Extrahepatic Shunt Research Articles

The Choice of Surgical Management in Patients with Portal Hypertension

Aim. To improve the results of surgical treatment of patients with syndrome of portal hypertension. Material and Methods. One hundred and sixty nine patients with syndrome of portal hypertension who underwent elective surgery were examined. 100 patients had liver cirrhosis and 69 patients – extrahepatic portal vein obstruction. Portocaval shunting was scheduled for all patients. Selective portocaval shunting was preferred in cirrhotic patients; maximal shunting using “side to side” or “H-type” mesentericocaval or splenorenal anastomoses was chosen for those who had extrahepatic portal vein obstruction. Results. Selective portocaval shunting was performed in 26 of 100 patients with liver cirrhosis. Seventy six patients underwent gastrotomy with direct oesophagogastric varices ligation. In case of extrahepatic portal vein obstruction portocaval shunting was applied in 69 patients and direct oesophagogastric varices ligation – in 26 patients. Shunt surgery was rejected due to high activity of the pathological process in liver, presence of hepatic encephalopathy, heart failure; and also according to ultrasonography and computed tomography of portal system. Satisfactory short-term results were obtained in 97% of patients. Overall mortality was 0,8%. Conclusion. Phased preoperative examination protocol should be applied for the optimization of surgical management in patients with portal hypertension: patients’ selection based on clinical and laboratory data, assessment of central hemodynamics and neurological status. It is necessary to determine hemodynamic and topographic features using ultrasonography and 3D computed tomography of portal and caval systems to forecast the ability to perform different types of portocaval shunting; to define type and extent of surgical intervention depending on intraoperative data

New perspectives on the development of extrahepatic portosystemic shunts.

In this essay we use clinical evidence and knowledge of anatomy to examine the relationship between blood flow and formation of congenital extrahepatic portosystemic shunts in dogs and cats. First we report on the clinical findings in a series of 50 dogs and 10 cats and then systematically review peer-reviewed data on the detailed anatomy of shunts in dogs and cats. In dogs four types of shunt: spleno-caval, left gastro-phrenic, left gastro-azygos and those involving the right gastric vein account for 94% of extrahepatic shunts. Cats also exhibit four types of shunt: spleno-caval, left gastrophrenic, left gastro-caval and left gastro-azygos, and the first three of these account for 92% shunts in this species. Our findings lead us to propose that preferential blood flow influences the subsequent formation of one of a number of defined and consistent congenital extrahepatic portosystemic shunts in dogs and cats.

Safety analysis of holmium-166 microsphere scout dose imaging during radioembolisation work-up: A cohort study

ObjectiveRadioembolisation is generally preceded by a scout dose of technetium-99m-macroaggregated albumin to estimate extrahepatic shunting of activity. Holmium-166 microspheres can be used as a scout dose (±250 MBq) and as a therapeutic dose. The general toxicity of a holmium-166 scout dose (166Ho-SD) and safety concerns of an accidental extrahepatic deposition of 166Ho-SD were investigated.MethodsAll patients who received a 166Ho-SD in our institute were reviewed for general toxicity and extrahepatic depositions. The absorbed dose in extrahepatic tissue was calculated on SPECT/CT and correlated to clinical toxicities.ResultsIn total, 82 patients were included. No relevant clinical toxicity occurred. Six patients had an extrahepatic deposition of 166Ho-SD (median administered activity 270 MBq). The extrahepatic depositions (median activity 3.7 MBq) were located in the duodenum (3x), gastric fundus, falciform ligament and the lesser curvature of the stomach, and were deposited in a median volume of 15.3 ml, which resulted in an estimated median absorbed dose of 3.6 Gy (range 0.3–13.8 Gy). No adverse events related to the extrahepatic deposition of the 166Ho-SD occurred after a median follow-up of 4 months (range 1–12 months).ConclusionThese results support the safety of 250 MBq 166Ho-SD in a clinical setting.Key Points• A holmium-166 scout dose is safe in a clinical setting.• Holmium-166 scout dose is a safe alternative for99mTc-MAA for radioembolisation work-up.• Holmium-166 scout dose potentially has several benefits over99mTc-MAA for radioembolisation work-up.

Echographic features and perinatal outcomes in fetuses with congenital absence of ductus venosus.

The purpose of this study was to describe the echographic features and perinatal outcomes of fetuses with absence of ductus venosus. Retrospective review of 10 cases with absence of ductus venosus diagnosed by prenatal ultrasonography between January 2014 and February 2016 at a single referral center. Prenatal findings, umbilical shunting type, perinatal outcomes, and autopsy reports were reviewed. A total of 11 491 fetuses underwent a first- and second-trimester screening during the study period. Ten cases of absence of ductus venosus were diagnosed. All of the fetuses presented an extrahepatic shunt: three fetuses from the umbilical vein to the right atrium and the seven remaining fetuses from the umbilical vein to the inferior vena cava. Major structural defects and fetal effusions were detected in six fetuses. There were two cases of chromosomal abnormalities. Five patients underwent legal termination of pregnancy and five decided to carry to term. In two of these, the absence of ductus venosus anomaly was isolated and had a normal outcome. In the remaining three cases, a follow up of the children showed a variety of adverse outcomes. The absence of ductus venosus is associated with high rates of adverse perinatal outcomes. The prognosis for this group of anomalies depends on the additional findings with targeted ultrasound. This pathology should lead to a detailed anatomical study and affected fetuses should be closely monitored for signs of congestive heart failure.

Characterization of the coagulation profile in children with liver disease and extrahepatic portal vein obstruction or shunt

ABSTRACTIntroduction: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. Materials and methods: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children. Results: Children with severe liver disease had coagulopathic changes, including either decreased concentration or activity of factor II, factor V, and factor VII. Nineteen percent (8/42) of the cohort had significant bleeding. Thrombophilic changes were also observed, including decreased concentration or activity of protein C, protein S, and antithrombin and increased concentration and activity of factor VIII and Von Willebrand factor. Similar coagulation factor changes were observed in children with extrahepatic portal vein obstruction or shunt. There was a trend toward greater changes in coagulation factor activity compared to concentration. Conclusion: This study provides a detailed description of the changes in both the concentration and activity of coagulation factors in pediatric liver disease and extrahepatic portal vein obstruction or shunt. Interestingly, procoagulant and anticoagulant factors were deranged in portal vein obstruction or shunt to a similar degree as in liver disease. An improved understanding of the coagulation profile in the pediatric setting will contribute to the improved management of liver disease and extrahepatic portal obstruction or shunt.Abbreviations: PELD: pediatric end-stage liver disease score; MELD: model for end-stage liver disease score; ELISA: enzyme-linked immunosorbent assay; MCRI: Murdoch Childrens Research Institute; FII: factor II; FV: factor V; FVII: factor VII; FVIII: factor VIII; AT: antithrombin III; A2M: alpha-2-macroglobulin; vWF: Von Willebrand factor; PC: protein C; PS: protein S; ISTH-BAT: International Society on Thrombosis and Haemostasis Bleeding Assessment Tool

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.

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

P03.11: Absent ductus venosus (ADV) with intra‐ and extrahepatic shunts demonstration using 4D STIC with power Doppler

P03.11: Absent ductus venosus (ADV) with intra‐ and extrahepatic shunts demonstration using 4D STIC with power Doppler

Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance.

To review our experience with fetal umbilical-portal-systemic venous shunts (UPSVS), to devise an in-utero classification system, and to analyze their clinical and prognostic characteristics. This was a retrospective review of cases of UPSVS examined at an academic tertiary referral center over the 14-year period from 2001 to 2014 inclusive. The anatomical origin and drainage of the fetal umbilical, portal, ductus venosus and hepatic venous systems, and the integrity of the intrahepatic portal venous system (IHPVS), were assessed using two- and three-dimensional color Doppler sonography. Fetomaternal clinical characteristics and long-term outcome were investigated by means of medical files, imaging documentation and telephone interviews with the mothers. Forty-four cases with UPSVS were reviewed. Prenatal classification was based on the anatomical origin of the shunt (umbilical, portal or ductal), with cases classified into four types: Type I, umbilical-systemic shunt (n = 9 (20.4%)); Type II, ductus venosus-systemic shunt (n = 19 (43.2%)); and Type III, portal-systemic shunt, divided into two subtypes: Type IIIa, intrahepatic portal-systemic shunt (n = 12 (27.2%)) and Type IIIb, extrahepatic portal-systemic shunt (n = 4 (9.1%)). Each type presented particular clinical and prognostic characteristics. The most important favorable prognostic factors were absence of associated major malformation and presence of the IHPVS. Thus, Type IIIa (intrahepatic portal-systemic shunt) had the best outcome. Fetal UPSVS are anomalies with a broad spectrum of manifestations and prognoses. Accurate mapping of the IHPVS is paramount for optimizing prenatal counseling and postnatal care. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Predicting outcome in 259 fetuses with agenesis of ductus venosus – a multicenter experience and systematic review of the literature

Objective: To evaluate prenatal predictors of postnatal survival in fetuses with agenesis of ductus venosus (ADV).Methods: This retrospective study reviewed our experience and the literature between 1991 and 2015. Prenatal findings were evaluated and perinatal morbidity and mortality was documented.Results: A total of 259 cases were included in the present analysis from our centers and 49 published studies (15 patients from our retrospective cohort review and 244 from literature review). The intrahepatic and extrahepatic shunts were present in 32.0% (73/226) and 67.7% (153/226), respectively. Cardiomegaly (n = 64/259, 24.7%), hydrops (n = 31/259, 12.0%) and amniotic fluid abnormalities (n = 22/259, 8.5%) were among the most frequent initial ultrasound findings. One hundred and forty-seven fetuses (56.8%) had ADV without structural anomalies while 112 (43.2%) had associated anomalies (cardiac anomalies (n = 66), extra-cardiac anomalies (n = 19) and both cardiac and extra-cardiac anomalies (n = 27)). The mean gestational age (GA) at ultrasound diagnosis was 22.9 ± 6.9 weeks while the mean GA at delivery was 34 ± 7.5 weeks. The overall neonatal survival was 57.1% (n = 148/259). The following factors were associated with survival: advanced maternal age, earlier GA at diagnosis, prematurity, increased nuchal translucency, pericardial effusion, associated cardiac defects (especially AVSD), chromosomal abnormalities, hydrops, hygroma and limb anomalies.Conclusion: Fetal hydrops, the presence of associated congenital anomalies and premature delivery are associated with poor prognosis in fetuses with ADV.

Organ doses from hepatic radioembolization with 90Y, 153Sm, 166Ho and 177Lu: A Monte Carlo simulation study using Geant4

90Y-radioembolization is a palliative treatment for liver cancer. 90Y decays via beta emission, making imaging difficult due to absence of gamma radiation. Since post-procedure imaging is crucial, several theranostic radionuclides have been explored as alternatives. However, exposures to gamma radiation throughout the treatment caused concern for the organs near the liver. Geant4 Monte Carlo simulation using MIRD Pamphlet 5 reference phantom was carried out. A spherical tumour with 4.3cm radius was modelled within the liver. 1.82GBq of 90Y sources were isotropically distributed within the tumour, with no extrahepatic shunting. The simulation was repeated with 153Sm, 166Ho and 177Lu. The estimated tumour doses for all radionuclides were 262.9Gy. Tumour dose equivalent to 1.82GBq 90Y can be achieved with 8.32, 5.83, and 4.44GBq for 153Sm, 166Ho and 177Lu, respectively. Normal liver doses by the other radionuclides were lower than 90Y, hence beneficial for normal tissue sparing. The organ doses from 153Sm and 177Lu were relatively higher due to higher gamma energy, but were still well below 1Gy. 166Ho, 177Lu and 153Sm offer useful gamma emission for post-procedure imaging. They show potential as 90Y substitutes, delivering comparable tumour doses, lower normal liver doses and other organs doses far below the tolerance limit.

Radioembolization with Y-90 Glass Microspheres: Do We Really Need SPECT-CT to Identify Extrahepatic Shunts?

PurposeSelective Internal Radiation Therapy (SIRT) with 90yttrium (Y-90) is an increasingly used therapeutic option for unresectable liver malignancies. Nontarget embolization of extrahepatic tissue secondary to vascular shunting can lead to SIRT associated complications. Our aim was to assess whether extrahepatic shunts can reliably be diagnosed based on hepatic digital subtraction angiography (DSA) or whether subsequent SPECT/CT data can provide additional information.Materials and Methods825 patients with hepatocellular carcinoma (n = 636), hepatic metastases (n = 158) or cholangiocellular carcinoma (n = 31) were retrospectively analyzed. During hepatic DSA 128 arteries causing shunt flow to gastrointestinal tissue were coilembolized (right gastric artery n = 63, gastroduodenal artery n = 29; branches to duodenum / pancreas n = 36). Technectium-99m-labeled human serum albumin (HSA) was injected in all 825 patients. SPECT/CT data was used to identify additional or remaining shunts to extrahepatic tissue.ResultsAn unexpected uptake of HSA in extrahepatic tissue was found by SPECT/CT in 54/825 (6.5%) patients (located in stomach n = 13, duodenum n = 26, distal bowel segments n = 12, kidney n = 1, diaphragm n = 2). These patients underwent repeated DSA and newly identified shunt vessels were coilembolized in 22/54 patients, while in 12/54 patients a more distal catheter position for repeat injection of HSA was chosen. In 20/54 patients the repeated SPECT/CT data still revealed an extrahepatic HSA uptake. These patients did not receive SIRT.ConclusionMost extrahepatic shunts can be identified on DSA prior to Y-90 therapy. However, SPECT-CT data helps to identify additional shunts that were initially not seen on DSA.

Use of C-Arm Cone Beam CT During Hepatic Radioembolization: Protocol Optimization for Extrahepatic Shunting and Parenchymal Enhancement.

PurposeTo optimize a C-arm computed tomography (CT) protocol for radioembolization (RE), specifically for extrahepatic shunting and parenchymal enhancement.Materials and MethodsA prospective development study was performed per IDEAL recommendations. A literature-based protocol was applied in patients with unresectable and chemorefractory liver malignancies undergoing an angiography before radioembolization. Contrast and scan settings were adjusted stepwise and repeatedly reviewed in a consensus meeting. Afterwards, two independent raters analyzed all scans. A third rater evaluated the SPECT/CT scans as a reference standard for extrahepatic shunting and lack of target segment perfusion.ResultsFifty scans were obtained in 29 procedures. The first protocol, using a 6 s delay and 10 s scan, showed insufficient parenchymal enhancement. In the second protocol, the delay was determined by timing parenchymal enhancement on DSA power injection (median 8 s, range 4–10 s): enhancement improved, but breathing artifacts increased (from 0 to 27 %). Since the third protocol with a 5 s scan decremented subjective image quality, the second protocol was deemed optimal. Median CNR (range) was 1.7 (0.6–3.2), 2.2 (−1.4–4.0), and 2.1 (−0.3–3.0) for protocol 1, 2, and 3 (p = 0.80). Delineation of perfused segments was possible in 57, 73, and 44 % of scans (p = 0.13). In all C-arm CTs combined, the negative predictive value was 95 % for extrahepatic shunting and 83 % for lack of target segment perfusion.ConclusionAn optimized C-arm CT protocol was developed that can be used to detect extrahepatic shunts and non-perfusion of target segments during RE.

Embolization of Spontaneous Splenorenal Shunt for After-Tips Hepatic Encephalopathy in a Patient With Cirrhosis and Variceal Bleeding

Potential conflict of interest: Nothing to report. Transjugular intrahepatic portosystemic shunt (TIPS) is a well‐established treatment for complications arising from portal hypertension.1 However, hepatic encephalopathy (HE) is a common complication after TIPS insertion. The incidence rate of HE after TIPS insertion is 15%‐48%.2 TIPS implantation with or without gastroesophageal variceal embolization remains questionable in general,2 but it is particularly questionable when TIPS is used in patients with splenorenal shunt (SRS). SRS is a spontaneous portosystemic shunt (SPSS) that persists in 14%‐21% of patients with cirrhosis.4 A previous study suggested that SPSS embolization alone is an effective and safe treatment for chronic HE.5 However, combining TIPS implantation with SPSS embolization for patients who present with variceal bleeding (VB) remains an issue. We report on a case of improved post‐TIPS HE after embolizing the coexisting SRS in a patient with cirrhosis with VB. Case Report A 34‐year‐old man who had experienced repeated melena and hematemesis three times (total, 600 mL) for 13 days with post–hepatitis B cirrhosis required hospitalization on March 16, 2012. The Child‐Pugh score was B/8 (total bilirubin level: 0.92 mg/dL; serum albumin: 3.17 g/dL; mild ascites, no encephalopathy), and the hemoglobin level was 9.2 g/dL. An upper endoscopy revealed significant gastroesophageal varices (GEV), and a large SRS (11 mm in diameter) was observed by angiography (Fig. 1A). Hematemesis and melena disappeared after pharmacological treatment. To further decrease the portal pressure and prevent variceal rebleeding, we implanted a TIPS using an 8‐mm Bard polytetrafluoroethylene‐covered stent. The portosystemic pressure gradient decreased from 36 to 15 mmHg. Diagnostic endoscopy showed improved GEV 2 days later, and ascites disappeared a few days after TIPS implantation. Given the lack of evidence in the literature regarding the role of embolotherapy during the TIPS procedure, SRS was not embolized at that time (Fig. 1B).Figure 1: Portal venogram of a patient with a spontaneous splenorenal shunt undergoing a TIPS procedure with subsequent embolization in the SRS. (A) A large spontaneous SRS (black arrow) is shown. (B) The SRS (black arrow) was not embolized after the addition of the TIPS (white arrow). (C) The former TIPS (white arrow) and a coexisting spontaneous SRS (black arrow) are shown before embolization. (D) SRS was embolized using an Amplatzer plugging device (black arrow) through the femoral vein, and the patent TIPS is shown (white arrow).The patient developed lethargy and disorientation 5 days after TIPS insertion, and a diagnosis of grade II HE was made. HE occurred repeatedly in the following days, despite a continuous treatment of branched‐chain amino acids and lactulose. To treat the post‐TIPS encephalopathy effectively, we embolized the SRS using an Amplatzer plugging device (18 mm in diameter) from the femoral vein through the left renal vein to the SRS 37 days after TIPS placement (Fig. 1C,D). After embolization, the HE symptoms gradually disappeared in approximately 6 hours. The Child‐Pugh score changed to A/5 (total bilirubin level: 1.15 mg/dL; serum albumin: 3.95 g/dL), and the hemoglobin level was 9.3 g/dL 1 year later. There was no recurrence of HE or any significant increase in the aggravation of preexisting varices at the 2‐year follow‐up. Discussion Recently, SPSS has received increasing attention because it appears in 46%‐70% of patients with refractory HE.5 In TIPS patients, the large SRS diverts portal flow, resulting in the diversion of portal venous flow into the left renal vein, which decreases both blood flow within the stent and hepatopetal perfusion.3 Consequently, the SRS may increase the incidence of shunt dysfunction and liver dysfunction. Moreover, the existence of two shunts induces excessive portosystemic shunts, thereby increasing the incidence of HE episodes. We have established that TIPS implantation decreases the portal pressure for the prophylaxis of rebleeding and that SRS embolization anatomically forces the splenic venous flow into the main portal vein, which increases the previously reduced blood flow. Therefore, this method may improve liver function and maintain stent patency. Furthermore, SRS embolization shifts the splenic venous flow from the extrahepatic shunt into the TIPS, which allows for only one intrahepatic shunt, thereby effectively lowering the risk of rebleeding and HE episodes. Currently, there is no clear therapeutic strategy regarding the management of SPSS patients in liver cirrhosis and no definite consensus on the treatment of post‐TIPS HE. This case demonstrated that the combined therapy may be superior to TIPS alone owing to the mitigation of post‐TIPS HE. TIPS insertion combined with SPSS embolization may be an alternative treatment in the management of such patients. Prospective, randomized trials are warranted to further confirm these findings. Authors names in bold designate shared co‐first authorship.

P02.25: Ductus venosus agenesis with extrahepatic shunt at rutin 11‐14 week by 3D/4D ultrasound scan

<scp>P</scp>02.25: Ductus venosus agenesis with extrahepatic shunt at rutin 11‐14 week by <scp>3D</scp>/<scp>4D</scp> ultrasound scan

The history and future of transjugular intrahepatic portosystemic shunt: food for thought.

The origins of transjugular intrahepatic portosystemic shunt (TIPS) date back to the 1960s with inadvertent portal access during the early years of transjugular cholangiography. TIPS is now the most frequently performed portal hypertension decompressive procedure performed by interventional radiologists, and has become the primary portosystemic shunt (surgical or percutaneous) performed in the United States. One of the least discussed major advantages of TIPS in the transplant era is that it is intrahepatic and thus is removed in situ during a liver transplant, while extrahepatic shunts (surgical or percutaneous) have to be ligated or revised during the liver transplantation. However, since the widespread clinical utilization of TIPS in the 1990s, it has been plagued with two major problems, namely, patency and hepatic encephalopathy. With the advent of commercially available expanded polytetraflouroethylenne (e-PTFE) covered stents a decade ago, 12- to 24-month TIPS patency has improved significantly (by ∼20-30%). However, hepatic encephalopathy (although not proven to have increased due to e-PTFE covered stents grafts) remains a significant morbidity problem. The article discusses the history of TIPS, critiques the retrospective encephalopathy data in the literature, and discusses futuristic TIPS-design ideas about the management of post-TIPS hepatic encephalopathy.

Isolated Absent Ductus Venosus with Intrahepatic Shunt: Case Report and Review of Literature

AbstractA 28-year-old primigravida with spontaneous conception and no complicating medical illness presented to the authors for an early morphology scan. Her scan was unremarkable with normal nuchal translucency for crown rump length, no stigmata for chromosomal abnormalities and no major detectable structural abnormality. A repeated search for ductus venosus revealed non visualization of the ductus with intrahepatic drainage of the umbilical vein. The karyotype was normal. Fetal echocardiogram, anomaly and serial growth scans were also within normal limits. She developed mild hydramnios in the late third trimester and delivered a normal healthy female child at 37 weeks. Postnatal echocardiogram showed a very small muscular ventricular septal defect. Mild pulmonary stenosis has been diagnosed at the age of 6 months. Ductus venosus is the key regulator of oxygenated blood in fetal life. Absent ductus venosus is associated with multiple structural, chromosomal, cardiac abnormalities partial or complete absence of portal venous system, hydrops and fetal death. In the absence of ductus venosus the umbilical vein may drain normally into the liver (intrahepatic shunt) or may drain in an aberrant location into the systemic circulation (e.g., iliac vein, inferior vena cava, right heart and coronary sinus) creating an extrahepatic shunt. In all the cases of absent ductus venosus, the prognosis depends on the presence or absence of associated abnormalities. In cases of extrahepatic aberrant drainage of the umbilical vein, the prognosis depends upon the presence or absence of portal venous system, abnormal shunt site and shunt diameter. In general, intrahepatic variant has a much better prognosis in the absence of other abnormalities.

The relationship between the percentage of lung shunting on Tc-99m macroaggregated albumin (Tc-99m MAA) scan and the grade of hepatocellular carcinoma vascularity

BackgroundHepatocellular carcinoma (HCC) stands for the majority of primary liver malignancy. HCC has variable grade of vascularity according to the histological tumor grade and underlying liver damage. Yttrium-90 radioembolization is a promising catheter-based hepatic intra-arterial therapy for HCC that necessitates proper planning. Technetium-99m macroaggregated albumin (Tc-99m MAA), a simulation of the treatment, is used to identify the shunting of microspheres to the lungs or gastrointestinal tract. ObjectiveTo evaluate the relationship between the percentage of lung shunting on Tc-99m MAA scan performed in treatment planning of Yttrium-90 (Y-90) microsphere selective internal radiation therapy (SIRT) for HCC and the grade of tumor vascularity shown on digital subtraction hepatic angiography. Materials and methodsThis study was conducted on 19 patients diagnosed to have HCC, based on typical computed tomography (CT) criteria and/or elevated alpha-fetoprotein (AFP) ⩾200ng/ml, and planned for Y-90 therapy. They were injected by Tc-99m MAA into the hepatic arterial territory with prior digital subtraction angiography. Tc-99m MAA scan was reviewed for extrahepatic shunting within the abdomen. Additionally, the hepatopulmonary shunt (HPS) fraction or the lung shunting (LS) percentage was calculated and further evaluated in relation to the grade of tumor vascularity shown on digital subtraction hepatic angiography. ResultsThere was no extrahepatic accumulation of Tc-99m MAA related to umbilical, gastroduodenal or splenic shunting. The LS percentage assorted among patients and ranged between 0.8% and 42.9% with a median of 5.5%. LS more than 20% occurred in only 1 patient. Subsequent therapy plan was changed accordingly in 5% of patients. The more vascular the lesion was, the higher lung shunting percentage was detected. ConclusionTc-99m MAA scan is fundamental prior to Y-90 microsphere SIRT as it minimizes the risk of post-radioembolization complications, hence, enhancing the safety of Y-90 microsphere subsequent administration. Moreover, the percentage of lung shunting varies considerably among patients with HCC relying on the grade of tumor vascularity.

Intrahepatic portocaval shunt in dogs: diagnostic approach and therapy

Portosystemic shunt (PSS) is a relatively common phenomenon in dogs. PSS may be either intrahepatic (IHPSS) or extraheptic (EHPSS), congenital or acquired. The most commonly identified shunts are of congenital origin and consist of single, large intrahepatic or extrahepatic shunts. Since the discovery of the condition, PSS has been treated by medical or surgical methods, or a combination of both. Surgical techniques for partial attenuation of EHPSS and IHPSS include silk suture attenuation, ameroid constrictor placement, cellophane banding, and hydraulic occluders. This article describes the diagnostic process and the treatment of a case of IHPSS in a 7-month-old male entire Bull Mastiff, and provides an in depth discussion regarding clinical and diagnostic features, surgical treatment, complications, follow-up and prognostic factors.

Multiple Congenital PSS in a Dog: Case Report and Literature Review

A 4 yr old spayed female mixed-breed dog presented with a 2 yr history of recurring increases in liver enzymes. Two congenital portosystemic shunts (PSSs) were identified using computed tomography (CT) angiography, which included a portoazygous and portorenal extrahepatic shunt. Double right renal veins were also identified. The shunts were successfully identified and attenuated with cellophane banding. Multiple congenital PSS is a rare phenomenon, but should be considered during exploratory laparotomy for PSS and in dogs with poor response to surgical attenuation of a single PSS. CT proved to be a crucial part of accurate diagnosis and surgical planning for this dog with multiple congenital PSS.