Intrahepatic Shunt Research Articles

Abstract No. 620 Incidence and pathophysiology of transjugular intrahepatic portsystemic shunt (TIPS) complicated with lower extremity edema

TIPS is consistently proven to benefit patients with ascites, hydrothorax and gastrointestinal bleeding, however adverse events do occur after TIPS. Lower extremity edema (LEE), an under reported complication of TIPS, can cause significant morbidity. This study aims to measure the incidence and pathophysiology of LEE after TIPS. Between 2006-2016, a total of 220 patients underwent TIPS at our institution. LEE was defined as new onset or worsened edema up to one year after TIPS. Changes in diuretic use, clinical outcome of LEE, etiology of cirrhosis and effectiveness of TIPS in reducing ascites/hydrothorax were documented. Cardiac ventricular function was evaluated by echocardiography. Patients without close follow-up were excluded. Improvement in ascites was defined 25% fewer paracenteses. 205 patients were eligible for inclusion. 132 (64%) were male. Mean (SD) age was 54 (11) years old. Mean portosystemic shunt gradient changed from 18 to 7 mm Hg with mean (SD) post TIPS gradient of 7 (3). 80 (39%) patients had LEE at baseline. 101 patients (49%) had new onset or worsened LEE with median onset at one month follow-up. 62 out of 101 patients with LEE (61%) subsequently improved their edema (9 with conservative measures, 53 with diuretics). LEE was persistent despite diuretics in 36 patients (35%) at median follow-up of 21 months. Of note 30 out of 80 (37%) with baseline LEE had worsening edema. Multiple logistic regression analysis showed no significant impact from age, gender, ejection fraction, portosystemic gradient change and improvement in ascites on LEE. Presence of baseline edema prior to TIPS was protective in multivariate analysis (OR = -2.7, p = 0.007). LEE can complicate TIPS in almost half of patients, independent of left ventricular function. Close clinical follow-up after TIPS, without initially changing diuretics is recommended. Baseline edema does not necessarily lead to worsening edema after TIPS. Further studies are needed to better clarify the pathophysiology of LEE after TIPS.

Transjugular liver biopsy after transjugular intrahepatic portosystemic shunt (TIPS) or direct intrahepatic portocaval shunt (DIPS): Is it feasible, effective, and safe?

To evaluate our experience with transjugular liver biopsies (TJLB) in patients with previously placed transjugular intrahepatic portosystemic shunt (TIPS) or direct intrahepatic portocaval shunt (DIPS). A single-institution retrospective review was performed looking at all TJLBs in patients with previously placed TIPS or DIPS over the past seven years. There were six men and one woman (mean age 57.9±8.8 [SD] years; range: 47-71 years). Patient demographics, indications, procedural details, laboratory data, complications, and pathology were recorded. Patients with occluded TIPS were excluded from this study. Seven TJLBs were performed, five in patients who had a TIPS and two who had a DIPS. Of the patients with TIPS, biopsies were performed from the same hepatic vein as TIPS in three procedures and from a different hepatic vein in two procedures. In DIPS patients, both biopsies were performed from the right hepatic vein. The reasons for the transjugular rather than the percutaneous approach to liver biopsy included ascites, coagulopathy, or need for concurrent TIPS/DIPS evaluation and/or revision. All procedures were technically successful with adequate samples obtained. There were no immediate or delayed complications. Our results suggest that if needed a TJLB can safely and effectively be performed in patients with previously placed TIPS or DIPS.

Non-Cirrhotic Hepatic Encephalopathy Presenting as a Spontaneous Intrahepatic Porto-systemic Shunt

Hepatic encephalopathy (HE) is a common manifestation of cirrhosis, which is treated medically. Cirrhotic patients who require TIPS may also develop HE. Spontaneous porto-systemic shunts are a rare occurrence. In patients without liver disease they can be either congenital or spontaneous. Many go undetected if patients are asymptomatic. We present a case of a non-cirrhotic patient with a spontaneous intrahepatic porto-systemic shunt presenting with encephalopathy. A 58-year-old female presented with acute onset encephalopathy and concern for seizures. She underwent an EEG which was consistent with complex partial status epilepticus. She had an ammonia level of 56 but ranged as high as 110 mcmol/L. Liver function tests, PT/INR were within normal limits. She had no prior history of liver disease. She was not on any medications to cause hyperammonemia. She was started on AEDs and lactulose but continued to have cognitive deficits, trouble with short-term memory, concentration and balance. An LP was performed and revealed pleocytosis. Neurology felt her presentation and symptomology was consistent with autoimmune/paraneoplastic encephalitis and she was placed on IV steroids. A paraneoplastic antibody panel was unrevealing. She was referred to Hepatology for evaluation of persistent hyperammonemia. Workup revealed a large porto-hepatic shunt on CT scan. Chronic liver disease workup was unremarkable. Symptoms persisted despite medical treatment for hyperammonemia. She was referred to Interventional Radiology for embolization of her spontaneous intrahepatic porto-systemic shunt as there was suspicion that her symptoms and presentation were due to non-cirrhotic hepatic encephalopathy secondary to the intrahepatic shunt. She underwent a successful embolization. Her ammonia levels normalized. She has had an improvement in her symptoms and has been without overt encephalopathy. She also remains seizure free. Further follow up will be needed to assess for concurrent autoimmune/paraneoplastic encephalitis versus symptoms entirely due to her porto-systemic shunt. Spontaneous intrahepatic porto-systemic shunts are rare with many patients being asymptomatic. Mildly symptomatic patients are initially managed conservatively with medications and dietary modifications, however if symptoms persist or are severe enough, they may require intervention such as IR guided embolization to redistribute blood flow within the portal system.Figure: large shunt between the right portal and hepatic veins.Figure: Portal venography demonstrating large portal vein-hepatic vein shunt with poor parenchymal opacification of the liver giving preferential flow through the shunt rather than into native intrahepatic portal veins.Figure: Following embolization there was near complete occlusion of the shunt itself with preferential flow into the intrahepatic portal veins. Following shunt embolization marked improvement in the degree of hepatic parenchymal opacification was noted.

A Rare Case of Flood Syndrome and a Perspective on Management

Flood Syndrome is a rare complication of cirrhosis accompanied by ascites. The syndrome is named for the exsanguination of ascitic fluid caused by the spontaneous rupture of an umbilical hernia. Very little data exists regarding its management as the literature is solely drawn from case reports. We present the case of a 42 year-old male with alcoholic cirrhosis presenting from home with Flood Syndrome treated in piecemeal fashion with complete resolution of symptoms. A 42 year-old male with alcoholic cirrhosis complicated by grade I esophageal varices, GI bleeds, massive ascites, and a large umbilical hernia presented to the ED with sudden and spontaneous rupture of his hernia leading to copious drainage of ascitic fluid from his umbilicus (Fig. 1) without inciting event. Over the first few hours the patient lost approximately 9 liters of exsanguinated ascetic fluid and was given IV albumin for fluid resuscitation to maintain intravascular oncotic pressure. The patient was evaluated by the gastroenterology and transplant surgery teams where a decision was made to perform an emergent transjugular intrahepatic portosystematic shunt (TIPS) to relieve portal pressures and subsequent intraabdominal pressure. The patient had resolution of symptoms following successful TIPS and was seen again by the surgery team to have an umbilical hernia repair. Flood syndrome is named for the “flood” of ascetic fluid that accompanies spontaneous rupture of an umbilical hernia and is a rare complication of cirrhosis with associated ascites. Cirrhotic patients with ascites have a 20% risk of umbilical hernia development during the course of their disease. In most cases (>75%), development of cutaneous infection and/or necrosis precedes hernia rupture. Any action that increases intra-abdominal pressure, such as coughing or vomiting, can precipitate this event. We present the case of a 42 year-old male presenting with Flood Syndrome treated in piecemeal with TIPS followed by successful surgical umbilical repair. Limited data exists regarding the treatment and is generally regarded as a surgical issue. We propose that these patients should be initially evaluated for TIPS to relieve the underlying intraabdominal pressure with subsequent referral for surgical repair shortly afterwards. We speculate that following reduction of portal pressure, complications of surgery such as wound dehiscence or recurrent episodes of exsanguination will be reduced.Figure: Necrotic Umbilical Hernia.

Case of Chylous Ascites: A Diagnostic Dilemma

We present a case of an 83 year-old male who is status post orthotopic liver transplant in November 2005 for alcoholic cirrhosis, presenting to Henry Ford Hospital with increasing abdominal girth. His symptoms began 2 months prior to presentation following cardiac pacemaker placement for heart block. He was hospitalized shortly after his procedure for shortness of breath and was found to have a pericardial effusion, which was thought to be inflammatory in nature and resolved with steroids. He had been following in the transplant clinic on a regular basis without issues, and was maintained on a stable immunosuppression regimen of mycophenolate and Sirolimus for several years. As part of the work up for his new-onset ascites, a paracentesis was performed. Fluid analysis demonstrated 702 WBCs with 77% lymphocytes, serum-albumin ascites gradient (SAAG) < 1.1 g/dL, total protein < 2.5 g/dL and triglycerides of 2360 mg/dL; consistent with chylous ascites. Lipase and amylase levels were within normal limits. Fungal cultures, acid-fast bacilli stains and cytology were all negative. Echocardiogram showed normal right-sided pressures with preserved ejection fraction and stable pericardial effusion. CT abdomen was unremarkable with no evidence of cirrhosis or portal hypertension. Lymphoscintigraphy study was unremarkable. Liver biopsy was performed without evidence of cirrhosis or rejection. Cardiology was consulted to determine whether the ascites was secondary to venous/lymphatic obstruction as a complication of pacemaker placement or heart failure; this was felt unlikely given preserved cardiac function and normal positioning of pacemaker leads on x-ray. Ultimately, Sirolimus was felt to be the culprit of his chylous ascites. Sirolimus was subsequently discontinued and cyclosporine was initiated with resolution of his ascites. Chylous ascites is a rare finding. It most commonly occurs in the setting of malignancy, lymphatic/vascular obstruction or cirrhosis. Less common etiologies include mycobacterial infection, congenital, inflammatory, post-operative, traumatic and medications such as calcium channel blockers and Sirolimus. Few cases of chylous ascites secondary to Sirolimus have been reported, particularly in renal transplant patients. There are no reported cases of Sirolimus-induced chylous ascites in liver transplant patients. The diagnosis is established by abdominal paracentesis, with fluid studies demonstrating a triglyceride level >200 mg/dL. SAAG and protein levels may vary. Acid-fast bacilli stain and ADA should be performed as well to rule out tuberculous ascites in those at high risk, along with amylase, lipase and standard fluid studies. Abdominal CT should be obtained to rule out pathologic lymph nodes and masses, and lymphoscintigraphy or lymphangiography can aid in detecting leakage from lymphatics and patency of the thoracic duct. Failure to identify a clear etiology following this workup should prompt an investigation into drug etiologies. Treatment is aimed at correction of the underlying etiology (or withholding the offending agent) along with therapeutic paracenteses as needed. A high protein and low fat diet with medium chain triglycerides is recommended. Occasionally, surgery may be performed for obstructive etiologies such as malignancy. Transjugular intrahepatic shunts may be performed for cirrhotic patients. There is also limited evidence for the use of orlistat, somatostatin and octreotride in the mitigation of chylous ascites.

Circulating fibroblast growth factor 21 in patients with liver cirrhosis.

Fibroblast growth factor 21 (FGF21) is an adipokine and hepatokine, and its hepatic expression is induced in the injured liver. Adiponectin, whose systemic levels are positively correlated with measures of hepatic injury in patients with liver cirrhosis, is a downstream effector of FGF21. The aim of the present study was to identify possible associations of serum FGF21 with measures of liver function in patients with liver cirrhosis. FGF21 was determined by ELISA in serum of 42 patients. FGF21 was not linked to disease severity assessed by the Child-Pugh and MELD score. Levels were not changed in those patients with varices and/or ascites. Systemic FGF21 did not correlate with markers of liver and kidney function, inflammatory proteins or adipokines like adiponectin. Levels in hepatic and portal vein of 37 patients were also measured, but there was no transhepatic FGF21 gradient. Three months after insertion of a transjugular intrahepatic shunt hepatic venous pressure gradient was markedly improved, while FGF21 in serum of these 13 patients was not changed. The present study shows that hepatic release and systemic FGF21 are not linked to measures of liver function in patients with liver cirrhosis.

Radiological Management of Hepatocellular Carcinoma Associated With Hepatic Venous Outflow Tract Obstruction

Background and Aim: Hepatocellular carcinoma (HCC) associated with hepatic venous outflow tract obstruction (HVOTO) is an unusual entity which has recently gained recognition. Treatment of both entities is essential in prolonging survival. Interventional radiologic techniques are the established therapeutic options for HVOTO and HCC and thus treatment becomes challenging. We illustrate two representative cases treated successfully treated at our centre. Methods: Patients with clinical diagnosis of HVOTO were evaluated by ultrasound Doppler, multiphase CT liver (MPCT)/dynamic MRI. HVOTO was confirmed by the presence of intrahepatic venous obstruction on Doppler and the treatment was based on the site/type of venous obstruction and included inferior vena cava(IVC) angioplasty or hepatic vein angioplasty (both with or without stenting) and trans-jugular intrahepatic port-systemic shunt (TIPS)/direct intrahepatic port-systemic shunt. On MPCT, HCC diagnosis was based on the modified EASL criteria. Staging and treatment options were according to the Barcelona clinic liver cancer (BCLC) staging. Results: Case one was diagnosed with absent all three hepatic veins with BCLC B stage HCC on MPCT and Doppler. TIPS was performed for HVOTO and subsequently TACE was undertaken for HCC. Follow up Doppler confirmed normal flow through the shunt and HCC depicted complete response. Case two was diagnosed with stenosis of all hepatic veins and IVC stenosis at cavo-atrial junction with intrahepatic collaterals. He also had BCLC B stage HCC on MPCT. IVC angioplasty was performed following which there was persistent stenosis on Doppler assessment, IVC stenting was subsequently performed which restored the normal calibre and flow in IVC. TACE was done for HCC and the residual tumor was completed treated by acetic acid ablation. Conclusion: Radiological techniques play a key role in the diagnosis and management of HVOTO-HCC. Both the entities need to be treated and this makes the treatment very challenging. The authors have none to declare.

The Improvement of Nutritional Status of Cirrhotic Patients After Transjugular Intrahepatic Portosysthemic Shunt is Associated With the Amelioration of Cognitive Impairment

The Improvement of Nutritional Status of Cirrhotic Patients After Transjugular Intrahepatic Portosysthemic Shunt is Associated With the Amelioration of Cognitive Impairment

Abstract No. 548 - Impact of intrahepatic veno-venous shunts on wedged hepatic and direct portal pressures

Abstract No. 548 - Impact of intrahepatic veno-venous shunts on wedged hepatic and direct portal pressures

Outcomes of surgical shunts and transjugular intrahepatic portasystemic stent shunts for complicated portal hypertension.

Transjugular intrahepatic portasystemic stent shunt (TIPSS), instead of surgical shunt, has become the standard treatment for patients with complicated portal hypertension. This study compared outcomes in patients who underwent TIPSS or surgical shunting for complicated portal hypertension. This was a retrospective study of all consecutive patients who received portasystemic shunts from 1994 to 2014 at a single institution. Patients who underwent surgical shunting were compared with those who had a TIPSS procedure following one-to-one propensity score matching. The primary study endpoints were overall survival and shunt failure, defined as major variceal rebleeding, relapse of refractory ascites, irreversible shunt occlusion, liver failure requiring liver transplantation, or death. A total of 471 patients received either a surgical shunt or TIPSS. Of these, 334 consecutive patients with cirrhosis who underwent elective surgical shunting (34) or TIPSS (300) for repeated variceal bleeding or refractory ascites were evaluated. Propensity score matching yielded 31 pairs of patients. There were no between-group differences in morbidity and 30-day mortality rates. However, shunt failure was less frequent after surgical shunting than TIPSS (6 of 31 versus 16 of 31; P = 0·016). The 5-year shunt failure-free survival (77 versus 15 per cent; P = 0·008) and overall survival (93 versus 42 per cent; P = 0·037) rates were higher for patients with surgical shunts. Multivariable analysis revealed that a Model for End-Stage Liver Disease (MELD) score exceeding14 and TIPSS were independently associated with shunt failure. In patients with MELD scores of 14 or less, the 5-year overall survival rate remained higher after surgical shunting than TIPSS (100 versus 40 per cent; P < 0·001). Surgical shunting achieved better results than TIPSS in patients with complicated portal hypertension and low MELD scores.

Agenesia del ductus venoso: dos casos clínicos tipo

La agenesia del ductus venoso (ADV) no es infrecuente. Tiene dos manifestaciones clínicas que presentan diferentes pronósticos. En la ausencia del ductus venoso, el flujo venoso empleará caminos alternativos. Hay dos posibilidades. En una de ellas habrá un shunt extrahepático, y se caracterizará por sobrecarga de volumen sistémico y tendencia a la insuficiencia cardiaca y en otra habrá un shunt intrahepático, con drenaje al seno portal, que presenta mejor pronóstico. Es necesario descartar anomalías estructurales asociadas y cromosopatías, ya que de ello depende el pronóstico. Especialmente importante es anali0zar el sistema venoso biliar. Presentamos dos casos clínicos con cada una de estas variantes, ninguna de la cuales tuvo asociación a cromosopatías.

Double Inferior Vena Cava (IVC) With Intrahepatic Interruption, Hemiazygos Vein Continuation, and Intrahepatic Venous Shunt

The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right IVC. In variant forms, the interruption of the intrahepatic segment of the IVC, azygos or hemiazygos continuation, or retroaortic course of the renal vein may be seen. An intrahepatic venous shunt accompanying a double IVC variant is an extremely rare anomaly. We report a case of 40-year-old female patient with double IVC, hemiazygos continuation, intrahepatic IVC interruption, and a transhepatic venous shunt.

Expanding Role of US in Diagnosis of Portal Hypertension and Cirrhotic Complications

Color Doppler Ultrasonography (CD-US) has an advantage of being non-invasiveness, so many attempts to investigate the hemodynamic alterations in cirrhosis and the response to medical treatment of portal hypertension as well as morphologic change of liver have been made. In particular, any CD-US parameter that could be a suitable substitute for the invasive current gold standard for assessing portal hypertension by measuring hepatic venous pressure gradient (HVPG) and hepatic fibrosis evaluated by liver biopsy, would be highly desirable. The presence of intrahepatic shunts resulting from portal hypertension can be helpful to diagnosis of portal hypertension and cirrhosis through novel characteristic CD-US index. It has been recently suggested that the extent of abnormality in Doppler hepatic vein (HV) waveform is associated with degree of HVPG as well as severity of hepatic fibrosis, and a change in HV waveform is closely correlated with that in HVPG. Abnormal flattened HV wave form is thought to be due to hemodynamically blunting the effect of variations in central venous pressure during the cardiac cycle, which is arising from increased HV inflow via intrahepatic shunts secondary to portal hypertension. Moreover, under microbubble contrast enhancement, CD-US detection of decreased hepatic vein transit time due to presence of intrahepatic shunts can be useful to assess the severity of portal hypertension and grade of fibrosis. However, for being reproducible and accurate, a cooperative training program of operators with strict examination protocol would be helpful to reduce the rate of intra-and inter-observer variation. Consequently, developments of novel CD-US indices and techniques are ongoing to overcome the limitations of conventional CD-US methods in diagnosis of portal hypertension and cirrhosis, and some of them are enough to potentially allow widespread clinical use.

Hepatorenal syndrome: role of the transjugular intrahepatic stent shunt in real life practice.

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SAT-043 - Sarcopenia represents a risk factor for the development of hepatic encephalopathy after transjugular intrahepatic portosysthemic shunt

SAT-043 - Sarcopenia represents a risk factor for the development of hepatic encephalopathy after transjugular intrahepatic portosysthemic shunt

Pros and cons of Danis stent application in esophageal bleeding

Aim. A critical analysis of world clinical experience of endoscopic hemostasis with self-expanding nitinol Danis stents application in acute bleeding from esophageal varices.Methods. Literature data of last 10 years about self-expanding nitinol Danis stents application at the peak of esophageal bleeding or in high risk of its recurrence were analyzed.Results. The advantages of Danis stent include minimal injury, good tolerance by the patient, providing physiological drainage of saliva and the possibility of fluid and food intake through the mouth, possibility of a repeated endoscopic inspection of the esophagus and stomach after the application of the stent, reducing the risk of aspiration pneumonia, the impossibility of removal or displacement by the patient in agitation. According to most authors, Danis stent allows saving time to get ready for safe transjugular intrahepatic portocaval shunt (TIPS), surgical intervention or liver transplantation. This time should be used for performing all procedures necessary to stabilize the patient’s condition and clarify the nature of the existing pathology. Disadvantages include high risk of migration from the esophagus into the stomach with loss of its plugging function, limitation for treatment of only esophageal varices, development of small erosions or ulcers on the mucous membrane of the esophagus after removal of the stent. When Danis stent was used as a final method of treatment in patients who do not fit for TIPS or liver transplant, the mortality rate reached 50%.Conclusion. To date, it is not yet clarified whether endoscopic hemostasis with self-expanding nitinol Danis stents should be firmly used in the treatment algorithm of patients with variceal bleeding; confirmation of initial clinical results in further comparative randomized controlled trials is necessary.

Hepatic vein transit time of second-generation ultrasound contrast agent: new tool in the assessment of portal hypertension

It has been demonstrated that Doppler waveform of the hepatic vein (normally triphasic) is transformed into a biphasic or monophasic waveform in cirrhotic patients. The compressive mechanism of liver tissue has been considered up till now the cause of this change. Moreover, cirrhotics show, after USCA injection, a much earlier HVTT due to intrahepatic shunts. Our aim was to prospectively evaluate the correlation between Doppler pattern of hepatic vein and HVTT of a second-generation USCA; we also correlated HVTT with the most common indexes of portal hypertension. We enrolled 38 participants: 33 cirrhotics and 5 healthy controls. Doppler shift signals were obtained from the right hepatic vein. To characterize the hepatic vein pattern, we used the hepatic vein waveform index (HVWI). This index becomes >1 with the appearance of the triphasic waveform. We recorded a clip from 20s before to 2min after a peripheral intravenous bolus injection of 2.4ml of USCA (sulfur hexafluoride).The time employed by USCA to cross the liver from the hepatic artery and portal vein to the hepatic vein was defined as HA-HVTT and PV-HVTT, respectively. Cirrhotics with low HVWI showed an earlier transit time; participants with higher HVWI had a longer transit time (p<0.001). HVTT was earlier as MELD, Child-Pugh score and spleen diameter increased. Patients with ascites and varices of large size had significantly shorter transit times. Abnormal hepatic vein Doppler waveform in cirrhotic patients could be due to intrahepatic shunts. HVTT could be useful in the non-invasive evaluation of portal hypertension.

A Case of Congenital Portosystemic Shunt Treated with Shunt Embolization

Congenital portosystemic shunt (CPS) is an anomaly of splanchnic venous flow which bypasses the liver and drains directly into the systemic circulation. It is associated with complications like neonatal cholestasis, hepatopulmonary syndrome, portopulmonary hypertension and encephalopathy. A female with microdeletion of chromosome 5q14.3, atrial septal defect, ventral septal defect and chronic oxygen requirement presented at 8 months with elevated AST/ALT (50) and irritability. Ammonia was elevated to 90. Abdominal CT showed a portosystemic shunt connecting portal vein to inferior vena cava. She was treated with lactulose/rifaxamin without any benefit. Due to concern for encephalopathy, she underwent interventional radiology (IR) procedure to close the shunt. A 9 mm shunt joining the main portal vein to inferior vena cava was found. Left portal vein, left hepatic vein and middle hepatic vein were draining into the shunt. A vascular plug was deployed. After four months, she had recurrence of hyperammonemia. Repeat IR procedure showed two intrahepatic portosystemic shunts emanating from left hepatic vein and persistent communication between enlarged left portal vein and the dominant shunt. The shunts were embolized with coil and dominant shunt was re-occluded with vascular plug. Follow up ultrasound showed successful closure of the shunts and patient has done well with decreased oxygen requirement and normalization of ammonia and AST. CPS are vascular malformations that lead to severe complications. These may not be diagnosed early due to presence of other comorbid conditions like congenital heart defects. Chronic hyperammonemia may lead to encephalopathy which can cause cognitive disorders, drowsiness, delirium, mental retardation, behavioral problems and attention hyperactivity disorders. Doppler ultrasonography (US) is the key imaging modality for diagnosis and monitoring. Computed tomography (CT) with contrast helps in documentation of location and anatomy of the shunt. The management is based on the size of the shunt. Small intrahepatic shunts may close spontaneously and may be monitored till age of 2 years. Large intrahepatic shunts, those with persistent ductus venosus and extrahepatic shunts need to be considered for closure, by endovascular methods or surgery. Our patient improved remarkably following IR guided embolization of her CPS shunt.Figure 1Figure 2Figure 3

Transjugular Intrahepatic Portal-Systemic Shunt for Palliative Treatment of Radioembolization-Induced Liver Disease in a Patient with Metastatic Lung Cancer

Introduction: Radioembolization induced liver disease (REILD) is a rare disease secondary to hepatic outflow obstruction after intra-arterial injection of radioactive embolic microspheres. It manifests as hepatomegaly, jaundice, and ascites shortly after therapy. Treatment for REILD is limited, but some studies suggest that portal-systemic shunting may potentially slow disease progression. This case report describes an instance where transjugular intrahepatic portal-systemic shunt (TIPS) provided palliative benefit in a cancer patient suffering from REILD. Case Presentation: A 64 year old female was diagnosed with metastatic pulmonary adenocarcinoma. Palliative chemotherapy with Crizotinib was initiated and the tumor burden significantly improved, however new liver metastasis were later identified and the patient underwent radioembolization with yttrium-90 (90Y-RE). One month later she developed new ascites and elevated bilirubin and increased transaminases. Crizotinib was discontinued and the patient was treated supportively for presumed REILD. Over the next 4 weeks her bilirubin peaked at 18.3 mg/dL and she was hospitalized with tense ascites and hydrothorax. She underwent transjugular intrahepatic portal-systemic shunt (TIPS) which improved her portal hypertension (HVPG from 15mmHg to 4mmHg). A liver biopsy was performed and sinusoidal obstruction syndrome (SOS), consistent with REILD, was identified (Figure 1). The patient continued to improve and on day 7 following TIPS she was discharged. Discussion: 90Y-RE is a radioactive embolization therapy that can be used as palliative, sometimes curative therapy in patients with unresectable tumor. REILD results from injury and eventual fibrosis of hepatic sinusoids; histologically identical to sinusoidal obstruction syndrome. REILD occurs more often in patients who have cirrhosis, are treated with larger doses of radiation, and those treated with radiation-sensitizing chemotherapeutic drugs. Crizotinib is not a known radiation sensitizing agent, but it may have contributed to this patient's REILD. TIPS has been utilized as a method of reducing portal hypertension and improving symptoms in patients with SOS, but there have been no prior case reports describing the use of TIPS for treatment of 90Y-RE induced REILD. Crizotinib may be a radiation-sensitizing chemotherapy that has yet to be recognized. TIPS procedure may be considered for select patients suffering from REILD in the future.

Modified technique for preparation of venous circulation resin casts in the cirrhotic liver.

This study describes two major adaptations for the preparation of resin casts in human cirrhotic liver, harvested at the time of transplantation. The first is the way of fixing the catheter in the ostia of the hepatic and portal veins through a cerclage, so as to prevent displacement of the catheter and / or leakage of the resin during its injection. The second is the extension of corrosion time in the NaOH solution, averaging 6.8 days, with daily replacement the solution until complete removal of parenchymal tissue. We applied the method in 14 cirrhotic livers, with good filling and coloring of the portal and hepatic vein territories, using different colors. This allows an anatomical study of these vessels, able to complement the knowledge of the histopathology in research work, and the planning of therapeutic procedures, such as the Trans-Jugular Intrahepatic Port-Systemic Shunt (TIPS). RESUMO Este estudo descreve duas importantes adaptações para o preparo de moldes de resina em fígado humano cirrótico, captado no momento do transplante: a primeira, é a maneira de fixação dos cateteres nos "óstios" das veias hepáticas e porta, através de uma "cerclagem" dos mesmos, de modo a evitar o deslocamento do cateter e/ou extravasamento da resina durante sua injeção, e a segunda, é o prolongamento do tempo de corrosão na solução de NaOH, atingindo a média de 6,8 dias, com a substituição diária da solução, até a remoção completa do tecido parenquimatoso. O método foi empregado em 14 fígados cirróticos com bom preenchimento e coloração dos territórios das veias porta e hepáticas, utilizando cores distintas. Isto permite um estudo anatômico desses vasos, capaz de complementar os conhecimentos da histopatologia em trabalhos de pesquisa, e planejar procedimentos terapêuticos como a derivação porto-sistêmica intra-hepática transjugular (TIPS - Transjugular Intrahepatic Postosystemic Shunt).