Intrahepatic Portosystemic Venous Shunt Research Articles

EP10.16: Prenatal ultrasonographic features and outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts

EP10.16: Prenatal ultrasonographic features and outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

BackgroundTo investigate the prenatal ultrasonographic features and case characteristics of the congenital intrahepatic portosystemic venous shunt (IHPSS) diagnosed during the foetal period and analyse its prognosis.MethodsWe conducted a retrospective cohort study of patients diagnosed with IHPSS between 2016 and 2021. IHPSS was defined as an abnormal connection between the foetal intrahepatic portal and the hepatic veins.ResultsIn this study, 19 foetuses were identified, including 12 cases of single shunt and 7 cases of multiple shunts, with a gestational age of 33.8 ± 4.5 (range 25–40) weeks at diagnosis. In the single-shunt group, the origin position of the shunts was all from the left branch of the portal vein (LPV), whereas in the multiple-shunt group, the origin position of the shunts was from the LPV in six cases. Common concomitant intrauterine abnormalities of IHPSS include foetal growth restriction (47.4%) and foetal cardiac enlargement (21.1%). The postnatal manifestations of IHPSS include biochemical abnormalities (increased gamma-glutamyl transferase and bilirubin levels), neonatal hypoglycaemia, neonatal hyperammonaemia, pulmonary hypertension, multiple intrahepatic hyperechoic nodules, and cutaneous haemangiomas. Spontaneous closure of shunts occurred in ten cases, and the mean time to shunt closure was 8.1 months (1–28 months).ConclusionsMost IHPSS found during the foetal period is located in the left branch of the portal vein, and the gestational age at diagnosis is usually in the late second or third trimester. Spontaneous closure of shunts can occur in most live births, and the prognosis is good.

S3320 An Intrahepatic Portosystemic Shunt Masquerading as a Tumor

Introduction: Portosystemic shunts can be congenital or acquired and are divided into extrahepatic and intrahepatic shunts. Intrahepatic portosystemic venous shunts (IPSVSs) are abnormal intrahepatic connections that occur between branches of the portal vein (PV) and hepatic veins (HV) or IVC. Patients with IPSVSs can be symptomatic or asymptomatic as most of the cases are detected incidentally on imaging. However, they can potentially lead to long term complications by allowing bypass of mesenteric venous return into systemic circulation without going through the liver. We herein describe the case of a 58-year-old woman who was incidentally found to have an IPSVS on imaging for which she was recommended periodic surveillance imaging. Case Description/Methods: A 58-year-old woman was undergoing workup for weight loss and was noted to have a 2.3 x 2.0 cm hyperdense nodular lesion in the posterior segment of the right hepatic lobe suspicious for hemangioma or a hypervascular neoplasm on CT abdomen pelvis. She was referred to the hepatobiliary clinic and a CTA abdomen pelvis was then performed and showed a lobulated 2.2 x 2.1 x 2.9 cm lesion in segment 6 consistent with an intrahepatic portosystemic shunt (figure 1). The patient was otherwise asymptomatic and therefore was advised to continue with periodic imaging surveillance. Discussion: IPSVSs are divided into 4 types depending on their morphology. In type 1, a single large vessel connects the PV or its right branch to the IVC. Type 2 involves a localized peripheral shunt within one hepatic segment that has one or more communications between peripheral branches of the PV and HV. In type 3, an aneurysmal connection exists between PV and HV. And finally, multiple communications between PV and HV in multiple lobes are present in type 4. In patients without a history of liver disease or trauma, the origin of the IPSVS is presumed to be congenital. CT scan, MRI and even color Doppler sonograms should provide visualizations of the feeding portal vein and draining hepatic vein. For symptomatic IPSVSs, endovascular closure is the standard of care. However, no consensus exists for asymptomatic cases and surveillance is an appropriate option in asymptomatic patients.Figure 1.: Axial CTA abdomen venous phase showing aneurysmal communication (red arrow) between right portal vein (yellow arrow) and middle hepatic vein (green arrow) in segment 6 of the liver.

Intrahepatic aneurysmal portosystemic venous shunt diagnosed on EUS

Video 1EUS demonstration of aneurysmal intrahepatic portosystemic venous shunt.

Congenital intrahepatic portosystemic venous shunt.

Congenital intrahepatic portosystemic venous shunt.

2452 Intrahepatic Portosystemic Venous Shunt: A Clinically Significant Rare Anomaly

INTRODUCTION: Intrahepatic Portosystemic Venous Shunts (IPSVS) are rare vascular malformations between the hepatic and portal veins. Most of the porto-venous shunts are asymptomatic and found incidentally on radiology imaging. These can be either congenital or acquired. Acquired shunts are typically less than 2 mm in size. Occasionally, complications such as encephalopathy, heart failure, pulmonary hypertension and elevated ammonia levels can occur. We report a case of incidental IPSVS in a patient who presented with complaints of fatigue and failure to thrive. CASE DESCRIPTION/METHODS: 82-year-old African American male with hypertension, diabetes mellitus and prostate cancer (treated) presented with complaints of fatigue and loss of appetite for one-month. Laboratory work up showed a hemoglobin of 11.6 g/dl, normal platelet count, and elevated aspartate aminotransferase of 45 U/L and decreased albumin of 2.0 g/dl. Serum ammonia level was normal. He was immune to hepatitis A, non immune to hepatitis B and hepatitis C antibody was negative. A computed tomography scan of the abdomen showed a contrast enhanced tubular shaped vascular connection between middle hepatic vein and the anterior branch of the right portal vein. The size of the shunt was approximately 10 mm (Figure 1). Liver appeared normal except for small calcifications within the parenchyma. Ultrasound Doppler of hepatic and portal veins were performed, and the porto-hepatic shunt was visualized (Figure 2). Patient had a complicated hospital course secondary to sepsis; however, he did not develop hyperammonemia or hepatic encephalopathy during the hospital stay. DISCUSSION: IPSVS in our patient was likely a congenital anomaly given the presentation, size and lack of related symptoms. IPSVS is divided into 4 types; Type 1- Shunt between portal vein and IVC; Type 2- connections between peripheral branches of portal and hepatic veins in one hepatic segment; Type 3- Aneurysmal connection between portal and hepatic vein; Type 4- multiple communications of portal and hepatic veins in both major lobes of the liver. Doppler sonogram with pulse doppler is highly sensitive and specific to evaluate for IPSVS. In symptomatic IPSVS, shunt closure has shown improvement in symptoms and correction of hyperammonemia. Clinicians should consider this rare vascular malformation in patients presenting with hepatic encephalopathy or hyperammonemia in the absence of cirrhosis where shunt closure can be curative.

Intrahepatic Portosystemic Venous Shunt in a Non-Cirrhotic Adult Female with Encephalopathy

Introduction: Intrahepatic portosystemic venous shunts (IPSVS) are rare anomalous vascular connections within the liver that are often asymptomatic. However, significant shunting of blood may present as encephalopathy. Cross sectional imaging and Doppler studies are essential to characterize the shunt and determine portosystemic shunt ratios. High flow shunts may be treated using coil embolization or surgery. Result: We present the case of a 57-year-old previously asymptomatic lady who was admitted for confusion and behavioral change. Work-up revealed deranged liver function and hyperammonemia. Triphasic CT scan showed an aneurysmal connection between the right anterior portal vein and the middle hepatic vein measuring 1.9 cm in widest diameter. The left portal vein was small. The liver had homogenous parenchyma and smooth borders. Aside from small hepatic cysts, there were no other lesions detected. Doppler ultrasound showed turbulent flow across the connection with a portovenous shunt ratio of 73%. The size of the shunt precluded safe coil embolization. Surgical treatment was then opted for and she underwent a hepatotomy from the superior border of the liver until the aneurysmal shunt connection was identified and isolated. The connection was then divided between clamps and the stumps sutured close. Blood flow in the left portal vein then increased immediately. Her postoperative course was unremarkable and she was discharged improved. She remains well 8 months after surgical correction of the shunt. Conclusion: A high index of suspicion should prompt the search for IPSVS in patients presenting with encephalopathy in the absence of liver cirrhosis. Imaging studies are essential in assessing the type and severity of the portovenous shunting and in guiding the management. Symptomatic patients with high shunt ratios should be treated promptly using interventional radiologic procedures or surgery.

Asymptomatic Intrahepatic Portosystemic Venous Shunt: Case Report and Review of Literature

A spontaneous portosystemic shunt is a rare malformation of the liver vessels. The etiology of these shunts is controversial. They can be divided into acquired (most commonly associated with liver cirrhosis) and congenital. Vascular shunts are asymptomatic in the majority of the patients, and when symptomatic were presented by severe complications. The specific way of management can be selected depended on whether the patient was symptomatic or not, and also on the size of the shunt, shunt ratio and whether it was associated with cancer. We will present a clinical case and discuss the importance of the radiological imaging in the screening, diagnosis and follow up of these anomalies.

Congenital Intrahepatic Portosystemic Venous Shunt With an Associated Aneurysm: Sonographic Diagnosis

Intrahepatic portosystemic venous shunts with associated aneurysms are extremely rare anomalous communications between intrahepatic portal veins and systemic veins through intrahepatic venous channels. Intrahepatic portosystemic venous shunts are usually asymptomatic but can be the cause of hepatic encephalopathy and hypoglycemia, especially when a high degree of shunting exists. The sonographic incidental finding of an aneurysmal vascular connection between the left portal vein and left hepatic vein as diagnosed by sonography is presented and discussed. When evaluating patients with suspected symptoms of hepatobiliary disease, sonographers and radiologists should carefully scan and meticulously assess the liver for signs of abnormal venous communications.

Transhepatic Embolization of Congenital Intrahepatic Portosystemic Venous Shunts with Associated Aneurysms.

Intrahepatic shunts between the portal and systemic venous systems with associated aneurysms are extremely rare. A middle aged woman presented with hepatic encephalopathy and was found to have two intrahepatic portosystemic venous shunts with associated aneurysms. Diagnosis was made by duplex ultrasound and was confirmed with contrast enhanced MRI. Treatment was performed percutaneously with an Amplatzer vascular plug.

Infusion of 50 % glucose solution to occlude an intrahepatic portosystemic venous shunt before percutaneous transhepatic portal embolization: report of a case

A 68-year-old man with cholangiocarcinoma underwent percutaneous transhepatic portal embolization to expand the indication for hepatic resection. Selective right posterior portography revealed an intrahepatic portosystemic venous shunt (IPSVS) connecting the segment VII branch to the right hepatic venous branch. An infusion of 50% glucose solution was given to occlude the shunt. This is novel management for IPSVSs when they are numerous, small, or torturous, and makes the subsequent procedures simpler, shorter, and less expensive.

Endovascular Treatment of Symptomatic Intrahepatic Portosystemic Venous Shunt Using Amplatzer Vascular Plug II

Endovascular Treatment of Symptomatic Intrahepatic Portosystemic Venous Shunt Using Amplatzer Vascular Plug II Spontaneous intrahepatic portosystemic venous shunt (IPSVS) is a rare condition that implies abnormal communication between portal and systemic venous system within the liver parenchyma. It can be asymptomatic or present with symptoms of hepatic encephalopathy. We show a case of 74-year-old female patient with symptomatic IPSVS and successful endovascular treatment using Amplatzer Vascular Plug (AVP) II.

Ultrasound diagnosis of congenital intrahepatic portosystemic shunt

To investigate the ultrasonographic features of congenital intrahepatic portosystemic venous shunt (CIPSVS) and to assess the clinical value of ultrasonography in the diagnosis of CIPSVS. Six cases of CIPSVS diagnosed in our hospital between March 2010 and March 2012 and confirmed by enhanced computed tomography (CT) were retrospectively reviewed. Five of the six cases had follow-up data that was included in the analysis. Among the six CIPSVS cases, only one was classified as Park's type II and the rest were classified as Park's type III. Five cases involved the right lobe of the liver and only one case involved the left lobe. The lesion shapes included cystic, tubular, and irregular with clear contour and appeared to be anechoic on CT scan. The lesions ranged in size from 1.1*0.6 cm to 2.0*1.7 cm. For all cases, the color Doppler ultrasound images showed blood flowing from the portal vein to the hepatic vein, and single-phase spectrum was detected in the diversion channel. The differences observed in level of lesion size and blood flow velocity at the shunt from the time of examinations at diagnosis and subsequent follow-up did not reach statistical significance (P = 0.223 more than 0.05 and P = 0.930 more than 0.05 respectively). Although cases of CIPSVS are rare, they share some specific sonographic features that may help in diagnosis. Color Doppler ultrasound findings have high diagnostic accuracy and may represent a preferred modality for follow-up monitoring.

An unusual case of intrahepatic portosystemic venous shunt

Intrahepatic portosystemic venous shunts are rare vascular anomalies that may be detected in asymptomatic patients, given the recent advances in radiological imaging techniques. Accurate shunt evaluation and classification can be performed with ultrasound and multi-detector computed tomography. We report an unusual case of an intrahepatic portosystemic venous shunt with an incidental finding of neurofibroma.

Metabolic improvements in intrahepatic porto-systemic venous shunt presenting various metabolic abnormalities by 4-phenylacetate

BackgroundIntrahepatic congenital portosystemic venous shunt (CPSVS) presents hyperammonemia, cholestasis, hypergalactosemia and imbalanced vasomediators. Especially, fluctuating plasma ammonia often causing neurological signs and symptoms is a serious problem in the daily life. 4-Phenylacetate (4-PA) has effects to eliminate blood ammonia, bile acids and bilirubin. 4-PA might be expected to improve the metabolic abnormalities in intrahepatic CPSVS. MethodsThree intrahepatic CPSVS children often receiving 4-PA from early life were enrolled. We analyzed biological and clinical changes by intravenous administration of 4-PA. Results4-PA improved hyperammonemia enough to subside the clinical presentations: headache, cognition dysfunction and attention deficit. Concurrently, this drug decreased serum total bilirubin and total bile acid levels. In their neonatal ages, 4-PA also decreased galactose and galactose-1-phosphate levels. In their preschool or school ages, 4-PA increased nitric oxide (NO) prompting vasodilation, but not changed amino acids controlling NO production and endothelin-1 prompting vasoconstriction. Plasma ammonia level returned to the pre-administration level within one day of the discontinuation, and serum total bilirubin and total bile acid levels were maintained to be reduced a few days after the discontinuation. Conclusion4-PA improves galactosemia and imbalanced vasomediators, together with liver functions, in CPSVS, although such effects retract after the discontinuation.

Congenital Portosystemic Venous Shunt in a Preterm Rh-Isoimmunized Infant

The authors report a preterm infant with Rh-isoimmunization, who had persistent hepato-splenomegaly with conjugated hyperbilirubinemia, transaminitis, and hyperammonemia. Ultrasound abdomen revealed an intrahepatic portosystemic venous shunt (PSVS). The child was managed conservatively. On follow up at 2.4 y of age, the child is having normal growth and development, but with persisting shunt. Severe Rh-isoimmunisation in a neonate can sometimes share some of the features of congenital PSVS and delay the diagnosis of the latter. The index case had shunt ratio >80 % during the neonatal period but did not require any intervention.

Congenital Portosystemic Shunts

The purpose of this study was to evaluate prenatal sonographic findings that could be helpful for diagnosis of congenital intrahepatic portosystemic venous shunts and the follow-up results. Six neonates with congenital shunts between the portal vein and hepatic vein were enrolled in this study. Prenatal sonography was performed in 5 cases. We categorized the cases according to a previously published classification of intrahepatic portosystemic venous shunts and retrospectively reviewed the prenatal and postnatal sonographic examinations to identify findings that might be helpful for diagnosing shunts prenatally. Follow-up sonographic examinations were done until closure of the shunts. Clinical features were also determined. According to the original reports, intrahepatic portosystemic venous shunts were diagnosed by prenatal sonography in 2 of 5 cases. In the remaining 3 cases, there were suggestive abnormal findings on retrospective review, including an abnormal intrahepatic tubular structure, a prominent hepatic vein, and congestive heart failure. Postnatal sonography showed type 2 shunts in all 6 cases. In 1 case, there were 2 type 2 lesions between two branches of the left portal vein and the middle and left hepatic veins. On follow-up sonography, 5 of the 6 congenital shunts had spontaneously closed by 11 months of age. One case was treated with coil embolization during the neonatal period. Intrauterine growth restriction was the most commonly clinical feature prenatally. Findings such as an abnormal tubular structure, a prominent hepatic vein, and congestive heart failure can be important clues for identifying congenital intrahepatic portosystemic venous shunts on prenatal sonography. The use of prenatal and postnatal sonography is feasible for detection and evaluation of these shunts.

Congenital Intrahepatic Portosystemic Venous Shunt Presenting with Paraparesis as the Initial Symptom

An 85-year-old woman was hospitalized with rapidly progressive paraparesis without altered consciousness, although she was not definitively diagnosed. She developed acute drowsiness and disorientation several days later. An intrahepatic portosystemic venous shunt (IPSVS) was observed on enhanced computed tomography, and hyperammonemia suggested leakage of neurotoxins from the shunt as the etiology of the patient's symptoms. Her neurological symptoms and hyperammonemia improved following transcatheter shunt embolization. We diagnosed her with hepatic myelopathy, which is a rare complication of liver cirrhosis and portosystemic venous shunts. Hepatic myelopathy resulting from a congenital IPSVS has not been previously reported. A diagnosis of hepatic myelopathy should be ruled out in diagnostically difficult cases of paraparesis.

Successful Treatment Using Coil Embolization of a Symptomatic Intrahepatic Portosystemic Venous Shunt Developing through a Patent Ductus Venosus in a Noncirrhotic Adult

The patient was a 60-year-old man with encephalopathy without liver cirrhosis. CT angiography revealed a patent ductus venosus between the anterior segmental branch of the portal vein and the middle hepatic vein. Coils were framed in the patent ductus venosus and then used to fill in the frame. After treatment, transarterial portography showed that the shunt flow of the ductus venosus had decreased significantly. After one day, the patient's disturbance of consciousness disappeared. Our case involved the adult-onset of a patent ductus venosus, which is extremely rare. This case is the first in which coil embolization was successfully achieved in a noncirrhotic elderly patient with a patent ductus venosus.

Percutaneous Embolization of Congenital Portosystemic Venous Fistula in an Infant with Down Syndrome

Congenital intrahepatic portosystemic venous shunts are rare vascular malformations often associated with severe complications. We describe a term male infant with Down syndrome with high output heart failure secondary to a congenital arterial to portal venous fistula that was diagnosed by Doppler ultrasound. Percutaneous embolizations of the left hepatic vein, portal vein, and communicating fistulas were performed without complications, resulting in clinical improvement. A subsequent hepatic ultrasound demonstrated resolution of the pathologic fistulous communication and shunting effects.