcer and Klippel-Trenaunay-Weber syndrome hereditary hemochromatosis (adult type, HFE-gene related, Schistosomiasis homozygous mutation). Surgical Procedures (OG varices band ligation) At first, clinical investigations showed a pale man with Tumour (pancreas, periportal) normal vital signs (blood pressure 125/74 mmHg, heart rate 80/min, respiratory rate 14/min). Digital rectal examination Thrombotic disorders and Coagulopathies was normal except for melena. Blood results showed a slight Trauma anaemia (haemoglobin 9.8 g/dL, haematocrit 30.1%) and Vascular anomalies a shortened APTT of 23.5 s. All other results were within normal range. An immediate esophagogastro-duodenoscopy could not identify any protruding varices, bleeding sites, bleeding from DV occurs, mortality is reported as high blood clots or other abnormalities. The endoscopy report as 40% (11). Management guidelines on ectopic (and states introduction up to the third part of the duodenum. duodenal) variceal bleeding are still lacking although various For further evaluation of bleeding sites, urgent CT- options are available. Classic, invasive surgical approaches angiography was performed and revealed a contrast blush (ligation, duodenectomy, open shunt surgery) have been out of a varix that protruded into the lumen of duodenum largely replaced by endoscopic (banding, sclerotherapy) part 3 (Figure 1). Further, extensive varicose veins were seen procedures, especially in the acute setting. In a later stage, at the transition of duodenal parts 3-4 and a near occlusion treatment is aimed at lowering the venous pressure and of the superior mesenteric vein (due to earlier thrombosis). therefore avoiding bleeding episodes and the formation of During the following hours, the patient became new varices. Open shunt surgery remains a viable option increasingly haemodynamic instable and developed although more recent radiological interventions like hematemesis. He underwent emergency laparotomy. Transjugular Intrahepatic Portosystemic Shunt (TIPS) or Duodenotomy showed three protruding veins, one of which Balloon-occluded Anterograde/Retrograde Transvenous was actively bleeding. These were ligated after which the Obliteration (BATO/BRTO) show good results. This case patient stabilised. After three days on intensive care, non- report describes a patient with active bleeding from DV ventilated, and 7 days on a conventional ward, he was located in the distal part of the duodenum and we continue discharged. Three months later, the patient underwent to discuss the diagnostics, treatment and etiology of DV. We a selective endovenous embolization of the DV with present the following article in accordance with the CARE cyanoacrylate (Glubran®, 3×1 mL). After initial surgery, the reporting checklist (available at http://dx.doi.org/10.21037/ only temporary complaint of the treatment received was a dmr-20-86). prolonged loss of appetite. The possible complications of selective DV embolization are shown in Table 2. Lifelong anticoagulation therapy was started (warfarin, target INR Case presentation 2-3) as our patient was in a hypercoagulable state due to his A 77-year-old male presented after having a sudden antithrombin III deficiency. onset, russet diarrhoea that was preceded by an episode of All procedures performed in studies involving human heavy sweating. Symptoms started the night before and participants were in accordance with the ethical standards of continued through the morning of admission. His medical the institutional and/or national research committee(s) and history showed superior mesenteric vein thrombosis, with the Declaration of Helsinki (as revised in 2013).

A B

C D

Figure 1 CT-scan (A) shows a slim splenic vein (arrow 1) and potent portal vein (arrow 2). Furthermore, no signs of cirrhosis. Image B shows a chronic, total occlusion of the superior mesenteric vein. Duodenal varices are shown on image C (arrow 1) and venous blush in the fourth part of the duodenum (arrow 2). Note the contrast in the proximal jejunum on image B and C whilst no per oral contrast was administered, correlating with a severe venous bleeding. Extravasation of contrast in the fourth part of the duodenum is show on image 4 (arrow 1).

Table 2 Based on Zamora et al. (12) Discussion Complications of selective duodenal variceal embolization Due to their low prevalence, the diagnosis and management Minor complications of DV remains challenging and most of the literature • Abominal pain or fever consists of case reports and small population studies.

• Extravasation of the embolization agent with local reaction Possible etiology of DV are shown in Table 1 (6,7). As previously noted, DV function as portosystemic shunts to • Variceal rupture (without hemodynamic instability) relieve PHT that is present in a majority of the cases. This • prolonged bleeding at puncture sites and/or hematoma is defined as a hepatic pressure venous gradient (HPVG) Major complications above 5 mmHg, although complications usually develop

• Those requiring an increased level of medical care, above the 10–12 mmHg threshold (13). DV are more including reactions to ethanolamine oleate frequently seen in the duodenal bulb and second part of

• conversion to surgery the duodenum with their frequency declining distally (2,4). Anatomy and its variations in EcV have been extensively • prolonged hospital stay described by Sharma et al. (14). Although rare, in this case • Any event related to the procedure that produced the left gonadal vein also drains the third and fourth part of hemodynamic instability) the duodenum (14,15).

The diagnosis is often made during endoscopy for duodenectomy or open shunt surgery) has been used with upper GI-tract bleeding. However, like in our case, a lack success and proves vital in patients with bowel ischemia of visualisation of the culprit vein due to food remnants, and extensive peritonitis (21). High volume studies on the duodenal folds, extensive clothing/bleeding or endoscopy outcome of open surgery are still lacking. Salzedas-Netto in a non-bleeding interval are among the diagnostic et al. presented a case with a modified meso-Rex shunt, challenges. Furthermore, it is the authors believe that, using the internal jugular vein as a conduit between due to the low prevalence, insufficient awareness of the the splenic and the left portal vein (22). The use of this existence of EcV in the distal duodenum D3-4 might also technique is however, limited to extrahepatic portal/ contribute to a lower detection rate. Early diagnosis in splanchnic vein obstruction. high-risk patients (any form of PHT or previous endoscopic sclerotherapy procedures for gastro-intestinal varices) Conclusions can be enhanced by screening, like in patients with liver cirrhosis, and by performing a full endoscopic inspection We presented a case of a distal DV bleeding that was of the entire duodenum, including D3 and D4 (16). The successfully managed with surgical ligation and, in a later benefit of performing an endoscopy is that it allows the phase, sclerotherapy. Our case is unique because there was operator to simultaneously diagnose and try to control no underlying liver pathology and formation of venous the bleeding with clipping, ligation or sclerotherapy, collaterals, at the level of D3/D4, was based on earlier SMV albeit at a high rate of re-bleeding and ulcers (17). obstruction due to superior mesenteric vein thrombosis. Iqbal et al. report a case of fatal GI bleeding after failed As bleeding from DV is seldomly seen, diagnosis of a more attempts to clip the culprit vessel (18). Because of these distal DV in a patient without liver cirrhosis or PHT is difficulties, an accurate diagnosis might be delayed or even even more challenging. Endoscopy plays a pivotal role in missed. Therefore, contrast enhanced imaging like MRI/ the diagnosis and acute management of DV and should be CT can be of assistance not only in identifying the focus of carried out with the possibility of more distal DV in mind. bleeding but also a possible underlying diagnosis (cirrhosis We advise that urgent endoscopic interventions are to be or splanchnic vein thrombosis) and guide following carried out by experienced clinicians as this might avoid therapies (15). open surgery. Guidelines, meta-analyses and high-quality Therapeutic options are plenty: endoscopy (clipping, reviews that compare different therapies are still lacking and ligation, sclerotherapy), interventional radiology (TIPS therefore further research is necessary. and BRTO or BATO, depending on the approach) and surgical interventions (duodenectomy, venous ligation, Acknowledgments open shunt surgery) are used. First and most important is to correctly assess the level of hemodynamic instability and Funding: None. whether there is sufficient time to allow for a minimally invasive endoscopic intervention. Swift decision making is Footnote crucial and one should not hesitate to proceed to invasive surgery. Whilst TIPS and BRTO/BATO have a favourable Reporting Checklist: The authors have completed the CARE outcome in gastric varices, both techniques have a high re- reporting checklist. Available at http://dx.doi.org/10.21037/ bleeding rate in EcV (1,19). Therefore, TIPS is a mainly dmr-20-86 to be considered a first-line therapy for bridging towards transplantation or for those patients unfit for surgery. Peer Review File: Available at http://dx.doi.org/10.21037/ Procedural risk and underlying etiology should be taken dmr-20-86 into account individually. BRTO/BATO are valid options in patients who are unfit for surgery or failed endoscopy, Conflicts of Interest: All authors have completed the ICMJE especially in those with existing hepatic encephalopathy uniform disclosure form (available at http://dx.doi. (19,20). The complexity of the procedure and lack of org/10.21037/dmr-20-86). MDM serves as the unpaid expertise limits its global use with successful reports mainly editorial board member of Digestive Medicine Research from originating from Japan (20). Open surgery (ligation, April 2020 to March 2022. The other authors have no

© Digestive Medicine Research. All rights reserved. Dig Med Res 2020;3:70 | http://dx.doi.org/10.21037/dmr-20-86 Digestive Medicine Research, 2020 Page 5 of 6 conflicts of interest to declare. bleed and a difficult diagnosis to make. BMJ Case Rep 2017;2017:bcr2016218669. Ethical Statement: The authors are accountable for all 8. Larson JV, Steensma EA, Burke LH, et al. Fatal upper aspects of the work in ensuring that questions related gastrointestinal bleed arising from duodenal varices to the accuracy or integrity of any part of the work are secondary to undiagnosed portal hypertension. BMJ Case appropriately investigated and resolved. All procedures Rep 2013;2013:bcr2013200194. performed in studies involving human participants 9. Helmy A, Al Kahtani K, Al Fadda M. Updates in the were in accordance with the ethical standards of the pathogenesis, diagnosis and management of ectopic institutional and/or national research committee(s) and varices. Hepatol Int 2008;2:322-34. with the Declaration of Helsinki (as revised in 2013). 10. Malik A, Junglee N, Khan A, et al. Duodenal varices Written informed consent was obtained from the patient successfully treated with cyanoacrylate injection therapy. for publication of this Case report and any accompanying BMJ Case Rep 2011;2011:bcr0220113913. images. 11. Khouqeer F, Morrow C, Jordan P. Duodenal varices as a cause of massive upper gastrointestinal bleeding. Surgery Open Access Statement: This is an Open Access article 1987;102:548-52. distributed in accordance with the Creative Commons 12. Zamora CA, Sugimoto K, Tsurusaki M, et al. Endovascular Attribution-NonCommercial-NoDerivs 4.0 International obliteration of bleeding duodenal varices in patients with License (CC BY-NC-ND 4.0), which permits the non- liver cirrhosis. Eur Radiol 2006;16:73-9. commercial replication and distribution of the article with 13. Vizzutti F, Schepis F, Arena U, et al. Transjugular the strict proviso that no changes or edits are made and the intrahepatic portosystemic shunt (TIPS): current original work is properly cited (including links to both the indications and strategies to improve the outcomes. Intern formal publication through the relevant DOI and the license). Emerg Med 2020;15:37-48. See: https://creativecommons.org/licenses/by-nc-nd/4.0/. 14. Sharma M, Rameshbabu CS. Collateral pathways in portal hypertension. J Clin Exp Hepatol 2012;2:338-52. 15. Bhat AP, Davis RM, Bryan WD. A rare case of bleeding References duodenal varices from superior mesenteric vein 1. Saad WE, Lippert A, Saad NE, et al. Ectopic varices: obstruction -treated with transhepatic recanalization and anatomical classification, hemodynamic classification, stent placement. Indian J Radiol Imaging 2019;29:313-7. and hemodynamic-based management. Tech Vasc Interv 16. Jakab SS, Garcia-Tsao G. Screening and Surveillance of Radiol 2013;16:158-75. Varices in Patients With Cirrhosis. Clin Gastroenterol 2. Hashizume M, Tanoue K, Ohta M, et al. Vascular anatomy Hepatol 2019;17:26-9. of duodenal varices: angiographic and histopathological 17. Jonnalagadda SS, Quiason S, Smith OJ. Successful therapy assessments. Am J Gastroenterol 1993;88:1942-5. of bleeding duodenal varices by TIPS after failure of 3. Al-Mofarreh M, Al-Moagel-Alfarag M, Ashoor T, et al. sclerotherapy. Am J Gastroenterol 1998;93:272-4. Duodenal varices. Report of 13 cases. Z Gastroenterol 18. Iqbal S, Likhtshteyn M, O'Brien D, et al. Duodenal 1986;24:673-80. Varix Rupture - A Rare Cause of Fatal Gastrointestinal 4. Tanaka T, Kato K, Taniguchi T, et al. A case of ruptured Hemorrhage: A Case Report and Review of Literature. duodenal varices and review of the literature. Jpn J Surg Am J Med Case Rep 2019;7:62-6. 1988;18:595-600. 19. Mukund A, Deogaonkar G, Rajesh S, et al. Safety and 5. Sato T, Akaike J, Toyota J, et al. Clinicopathological Efficacy of Sodium Tetradecyl Sulfate and Lipiodol Foam features and treatment of ectopic varices with portal in Balloon-Occluded Retrograde Transvenous Obliteration hypertension. Int J Hepatol 2011;2011:960720. (BRTO) for Large Porto-Systemic Shunts. Cardiovasc 6. Bommana V, Shah P, Kometa M, et al. A Case of Isolated Intervent Radiol 2017;40:1010-6. Duodenal Varices Secondary to Chronic Pancreatitis with 20. de Franchis R, Baveno VIF. Expanding consensus in Review of Literature. Gastroenterology Res 2010;3:281-6. portal hypertension: Report of the Baveno VI Consensus 7. Bhagani S, Winters C, Moreea S. Duodenal variceal Workshop: Stratifying risk and individualizing care for bleed: an unusual cause of upper gastrointestinal portal hypertension. J Hepatol 2015;63:743-52.

21. Rajesh S, Mukund A, Arora A. Imaging Diagnosis of Variation of the Rex shunt for treating concurrent Splanchnic Venous Thrombosis. Gastroenterol Res Pract obstruction of the portal and superior mesenteric veins. J 2015;2015:101029. Pediatr Surg 2011;46:2018-20. 22. Salzedas-Netto AA, Duarte AA, Linhares MM, et al.

doi: 10.21037/dmr-20-86 Cite this article as: Dries P, de Maat M, De Schepper B, D’Archambeau O, Hubens G, Beunis A. Gastrointestinal hemorrhage from a duodenal varix rupture: a case report. Dig Med Res 2020;3:70.