Duodenal varices are relatively rare, present an uncommon endoscopic finding and an unusual surgical problem (1). Hemorrhage associated with duodenal varices is a rare, but serious and often fatal event (1-3).
The definitive treatment for bleeding duodenal varices has not been established yet. Up to date, a variety of treatment options for bleeding duodenal varices have been proposed including endoscopic injectional sclerotherapy (EIS) (3-9), endoscopic rubber band ligation (EBL) (10-12), transjugular intrahepatic portosystemic shunt (TIPS) (13,14), balloon-occluded retrograde transvenous obliteration (BRTO) (10,15-17). However, surgical procedures such as portosystemic shunting (18,19) and direct operation (simple oversewing of the duodenal varices through duodenotomy, resection of a segment of the bleeding site, duodenal dearterialization with stapler) were reserved for endoscopic failure cases (20,21). There is no consensus regarding the best treatment method for bleeding duodenal varices and management policy depends on physician’s experience and is decided on a case-by-case basis.
We report the first case of bleeding duodenal varices successfully managed with endoscopical mini-loop ligation. The pathophysiological characteristics of these ectopic vatices, as well as the therapeutic options are discussed in the context of the available relevant literature.
A 47-year-old female was admitted to our unit with a 13 hours history of hematemesis, melena and orthostatic hypotension. The patient had a history of viral hepatitis B liver cirrhosis since 2003.
On admission to the intensive care unit (ICU) her vital signs were as follows: blood pressure 90/60 mm Hg, heart rate 120 beats/min, respiration 20/min. Abdominal examination revealed no surgical scars and tenderness, moderate ascites was determined. No hepatomegaly was established. Rectal examination revealed melena. The laboratory values on admission were: hemoglobin 6.4 g/dl; red blood cell count 2.1X1012/L; white blood cell count 6.8X109/L; hematocrit – 31%, total protein 57 g/l; total bilirubin 73 mmol/l; aspartate aminotransferase 64 U/L; alanine aminotransferase 30 U/L. Liver function test revealed Child-Pugh B class.
Initial emergency upper esophagogastroduodenoscopy (FEGDS) revealed small non-bleeding esophageal varices (F1 Li RCS -) (22), and a large amount of blood and clots in the stomach and duodenum, as well as large nodular varices with spurting bleeding in the first portion of the duodenum (Fig.1 a, b). Endoscopic hemostasis was obtained using a ligating device (HX-21L-1; Olympus Optical Co., Ltd., Tokyo, Japan), and five detachable nylon loops (MAJ-339; Olympus) (Fig.1 c, d). The bleeding varix was ligated first using one endo-loop and four aditional loops were aplied on the non-bleeding duodenal varices. The patient’s hemo-globin level was stable and there was no evidence of rebleeding episodes and she was discharged 9 days following the admission. Doppler ultrasound revealed portal vein cavernous transformation. FEGDS performed four weeks later demonstrated complete duodenal varices eradication and scars on the ligation sites (F0 RCS- Sc+) (22). The patient is free of upper gastrointestinal (GI) bleeding during a 6 months follow-up period.
Alberti first described duodenal varices in 1931 (23), and since then, a PubMed search revealed approximately 160 cases of these ectopic varices being reported in the literature.
Duodenal varices are rare, have no clinical manifestations and are usually found incidentally at FEGDS. When the duodenal varices present symptoms, these are always the symptoms of an active, massive and life-threatening bleeding (2, 6, 10, 24, 25). It is a well established fact that duodenal varices are more likely to appear in those patients with portal hypertension in whom the esophageal varices have been eradicated either by EBL or EIS (8).
From the anatomical point of view duodenal varices represent an ectopic portosystemic shunt (19). The anatomical features of the duodenal varices were described by Hashizume M et al (26), who noted that duodenal varices were typically located in the submucosal layer of the posterior wall in the second and third portions of the duodenum and consist of an afferent and an efferent vessel. The afferent vessel usually emerges from the superior or the inferior pancreaticoduodenal vein originating from the portal vein or the superior mesenteric vein while the efferent vessel drains through the Retzius’s veins into the inferior vena cava (26). On the other hand, previously published reports describe deeper located duodenal varices, mainly on the serosa of the duodenum, thus hemorrhage from these ectopic varices appears to be rare (27).
According to the endoscopic findings, the duodenal bulb is the most common site of ectopic varices, same as in our patient, while the second portion of duodenum appears to be the next most common location of the duodenal varices (2,18).
Generally, duodenal varices appear as a result of extra-hepatic portal hypertension and about 50% of these develop as the result of portal or splenic vein obstruction (26,28,29). The most common cause of splenic or portal vein obstruction is considered to be thrombosis, infection and tumors (26). The most common intrahepatic cause of duodenal varices is considered to be liver cirrhosis, accounting about 30% of all reported cases (2,4,6,9,13), as well as diffuse hepatoma (18).
Exceptionally, duodenal varices can appear as a result of localized portal hypertension (fistula between the hepatic artery and the portal vein) (30,31). Duodenal varices can occur as a result of splenic or superior mesenteric vein obstruction, providing an alternative pathway around the obstruction, connecting branches of the superior mesenteric or splenic vein upstream to reconstitute intrahepatic blood flow (6,32).
Successful treatment of bleeding duodenal varices has been reported with EIS with various agents, such as human thrombin (33,34), sodium tetradecyl sulfate (6), N-butyl-2-cyanoacrylate (7,9,35) and ethanolamine oleate (36). On the other hand, EIS may result in severe complications, because the duodenal wall is thinner than the esophageal wall and the esophageal varices are usually submucosal, while the duodenal varices are located mainly outside the duodenal wall resulting in a high risk of penetration and perforation of the duodenal wall with repeated EIS (36,37). Moreover, EIS with conventional sclerosants may not occlude immediately the variceal lumen and could induce sclerosant leakage into the systemic circulation because duodenal varices represent a major portosystemic shunt with rapid and abundant blood flow (1,28,36).
Recently, occasional reports of bleeding duodenal varices treatment by endoscopic clipping and EBL were published (1,10-12), but the results are controversial. These endoscopic methods result in little damage to the local tissue unlike the EIS, but the risk of the major duodenal papilla damage due to poor visualization through the banding chamber must be considered (12). However, taking into consideration the potential risks of the above described methods (early slippage of the rubber bands) (38), we hypnotized that endo-loop is more suitable for duodenal varices treatment.
Interventional radiology techniques (IVR) by BRTO or TIPS in the cases of failed endoscopic hemostasis have been advocated for the treatment of duodenal variceal bleeding (10,13-17,39). Most recent reports described successful control of bleeding duodenal varices by TIPS (13, 14), and BRTO (10,15-17,39). However, IVR demand considerable technical skill and are not suitable for emergency cases (1).
Up to day surgery is the ultimate option for duodenal varices and is indicated when endoscopical hemostasis fails to control bleeding (18,19,21,32). Surgical management for bleeding duodenal varices include variceal ligation (32), variceal resection, ligation of the gastroduodenal and splenic arteries, splenectomy, stapling of the duodenum and gastro-enterostomy (21) as well as porto-systemic shunt surgery (18,19), but the reported death rate after the surgical treatment of duodenal varices is 30 – 40% (1,32). The same authors recommend these patients to be further managed by installing a surgical portosystemic shunt, when ligation of the bleeding duodenal varices or duodenal resection fails to control the bleeding (18,19,32); a serious surgical challenge represent the patients with “unshuntable” portal hypertension (complete splenomesoportal thrombosis).
Furthermore, the rebleeding rate after surgery is high, Khouqeer et al report a rebleeding rate of 57% after variceal ligation and 40% after duodenal resection (32).
In summary, bleeding duodenal varices are an uncommon situation and there are no randomized studies concerning the optimal treatment modality of this dramatic condition. Our case report suggest mini-loop as a possible treatment option for bleeding duodenal varices. It is not clear which treatment method is more appropriate, and the literature review demonstrates that the management of this clinical entity depends on the physician’s experience, patient’s condition, and additional clinical material must be collected before the final conclusions will be reached.
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