Ectopic Varices Research Articles

A73 PHARYNGEAL VARICES AS A COMPLICATION OF PORTAL HYPERTENSION: A CASE SERIES

Abstract Background One of the complications of portal hypertension, with or without the presence of cirrhosis, is the development of varices along the length of the gastrointestinal tract. The commonest sites are along the esophagus or in the stomach. Ectopic varices in the small and large bowels can also be observed, but ectopic varices in the pharynx are extremely uncommon. Aims To present a case series and review the literature regarding pharyngeal varcies. Methods - Results Three elderly female patients presented for esophagogastric varices surveillance gastroscopy were diagnosed with pharyngeal varices. One patient has hepatitis C cirrhosis, while the other two non-cirrhotic patients have myeloproliferative neoplasm (MPN). None of the patients had thromboses of the portal vein or its tributaries. All three patient have concomitant esophageal varices, but only one required band ligation of her esophageal varices. All patients are asymptotic except for mild dysphagia. No patient has bled from their pharyngeal varices to date. Two patients have had prophylactic treatment of their portal hypertension with non-selective beta blocker (NSBB), while the third one has not received NSBB prophylaxis because of her age. Conclusions Pharyngeal varices are extremely rare. To date, there are three case reports in the literature, however, we have been able to identify three cases in our practice. The previous two cases reported possible left-sided portal hypertension with splenic vein thrombosis, leading to the development of collateral vessels including a gastrocaval shunt, which by some contiguous route connects to the brachiocephalic vein; and a third case was a complication of neck dissection surgery. In our case series, none of our patients had splenic vein thrombosis. However, none of them has had a careful CT angiogram to delineate the portal vein tributaries and the collateral vessels, which may further help to define their pathogenesis. It is unclear whether NSBB would be effective as primary prophylaxis against their bleeding, The plan is to continue to monitor these patients to learn about the natural history of these pharyngeal varices. Funding Agencies None

A174 RARE BUT DEADLY: AN APPROACH TO MANAGEMENT OF DUODENAL VARICEAL HEMORRHAGE

Abstract Background Common etiologies of upper gastrointestinal bleeding (UGIB) in cirrhotic patients with portal hypertension include gastroesophageal varices (GOV), portal hypertensive gastropathy (PHG) and gastric antral vascular ectasia (GAVE). Less commonly, patients with portal hypertension develop varices in ectopic sites, including the rectum, biliary tree and duodenum. Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Aims To describe the management of duodenal variceal hemorrhage. Methods A case report and literature review was performed. Results Case report: We present a case of recurrent UGIB in a 59-year-old male with decompensated cirrhosis due to non-alcoholic steatohepatitis (CP-C; Meld 14). Initial endoscopy was negative for GOV, peptic ulcer, PHG, and GAVE, but an erosion over a mucosal bulge in the third segment of the duodenum was identified, raising possibility of vascular malformation versus ectopic varix. There was active bleeding after water provocation, so clips were deployed for hemostasis. CT imaging showed mesenteric venous collaterals abutting the duodenum, again raising possibility of duodenal varix, which was ultimately confirmed by endoscopic ultrasound (EUS). Patient had recurrent overt bleeding despite beta-blockage for prophylaxis, endoscopic clipping on four separate occasions, attempted angioembolization by interventional radiology, and cyanoacrylate glue. Transjugular intrahepatic porto-systemic shunt (TIPS) was not possible due to portal vein occlusion, so he underwent EUS-guided cyanoacrylate glue a second time. Literature review: Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Duodenal variceal hemorrhage can lead to hemorrhagic shock, and is potentially life threatening, with quoted mortality rates of 40%. Unfortunately, duodenal varices can be difficult to identify. Diagnosis is often delayed due to a combination of lower awareness and endoscopic challenges given the unusual serosal and submucosal location. Evidence-based guidelines for the management of ectopic varices are limited. For this reason, our current management strategies rely heavily on local expertise. Splanchnic vasoconstrictor medication, endoscopic ligation, EUS guided gluing, interventional radiology guided embolization, TIPS, balloon retrograde transvenous obliteration and surgical shunts are potential therapeutic options to manage the acutely bleeding varix. Following a variceal bleed, liver transplantation should be considered in eligible patients with no other contraindications. Conclusions Duodenal varices are a rare, potentially fatal, and underrecognized cause of gastrointestinal bleeding in patients with portal hypertension. Definitive therapy currently relies upon local expertise in the absence of clear guideline-based therapy. Funding Agencies None

Sigmoid Ectopic Varix Complicating Primitive Biliary Cholongitis Treated by Sclerotherapy

Sigmoid Ectopic Varix Complicating Primitive Biliary Cholongitis Treated by Sclerotherapy

Bleeding from gastrointestinal ectopic varices is not associated with haemorrhage from oesophageal or gastric varices.

IntroductionEctopic varices are those that appear in a different region of the gastroesophageal junction. Bleeding from ectopic varices is rare but is usually massive and deadly.AimTo identify the possible factors that cause bleeding from ectopic varices in patients with portal hypertension.Material and methodsA cross-sectional and retrospective study; the data were collected between January 2004 and June 2014. We included patients with portal hypertension and gastrointestinal ectopic varices diagnosed by endoscopy.ResultsWe found 31 patients with gastrointestinal ectopic varices. Of these, 25 had liver cirrhosis, and six showed non-cirrhotic portal hypertension. There were 16 men and 15 women in the study. The median age of the patients was 60 years (range minimum–maximum of 27 to 80 years). Nineteen (61%) patients had rectal varices, 10 (32%) had duodenal varices, 1 (3%) had ileal varices, and 1 (3%) had colonic varices. We found bleeding in 4 (13%) of the 31 patients with ectopic varices; two belonged to the cirrhosis group, and the other two were from the non-cirrhotic portal hypertension group. Three of the 4 patients with bleeding from gastrointestinal ectopic varices had exhibited haemorrhage from oesophageal varices (odds ratio = 4.09, 95% CI: 0.37–44.78, p = 0.249), but none of them showed bleeding from gastric varices.ConclusionsBleeding from gastrointestinal ectopic varices is not necessarily associated with bleeding from oesophageal or gastric varices.

Transjugular intrahepatic portosystemic shunt (TIPS): current indications and strategies to improve the outcomes.

Transjugular intrahepatic portosystemic shunt (TIPS) represents a very effective treatment of complications of portal hypertension. Established indications to TIPS in cirrhotic patients include portal hypertensive bleeding and refractory ascites. Over the years additional indications have been proposed, such as the treatment of vascular disease of the liver, hepatic hydrothorax, hepatorenal syndrome and bleeding from ectopic varices. Indications under evaluation include treatment of portal hypertension prior to major abdominal surgery and treatment of portal vein thrombosis. In spite of these advances, there are still uncertainties regarding the appropriate workup for patients to be scheduled for TIPS. Moreover, prevention and management of post-TIPS complications including hepatic encephalopathy and heart failure are still suboptimal. These issues are particularly relevant considering aging in TIPS candidates in Western countries. Correct selection of patients is mandatory to prevent complications which may eventually frustrate the good hemodynamic results and worsen the patient's quality of life or even life expectancy. The possible role of small diameter TIPS to prevent post-procedural complications is discussed.

Clinical characteristics and efficacy of endoscopic treatment of gastrointestinal ectopic varices: A single-center study.

Background:Ectopic varices (EcVs) may cause massive bleeding, which can be difficult to control, with a high rate of mortality. The purpose of this study was to analyze the clinical characteristics of EcVs and the efficacy of endoscopic treatment.Methods:From January 2008 to July 2017, the clinical data of 150 patients with EcVs in our center were retrospectively collected and analyzed.Results:One hundred and fifty patients with EcVs (male 74.7%), with a mean age of: 54.1 ± 14.6 years were included. The prevalence of EcVs was 0.92% in gastrointestinal varices. Cirrhosis was the most common cause of EcVs (67.0%). The rates of bleeding were 57.14%, 4.34%, 30.0%, 33.3%, and 100% in the duodenal varices rectal varices, colonic varices, anastomotic varices, and small bowel varices, respectively. An age under 55 years, varices in the duodenum, and erythema were considered risk factors for EcV bleeding. Endoscopic treatments were performed in 15 patients with EcV bleeding. The follow-up period of the patients who underwent endoscopic treatment ranged from 0.5 to 24 months. The overall rate of treatment success was 73.33% for endoscopic treatment of EcV bleeding. The overall rates of rebleeding and mortality during 2 years were as high as 53.3% and 26.7%, respectively.Conclusion:Age, erythema, and the location of EcVs are considered risk factors for EcV bleeding, and the rate of bleeding is higher in patients with duodenal varices than in those with other EcVs. Endoscopic treatment is safe, effective, and feasible for controlling EcV bleeding.

Ectopic Ascending Colonic Variceal Bleeding Treated with Balloon-Occluded Retrograde Transvenous Obliteration in a Decompensated Liver Cirrhosis Patient—A Hepatology Perspective

Ectopic colonic varices development from liver cirrhosis and portal hypertension is uncommon. They are part of the spectrum of portal hypertensive colopathy. Colonic variceal bleeding remains a rare cause of lower gastrointestinal tract (GI) bleeding. Due to the paucity of cases, there are no well-established conventional treatments for bleeding colonic varices. Different treatments have been reported. Here, we report a case of a 55-year-old gentleman, with a history of alcoholic liver cirrhosis, presenting with severe lower GI bleeding and symptomatic anaemia. An esophagogastroduodenoscopy revealed large esophageal varices with high-risk bleeding stigmata requiring endoscopic variceal ligation. A cross-sectional computed tomography scan showed colonic portosystemic shunts. In light of this and that the severe lower GI bleeding seemed out of proportion to the esophageal varices seen on upper endoscopy, an urgent unprepped colonoscopy was performed which revealed possible bleeding diverticula disease which required endoscopic mechanical hemoclip therapy. However, despite this, patient had recurrence of lower GI bleeding prompting a second colonoscopy. This relook colonoscopy showed ectopic ascending colon varices with high-risk bleeding stigmata. High-dose intravenous vasoactive agent somatostatin (500 mcg/hour) and subsequently terlipressin (2 mg every 4 hours) were used. The patient subsequently underwent successful balloon-occluded retrograde transvenous obliteration (B-RTO) and sclerotherapy. The non-selective beta-blocker (NSBB) carvedilol was started and bridged together with the vasoactive agent until stabilisation of portal hypertension. This difficult case illustrates the dynamic nature of portal hypertensive bleeding. It also highlights the presence of confounding non-variceal pathology complicating diagnosis of portal hypertensive colonic variceal bleeding, and that ectopic ascending colonic variceal bleeding can be treated successfully with B-RTO and sclerotherapy, with meticulous titration of high-dose vasoactive agents and NSBB, in a decompensated alcoholic liver cirrhosis patient.

Not so innocent bystander - gallbladder varices without portal vein thrombosis

Introduction. Gallbladder varices (GBV) represent a rare form of ectopic varices that usually occur in patients with portal hypertension and portal vein thrombosis. Case outline. We present a case of a 38-year-old woman with decompensated autoimmune liver cirrhosis who was referred to our institution for evaluation for liver transplantation. She was incidentally discovered to have GBV during a routine B-mode abdominal ultrasonography as part of pre-transplant evaluation. GBV were confirmed by the Color Doppler Sonography, and multi detector computed tomography angiography. Interestingly, portal vein was patent and without thrombus. Conclusion. Despite being asymptomatic in most cases, the presence of GBV is valuable information for a surgeon because they might be a source of potentially catastrophic bleeding, which is particularly poorly tolerated by patients with decompensated liver cirrhosis. Ultrasound has the irreplaceable role not only in discovering GBV, but in prompt diagnosis of rare, but unpredictable and fatal complications as well.

Cyanoacrylate injection of an ectopic variceal bleed at a choledochojejunal anastomotic site in a patient with post-Whipple anatomy.

Cyanoacrylate injection of an ectopic variceal bleed at a choledochojejunal anastomotic site in a patient with post-Whipple anatomy.

2496 Management of Recurrent Ectopic Variceal Bleeding in Ileal Conduit With TIPS

INTRODUCTION: The simplest classification of portosystemic collateral veins classifies all non-esophagogastric varices as ectopic varices. Ileal varices are an example of ectopic varices formed from the ileal veins (tributaries of SMV) and draining into the mesenteric vessels. We present a case of an ileal conduit complicated by recurrent ectopic variceal bleeding amenable to a transjugular intrahepatic portosystemic shunt (TIPS) procedure. CASE DESCRIPTION/METHODS: A 45 year-old male with Hepatitis C and alcohol related cirrhosis complicated by portal hypertensive gastropathy and non-bleeding esophageal varices (EV) presented with a one month history of recurrent hematuria via his ileal conduit. He had a history of colon cancer stage IIB status post cystoprostatectomy and sigmoidectomy with ileal conduit in 2013. He had required three hospitalizations for his recurrent bleeding, needing multiple blood transfusions. Previous workup included a CT abdominal angiogram showing porto-systemic collaterals without active extravasation of contrast, an ileal conduit loopogram showing good functionality, and an ileal conduit endoscopy showing no visible ileal varices or active bleeding. On admission to our institution, he was hemodynamically stable and did not required additional blood transfusions. An abdominopelvic CT scan showed a normal ileal loop conduit and again small mesenteric portosystemic collaterals were evident. There was a high suspicion for ectopic varices in the ileal conduit, and a TIPS procedure was performed. A pre-TIPS portogram showed the presence of large ectopic ileal varices filled with contrast with resolution after TIPS placement. TIPS was successful with decrease of hepatic venous pressure gradient from 18 mmHg to 9 mmHg. He was discharged after TIPS procedure and has not required repeat hospitalization for hematuria. DISCUSSION: Ectopic varices account for up to 5% of variceal hemorrhage, and there is no consensus for therapeutic management. This patient had ileal conduit varices causing recurrent clinically significant hematuria. TIPS placement successfully controlled his recurrent ectopic variceal hemorrhage.

1921 A Case of Ovarian Varices Bleeding Presenting as an Obscure GI Bleed and Abdominal Pain

INTRODUCTION: Upper GI bleed is one of the most common complications in cirrhotic patients with portal hypertension. However, when GI bleed cannot be found after performing endoscopy, one should consider an ectopic gastrointestinal variceal bleeding. Ectopic varices are portosystemic collaterals in locations other than the gastroesophageal region. We present you a case of 44 year old cirrhotic patient, who was seen for nausea, vomiting and abdominal pain with acute blood loss anemia. She was found to have an ectopic gastrointestinal variceal bleeding after a negative endoscopy. CASE DESCRIPTION/METHODS: 44 years old female with past medical history of cirrhosis secondary to NASH presented to the ER with complaint of abdominal pain associated with nausea and vomiting while she was having sexual intercourse. Patient had CT Abdomen and Pelvis with IV contrast which was consistent with evidence of portal hypertension including large venous collaterals and splenomegaly. There was hyperdense ascitic fluid identified on the CT scan. On the left side of the hemiabdomen the fluid was hyperdense, suggestive of blood. But no site of bleeding could be identified on the study. Patient’s hemoglobin dropped by 2 grams in the ER. GI recommended to place NG tube and evaluate for blood contents. Gastric content via NG tube was non-bloody.IR was consulted who, after reviewing her CT scan images, noticed an unusual large IMV supplying large pelvic varices presumably as portal venous compression sites into systemic venous system. IR performed transhepatic portal access with pelvic variceal embolization. Interestingly, they did not find active bleed at the time of procedure. But, given recent history and CT findings, empiric embolization was performed of these varices. At completion, no residual pelvic varices was seen. Patient has successful sclerotherapy and coil embolization of the 2 pelvic-to-ovarian venous plexus varicosities for treatment of presumed source of variceal intraperitoneal bleeding. Patient was discharged home with stable hemoglobin. DISCUSSION: When GI bleed cannot be found after performing endoscopy, one should consider an ectopic gastrointestinal variceal bleeding. Out of all the variceal bleeding, 5% of them are ectopic variceal bleeds. As GI bleed with acute blood loss anemia can be fatal and outcome being time-sensitive, it is necessary to establish diagnosis early. Multi-slice computed tomography with contrast plays an important role in the early diagnosis of ectopic variceal bleed.

1977 A Rare Cause of Small Bowel Bleeding From a Duodenal Varix

INTRODUCTION: Ectopic varices are rare and by definition occur outside of the esophagus and stomach. They can be found in the rectum, duodenum or be peristomal. An estimated 2–5% of variceal bleeds are due to ectopic varices with the majority occurring either in the rectum or duodenum. The diagnosis of ectopic duodenal varices can be challenging given their location. There is a paucity of data regarding optimal diagnosis and management of bleeding duodenal varices. CASE DESCRIPTION/METHODS: A 46 year old woman with a history of cirrhosis from alcohol and nonalcoholic steatohepatitis was referred to our center for ongoing small bowel bleeding. Previous endoscopic evaluation included an unremarkable EGD, colonoscopy revealed blood through the colon without lesions, and capsule endoscopy showed blood in the proximal small bowel (at less than 30% small bowel transit time). We performed a push enteroscopy which showed a submucosal bulge with overlying ulceration and intermittent active bleeding in the third portion of the duodenum. Hemostasis was achieved with two hemoclips and the location was marked with tattoos. On initial discovery, the lesion was most consistent with a submucosal tumor such as a GIST (Figure 1) and she was referred for surgical resection. However, surgery was unsuccessful because of dense adhesions from a prior unrelated surgery. A repeat examination of the lesion 6 weeks later with endoscopic ultrasound showed a large varix (Figures 2 and 3). The patient has otherwise been doing well without any recurrent GI bleeding. DISCUSSION: Varices typically appear as bluish enlarged vessels or bulges and are commonly tortuous and easily compressed with instrumental pressure. Ectopic varices can be confused with thickened folds or submucosal lesions as in this case. Differentiation of these lesions is paramount in choosing appropriate management. It is important to consider ectopic varices on the differentials of both GI hemorrhage and submucosal lesions. EUS is the preferred test to characterize such lesions. Varices typically appears as round anechoic structures arising from the lamina propria or submucosa. Color Doppler is used to detect blood flow, which will confirm the vascular nature of the structure. Options of endoscopic hemostasis of bleeding duodenal varices include hemoclips, sclerotherapy, TIPS, balloon-occluded retrograde transvenous obliteration or surgical resection.

2197 Successful EUS-Guided Treatment of Duodenal Varices With Coil Embolization

INTRODUCTION: Ectopic varices are unusual and can involve any site along the GI tract. Only 5% of variceal bleeding is related to ectopic varices such as duodenal varices. Ectopic variceal bleeding is usually massive, difficult to treat, and associated with high mortality rates. High clinical suspicion is warranted. No standardized treatment is recommended, and the management remains challenging with limited success in controlling recurrent bleeding. EUS-guided therapy has evolved as an effective therapeutic modality in gastric varices and this case demonstrates the role of EUS-guided coil embolization (EUS-CE) with cyanoacrylate (CYA) injection in the management of duodenal variceal bleeding. CASE DESCRIPTION/METHODS: 81-year-old female with history of Diffuse large B cell lymphoma on chemotherapy presented with intermittent massive upper gastrointestinal bleeding. CT abdomen showed cirrhotic liver and portal vein thrombosis due to lymphoma involvement. Three endoscopies performed over a period of 5 days revealed 1 cm non-bleeding duodenal ulcer at the duodenal sweep without stigmata of recent bleeding. Due to recurrent bleeding, empiric coil embolization of the gastroduodenal artery and multiple duodenal branches through angiography was done by interventional radiology. However, severe bleeding recurred after the embolization, and the fourth urgent EGD showed large subepithelial violaceous lesions next to the deep penetrating ulcer, highly suspicious for duodenal varices. EGD-EUS confirmed these lesions were duodenal varices. By the fifth day of admission, patient had required 8 units of PRBC. Therefore, the decision was made to treat with EUS-CE. Two coils of 8 and 9 mm were deployed into the larger varix through a 19G EUS-FNA needle followed by 2 ml injection of CYA. A third 6 mm coil was also deployed in the feeder vessel also followed by CYA. Both lead to embolization and cessation of blood flow as demonstrated through Doppler investigation at the end of the procedure. Bleeding stopped and patient was discharged home to complete chemotherapy. DISCUSSION: Bleeding from duodenal varices require high suspicion as vessels could be miss if collapsed soon after bleeding. High clinical suspicion can result in early angiographic evaluation and intervention. Ideal management of ectopic variceal bleeding is still unclear. Multiple endoscopic technique modalities have been reported. EUS-CE with or without CYA injection is a safe endoscopic technique with effective variceal obliteration in most cases.

1958 An Unusual Ca(u)se of Upper Gastrointestinal Bleeding

INTRODUCTION: Duodenal varices (DVs) represent 17% of ectopic varices (EVs), have a 4-fold increased bleeding risk when compared to esophago-gastric varices (EGVs), and mortality rate up to 40%. There are no guidelines on the ideal management of DVs. We report a case of massive hemorrhage secondary to DV managed successfully with transjugular intrahepatic portosystemic shunt (TIPS). CASE DESCRIPTION/METHODS: A 69-year-old gentleman presented with hematemesis, hematochezia, dizziness and near-syncope. He was diagnosed with cryptogenic cirrhosis, non-alcoholic steatohepatitis in the past after negative autoimmune and infectious work-up. Prior doppler ultrasound showed elevated portal flow velocity; esophagogastroduodenoscopy (EGD) a month prior revealed portal hypertensive gastropathy (PHG) but no EGVs, and normal duodenum. Given prior hepatic encephalopathy, TIPS was not performed. He had abdominal surgeries in the past. He was hemodynamically unstable with benign abdominal exam. Labs revealed acute blood loss anemia with Hgb 9.1 (baseline 13), thrombocytopenia and coagulopathy. Following initial resuscitation with fluids, blood products, octreotide and pantoprazole infusion, urgent EGD was performed. There were no EGVs, but features of diffuse PHG, as well as a compressible mucosal bulge with a nipple sign representing a varix in the second portion of the duodenum were seen. Given the insufficient evidence to support endoscopic therapy in ectopic varices, interventional radiology was consulted. CT angiogram demonstrated patent portosystemic veins. TIPS was performed with a decrease in portal gradient from 16 to 2 mmHg. Rifaximin, lactulose and zinc were commenced. He remained hemodynamically stable following this and did not require further intervention. A year later, he remains free of further bleeding episodes or hepatic encephalopathy. DISCUSSION: Bleeding DVs are rare and potentially life-threatening. Diagnosis should be suspected in all cases of gastrointestinal hemorrhage, particularly in the absence of EGV or another source. Standard management has not yet been established. TIPS is a relatively safe and effective means of controlling acute DV bleeding. EVs may re-bleed despite a reduction of portosystemic pressure gradient < 12 mmHg or by 25–50% of baseline; hence the “12 mmHg rule” does not apply. Our patient recovered successfully following TIPS without needing additional therapy. Reducing the pressure gradient to very low values can result in successful outcomes with TIPS in DVs.

2209 Abdominal Arterial Aneurysms in a Patient With Budd-Chiari Syndrome

INTRODUCTION: Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms. CASE DESCRIPTION/METHODS: A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours. DISCUSSION: Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.

1933 A Case of a Hidden Varix: Massive Duodenal Variceal Hemorrhage Requiring Emergent TIPS

INTRODUCTION: Variceal hemorrhage is a potentially life-threatening cause of bleeding in cirrhotic patients. While typically associated with esophageal or gastric varices, clinically significant variceal hemorrhage may occasionally originate from the small bowel, colon, rectum or biliary tract. Duodenal variceal hemorrhage is an uncommon cause of GI tract bleeding and is associated with a mortality rate up to 40%. We present a case of severe duodenal variceal hemorrhage necessitating emergent transjugular intrahepatic portosystemic shunt (TIPS) and variceal coil embolization. CASE DESCRIPTION/METHODS: A 58-year-old female with a history of cryptogenic cirrhosis decompensated by ascites presented to the ER with melena and dull, generalized abdominal pain over 2 days. Vital signs were notable for tachycardia to 114 bpm and she was normotensive. Her abdomen was non-tender to palpation and rectal exam was notable for melenic stools. Her hemoglobin was 9.5 g/dL on presentation. She continued to have melena over the course of the day with associated tachycardia and a decrease in hemoglobin to 5.6 g/dL. She was transferred to the intensive care unit where she underwent an upper endoscopy revealing small esophageal varices with no stigmata of bleeding and a normal duodenum. However, she continued to have melena unresponsive to blood product resuscitation leading to profound hypotension. A stat CT Angiogram showed intraluminal hyperdensities within the second portion of the duodenum due to hemorrhage from adjacent varices. Interventional Radiology was consulted and performed a right hepatic vein to right portal vein TIPS. They also performed fluoroscopic guided coil embolization of large duodenal varices arising from the main portal vein. The patient’s hemoglobin stabilized and she had no further bleeding during the course of her hospitalization. DISCUSSION: Duodenal variceal hemorrhage is a rare and life-threatening manifestation of portal hypertension. Due to the rarity of the condition, there are no established guidelines regarding treatment. Endoscopic therapy aimed at achieving hemostasis, such as sclerotherapy and clipping have shown limited success in case reports. TIPS, as seen in this case, is a viable treatment option. Our case illustrates the importance of considering ectopic varices in cirrhotic bleeders. Moreover, it highlights the importance of utilizing angiographic evaluation for patients in whom the source of bleeding is not identified on endoscopy.

1536 An Uncommon Case of Small Bowel and Pan-Colonic Varices

INTRODUCTION: We present a case of idiopathic varices in the small bowel and colon discovered after extensive work up for iron-deficiency anemia and portal hypertension. CASE DESCRIPTION/METHODS: 41-year-old with no known medical history underwent esophagogastroduodenoscopy (EGD) and colonoscopy for evaluation of iron-deficiency anemia. EGD was normal. Colonoscopy revealed diffuse colonic varices extending from the cecum to the rectum (Figure 1). Computed tomography enterography showed numerous varices of the mid jejunum. Magnetic resonance imaging of the abdomen showed patent portal circulation and no other abnormalities. Echocardiogram did not show any evidence of right heart failure. Transjugular liver biopsy showed a portosytemic pressure gradient of 4 mm of Hg indicating the absence of portal hypertension. Liver biopsy only showed mild steatosis and no fibrosis. A direct measurement of portal pressure was 3 mmof Hg again indicating the absence of portal hypertension. DISCUSSION: Given the extensive workup with no evidence of portal hypertension, patient is thought have idiopathic varices in the small bowel and colon. Ectopic varices, especially in the colon are rare and are usually associated with portal hypertension. Idiopathic colonic varices in association with small bowel varices are even more infrequently described in existing literature. Clinical presentation has been reported with massive hemorrhage, recurrent bleeds and even as incidental findings in endoscopies and imaging performed for other reasons. Management options include conservative frequent follow up or surgery if there is evidence of recurrent bleeds or massive hemorrhage.

2335 Bladder Varices Causing Hematuria and a New Diagnosis of Cirrhosis

INTRODUCTION: A known consequence of portal hypertension is the development of varices, which are described as “ectopic” when located at unusual sites in the abdomen. Ectopic varices carry a mortality rate as high as 40% after initial hemorrhagic episode. We report a rare case of bladder varices as the presenting symptom for a new diagnosis of cirrhosis. CASE DESCRIPTION/METHODS: A 62yo female with a history of treated chronic hepatitis C (HCV) presented to an outside hospital with two days of gross hematuria. She was transferred to our center for further management. She had completed eight weeks of glecaprevir-pibrentasvir a few months prior to admission. On physical exam, she was an obese female without scleral icterus or jaundice, nor cutaneous stigmata of liver disease. Past imaging from six months prior to admission was notable for hepatomegaly only and not cirrhosis. Labs were significant for a hemoglobin of 7.6 and MELD-Na = 7. HCV RNA was undetectable. CT imaging of her abdomen/pelvis revealed cirrhosis with varices present along the right bladder wall in connection with the right internal iliac vein and the portal venous system. We performed a transvenous hepatic venogram and pressure study with biopsy. Liver pathology confirmed the presence of chronic hepatitis and cirrhosis and the hepatic venous pressure gradient was 13mm Hg. Urology placed the patient on continuous bladder irrigation and then performed a cystoscopy that revealed large, tortuous veins along the right lateral and posterior bladder, consistent with varices. She received an IV octreotide infusion with some improvement in hematuria, but not cessation. Multidisciplinary discussion resulted in our interventional radiology (IR) team performing a modified balloon-occluded retrograde transvenous obliteration, accessing the varix successfully via percutaneous transhepatic approach through the superior mesenteric vein and into the bladder varix. The hematuria resolved and the patient was safely discharged two days later. DISCUSSION: The bladder is an uncommon site for ectopic varices to form and hematuria is not often a symptom associated with cirrhosis or portal hypertension. This presentation was even more uncommon as the patient was not previously known to have cirrhosis. Cross-sectional imaging was key to the diagnosis in this case. Recognition of this rare event, combined with multidisciplinary management, helped this patient have a successful outcome.

2623 Isolated Duodenal Variceal Bleeding Secondary to Metastatic Carcinoid Tumor Obstructing the Superior Mesenteric Vein

INTRODUCTION: Gastrointestinal bleeding secondary to duodenal varices is an uncommon finding, and is typically seen in patients with portal hypertension due to cirrhosis. We report a case of a patient without evidence of liver disease who developed significant bleeding from isolated duodenal varices caused by mesenteric venous obstruction from a carcinoid mass. CASE DESCRIPTION/METHODS: A 71-year-old male with a history of a metastatic mesenteric carcinoid tumor presented with recurrent gastrointestinal bleeding. He was initially diagnosed with carcinoid of the mesentery several years prior, for which he underwent resection followed by Y40 ablation of liver metastases. He had been admitted multiple times at a local hospital for recurrent gastrointestinal bleeding, for which he had undergone extensive endoscopic evaluation. He was then transferred to our facility for a higher level of care. A double balloon push enteroscopy and endoscopic ultrasound were performed with findings of a large cluster of bleeding duodenal varices in the third portion of the duodenum not amenable to endoscopic intervention. CT abdomen and pelvis with contrast and CT angiography of the abdomen showed an irregular 3 cm calcified mass at the root of the mesentery. Additionally, several branches of the superior mesenteric artery and vein (SMA and SMV) were noted to be occluded near the mass. Interventional radiology (IR) performed an IR-guided transhepatic portogram revealing obstruction of the SMV at the level of the mesenteric mass. There appeared to be a large colic branch communicating with an abnormal cluster of varices near the third portion of the duodenum. Two overlapping covered stents were placed at the large colic branch into the SMV, decompressing the varices. The patient was started on plavix, propranolol, and octreotide depot and safely discharged home without further bleeding. DISCUSSION: Duodenal varices are an uncommon but potentially serious complication of portal hypertension, and are most often seen as a sequelae of liver disease. The development of duodenal varices as a result of venous obstruction secondary to a desmoplastic reaction induced by a carcinoid tumor has seldom been reported in the literature. This case illustrates a noteworthy example of bleeding ectopic varices in a patient without liver disease, a rare cause of obscure gastrointestinal bleeding.

2011 Jejunal Varices: A Diagnostic and Therapeutic Nightmare!

INTRODUCTION: Gastrointestinal hemorrhage is a common complication among cirrhotic patients with portal hypertension, due to the development of bleeding varices. While esophagus and stomach are the most common location for development of varices, it can also rarely occur in ectopic locations, such as the jejunum, ileum, rectum and duodenum. Here, we report a case of a patient with severe gastrointestinal hemorrhage due to a bleeding jejunal varix. CASE DESCRIPTION/METHODS: A 55-year-old male with a history of hypertension, splenectomy, and alcoholic cirrhosis had presented initially to an outside ED with complaints of bright red blood per rectum. At admission, he required multiple infusions of pRBCs, and several upper endoscopies due to refractory bleeding with unclear source. Initial endoscopies showed non-bleeding esophageal varices, for which he underwent ligation banding. However, he continued to deteriorate, developing recurrent melena, hypotension, and syncope. On admission to our hospital, he had a repeat upper endoscopy, lower endoscopy, CT angiography and tagged red blood cell study; but we were unable to localize the source of the bleeding. He continued to deteriorate, and also developed a PEA arrest in the setting of a profuse bright red blood per rectum, requiring massive transfusion protocol. After successful resuscitation, he underwent a small bowel enteroscopy, which finally revealed a bleeding jejunal varix. However, the varix was not amenable to any endoscopic intervention. Subsequently, he underwent a successful balloon-occluded retrograde transvenous obliteration (BRTO) of varix. Patient’s condition stabilized and after adequate recovery, he was extubated. DISCUSSION: Early diagnosis of ectopic varices is difficult to achieve due to low likelihood of presentation and low sensitivity to various diagnostic tests. Due to its low prevalence, there are no protocols for its management. As in our patient, endoscopic intervention such as sclerotherapy and band ligation can be difficult due to the narrow lumen of the small bowel. Alternatively, BRTO and TIPS are also considered effective therapeutic options. However, due to his poor liver function, BRTO was a better alternative. In conclusion, through this case report, we want to emphasize the importance of early recognition and appropriate management of ectopic varices due to its potential for massive, even fatal hemorrhage.