Splenorenal Shunt Research Articles

Abstract No. 205: The Preliminary Study of CT Perfusion for the Graft Hemodynamic Changes after Liver Transplantation

Abstract No. 205: The Preliminary Study of CT Perfusion for the Graft Hemodynamic Changes after Liver Transplantation

Nephronophthisis complicated with hepatic fibrosis: an autopsy case with rupture of the splenic artery after renal transplantation

Nephronophthisis (NPHP) is a disease characterized by a genetic cause of chronic renal failure in children and adolescents, complicated with several extra-renal manifestations such as retinal defect and/or liver fibrosis. Although it is difficult to establish the correct diagnosis, mutations in six genes (NPHP 1-6) have recently been identified. Here we report the case of a 25-year-old male with NPHP with congenital hepatic fibrosis. He showed microscopic hematuria and moderate proteinuria at 20 years. Renal biopsy revealed severe interstitial fibrosis, diffuse tubular atrophy and microcysts at this time with chronic kidney disease stage III (Cr 2.43 mg/dl). C3c was positive in glomeruli in direct immunofluorescent study. Although his mother belongs to a family with polycystic kidney disease, he did not have a novel genetic background of Arg585Cys mutation in exon 8 of the PKD1 gene. Magnetic resonance angiography (MRA) showed typical portal hypertension with spleno-renal shunt caused by biopsy-proven liver fibrosis. Thus, we diagnosed him as having undetermined renal cystic or tubulo-interstitial disease complicated with membranoproliferative glomerulonephritis (MPGN). Renal transplantation was performed in January 2005 after 2 years of dialysis therapy. He was transported to our emergency room because of severe abdominal pain in December 2005. A computed tomographic scan showed massive ascites, which were caused by rupture of the splenic artery. Despite full intensive care including intraluminal coiling of the ruptured aneurysm and extensive blood transfusion, we failed to rescue him on the next day. The autopsy findings revealed severe atrophy of the bilateral kidney with multiple cysts along the cortico-medullary border. Obvious portal hypertension, resulting from congenital hepatic fibrosis, could account for the rupture of the splenic artery with aneurysm formation under pressure/volume overload. This is the first report of a NPHP patient with the complication of hepatic fibrosis emerging from an ADPKD family. As it remains elusive on the phenotype-genotype of the Japanese NPHP population, a registration system of cystic disease of the kidney is required.

The Usefulness of Distal Splenorenal Shunt in Children with Portal Hypertension for the Treatment of Severe Thrombocytopenia and Leukopenia

In the current era of transplantation and therapeutic endoscopy, the role of the distal splenorenal shunt (DSRS) for portal hypertension (PH) has diminished. We reviewed the outcome of the use of DSRS in children to determine the usefulness of this operation. In the follow-up course for PH from 1987 to 2006, 15 patients who developed severe thrombocytopenia (platelet count <50 x 10(3)/mm(3)) and/or leukopenia (WBC count <3000/mm(3)) with normal liver function were referred for DSRS. Primary diagnosis was portal vein thrombosis (N=10) and congenital hepatic fibrosis (N=5). Platelet, WBC count, liver function test, and spleen size were checked before and after DSRS. Shunt patency was accessed postoperatively. Operative morbidity, mortality, and long-term outcomes were measured. Platelet count and WBC count increased in individual patients. Mean value of each count increased significantly after DSRS (p=0.002, .004, respectively). Spleen size decreased significantly (N=7, p=0.018). Shunt patency rate was 100%. There was one postoperative complication and no postoperative mortality. Two patients developed portopulmonary hypertension. No patients underwent subsequent transplantation or endoscopic treatment for gastroesophageal varices after DSRS. DSRS is an effective and reliable procedure for children with PH and is still useful for selected pediatric patients.

Renal Hypoperfusion Associated with Splenorenal Shunts in Liver Cirrhosis

Purpose: To determine whether spontaneous a splenorenal shunt can be used as an imaging predictor of early renal hemodynamic changes in patients with cirrhosis. Materials and Methods: The study included 82 cirrhotic patients and 41 control subjects. Three-phase CT was performed and CT attenuation values (Hounsfield units) of the renal cortex in three phases were measured to evaluate renal perfusion. Likelihood ratio tests for trend were conducted for age, presence of ascites, and Child’s grade. Results: The mean CT attenuation values of the renal cortex in cirrhotic patients were significantly lower than the values of control subjects in three phases: 153.3 ± 37.9 versus 173.3 ±25.2 in the arterial phase, 172.6 ± 41.0 versus 197.6 ± 26.5 in the portal phase and136.9 ± 26.0 versus 152.7 ± 20.0 in the delayed phase, respectively. The mean CT attenuation value of cortices in patients with renal hypoperfusion was 119.9 ± 11.8 in the portal phase. Child’s class C (aOR: 58.4, 95% CI: 3.6- 956.2; p < 0.01) and the presence of a renal shunt (aOR: 7.5, 95% CI: 1.8- 30.5; p < 0.01) were associated with renal hypoperfusion. The incidence of renal hypoperfusion was associated with Child’s grade (trend: p < 0.01), and not with the grade of ascites or age. Conclusion: A dilated spontaneous splenorenal shunt may be a risk factor for renal hypoperfusion in cirrhosis.

Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt

Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.

The outcome of living donor liver transplantation with prior spontaneous large portasystemic shunts

We investigated the outcome of living donor liver transplantation (LDLT) with prior spontaneous large portasystemic shunts. Thirty-three patients of 155 patients (21.2%) undergoing LDLT had spontaneous large portasystemic shunts. Portal venous hemodynamics, surgical procedures for shunts, and morbidity and mortality rates were investigated in three types of shunts: splenorenal shunt (SRS group; n = 11), shunt derived from coronary vein (CVS group; n = 6) and umbilical vein shunt (UVS group; n = 15). The two groups of patients (SRS/CVS) received prophylactic surgical repair of shunts during LDLT except for one patient in the SRS group. The flow direction of main portal vein and grade of steal of superior mesenteric vein flow by shunt were significantly different among three groups. No significant differences were observed among three groups in operative parameters, hospitalization and morbidity except for postoperative portal complication. There was no significant difference in the actuarial survival rate among three groups of SRS, CVS and UVS (81.8% vs. 83.3% vs. 86.6% at 1 year respectively). In the SRS group, two patients had postoperative steal of graft portal venous flow by residual SRS that needed further treatment. The outcome of LDLT with prior spontaneous large portasystemic shunts is satisfactory, despite the complexity of the transplant procedures.

Prevention of Recurrent Esophageal Variceal Hemorrhage

Variceal rebleeding is a very frequent and severe complication in cirrhotic patients; therefore, its prevention should be mandatory. Lately several studies demonstrated that the rate of rebleeding was decreased by 40% and overall survival is improved by 20% with beta-blockers. However, this treatment presents some problems, such as the number of nonresponders and contraindications for its use. Recent trials found that the combination of beta-blockers with mononitrate of isosorbide to be superior to beta-blockade alone. Furthermore, endoscopic band ligation also shown to decrease the frequency of rebleeding, complications, and death compared with sclerotherapy and should be the preferred endoscopic treatment. In addition, the comparison between combined pharmacologic treatment with endoscopic treatment present similar rebleeding and mortality rates. More recently, the addition of nadolol to endoscopic band ligation increased the efficacy of endoscopy alone in the prevention of variceal rebleeding. These studies suggest that banding plus drugs could be the treatment of choice for the prophylaxis of rebleeding. When these treatments fail, the recommendation is to use transjugular intrahepatic portosystemic shunt (TIPS) or surgical shunts. Both treatments are effective in preventing rebleeding; however, they are associated with a greater risk of encephalopathy. The comparison of portacaval shunts with TIPS demonstrated that TIPS patients presented higher rebleeding, treatment failure, and transplantation. Another randomized controlled trial comparing distal splenorenal shunt with TIPS shows that variceal rebleeding was similar in both groups without differences in encephalopathy and mortality. The only difference observed was the higher rate of reintervention observed in the TIPS group to maintain his patency.

Hepatic Encephalopathy from Dual Splenorenal Shunts Treated with Balloon-Occluded Retrograde Transvenous Obliteration by Using a Double-Balloon Technique

The authors report on their experience with a 61-year-old woman with hepatic encephalopathy and two large splenorenal shunts. She was treated with dual balloon-occluded retrograde transvenous obliteration (B-RTO). Retrograde injection of the sclerosant into the shunt was performed with balloon occlusion of the two vessels draining into the left renal vein, and complete occlusion was achieved without complications. After B-RTO, the patient's clinical symptoms improved substantially and contrast-enhanced computed tomography (CT) performed 3 months later demonstrated complete thrombosis of the splenorenal shunts.

Cost of preventing variceal rebleeding with transjugular intrahepatic portal systemic shunt and distal splenorenal shunt

We examined the cost and cost effectiveness of distal splenorenal shunt (DSRS) and transjugular intrahepatic portosystemic shunt (TIPS) in the prevention of variceal rebleeding. Patients participated in a randomized controlled trial comparing DSRS to TIPS. Quality of life (QOL) was measured using SF-36 preceding randomization and yearly thereafter. Cost utility analysis was performed using TreeAge DATA. Costs for both in- and out-patient events and interventions were obtained for each patient. Costs using coated stents were estimated using different rates of stenosis. Incremental cost effectiveness ratios (ICERs) were determined at 1, 3 and 5 years. The average yearly costs of managing patients after TIPS and DSRS over 5 years were similar, $16,363 and $13,492, respectively. Cost of TIPS for surviving patients exceeded the cost of DSRS at years 3 and 5 but not significantly. ICERs per life saved favored TIPS at year 5 ($61,000). If coated rather than bare stents were used the cost effectiveness of TIPS increased slightly. TIPS is as effective as DSRS in preventing variceal rebleeding and may be more cost effective. TIPS, in all aspects, is equal to DSRS in the prevention of variceal rebleeding in patients who are medical failures.

Hypertension portale idiopathique associée à une maladie cœliaque : à propos d’un cas

We describe for the first time a case of idiopathic portal hypertension (IPH) in a 31 year old patient with coeliac disease (CD). She had splenomegaly, pancytopenia and an unexplained portal hypertension in the absence of any histological abnormalities of the liver. The diagnosis of coeliac disease was based on histological and serological data. Treatment included a splenectomy and a splenorenal shunt.

Jejunal Varices Detected on Capsule Endoscopy as the Cause of Recurrent GI Bleed

Purpose: We present a case of a 45 year old male with history of abdominal gunshot wound in 1980's and small wedge resection of the liver for laceration. Patient developed GI hemorrhage in 2004, and workup revealed hepatic hemangiomas as the likely source. Patient reportedly had embolization with coils without success and continued to have bleeding episodes. Several months later, he had right hepatectomy and Roux-en-Y hepaticojejunostomy since hepatic hemangiomas were still the suspected etiology of his GI bleed. However, he continued to have bleeding. At some point around this time, he was diagnosed with portal vein thrombosis and portal hypertension, though the exact details of this diagnosis are not known. He had extensive evaluations including multiple endoscopies at two teaching medical centers with no definitive conclusion. Patient was then seen at a different hospital for a massive GI hemorrhage in 2006. During that admission, EGD/enteroscopy showed grade I esophageal varices, questionable gastric varices, and mild portal gastropathy. Colonoscopy showed mild portal colopathy and small internal hemorroids. Tagged RBC scans and angiogram did not locate the source of bleed. CT scan suggested the possibility of varices within jejunum. This is when the patient was referred to our facility for capsule endoscopy, which demonstrated four clusters of varices in jejunum with fresh blood approximately 2 hours and 40 minutes into the study. The patient was not a candidate for TIPS because of his extensive portal vein thrombosis and underwent surgery for splenorenal shunt with an associated splenectomy. He currently remains stable without any further episodes of gastrointestinal bleeding. Bleeding from jejunal varices is an uncommon manifestation of portal hypertension. Recently, capsule endoscopy has been used in the workup of obscure-overt GI bleed as in our case. Our case represents one of the first reports of jejunal varices detected on capsule endoscopy as the cause of GI hemorrhage.[figure1]Figure

Intraoperative Flow Measurement of Native Liver and Allograft During Orthotopic Liver Transplantation in Children

Hepatic hemodynamic changes during liver transplantation (OLT) in children have not yet been studied. We measured intraoperative portal vein flow (PVF) and hepatic arterial flow (HAF) (mL/min) in 53 children and 58 grafts during OLT. Flows were measured in the native organ and in the allograft. In the native liver, PVF and HAF are similar; after transplantation they return to the physiological situation. No flow differences were seen between whole and partial grafts. Among the 8 (14%) portal vein thromboses, PVF was lower in both the native liver and the graft than in the no thrombosis group ( P < .05). PVF <5 mL/min/kg was a risk factor to develop PV thrombosis. No graft loss occurred in 3 cases without PVF at the time of OLTs despite the observation that repermeabilization was not possible. In 4 patients with PVF <5 mL/min/kg, after tying a spontaneous spleno-renal shunt (n = 3) or performing a porto-renal vein anastomosis (n = 1), PVF reached >20 mL/min/kg, avoiding thrombosis. In conclusion, PVF and HAF measurements during pediatric OLT may predict patients at high risk for development of PV thrombosis.

Variceal pseudoaneurysm in sinistral portal hypertension

Localized, left-sided portal hypertension, also known as sinistral portal hypertension, is a rare entity most commonly seen as a result of splenic vein thrombosis. Aneurysms of the portal venous system usually occur either at the hilum or at the splenomesenteric confluence. Aneurysms of portal venous collaterals are rare, and pseudoaneurysms have not been described. We report the case of an elderly woman with asymptomatic sinistral hypertension who developed a pseudoaneurysm of a splenorenal shunt following blunt trauma.

Subtotal Splenectomy and Central Splenorenal Shunt for Treatment of Bleeding from Roux En Y Jejunal Loop Varices Secondary to Portal Hypertension

To present subtotal splenectomy and splenorenal shunt as a surgical option to treat severe bleeding from a Roux en Y jejunal loop varices secondary to portal hypertension. A 64-year-old white woman presented severe episodes of bleeding from varices inside a Roux en Y jejunal loop secondary to portal hypertension due to cirrhosis. Subtotal splenectomy was performed with preservation of the upper splenic pole supplied by the splenogastric vessels. This procedure was combined with a central splenorenal shunt to divert part of portal blood to systemic flow. This procedure was safely performed with no complications. A 2-year post-operative follow-up of the patient has been uneventful. No re-bleeding occurred during this period and she returned to her normal life. Subtotal splenectomy combined with central splenorenal shunt seems to be a safe procedure useful for the treatment of enteral bleedings due to portal hypertension.

Interventional Radiological Management of Prehepatic Obstruction the Splanchnic Venous System

The purpose of this study was to retrospectively evaluate interventional radiological management of patients with symptomatic portal hypertension secondary to obstruction of splanchnic veins. Twenty-four patients, 15 males and 9 females, 0.75 to 79 years old (mean, 36.4 years), with symptomatic portal hypertension, secondary to splanchnic venous obstruction, were treated by percutaneous methods. Causes and extent of splanchnic venous obstruction and methods are summarized following a retrospective evaluation. Obstructions were localized to the main portal vein (n = 22), intrahepatic portal veins (n = 8), splenic vein (n = 4), and/or mesenteric veins (n = 4). Interventional treatment of 22 (92%) patients included recanalization (n = 19), pharmacological thrombolysis (n = 1), and mechanical thrombectomy (n = 5). Partial embolization of the spleen was done in five patients, in two of them as the only possible treatment. TIPS placement was necessary in 10 patients, while an existing occluded TIPS was revised in two patients. Transhepatic embolization of varices was performed in one patient, and transfemoral embolization of splenorenal shunt was performed in another. Thirty-day mortality was 13.6% (n=3). During the follow-up, ranging between 2 days and 58 months, revision was necessary in five patients. An immediate improvement of presenting symptoms was achieved in 20 patients (83%). We conclude that interventional procedures can be successfully performed in the majority of patients with obstruction of splanchnic veins, with subsequent improvement of symptoms. Treatment should be customized according to the site and nature of obstruction.

Porto-systemic shunt using adrenal vein as a conduit; an alternative procedure for spleno – renal shunt

BackgroundCurrently, portal hypertension is still big problem for the patients with serious liver diseases. Variceal bleeding is one of the most important complications of portal hypertension. In case of failure of endoscopic and combined medical treatments, surgical decompressive shunts are required. We emphasized an alternative splenorenal shunt procedure using adrenal vein as a conduit.Case presentationA 26-year-old male suffered from recurrent variceal bleeding was considered for surgical therapy. Although we planned to perform a distal splenorenal shunt procedure, it was observed to be difficult. Therefore left adrenal vein was used as a conduit between left renal vein and splenic vein after splenic artery was ligated. He did well and was discharged from the hospital on the postoperative day 6. In the follow up period for nine months, endoscopic and ultrasonographic examinations were normal.ConclusionWe concluded that, in case of failure to perform distal splenorenal shunt due to technical problems, alternative porto-systemic shunt procedure using the adrenal vein as a vascular conduit can be safely employed.

Influência do grau de insuficiência hepática e do índice de congestão portal na recidiva hemorrágica de cirróticos submetidos a cirurgia de Teixeira-Warren

Bleeding from esophagogastric varices is the worst and most lethal complication of cirrhotic portal hypertension. Distal splenorenal shunt (Warrens surgery) is used in the therapeutic of this patients, Child A and B, with rebleeding after clinical endoscopic therapy. The portal vein congestion index is elevated in cirrhotic portal hypertension and could predict rebleeding after Warrens surgery in these patients. To verify if the portal vein congestion index or liver function (Child-Pugh) at preoperative are predictive factors of rebleeding after Warrens surgery. Sixty-two cirrhotic patients were submitted to Warrens surgery at "Santa Casa" Medical School and Hospital - Liver and Portal Hypertension Unit, São Paulo, SP, Brazil. Fifty-eight were analyzed for Child-Pugh class and 36 for portal vein congestion index, divided in two groups: with or without rebleeding and statistical analysis was performed. In the rebleeding group, 69% were Child B, with portal vein congestion index = 0.09. The group without rebleeding show us 62% patients Child A with portal vein congestion index = 0.076. The difference was significant for Child-Pugh class but not to portal vein congestion index. Portal vein congestion index was not predictive of rebleeding after Warrens surgery, but cirrhotics Child B have more chance to rebleed after this surgery than Child A.

Splenic Artery Embolization and Balloon Occlusion Retrograde Alcohol Embolization in a Patient with Bleeding Gastric Varices

We present a patient with a large spontaneous splenorenal shunt secondary to isolated splenic vein thrombosis who developed severe bleeding from fundal gastric varices. The patient was managed emergently with splenic artery embolization and balloon occlusion retrograde embolization of the varices with alcohol. We discuss the clinical presentation, embolization techniques, and a potential complication of the use of alcohol for this purpose.

Distal splenorenal shunt with partial spleen resection

Hypersplenism is a common complication of portal hypertension. Cytopenia in hypersplenism is predominantly caused by splenomegaly. Distal splenorenal shunt (Warren) with partial spleen resection is an original surgical technique that regulates cytopenia by reduction of the enlarged spleen. The aim of our study was to present the advantages of distal splenorenal shunt (Warren) with partial spleen resection comparing morbidity and mortality in a group of patients treated by distal splenorenal shunt with partial spleen resection with a group of patients treated only by a distal splenorenal shunt. From 1995 to 2003, 41 patients with portal hypertension were surgically treated due to hypersplenism and oesophageal varices. The first group consisted of 20 patients (11 male, mean age 42.3 years) who were treated by distal splenorenal shunt with partial spleen resection. The second group consisted of 21 patients (13 male, mean age 49.4 years) that were treated by distal splenorenal shunt only. All patients underwent endoscopy and assessment of oesophageal varices. The size of the spleen was evaluated by ultrasound, CT or by scintigraphy. Angiography was performed in all patients. The platelet and white blood cell count and haemoglobin level were registered. Postoperatively, we noted blood transfusion, complications and total hospital stay. Follow-up period was 12 months, with first checkup after one month. In the first group, only one patient had splenomegaly postoperatively (5%), while in the second group there were 13 patients with splenomegaly (68%). Before surgery, the mean platelet count in the first group was 51.6 +/- 18.3 x 10(9)/l, to 118.6 +/- 25.4 x 10(9)/l postoperatively. The mean platelet count in the second group was 67.6 +/- 22.8 x 10(9)/l, to 87.8 +/- 32.1 x 10(9)/l postoperatively. Concerning postoperative splenomegaly, statistically significant difference was noted between the first and the second group (p < 0.05). Comparing the postoperative platelet count between the first and second group, we found that there was a very significant statistical difference, too (p < 0.01). Distal splenorenal shunt (Warren) with partial spleen resection is a very reliable surgical technique in treatment of hypersplenism and decompression of oesophageal varices caused by portal hypertension and has advantage in treatment of hypersplenism over the distal splenorenal shunt method.

Pre- and postoperative systemic hemodynamic evaluation in patients subjected to esophagogastric devascularization plus splenectomy and distal splenorenal shunt: A comparative study in schistomomal portal hypertension

To investigate the systemic hemodynamic effects of two surgical procedures largely employed for treatment of schistosomal portal hypertension. Thirty-six patients undergoing elective surgical treatment of portal hypertension due to hepatosplenic mansonic schistosomiasis were prospectively evaluated. All patients were subjected to preoperative pulmonary artery catheterization; 17 were submitted to esophagogastric devascularization and splenectomy (EGDS) and 19 to distal splenorenal shunt (DSRS). The systemic hemodynamic assessment was repeated 4 d after the surgical procedure. Preoperative evaluation revealed (mean +/- SD) an increased cardiac index (4.78 +/- 1.13 L/min per m(2)), associated with a reduction in systemic vascular resistance index (1457 +/- 380.7 dynes.s/cm(5).m(2)). The mean pulmonary artery pressure (18 +/- 5.1 mmHg) as well as the right atrial pressure (7.9 +/- 2.5 mmHg) were increased, while the pulmonary vascular resistance index (133 +/- 62 dynes x s/cm(5) x m(2)) was decreased. Four days after EGDS, a significant reduction in cardiac index (3.80 +/- 0.4 L/min per m(2), P < 0.001) and increase in systemic vascular resistance index (1901.4 +/- 330.2 dynes x s/cm(5) x m(2), P < 0.001) toward normal levels were observed. There was also a significant reduction in pulmonary artery pressure (12.65 +/- 4.7 mmHg, P < 0.001) and no significant changes in the pulmonary vascular resistance index (141.6 +/- 102.9 dynes x s/cm(5) x m(2)). Four days after DSRS, a non-significant increase in cardiac index (5.2 +/- 0.76 L/min per m(2)) and systemic vascular resistance index (1389 +/- 311 dynes x s/cm(5) x m(2)) was observed. There was also a non-significant increase in pulmonary artery pressure (19.84 +/- 5.2 mmHg), right cardiac work index (1.38 +/- 0.4 kg x m/m(2)) and right ventricular systolic work index (16.3 +/- 6.3 g x m/m(2)), without significant changes in the pulmonary vascular resistance index (139.7 +/- 67.8 dynes xs/cm(5) x m(2)). The hyperdynamic circulatory state observed in mansonic schistosomiasis was corrected by EGDS, but was maintained in patients who underwent DSRS. Similarly, the elevated mean pulmonary artery pressure was corrected after EGDS and maintained after DSRS. EGDS seems to be the most physiologic surgery for patients with schistosomal portal hypertension.