Intrahepatic Arterioportal Fistula Research Articles

Transhepatic ultrasound guided embolization as a successful novel technique in treatment of pediatric complex intrahepatic arterioportal fistula: a case report and review of the literature

IntroductionIntrahepatic vascular shunts “IHVS” are abnormal communications between intra-hepatic vasculature involving the arterial, portal, or hepatic venous system. Arterio-portal fistula “APF” is an intrahepatic communication between the hepatic arterial system and the portal venous system without any communication with the systemic venous circulation. APF is considered a rare cause of portal hypertension and gastrointestinal bleeding in infancy.Case presentationA 3-month-old Mediterranean female with known cardiac congenital anomalies presented to us with abdominal distension and diarrhea. Ultrasonography revealed massive ascites and computerized tomography (CT) abdomen with intravenous (IV) contrast revealed a left hepatic lesion. On further evaluation, an intrahepatic arterio-portal vascular malformation was detected. Attempted trans arterial embolization failed and radiology team successfully carried out direct trans hepatic ultrasound guided coiling of the aneurysmal venous sac followed by successful resection of segment 4 of the liver with the vascular malformation avoiding life threatening intra operative bleeding.ConclusionAny child with recurrent gastrointestinal bleeding, failure to thrive, vomiting, diarrhea, steatorrhea, splenomegaly, or ascites should be investigated for intrahepatic arterio-portal fistula “IAPF”. Our novel technique of direct trans hepatic ultrasound guided coiling is an alternative method if trans arterial embolization “TAE” failed.

A case of agnogenic intrahepatic arterioportal fistula complicated by gastric ulcer bleeding and progressive portal thrombosis

AbstractArterioportal fistulas (APFs) are defined as vascular communications between the systemic arteries and portal circulation. APFs are classified as congenital or acquired, large or small, intrahepatic or extrahepatic, and traumatic or spontaneous. The consistent abnormal connection may result in severe presinusoidal portal hypertension, leading to gastrointestinal bleeding (GIB), ascites, elevated liver function enzymes, heart failure, diarrhea, and even pancreatitis or hematobilia in some unusual cases. Indeed, less than half reported cases have GIB, predominately caused by variceal rupture. Peptic ulcer bleeding has rarely been reported in the setting of APFs. Herein, we describe a case in which intrahepatic APF manifested as gastric ulcer bleeding, progressive portal thrombosis, and massive ascites, with these symptoms successfully relieved by endovascular treatment.

Simultaneous embolization of a spontaneous porto-systemic shunt and intrahepatic arterioportal fistula: A case report

BACKGROUNDHepatic encephalopathy (HE) is a frequent and debilitating complication of chronic liver disease. Recurrent HE is strongly linked with spontaneous portosystemic shunts (SPSSs). Intrahepatic arterioportal fistulas (IAPFs) occur rarely but pose a major clinical challenge and may lead to or worsen portal hypertension. Herein, we present a rare case of recurrent HE secondary to a SPSS combined with an IAPF.CASE SUMMARYA 63-year-old female with primary biliary cirrhosis presented with recurrent disturbance of consciousness for 4 mo. SPSS communicating the superior mesenteric vein with the inferior vena cava and IAPF linking the intrahepatic artery with the portal vein were found on contrast-enhanced abdominal computed tomography. The patient did not respond well to medical treatment. Therefore, simultaneous embolization of SPSS and IAPF was scheduled. After embolization, the symptoms of HE showed obvious resolution.CONCLUSIONThe presence of liver vascular disorders should not be neglected in patients with chronic liver disease, and interventional therapy is a reasonable choice in such patients.

A clinical case of endovascular treatment of congenital intrahepatic arterioportal fistulae

The development and wide use of instrumental investigational methods, including multispiral computed tomography, computed tomography with angiography, duplex scanning of blood vessels, subtraction angiography, enabled diagnosis of rare diseases, in particular, of the intrahepatic arterioportal fistulae (IAPF). Methods of surgical treatment of this pathology include both open surgery and endovascular treatment with the use of embolizing implants or coils. The article presents a clinical case of diagnosis and endovascular treatment of IAPF in a 33‑year‑old patient, hospitalized in the Center of Interventional Neuroradiology at Feofaniya Clinical Hospital. The choice of the surgical treatment was based on the account of indications and contraindications to the intervention. The endovascular method was used: exclusion of the intrahepatic arterioportal fistulae by means of implantation of micro‑coils Nester 20 мм, MReye 20 mm, Tornado 10–4 mm. Positive dynamics was observed in the long‑term postoperative period (after 3 months), the control was performed based on such criteria as complaints’ absence, normalization of laboratory parameters, positive dynamics of the data of control hepatic angiography. IAPF elimination promoted the reduction of portal hypertension, and improvement of functional liver state. Endovascular method of treatment of patients with arterioportal fistulae is the method of choice at this pathology. The advantages of endovascular method include the minimization of intraoperative and postoperative complications, the shorter postoperative rehabilitation period. In case of the necessity for other method of surgical treatment, one should follow the indications and contraindications to the surgical intervention.

Huge portal venous aneurysm incidentaloma caused by intrahepatic arterioportal fistula accompany with hepatobiliary stones and cholangitis

Huge portal venous aneurysm incidentaloma caused by intrahepatic arterioportal fistula accompany with hepatobiliary stones and cholangitis

Ultrasonographically guided percutaneous transhepatic liver biopsy after pediatric liver transplantation.

Complications associated with ultrasonographically guided percutaneous transhepatic liver biopsy (PTLB) after liver transplantation (LT) have been rarely reported, and there is no consensus about its safety. We retrospectively reviewed the safety and outcomes of PTLB after pediatric LT. Between January 2008 and December 2019, 8/1122 (0.71%) pediatric patients who underwent ultrasonographically guided PTLB after LT developed complications. The median age at PTLB was 7.8years (range 0.1-17.9). Grafts included left lobe/left lateral segment in 1050 patients and others in 72. PTLB was performed using local anesthesia±sedation in 1028 patients and general anesthesia in 94. Complications after PTLB included acute cholangitis in 3 patients, sepsis in 2, respiratory failure due to over-sedation in 1, subcapsular hematoma in 1, and intrahepatic arterioportal fistula in 1. The incidence of complications of PTLB in patients with biopsy alone and those with simultaneous interventions was 0.49% and 3.19%, respectively (p=.023). Patients who developed acute cholangitis, respiratory failure, subcapsular hematoma, and arterioportal fistula improved with non-operative management. Of two patients with sepsis, one underwent PTLB and percutaneous transhepatic portal vein balloon dilatation and developed fever and seizures the following day. Sepsis was treated with antibiotic therapy. Another patient who underwent PTLB and exchange of percutaneous transhepatic biliary drainage catheter developed fever and impaired consciousness immediately. Sepsis was treated with antibiotic therapy, mechanical ventilation, and continuous hemofiltration. Percutaneous transhepatic liver biopsy after pediatric LT is safe. However, combining liver biopsy with simultaneous procedures for vascular and biliary complications is associated with an increased risk of complications.

Early surgical management of severe liver trauma with vascular complication can lead to early discharge

Intrahepatic arterio-portal fistulas are rare complications of blunt hepatic trauma. We describe a case of a 35-year-old male sustaining blunt abdominal trauma resulting in a grade IV liver injury complicated by arterio-portal fistula, portal venous pseudoaneurysm and concomitant bile duct injury. Although arterial embolisation is the mainstay of treatment for arterio-portal fistula, we describe a rationale for early involvement of a hepatobiliary surgeon for multidisciplinary management. Hepatic resection for acute hepatic trauma can, in selected cases, promptly manage all elements of portal triad injury, and in this particular case facilitated early uncomplicated discharge.

Successful surgical treatment for intrahepatic arterioportal fistula with severe portal hypertension: a case report

BackgroundIntrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension. Interventional radiology (IVR) is generally selected as the first-line therapeutic option. Surgical treatment for IAPF is required in refractory cases of IVR. As the treatment success rate with IVR is high, cases requiring surgical treatment are extremely rare.Case presentationA 54-year-old man was admitted to another hospital complaining of hematemesis due to rupture of the esophageal varices. A computed tomography revealed ascites and arterioportal fistula in the left lobe of the liver. Transcatheter arterial embolization (TAE) was performed to occlude the fistula; however, it could not reach complete occlusion. Thereafter, there were a total of four hematemeses, and six endoscopic variceal ligations were required. The second TAE also failed to reach complete occlusion. He was transferred to our hospital for further treatment. Because liver function was low due to frequent hematemeses and there was also uncontrollable ascites, it was confirmed that hepatectomy could not be performed safely at this time. Therefore, we ligated the left portal branch and ligated and dissected the left gastric vein to decrease portal vein pressure. However, on the 5th day after surgery, the esophageal varices reruptured. As the disappearance of ascites was observed in the postoperative course and the general condition also improved, left hepatectomy was performed to remove IAPF. There was no recurrence of portal hypertension for 1 year and 3 months since hepatectomy.ConclusionsThis case was difficult to treat with IVR and required surgical treatment. Our experience in the present case suggests that hepatectomy to remove arterioportal fistula was considered effective for improving portal hypertension due to IAPF. However, careful treatment selection according to the patient’s overall condition and clinical course is necessary for IAPF presenting with severe portal hypertension.

Case Report of Multiple Bilobar Hepatic Arterio-Portal Fistulas Post–Liver Transplantation Managed Conservatively

Multiple intrahepatic arterio-portal fistulas are rare. The majority are isolated and occur secondary to liver trauma including iatrogenic interventions such as liver biopsy. Post–liver transplantation 18 cases have been reported, all secondary to an interventional radiological procedure. We report multiple bi-lobar arterio-portal fistulas in a liver transplant recipient recognized 1 year after transplantation. The donor died due to intracerebral bleeding following blunt head and abdominal trauma. In the present case, the etiology is not very clear. The patient was managed conservatively and to date has not required intervention.

Therapeutic evaluation of arterio-portal fistula-related gastroesophageal variceal bleeding.

Background & AimsIntrahepatic arterio-portal fistula is an uncommon etiology of portal hypertension, which presents diagnostic and therapeutic challenges. This study aimed to assess the efficacy and outcomes of gastroesophageal variceal bleeding caused by arterio-portal fistula using different therapeutic approaches.MethodsMedical records of 451 consecutive patients with arterio-portal fistula were reviewed from January 1, 2009, to July 15, 2016, and patients suffered variceal bleeding were eligible for the study.ResultsAmong 57 patients with arterio-portal fistula, hepatocellular carcinoma was existed in 61.4% patients. A combination of radiological intervention and endoscopic treatment was performed in 8 (14.0%) patients; the remainder were treated using radiological intervention alone (n = 20, 35.1%), endoscopic treatment alone (n = 18, 31.6%), or without any intervention (n = 11, 19.3%). No patient died in the combination group, while 20 patients in the single-treatment group and 6 in the untreated group died during follow-up. A significant difference in the survival rate was found between the combination group and the other two groups. Treatment selection between combination and untreated groups was the only parameter significantly associated with survival (p = 0.002).ConclusionsFor patients diagnosed with arterio-portal fistula, combination treatment is the most optimal strategy in managing variceal bleeding, especially in patient with severe type of fistula.

自然閉鎖した肝内動脈門脈瘻の一例

症例は67歳男性．吐血を主訴に救急搬送された．緊急上部消化管内視鏡検査（EGD）にて食道静脈瘤（Ls F3 Cb RC2）ならびに胃静脈瘤（Lg-cf F3 RC1）を認め，出血源と考えられた胃噴門部の静脈瘤に対して内視鏡的静脈瘤結紮術（EVL）を施行した．腹部造影CTで脾腫，腹水のほか，左肝動脈から門脈左枝に流入する肝内動脈門脈瘻を認め，肝生検では肝硬変の所見を認めなかった．血管造影検査にて左肝動脈から門脈左枝に連絡する肝内動脈門脈瘻を確認した．その後の経過観察中に腹水は改善し，EVL施行から9カ月後のEGDでは食道静脈瘤はF1形態で白色化し，胃静脈瘤はF0形態となりいずれも著明な改善を認めた．血管造影検査では，肝内動脈門脈瘻は閉鎖しており，これにより門脈圧が正常化することで食道胃静脈瘤が改善したと考えられた．門脈圧亢進症を呈する肝内動脈門脈瘻の自然閉鎖は極めて稀であると考え，報告する．

Portal Vein Thrombosis and Arterioportal Fistula in Post Liver Transplant Recipient: A Case Report

An intrahepatic Arterioportal Fistula Refers (APF) to abnormal shunt or fistulous connection between the portal venous system and a hepatic arterial system within the liver. Here, we present a case of portal vein thrombosis with APF in post-transplant liver, developed 2 years and 6 months after transplantation. The condition was diagnosed by Triphasic CT angiography. In this case report we have discussed various causes and pathophysiology of APF with its imaging findings.

PO-0606 Radiologic Intervention Of Portal Hypertension In An Infant With Down Syndrome Due To Diffuse Arterioportal Fistulae

Introduction Portal hypertension isdefined as an elevation of portal pressure >10–12 mm Hg. 1 There are manycauses of portal hypertension including heterogeneous group of diseases due to intrahepatic or extrahepatic etiologies. 2 Hepatic vascularmalformations usually occur secondary totrauma, percutaneous interventions, neoplasms and cirrhosis. Arteriovenousfistulae may also be congenital. 3 Patients withDown syndrome might have a reduced riskof vascular anomalies compared with the general population. 4 Althoughvarious vascular abnormalities were described in patients with Down syndrome,arteriovenous fistulae between hepatic artery and portal vein is rarely not reported. Case report We describe a three month old infant with trisomy 21 (Down syndrome) andarterioportal fistula (APF) associated with extrahepatic portal hypertensionand massive ascites. Sonographic examination of the upper abdomen showed severe ascites and hepatosplenomegaly. Multiple aneurismatic arteriovenous fistulae betweenhepatic artery and left portal vein were demonstrated by portal Doppler sonography. Celiacarteriography was performed for further evaluation and coil embolization;demonstrating that there were diffuseconnections between left portal vein and main hepatic artery, gastroduodenal artery,second and third branches of right and left hepatic artery (Figure 1A and 1B). In addition to transarterial interventionpercutaneus transhepatic access to the left portal vein was performed and lefthepatic artery to left portal vein fistulae were embolized with n-butyl 2cyanoacrylate which is a liquid embolic agent. Right hepatic artery and mainhepatic artery were embolized with multiple metalic coils bymicrocatheterization technique. Following the embolization procedure celiacarteriography revealed only partialocclusion of APF due to diffuse involment (Figure 2). Discussion Our case isunique as it is the first case who wasattempted to be treated with percutaneous transvascular coil embolization of the APF. Hepaticangiography is the gold Standard to confirm the diagnosis and to demonstratethe vascular anatomy (5). References 1 Suchy FJ. Portal Hypertension and Varices, Nelson Text Book Of Pediatrics, 18 th ed. Philadelphia, WB Saunders,2007, pp:1709–1712 2 Sarin SK, Kumar A. Noncirrhotic portal hypertension. Clin Liver Dis . 2006;10(3):627–51 3 Landau YE, Schwarz M, Belenky A, Shapiro R, Amir J. Arteriovenous Fistula and Portal Hypertension in a Child with Down Syndrome. IMAJ 2007;9:825–826 4 Greene AK, Kim S, Rogers GF, Fishman S, et al . Risk of Vascular Anomalies With Down Syndrome. Pediatrics 2008;121(1):135–140 5 Karnak I, Cil BE, Akay H. Congenital Intrahepatic Arterioportal Fistula:An Unusual Cause of Portal Hypertension Treated by Coil Embolization in an Infant. Eur J Pediatr Surg . 2009;19:251–271

Non-cirrhotic Portal Hypertension from Chemotherapy-induced Intrahepatic Non-tumorous Arterioportal Fistula

Purpose: Intrahepatic arterioportal fistulas (APF) are rare, are mostly caused by trauma or malignancy, and can cause presinusoidal portal hypertension. We report a case of chemotherapy-induced non-tumoral intrahepatic APF (CI-APF) presenting with non-cirrhotic portal hypertension (NCPH). A 57-year-old white man presented with chronic abdominal pain and abnormal liver tests for one year (3/13). He underwent right hemicolectomy for cecal adenocarcinoma (2/12), and received adjuvant chemotherapy with FOLFOX (4/12-9/12). His laboratory data showed thrombocytopenia and fluctuating liver tests (AST 43-90 U/L; ALT 49-91U/L; alkaline phosphatase 173-280U/L, and total bilirubin 0.4-1.7mg/dL). Viral and autoimmune hepatitis were excluded. Physical exam was normal. Upper endoscopy showed new trace esophageal varices. Abdominal magnetic resonance imaging reported a large APF in both lobes of the liver, with hyperarterial vascularity perfusing the right hepatic lobe, early shunting to the portal vein, moderate atrophy of right lobe of the liver, distension of the portal vein, and splenomegaly with otherwise patent hepatic vasculature (A). Trans-jugular liver biopsy with wedge pressure and hepatic arteriography showed hepatic vein pressure gradient of 3 mmHg and hepatic arterial to portal vein shunting (B). Liver biopsy was notable for focal sinusoidal dilatation, unremarkable bile ducts, portal tracts, and hepatocytes, with no fibrosis. Prior imaging showed normal hepatic anatomy (2/2012, 6/2012) and fatty infiltration of the liver without vascular abnormalities (11/2012). The occurrence of APF with presinusoidal portal hypertension and the temporal relationship with chemotherapy led to the final diagnosis of NCPH from CI-APF. Several cases of NCPH in patients undergoing chemotherapy for metastatic colorectal cancer have been reported. It is believed that severe hepatic injury from chemotherapy results in benign transformation of the hepatic parenchyma into nodular regenerative hyperplasia (NRH). In our case, the presence of pre-sinusoidal portal hypertension is more supportive of obliterative portal venopathy leading to APF, rather than NRH. Shunt reduction with minimally invasive techniques such as transcatheter hepatic artery embolization have been successfully performed without severe complications.Figure

A patient with hemodialysis-related hyperammonemic encephalopathy: a delayed presentation of congenital arterioportal fistulas

We present a 32-year-old woman who developed hyperammonemic encephalopathy during hemodialysis. She was rather well before 2009 when receiving peritoneal dialysis due to chronic interstitial nephritis. Due to a refractory peritonitis, the treatment was shifted to hemodialysis in January 2009. About 1 year later, she was found with consciousness disturbance during hemodialysis then admitted to the hospital because of hyperammonemia (165 μg/dl). During hospitalization, the patient's abdominal Doppler sonography showed a hepatofugal flow in the portal trunk while the hepatic artery angiography demonstrated multiple intrahepatic arterioportal fistulas. Her general condition was improved after the treatment of lactulose and hepatic artery embolization. With the occurrence of arterioportal fistulas induced portal hypertension, we speculated that the portal-systemic shunt was enhanced during hemodialysis because of venous hypotension which then resulted in the transient hyperammonemia. To the best of our knowledge, this is the first patient who developed hemodialysis- related hyperammonemic encephalopathy due to multiple arterioportal fistulas.

Isolated arterioportal fistula presenting with variceal hemorrhage

We report a case of life-threatening hematemesis due to portal hypertension caused by an isolated arterioportal fistula (APF). Intrahepatic APFs are extremely rare and are a cause of presinusoidal portal hypertension. Etiologies for APFs are comprised of precipitating trauma, malignancy, and hereditary hemorrhagic telangiectasia, but these were not the case in our patient. Idiopathic APFs are usually due to congenital vascular abnormalities and thus usually present in the pediatric setting. This is one of the first cases of adult-onset isolated APF who presented with portal hypertension and was successfully managed through endoscopic hemostasis and subsequent interventional radiological embolization.

Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up

Congenital intrahepatic arterioportal fistulas (APFs) are a rare cause of portal hypertension in children. Doppler US is a useful diagnostic imaging modality. Transarterial embolization is a minimally invasive and effective therapy allowing occlusion of the fistula and restoration of liver hemodynamics. To describe the clinical and radiologic findings, percutaneous treatment and role of D-US in the postembolization follow-up of children with APF. Between 2002 and 2011, four children with APF were treated. Initial diagnosis and follow-up was performed with D-US and confirmed by arteriography, followed by endovascular embolization in all patients. D-US demonstrated abnormal arterioportal communications in all patients. Six endovascular procedures were performed in these four children. In two children, no residual fistula was seen on D-US after the first procedure and symptoms resolved. In the other two children, D-US demonstrated residual flow through the fistula, with resolution of pathological D-US findings and symptoms after the second endovascular procedure. All four children were successfully treated and asymptomatic at the end of follow-up. The mean follow-up was 24 months. Interventional radiology has a key role in the treatment of congenital APF. D-US is a noninvasive and effective tool for the diagnosis and follow-up of these patients.

Arterioportal Fistulas in Liver Transplant Recipients

Arterioportal fistulas (APFs) are classified into intrahepatic (>75% of all reported) and extrahepatic (<25% of all reported ). Anecdotally, investigators are more likely to report more sensational cases (typically extrahepatic APFs), so the actual prevalence of intrahepatic APFs is probably much higher (likely >90% of APFs). All reported APFs in liver transplant recipients have been intrahepatic. Hemodynamically significant APFs in liver transplant recipients are rare, occurring in 0.2%; however, APFs (hemodynamically significant or not) are not uncommonly seen in hepatic angiograms of liver transplant recipients (up to 5.4% of hepatic arteriograms in transplants). Interestingly, hemodynamically significant APFs warranting endovascular treatment are reported more commonly in the literature in native compared with transplanted livers (n >280-300 versus n = 13, respectively). This article discusses APFs that are specific to liver transplant recipients; their incidence, etiology, pathogenesis, natural history, clinical presentation, and endovascular management are discussed in detail.

Gastric variceal bleeding caused by an intrahepatic arterioportal fistula that formed after liver biopsy: a case report and review of the literature

An intrahepatic arterioportal fistula is a rare cause of portal hypertension and variceal bleeding. We report on a patient with an intrahepatic arterioportal fistula following liver biopsy who was successfully treated by hepatectomy after unsuccessful arterial embolization. We also review the literature on symptomatic intrahepatic arterioportal fistulas after liver biopsy. A 48-year-old male with bleeding gastric varices and hepatitis B virus-associated liver cirrhosis was transferred to our hospital; this patient previously underwent percutaneous liver biopsies 3 and 6 years ago. Abdominal examination revealed a bruit over the liver, tenderness in the right upper quadrant, and splenomegaly. Ultrasonographic examination, computed tomography, and angiography confirmed an arterioportal fistula between the right hepatic artery and the right portal vein with portal hypertension. After admission, the patient suffered a large hematemesis and developed shock. He was treated with emergency transarterial embolization using microcoils. Since some collateral vessels bypassed the obstructive coils and still fed the fistulous area, embolization was performed again. Despite the second embolization, the collateral vessels could not be completely controlled. Radical treatment involving resection of his right hepatic lobe was performed. For nearly 6 years postoperatively, this patient has had no further episodes of variceal bleeding.

An unusual presentation of a congenital intrahepatic arterioportal fistula in an infant with Down syndrome

Congenital intrahepatic arterioportal fistula is a rare entity that most often presents with symptoms of portal hypertension and malabsorption. We discuss a patient who presented with distributive shock, congestive heart failure, pulmonary hypertension, ascites, and a history of severe constipation.