Splenorenal Shunt Research Articles

Two surgical procedures for esophagogastric variceal bleeding in patients with portal hypertension

To determine the clinical value of a splenorenal shunt plus pericardial devascularization (PCVD) in portal hypertension (PHT) patients with variceal bleeding. From January 2008 to November 2012, 290 patients with cirrhotic portal hypertension were treated surgically in our department for the prevention of gastroesophageal variceal bleeding: 207 patients received a routine PCVD procedure (PCVD group), and 83 patients received a PCVD plus a splenorenal shunt procedure (combined group). Changes in hemodynamic parameters, rebleeding, encephalopathy, portal vein thrombosis, and mortality were analyzed. The free portal pressure decreased to 21.43 ± 4.35 mmHg in the combined group compared with 24.61 ± 5.42 mmHg in the PCVD group (P < 0.05). The changes in hemodynamic parameters were more significant in the combined group (P < 0.05). The long-term rebleeding rate was 7.22% in the combined group, which was lower than that in the PCVD group (14.93%), (P < 0.05). Devascularization plus splenorenal shunt is an effective and safe strategy to control esophagogastric variceal bleeding in PHT. It should be recommended as a first-line treatment for preventing bleeding in PHT patients when surgical interventions are considered.

Ligation of Left Renal Vein for Spontaneous Splenorenal Shunt to Prevent Portal Hypoperfusion after Orthotopic Liver Transplantation

We report a case of recovered portal flow by ligation of the left renal vein on the first postoperative day after orthotopic liver transplantation of a 54-year-old female with alcoholic liver cirrhosis, chronic kidney failure, and spontaneous splenorenal shunt. After reperfusion, Doppler ultrasonography showed almost total diversion of the portal flow into the existing splenorenal shunt, but because of severe coagulopathy and diffuse bleeding, ligation of the shunt was not attempted. A programmed relaparotomy was performed on the first postoperative day, and the left renal vein was ligated just to the left of the inferior vena cava. Portal flows subsequently increased to 37 cm/sec, and the patient presented a good and stable liver function. We conclude that patients with known preoperative splenorenal shunts should be closely monitored, and if the portal flow becomes insufficient, ligation of the left renal vein should be attempted in order to optimize the portal perfusion of the liver.

Liver function impairment in liver transplantation and after extended hepatectomy

Extended hepatectomy, or liver transplantation of reduced-size graft, can lead to a pattern of clinical manifestations, namely "post-hepatectomy liver failure" and "small-for-size syndrome" respectively, that can range from mild cholestasis to irreversible organ non-function and death of the patient. Many mechanisms are involved in their occurrence but in the recent past, high portal blood flow through a relatively small liver vascular bed has taken a central role. Therefore, several techniques of inflow modulation have been attempted in cases of portal hyperperfusion first in liver transplantation, such as portocaval shunt, mesocaval shunt, splenorenal shunt, splenectomy or ligation of the splenic artery. However, high portal flow is not the only factor responsible, and before major liver resections, preoperative assessment of the residual liver function is necessary. Techniques such as portal vein embolization or portal vein ligation can be adopted to increase the future liver volume, preventing post-hepatectomy liver failure. More recently, a new surgical procedure, that combines in situ splitting of the liver and portal vein ligation, has gradually come to light, inducing remarkable hypertrophy of the healthy liver in just a few days. Further studies are needed to confirm this hypothesis and overcome one of the biggest issues in the field of liver surgery.

Congenital hepatic fibrosis

Congenital hepatic fi brosis (CHF) is a rare developmental disorder of the portobiliary system and most commonly associated with polycystic kidney disease. The pattern of inheritance of this disorder is autosomal recessive. The exact prevalence of CHF is unknown (estimated from 1:10000 to 1:20000). Sequelae of CHF and portal hypertension have been found in less than half of the all CHF patients and were associated with age. We present a case study of a boy with CHF complicated by portal hypertension, splenomegaly and hypersplenism. Tis patient was diagnosed with cholestatic syndrome as a neonate. A transcutaneous liver biopsy was performed and repeated at the age of 9 months. Diagnosis of cholestatic syndrome was made based on the findings of a histopathological examination. The ultrasound examination showed polycystic kidneys; however, global renal function remained normal. At the age of 8 years and 6 months, portal hypertension was confirmed by Doppler ultrasonography, and endoscopic examination revealed oesophageal varices of second and third grade, which was also observed in the splenic portography. Trombocytopenia due to hypersplenism was identifi ed by a platelet count of 75.2 x 103. To prevent variceal bleeding, a a splenorenal shunt and a partial spleen resection were performed. The differential of cholestatic syndrome in infants should include CHF. This type of disease may suggest early developing complications of CHF, such as portal hypertension and hypersplenism. Portosystemic shunt surgical treatment is justified in CHF cases with cholestatic syndrome .

Multiple Splenic Artery Aneurysms Secondary to Extra Hepatic Portal Vein Obstruction

A 14 year old girl presented with vague abdominal pain, recurrent, well tolerated haematemesis and feeling of presence of a lump in the abdomen. Her abdominal examination revealed gross splenomegaly. The laboratory tests, haematological counts, liver enzymes, platelet counts and the international normalized ratio were within the normal limits. Upper gastrointestinal endoscopy showed grade II oesophageal varices. Ultrasonography showed that the portal vein was replaced by multiple, dilated, collateral venous channels around the porta, with collaterals around the pancreas and splenic hilum. There was splenomegaly with a suspicious aneurysm of the splenic artery at the hilum. She was recommended Computed Tomography (CT) angiography, as it was difficult to differentiate aneurysm from collaterals at the splenic hilum on ultrasonography. CT angiography revealed multiple aneurysms in the main splenic artery, at the splenic hilum and at intra parenchymal branches, with largest being seen at the splenic hilum, which measured 18 x 15mm [Table/Fig-1A and B]. [Table/Fig-1A and B]: CT angiography axial section in 14 year old girl showing tortuous splenic artery with large aneurysm in the distal part of the splenic artery (arrow) and another smaller one in the intra parenchymal branch (arrow head). Portovenous phase CT showed multiple dilated venous collaterals replacing the main portal vein with multiple collaterals around the pancreatic head and the splenic hilum. There was gross splenomegaly [Table/Fig-1C and D]. [Table/Fig-1C and D]: 3D volume rendered CT angiography image better depicting the SAA (arrow) and small aneurysms in intraparenchymal branches (arrow heads). The aneurysms were indistinguishable from the dilated collaterals on portovenous phase CT. A diagnosis of extra hepatic portal vein obstruction with splenomegaly and multiple splenic aneurysms was made. Splenectomy, aneurysmectomy and a splenorenal shunt were performed. The post operative period was uneventful.

Rectal arterio-portal fistula: An unusual cause of persistent bleeding per rectum following a proximal spleno-renal shunt

Gastrointestinal arterio-venous malformations are a known cause of gastrointestinal bleeding. We present a rare case of persistent rectal bleeding due to a rectal arterio-portal venous fistula in the setting of portal hypertension secondary to portal vein thrombosis. The portal hypertension was initially surgically treated with splenectomy and a proximal splenorenal shunt. However, rectal bleeding persisted even after surgery, presenting us with a diagnostic dilemma. The patient was re-evaluated with a computed tomography mesenteric angiogram which revealed a rectal arterio-portal fistula. Arterio-portal fistulas are a known but rare cause of portal hypertension, and possibly the underlying cause of continued rectal bleeding in this case. This was successfully treated using angiographic localization and super-selective embolization of the rectal arterio-portal venous fistula via the right internal iliac artery.The patient subsequently went on to have a full term pregnancy. Through this case report, we hope to highlight awareness of this unusual condition, discuss the diagnostic workup and our management approach.

Spleno-adrenal shunt: A novel alternative for portosystemic decompression in children with portal vein cavernous transformation

PurposeChildren with portal vein cavernous transformation (PVCT) can develop life-threatening variceal hemorrhage from progressive portal hypertension. While spleno-renal shunt±splenectomy is the most common portosystemic decompression surgery performed in children, we have adopted a modified spleno-adrenal (SA) shunt for complicated PVCT. We describe our 10year experience focusing on technique evolution and treatment efficacy. MethodsBetween 2001 and 2011, 15 children (9 girls and 6 boys, ages 3–11years, median: 6years) with PVCT, portal hypertension, and hypersplenism were treated with SA shunt with splenectomy in Shanghai Children's Medical Center. All children in the study had endoscopy proven active esophageal variceal bleeding requiring multiple transfusions (mean: 4.2 units) with failed sclerotherapy (mean: 2.6 times). Greater omental vein pressure (GVP) approximating portal venous pressure was measured pre- and post-SA shunt. Pre- and post-operative ammonia levels were obtained. Follow-up ranged from 6months to 10years (mean: 4.2±2years). ResultsIntra-operative adrenal vein diameter and length ranged from 0.7 to 1.8cm and 2 to 3cm, respectively. Intra-operative GVPs pre-and post-SA shunt were (30±11) and (22±7) mmHg, respectively (p<0.01). On follow-up, there have been no recurrences of GI bleeding. Liver function tests remained normal in all children with the exception of elevated post-operative mean blood ammonia levels [Pre (18±7) mmol/L, post (60±17) mmol/L (p<0.05)] in all children. Ammonia levels normalized in all cases on outpatient follow-up. There have been no cases of hepatic encephalopathy, and all have normal age appropriate neurodevelopment (Bayley's assessment). Barium swallow and/or upper endoscopy showed interval resolution of esophageal varices in all children, and vascular ultrasound showed patent shunt anastomosis without stricture in 14 (93%). ConclusionsThe left adrenal vein is a viable conduit for effective selective portosystemic decompression. Similar to the more traditional spleno-renal shunt, SA appears also to have the advantage of preventing hepatic encephalopathy preserving neurodevelopment, although the rise in post-operative ammonia levels was unexpected. Longer follow-up is needed to look for late signs of encephalopathy assessing neurodevelopment long term.

Gastric Varices: Is There a Role for Endoscopic Cyanoacrylates, or Are We Entering the BRTO Era?

Bleeding from portal hypertension-related gastric varices arising in the cardiofundal region of the stomach presents a challenge due to the unique underlying vascular anatomy which is sometimes underappreciated in endoscopic classification schemes. They often have dominant tributaries from the splenic vein or splenic hilum and terminate in the left renal vein (spontaneous splenorenal or gastrorenal shunts). This may limit the applicability of a transjugular intrahepatic portosystemic shunt (TIPS), because of the shunt's distance from the hilum of the liver. Endoscopically, the presence of a large systemic outflow track also may influence the performance of different cyanoacrylates. However, this anatomy allows an alternative approach, balloon-occluded retrograde transvenous obliteration (BRTO), which accesses the varix via the outflow pathway. Definitive comparisons between TIPS, endoscopic cyanoacrylate, and BRTO will be challenging because the incidence of this type of varix is insufficient for large trials. Here, I provide a perspective based on existing literature, 15 years of experience with various cyanoacrylates, and 4 years of experience with BRTO.

Natural course of splenic artery aneurysm with associated spontaneous splenorenal shunt in non-cirrhotic liver: An 18-year observational follow-up and review of literature

Through a review of the literature, a splenic artery aneurysm (SAA) with associated spontaneous splenorenal shunt (SSRS) was only reported in patients with liver cirrhosis and portal hypertension. However, a natural course of a SAA with associated SSRS was found in a non-cirrhotic male patient during an 8-year observational follow-up, and thus reported. Initially, splenomegaly and thrombocytopenia were noted; SSRS was observed later with a tortuous dilated splenic artery, and a SAA was then progressively formed and found. The patient received splenectomy with aneurysm resection and SSRS was preserved. Post-operative follow-up revealed that the size of the SSRS was reduced. Through the course, no abnormalities of liver enzymes, portal hypertension, or esophageal-gastric varicose were found in the patient. No positive association was demonstrated between the formation of SSRS and the severity of liver cirrhosis in patients, implying some other factors, e.g., vascular endothelial growth factor (VEGF) mentioned in the literature, might be involved.

Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism

To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published. Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade in all patients and band ligation or endoscopic sclerotherapy had been done for children with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen (5-15 cm below the costal margin) and symptomatic hypersplenism with leucopenia, thrombocytopenia, and anemia. Partial resection of the spleen was performed, starting with ligation of the branches and tributaries of the caudal two-thirds. When an ischemic line demarcated the splenic parenchyma, it was transected using electrocautery or LigaSure, leaving 20-30 % of splenic tissue. After the spleen resection, a Warren shunt was performed. Platelet and white blood cell counts and liver function tests were performed before and after the operation. Growth was assessed using SD scores (z scores) for height, weight, and body mass index at the time of surgery and 1 year later. Postoperative recovery was uneventful and the leukocyte and platelet counts normalized. The shunt patency rate was 100 %. Two cases of shunt stenosis were treated successfully with percutaneous angioplasty. There was no postoperative mortality. During the follow-up period, from 1 to 7 years, all 16 children were asymptomatic, with improved quality of life, growth, and nutrition. No episodes of variceal bleeding, sepsis or encephalopathy occurred. Our results demonstrate that shunt surgery with partial resection of the spleen is effective and safe for pediatric patients with massive splenomegaly and severe hypersplenism secondary to EHPVO.

Ligation of Left Renal Vein for Spontaneous Splenorenal Shunt to Prevent Portal Hypoperfusion after Orthotopic Liver Transplantation: A Case Report

Low portal vein flow after orthotopic liver transplantation due to spontaneous splenorenal shunt is associated with hepatic hypoperfusion and poor allograft survival. We describe a case of recovered portal flow by ligation of the left renal vein on the first postoperative day after orthotopic liver transplantation of a 54-year-old female with alcoholic cirrhosis, chronic kidney failure and spontaneous splenorenal shunt. After reperfusion portal flows were extremely low, but because of severe coagulopathy and diffuse bleeding, ligation of the left renal vein was not attempted. A relaparotomy was performed on the first postoperative day and the left renal vein was ligated at its confluence to the inferior vena cava. Portal flows subsequently increased and the patient presented a good and stable liver function. We conclude, that patients with known preoperative splenorenal shunts should be closely monitored and if the portal flow becomes insufficient, ligation of the left renal vein should be attempted in order to optimize the portal perfusion of the liver.

Portosystemic Myelopathy: A Rare Neurological Presentation of Portosystemic Shunts

Portosystemic myelopathy is an unusual complication in patients with chronic liver disease with hepatic encephalopathy and portosystemic shunts. Here we present a case of 35-year-old male patient who presented to us with difficulty in walking and progressive stiffness in both lower limbs for two months. He had undergone splenectomy with distal splenorenal shunt 20 years back. On physical examination, he had spasticity in both lower limbs of grade 3, with minimal pyramidal weakness in lower limbs, brisk knee and ankle jerks. The plantar response was extensor. Upper limb examination was normal. On investigations, he had hypoalbuminemia, hyperbilirubinemia, increased plasma ammonia levels. Contrast enhanced CT scan abdomen revealed dilated splenorenal shunt and MRI spine showed no spinal cord compression. Electromyoneurogram was also normal. Spastic paraparesis due to portosystemic shunts was diagnosed. Liver transplantation can reverse the myelopathy only in earlier stages, hence early and accurate diagnosis is important.

Risk factors for the exacerbation of esophageal varices or portosystemic encephalopathy after sustained virological response with IFN therapy for HCV-related compensated cirrhosis

We aimed to identify risk factors contributing to the exacerbation of esophageal varices (EV) or portosystemic encephalopathy after hepatitis C virus (HCV) eradication with interferon (IFN) therapy in patients with compensated cirrhosis. Also, the prognosis after HCV eradication was analyzed. Fifty-two patients with sustained virological response to IFN treatment for HCV-related compensated cirrhosis were enrolled in this retrospective cohort study. At the achievement of HCV eradication, in 31 of the 52 patients (60%), feeding vessels for EVs (left gastric vein, posterior gastric vein, short gastric vein) were shown, and in 18 patients (35%) there were extrahepatic portosystemic shunts (paraesophageal vein, paraumbilical vein, and splenorenal shunt). Although the HCV eradication was successful, significant improvements were not observed in portosystemic collateral vessels 1year after HCV eradication, and EVs were exacerbated in 19 (36%) patients. The cumulative 1- and 3-year rates of EV exacerbation were 13% and 49%, respectively. By multivariate analysis, the existence of feeding vessels for EVs at HCV eradication was an independent predictive factor for the exacerbation of EVs (P=0.009). Seven patients who had an extrahepatic portosystemic shunt at HCV eradication developed portosystemic encephalopathy during follow up. The 1-, 3-, and 5-year incidences of portosystemic encephalopathy were 6, 21, and 34%, respectively. The cumulative 5-year survival rate of the cohort was 81%. Two patients died of hepatocellular carcinoma (HCC). Our findings suggest that the existence of radical portosystemic collateral vessels at successful HCV eradication increases the risk of the exacerbation of EVs and the incidence of portosystemic encephalopathy in patients with HCV-related cirrhosis.

Non-Cirrhotic Portal Hypertension of Unknown Etiology, May Be from Thrombosis of the Extrahepatic Portal Vein

Purpose: 55-year-old adult female with h/o colon cancer 3 years ago had right hemicolectomy and chemotherapy, presented with GERD symptoms and found to have large esophageal varices. Liver function is preserved. Screening tests for specific liver diseases including HBsAg, autoantibodies, serum ferritin, ceruloplasmin were all normal. CT of abdomen revealed extra hepatic portal vein thrombosis; hematology evaluation to evaluate other causes of hypercoaguable state is negative. She has multiple cysts throughout the liver on imaging and later patient developed esophageal variceal hemorrhage. After ultrasonographic and biopy evaluation, a diagnosis of idiopathic non-cirrhotic portal hypertension was made. Percutaneous liver biopsy showed non-specific changes and did not provide definitive diagnostic histologic evidence of either hepatic venous outflow obstruction or of pathology involving the major portal vein. Endoscopy showed extensive oesophageal varices with stigmata of bleeding. She has undergone esophageal variceal ligation several times electively and another time for a bleed. She saw a liver surgeon regarding whether she might be a candidate for a shunt procedure such as a distal splenorenal shunt. Conclusion: Noncirrhotic portal hypertension results from thrombosis of the extrahepatic portal vein. Multiple etiologies may cause the disorder, although nearly half are idiopathic. In this case, it's not certain whether or not the large number of liver cysts decreased liver parenchyma contributed to increased portal pressure and esophageal varices. Endoscopic management of esophageal variceal bleeding is the preferred therapy. However, when endoscopic treatment fails to control variceal hemorrhage, a distal splenorenal shunt is likely to be the most successful operation.

Fulminant Hepatic Failure and Gas Gangrene Secondary to Clostridium Perfringens Septicemia

Purpose:Clostridium perfringens is an exotoxin producing bacteria, and can be found in the intestinal tract of humans. Their invasive nature as pathogens can be attributed to physical trauma or surgical procedures, with resulting evidence of myonecrosis. We present a unique case of fulminant liver failure with gas gangrene, pneumobilia, myonecrosis, and splenorenal shunt phlebitis in an Asian female, without recent history of physical trauma or surgery. An 81-year-old female with history of Diabetes Mellitus presented with acute complaints of fever and altered mental status. Exam was significant for an erythematous rash on the right knee, without evidence of trauma. Labs were significant for leukocytosis, elevated LFTs (AST/ALT >2600 IU/L), and coagulopathy. CT scan of the abdomen revealed two necrotic masses in the right hepatic lobe, pneumobilia, and splenorenal shunt septic phlebitis. Additionally, multiple areas of intramuscular air were noted within the gluteus maximums muscles. The patient was started on broad spectrum antibiotics but expired within 12 hours. Blood cultures revealed C. perfringens after 24 hours of incubation. C. perfringens, is a gram-positive, rod-shaped, anaerobic, spore forming bacterium. These environmental bacteria enter the muscle tissue via a wound, resulting in tissue necrosis and systemic spread. Gas gangrene found in the liver with fulminant hepatic failure is exceedingly rare, especially in constellation with pneumobilia, myonecrosis, and splenorenal shunt septic phlebitis. Management consists of medical therapy with penicillin and clindamycin. Aggressive surgical debridement and excision of affected tissue is vital to increasing the likelihood of survival. A few rare cases of spontaneous gas gangrene have been described in the literature attributed to C. perfringens. None have shown the manifestations of fulminant hepatic failure with gas gangrene along with pneumobilia, splenorenal shunt phlebitis, and myonecrosis without preceding surgery or physical trauma. The exact incidence of this infectious process remains unknown because it is rapidly progressing and is often unrecognized in the emergency setting. Hematogenous seeding of muscles with bacteria from a gastrointestinal tract port of entry should be entertained. Early detection and diagnosis, together with proper supportive treatment, can improve patient outcomes.Figure: [1008]

Small-Intestinal Variceal Hemorrhage: Successful Treatment with Transjugular Intrahepatic Portosystemic Shunt

Purpose: A 48 y/o female with a history of alcoholic cirrhosis and portal hypertension presented with 2 episodes of large hematochezia with severe epigastric and periumbilical abdominal pain. Labs revealed an acute decline in hemoglobin (Hgb) from 11 to 8 gm/dL. An abdominal CT obtained to evaluate her abdominal pain revealed changes suggestive of portal hypertension including multiple collaterals, small intestinal (SI) varices, splenorenal shunting, and a recanalized paraumbilical vein. Intraluminal blood in the distal SI was noted, thought to be in the region of a SI varix and potentially the etiology of her symptoms. Fluid resuscitation, blood transfusion, IV proton pump inhibitor and octreotide therapy were initated. However, she continued to have persistent hematochezia with a further drop in Hgb to 6.3 gm/dL. Emergent EGD did not demonstrate any lesion as a source of bleeding. Subsequent CT angiography showed pooling of contrast in the distal loop of ileum with large SI varices. Attempts at angiographically controlling bleeding were unsuccessful. Given the findings of portal enteropathy with large SI varices, emergent therapeutic TIPS was performed for acute control of SI bleeding. Post-TIPS HVPG was documented as 7 mmHg. Subsequently, her bleeding stopped and her Hgb improved. Overall, she received 10 units of packed RBC, 6 units of FFP and 4 units of platelet transfusion. Portal hypertension, usually a complication of cirrhosis, presents as ascites, splenomegaly, and bleeding from esophageal, gastric, and ectopic varices. Ectopic enteric varices including duodenal, mesenteric, anorectal, peristomal, and rarely ileal varices are an unusual cause of GI bleeding accounting for 1-5% of all variceal bleeding. While the incidence of bleeding is low, this may result in severe, life threatening hemorrhage with a mortality approaching 40%. Therapeutic options for variceal bleeding include endoscopic sclerotherapy, vasoactive drugs, segmental bowel resection, portosystemic shunt surgery, oversewing of varices, and TIPS procedures with or without focal variceal embolization. Endoscopic management is limited as very often visualization of SI varices is not achieved because of their distal location and relatively small size. Unless the underlying portal hypertension is treated, suture ligation of varices as well as sclerotherapy have high rebleeding rates. Surgical portocaval shunting is effective with low rebleeding rates, but is only recommended in patients who can tolerate a major surgical procedure. TIPS can effectively reduce the portocaval gradient, and consequently, the risks of bleeding decrease drastically. This is especially indicated for patients that are poor surgical candidates.

A Rare Cause of Bleeding: Recurrent Hemobilia Due to Ectopic Varices

Purpose: Esophageal varices are a common cause of upper GI bleeding second to peptic ulcer disease, whereas ectopic varices account for only 5% of all variceal bleeding. Ectopic varices comprise large portosystemic collaterals anywhere other than the gastro-esophageal region. Clinicians must be astute in suspecting an ectopic variceal bleed since the mortality rate can reach 40% if missed. We describe a case of recurrent GI bleeding over multiple years in a patient found to have portal vein thrombosis leading to choledochal varices and recurrent hemobilia. A 64-year-old female presented to the hospital with melena and a hemoglobin of 4.8 g/dL. Her history is significant for a gastric banding procedure in 1986 complicated two years later with erosion of the band leading to perforation. It was removed with creation of an omental patch and a Roux-en-Y cholecystojejunostomy for biliary obstruction found at the time of the repair. Over the years, the patient reports multiple episodes of melena coinciding with fevers, jaundice, and abdominal pain suggestive of cholangitis. Numerous EGD and colonoscopies in the past did not reveal an active source of bleeding and every episode resolved without intervention. An EGD performed during this admission was unremarkable and her colonoscopy revealed clotted blood in the colon and terminal ileum. An antegrade spiral endoscopy to assess the cholecysto-jejunal anastomosis was unsuccessful due to acute angularity. An initial MRCP showed filling defects within the common bile duct, but subsequent imaging revealed the absence of any ductal material. There was portal vein occlusion with extensive varices in the vicinity of the cholecystojejunostomy and other changes consistent with portal hypertension, thought as a consequence of her previous surgery. She was managed medically requiring a total of 11 units of packed red blood cells and had no further bleeding. She was referred to surgery given that this episode and likely prior were a function of recurrent ectopic variceal bleeding. Attempts at a splenorenal shunt were abandoned due to the extent of adhesions and she underwent a complicated procedure involving removal of the small bowel and gallbladder containing the varices, and the creation of a new Roux-en-Y hepaticojejunostomy. Now 4 months after the procedure, the patient is doing well with no further episodes of GI bleeding or cholangitis. Although 80% of upper GI bleeding is self-limited, this case highlights the need for continued evaluation particularly in the setting of recurrent episodes. It also demonstrates the difficulty in identifying an obscure source can be overcome using the clinical history as a guide.

Renoportal anastomosis in right lobe living donor liver transplantation: report of a case

End-stage liver disease is often accompanied by thrombosis of the portal vein and the formation of splanchnic collateral vessels. Successful liver transplantation in such situations is more likely if the surgeon uses a strategy to establish a graft inflow. A 59-year-old male with a decompensated liver secondary to idiopathic portal hypertension underwent living donor liver transplantation (LDLT) using a right lobe liver graft donated from his son. His portal venous trunk was atrophied and a splenorenal shunt drained the mesenteric venous flow into the systemic circulation. LDLT was performed with renoportal anastomosis (RPA) using his right internal jugular vein as an interposed venous graft, without dissecting the collateral vessels. Although he developed temporary functional hyperbilirubinemia, he was discharged from the hospital 23 days after LDLT. This case suggests that RPA is a useful technique to manage patients with an obstructed portal vein and a splenorenal shunt.

Spontaneous Hemoperitoneum in Non Cirrhotic Portal Hypertension: A Case Report

Purpose: We report a rare case of non -irrhotic portal hypertension with spontaneous intraperitoneal hemorrhage. This report details patient's presentation, management and literature review. Case Report: 29-year-old Ethiopian male with history of splenomegaly and thrombocytopenia presented with sudden onset of severe diffuse abdominal pain, associated with generalized weakness. Examination was significant for mild tachycardia, diffuse abdominal tenderness without guarding and splenomegaly. Lab work was significant for Hemoglobin 9.3 (previous hemoglobin 14.7), platelets 27, WBC 2.1, normal Bun/Cr, total protein 6.0, albumin 3.4, AST 30, ALT 23, Alkaline phosphatase 62 and total bilirubin 2.3. CT abdomen with contrast was remarkable for massive splenomegaly, associated with extensive varices as well as spontaneous splenorenal shunt. Portal and splenic vein appeared to be intact. There was fluid of high density adjacent to the liver as well as within the bilateral pericolic gutters. Density was compatible with hemorrhagic fluid in nature. It was decided for initial conservative management. Upper GI endoscopy showed very large esophageal varices that were banded along with gastric varix and portal HTN gastropathy. Paracentesis was done that showed 3 million red blood cells confirming hemoperitoneum and a low albumin with a high SAAG, consistent with portal hypertension. A comprehensive evaluation for causes of liver disease including hepatitis serology, autoimmune causes, metabolic and schistosoma antibodies was done and was found negative. Liver biopsy was consistent with hepatoportal sclerosis and no evidence of cirrhosis. He remained hemodynamically stable, so conservative management was continued and he was discharged after five days of monitoring. Conclusion: Non-cirrhotic portal hypertension is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction. The most common clinical presentation is variceal bleeding, which (in contrast to variceal bleeding in cirrhosis) is often relatively well tolerated due to the preserved liver function. Intraperitoneal rupture of ectopic varices is a rare complication of portal hypertension. Very Few case reports have been published in the literature and little is known about the best management strategy for these patients. The management is individualized according to the condition of the patient and the resources. Objectives of management include suspicion for diagnosis, aggressive resuscitation, and correction of coagulopathy, reduction of portal hypertension and if required, surgical interventions for direct control of the bleeding vessel.

Treatment of Portosystemic Shunt Myelopathy with a Stent Graft Deployed through a Transjugular Intrahepatic Route

A case of surgically created splenorenal shunt complicated with shunt myelopathy was successfully managed by placement of a stent graft within the splenic vein to close the portosystemic shunt and alleviate myelopathy. To our knowledge, this is the first report of a case of shunt myelopathy in a patient with noncirrhotic portal fibrosis without cirrhosis treated by a novel technique wherein a transjugular intrahepatic route was adopted to deploy the stent graft.