Congenital 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.

Congenital intrahepatic portosystemic venous shunt.

Congenital intrahepatic portosystemic venous shunt.

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.

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.

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.

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.

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.

Color Doppler ultrasonography and contrast-enhanced ultrasound as a diagnostic tool for congenital intrahepatic portosystemic shunts

Objective To evaluate the color Doppler ultrasonography and contrast enhanced ultrasound (CEUS) findings of congenital intrahepatic portosystemic venous shunts (IPSVS).Methods Nineteen patients of congenital IPSVS were examined with color Doppler ultrasonography and CEUS.All patients were confirmed by CT angiography.The hepatic artery arrival time (HAAT),portal vein arrival time (PVAT),and hepatic vein arrival time (HVAT) on CEUS were recorded.The interval time between hepatic artery arrival time and hepatic vein arrival time (HA-HVTT) and the interval time between portal vein arrival time and hepatic vein arrival time (PV-HVTT) were calculated.Results The types of IPSVS between portal and systemic veins were based on Park's classification.Color Doppler ultrasonography showed abnormal communication between the portal vein branch and the hepatic veins,duplex Doppler ultrasonography showed abnormal spectral pattern from the portal vein such as undulating,triphasic waveform mimicked that of the hepatic vein.CEUS demonstrated abnormal communication between portal vein branch and hepatic vein.HVAT,HA-HVTT,and PV-HVTT were shorter statistically in congenital IPSVS group than those in cirrhosis and normal groups.Conclusions Congenital IPSVS is a rare vascular abnormality that is usually asymptomatic.Color Doppler ultrasonography is a useful tool for diagnosis of congenital IPSVS.CEUS provides helpful data for the diagnosis and differential diagnosis of congenital IPSVS. Key words: Ultrasonography; Microbubbles ; Congenital intrahepatic portosystemic shunts

Congenital Intrahepatic Portosystemic Venous Shunt and Liver Mass in a Child Patient: Successful Endovascular Treatment with an Amplatzer Vascular Plug (AVP)

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14-month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112micromol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Large Persistent Ductus Venosus: Retrograde Transvenous Embolization with a Double-catheter Technique

Previous investigators have reported on cases with patent ductus venosus (PDV) successfully treated with transcatheter embolization (1–3). There can be some cases, however, in which it is difficult to place the embolic materials because of the size and shape of the PVD. Herein, we report on a rare case of large persistent ductus venosus that was successfully treated with coil embolization. We attempted the double-catheter method for the case with a large PDV. A 2-year-old girl with hyperammonemia and hypergalactosemia detected with newborn screening tests was diagnosed with a congenital intrahepatic portosystemic venous shunt at abdominal ultrasonography (US).

The clinical anatomy of congenital portosystemic venous shunts

Congenital portosystemic venous shunts are rare. Their gross anatomy has not been well defined. Four different varieties of congenital portosystemic venous shunts are described in six children seen during a 10-year period, focusing on the anatomy of the shunt as determined by imaging studies and surgery. A detailed review of the literature indicates that congenital portosystemic venous shunts are best classified as: extrahepatic or intrahepatic. Extrahepatic shunts may be further subdivided into portocaval shunts (type 1 end-to-side and type 2 side-to-side) and others. Intrahepatic shunts are due to an abnormal intrahepatic connection between the portal vein and hepatic vein/inferior vena cava or a persistent patent ductus venosus. Additional congenital anomalies, particularly cardiac malformations, may be associated with any type. Some congenital intrahepatic portosystemic venous shunts close spontaneously in infancy; all other congenital portosystemic venous shunts tend to remain patent. To a variable extent, depending largely on the volume and duration of the shunt, affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor. The key to understanding the pathogenesis of these shunts lies in the normal developmental mechanisms underlying the formation of the portal vein and inferior vena cava in the embryo.

Congenital intrahepatic portosystemic venous shunt: An unusual feature in LEOPARD syndrome and in neurofibromatosis type 1

Congenital intrahepatic portosystemic venous shunt: An unusual feature in LEOPARD syndrome and in neurofibromatosis type 1

Congenital Intrahepatic Portosystemic Venous Shunt in an Infant with Down Syndrome

Congenital Intrahepatic Portosystemic Venous Shunt in an Infant with Down Syndrome

P0384 THREE CASES OF CONGENITAL INTRAHEPATIC PORTOSYSTEMIC VENOUS SHUNT IN NEONATES.

Introduction: Congenital intrahepatic portosystemic venous shunt (CIHPSS) are very unusual abnormalities. Only 50 publications have been counted. They are defined as macroscopic communications between the portal and the systemic venous circulation. They are entirely or partially located within a heathy liver that functions without portal blood. When discovered early in life, they are in favour of a malformative origin of the shunt. HEREIN, we report three neonate observations with their icon-çography. For the case n°1, it was a color Doppler and ultrasound scanning for a fever checkup,, which diagnosed the CIHPSS. between the right portal vein and the right-sus hepatic vein. In the two other one, it was the antenatal color Doppler and ultrasound scanning which diagnosed shunts during the third term of pregnancy. Those three shunts were considered as type II, according to the PARK’s classification system for IHPSS anormalies, because they link up a portal branch to an hepatic vein in a same parenchymal segment. Methods: Case n°1 is now 6 years old and is still asymptomatic. A clinical, biological and ultrasonographic examination, once a year is sufficient. The vascular fistula resolved spontaneously when case n°2 was 28 months old and when case n°3 was 8,5 months old. Results: DISCUSSION: Normally, toxic metabolites of nutrients and residual activity of pancreatic enzymes are degraded by the liver. Here, due to the shunt, they bypass directly into the systemic circulation. Then, hyperammoniemia, trimethylaminuria, hyperinsulinemia, pulmonary hypertension or congestive heart failure can appear. THERAPY: Because of the rarity of this disorder, the choice of treatment is controversial. It depends on the natural course of the disease, but the majority of patients did not have symptoms until their 6th decade. Initially, the spontaneous resolution is expected. If hepatic encephalopathy or heart failure progress, some treatment may indicate: angiographic intervention with percutaneous coil embolisation, surgical intervention such as shunt ligation and hepatic resection or realisation of an alternative portosystemic shunt. Conclusion: CIHPSS have been thought to be uncommon, but improvement of imaging (US, CT, MRI) have shown even asymptomatic intrahepatic shunts in an increasing number of patients. Spontaneous resolution is frequent. Metabolic and hemodynamic repercussions will guide the management in other cases.

2 P US diagnosis of congenital intrahepatic portosystemic venous shunt (IPVS): A case report

2 P US diagnosis of congenital intrahepatic portosystemic venous shunt (IPVS): A case report

Diagnosis and follow-up of congenital intrahepatic portosystemic venous shunt by ultrasounds

Acta PaediatricaVolume 90, Issue 10 p. 1209-1210 Diagnosis and follow-up of congenital intrahepatic portosystemic venous shunt by ultrasounds VA Caiulo, VA Caiulo Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorG Presta, G Presta Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorG Latini, Corresponding Author G Latini Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi. Giuseppe Latini, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Division of Paediatrics, Piazza A. Di Summa, IT-72100 Italy Brindisi, (e-mail, gilatini@tin.it)Search for more papers by this authorG Mattiolr, G Mattiolr Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorV Jasonni, V Jasonni Division of Paediatric Surgery, Istituto G. Gaslini, Genoa, Italy.Search for more papers by this author VA Caiulo, VA Caiulo Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorG Presta, G Presta Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorG Latini, Corresponding Author G Latini Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi. Giuseppe Latini, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Division of Paediatrics, Piazza A. Di Summa, IT-72100 Italy Brindisi, (e-mail, gilatini@tin.it)Search for more papers by this authorG Mattiolr, G Mattiolr Division of Paediatrics, Azienda Ospedaliera A. Di Summa, Ospedale A. Perrino, Brindisi.Search for more papers by this authorV Jasonni, V Jasonni Division of Paediatric Surgery, Istituto G. Gaslini, Genoa, Italy.Search for more papers by this author First published: 02 January 2007 https://doi.org/10.1111/j.1651-2227.2001.tb03259.xCitations: 11AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume90, Issue10October 2001Pages 1209-1210 RelatedInformation

Diagnosis and follow‐up of congenital intrahepatic portosystemic venous shunt by ultrasounds

Intrahepatic portosystemic venous shunt (IPSVS) is mostly observed in adults in association with portal hypertension or cirrhosis and diagnosed by angiography. The congenital form of IPSVS occurs less frequently, without primary liver disease, and can lead to hepatic encephalopathy(HE). Usually, small portosystemic shunts, less than 2 mms. in size, are present in patients with cirrhosis, while the largest ones have a congenital origin. The etiology of the congenital form is controversial, but it is likely due to an incomplete fetal development of vitellin veins that give rise to portal venous system. Literature reports that in infants the disease is usually discovered casually either through routine metabolic screening for galactosaemia (1) or through follow up of a cystic liver detected by prenatal sonogram (2). With improvements in diagnostic imaging techniques, an increasing number of infants and children with asymptomatic IPSVS has been diagnosed. To date, the natural course and indications regarding surgical closure of the shunt have still not been well established (3). Now we are reporting a case of a 5-month-old infant admitted to our department for poor weight gain and diarrhoea. Physical examination and laboratory data including liver function tests were normal. Only lactose tolerance test showed lactose maldigestion. Abdomen ultrasonography showed an anechoic lesion in the anterior segment of the right lobe of the liver that was supplied by a branch of the right portal vein and drained by an enlarged branch of the right hepatic vein, without evident