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BACKGROUND Hepatic 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 SUMMARY A 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. CONCLUSION The 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.

BACKGROUND Most intrahepatic arterioportal fistulae (IAPF) are acquired. The few cases of congenital fistulae are diagnosed in infants and children. CASE SUMMARY We report a 31-year-old female patient presenting with haematemesis and melena three weeks after delivering her second child. The patient had a 20-year history of abdominal distention and nausea. IAPF, along with splenomegaly and ascites, was found by Doppler sonography and confirmed by computed tomography angiography. The patient was treated with endovascular coil embolization, resulting in occlusion of the fistula. CONCLUSION This was an unusual case of possible congenital IAPF that manifested during a second pregnancy and was complicated by portal hypertension.

BACKGROUND Congenital intrahepatic arterioportal fistula (IAPF) is a rare vascular malformation in infants that causes severe portal hypertension (PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children. Furthermore, indications are not defined. CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multi-step endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised. The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascular-surgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported. CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

Congenital intrahepatic arterioportal fistulas are rare causes of presinusoidal portal hypertension in children. A rare case of arterioportal fistula in an infant is being reported. This report also highlights the need for long-term surveillance in such cases, despite being a benign pathology.

Background Massive arterioportal fistula (APF) is naturally irreversible and can induce portal hypertension and portal vein thrombosis (PVT), worsening survival outcomes. Purpose To evaluate the clinical course and details of transarterial embolization (TAE) procedures for massive APF. Material and Methods This retrospective single-center observational study evaluated the time until embolization after puncture, imaging, embolization methods, and laboratory data of 10 consecutive patients who were diagnosed with massive APF after puncture and underwent TAE at our hospital from 1 April 2012 to 30 September 2019. Results Out of 10 cases, eight demonstrated a simple type and the other two cases a complex network type on the digital subtraction angiography pattern of massive APF. In two simple-type cases for which re-embolization was required, other subsegmental branches were embolized. The two cases showing a complex network type had been embolized via not only the subsegmental branch, but also the extrahepatic and multiple subsegmental branches. Child–Pugh scores were improved in eight of the ten cases. PVT was seen in six cases before embolization, but disappeared after embolization in all cases, despite the fact that three cases had not received anticoagulant therapy. Six cases had digestive varices before embolization, suggesting portal hypertension, and two of the six cases with esophageal varices and one with gastric varices decreased after embolization. Conclusion TAE for massive APF contributed to the improvement of hepatic reserve, the disappearance of PVT, and the improvement of portal hypertension; however, embolization of multiple branches may still be required in some cases.

Intrahepatic arterioportal fistulas may be complicated by portal hypertension. An associated portal venous aneurysm (PVA) may impinge upon adjacent structures or rupture. We present a 65-year-old man with an intrahepatic Intrahepatic arterioportal fistula and 6.4 × 5.8 cm right portal vein aneurysm extending within 0.4 cm of the hepatic margin, associated with pain concerning for impending rupture. The PVA was refractory to transarterial embolization due to recruitment of arterial collaterals. Therefore, it was additionally excluded from the portal vein with a 12 mm × 9.5 cm venous stent graft. Although endovascular therapy thrombosed the aneurysm and improved symptoms, it was complicated by a type 2 endoleak into the PVA. Keywords: Arterial-portal fistula, Portal vein aneurysm, Type 2 endoleak

Arterioportal fistulas (APFs) are uncommon vascular abnormalities with a heterogeneous etiology. In pediatric orthotopic liver transplantation (OLT), APFs are frequently iatrogenic, following percutaneous liver interventions. The aim of this study was to report the 10-year experience of a tertiary referral center for pediatric OLT in the interventional radiological (IR) and conservative management of acquired APFs. A retrospective search was performed to retrieve pediatric patients (&lt, 18 years old) with a diagnosis of APF at color Doppler ultrasound (CDUS) or computed tomography angiography (CTA) from 2010 to 2020. Criteria for IR treatment were the presence of hemodynamic alterations at CDUS (resistive index &lt, 0.5, portal flow reversal) or clinical manifestations (bleeding, portal hypertension). Conservatively managed patients served as a control population. Clinical and imaging follow-up was analyzed. Twenty-three pediatric patients (median age, 4 years, interquartile range = 11 years, 15 males) with 24 APFs were retrieved. Twenty patients were OLT recipients with acquired APFs (16 iatrogenic). Twelve out of twenty-three patients were managed conservatively. The remaining 11 underwent angiography with confirmation of a shunt in 10, who underwent a total of 16 embolization procedures (14 endovascular, 2 transhepatic). Technical success was reached in 12/16 (75%) procedures. Clinical success was achieved in 8/11 (73%) patients, three clinical failures resulted in one death and two OLTs. After a median follow-up time of 42 months (range 1–107), successfully treated patients showed an improvement in hemodynamic parameters at CDUS. Conservatively managed patients showed a stable persistence of the shunts in six cases, spontaneous resolution in four, reduction in one and mild shunt increase in one. In pediatric patients undergoing liver interventions, APFs should be investigated. Although asymptomatic in most cases, IR treatment of APFs should be considered whenever hemodynamic changes are found at CDUS.

Superior mesenteric arteriovenous fistulae (SMAVF) are a rare complication from trauma or iatrogenic surgical intervention. There are less than 50 cases reported in the literature and no clear guidelines as to the best practices for diagnosis and treatment. SMAVF are often asymptomatic but can present with nonspecific abdominal symptoms ranging from nausea and vomiting to gastrointestinal bleeding and mesenteric ischemia. Symptom onset, when present, is often delayed years after the inciting event, further complicating the diagnosis. We present a case of a 71-year-old man presenting with mesenteric ischemic symptoms secondary to a large SMAVF that was successfully treated with coil embolization. We describe our approach to treatment and describe the classical imaging findings. We, then, review the current literature and management recommendations.

Hepatocellular carcinoma (HCC) patients often developed hepatic arterioportal fistula (APF). The aim of this study is to evaluate the impact of APF on future liver remnant (FLR) regeneration and surgical outcomes after the first stage of associating liver partition and portal vein ligation for staged hepatectomy (stage-I ALPPS). Consecutive HCC patients who underwent ALPPS at our center between March 2017 and May 2019 were retrospectively studied. Data for the association between APF and clinicopathological details, liver volume, and surgical outcomes were analyzed. The enrolled 35 HCC patients were divided into three groups: 15 patients with preoperative APF were classified as the APF I group, 10 patients developed APF after stage-I ALPPS as the APF II group, whereas the other 10 patients without APF before and after stage-I ALPPS as the control group. After stage-I ALPPS, patients in the APF I and APF II groups had lower kinetic growth rate (KGR) of FLR volume (6.1±3.2%, 11.4±8.4%, 25.0±8.8% per week, respectively, P

Background Arterioportal fistulas are rare vascular disorders of the abdominal viscera. They are arteriovenous communications between the splanchnic arteries and the portal vein or its tributaries. We herein report a case of an extrahepatic arterioportal fistula that was caused by rupture of a pseudoaneurysm of the pancreaticoduodenal artery and successfully treated with embolization using a combination of the arterial and percutaneous transhepatic portal venous approaches. Case presentation A 79-year-old man was transferred to our hospital because of the sudden appearance of a hematoma containing a large pseudoaneurysm in the mesentery of the duodenum. Emergency abdominal angiography revealed that a pseudoaneurysm of the anterior inferior pancreaticoduodenal arterial branch had perforated into the portal system (arterioportal fistula). We performed coil embolization via the inflow artery and portal vein using a percutaneous transhepatic approach. The patient recovered without complications and was discharged. Conclusion This rare vascular disorder was successfully treated with an unplanned combination therapy. We believe that flexible strategy changes led to the successful treatment in this case.

Resumo A síndrome da fístula artério-portal (FAP) é uma rara e reversível causa de hipertensão portal pré-sinusoidal, ocasionada pela comunicação de uma artéria visceral com o sistema venoso portal. A maioria dos pacientes é assintomática, mas quando desenvolvem sintomas, estes são mais relacionados com sangramento gastrointestinal, ascite, insuficiência cardíaca congestiva e diarreia. Este desafio terapêutico apresenta um caso de FAP decorrente de ferimento antigo por arma branca e subsequente evolução clínica desfavorável, com grave desnutrição e frequentes hemorragias digestivas. O caso foi solucionado através de oclusão da FAP por meio de tratamento endovascular.

Two cats presented with large volume ascites and the cause was suspected to be portal hypertension. On contrast CT they both showed enhancement of the main portal vein during the arterial phase and an anomalous connection between the celiac artery and extrahepatic portal vasculature, prompting a diagnosis of extrahepatic arterioportal fistula. An extrahepatic arterioportal fistula is a connection between any artery and the portal vein outside the liver and, to our knowledge, this is the first report in cats.

A 9‐month‐old domestic short‐haired cat presented with stunted growth and chronic gastrointestinal signs. Tachypnoea, a heart murmur and cranial abdominal bruit were detected on physical examination. Echocardiography revealed volume overload in all heart chambers. CT angiography identified an abnormal communication between the hepatic arterial circulation and the portal vein, along with multiple acquired shunts. The abnormal vascular communication was surgically ligated. Echocardiography documented improvement in cardiac parameters following surgery and the cat continues to have no clinical signs 39 months after surgery. This report describes successful surgical management of feline hepatic arterioportal fistula for the first time.

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Background Intrahepatic 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 presentation A 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. Conclusions This 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.

Highlights • Intrahepatic arterioportal fistula is an unusual complication of liver biopsy. • Intrahepatic arterioportal fistula can cause variceal bleeding. • Angioembolization is an effective treatment of variceal bleeding., Introduction and importance Variceal bleeding due to intrahepatic arterioportal fistula is an unusual complication of percutaneous liver biopsy. As majority of variceal bleeding are cirrhotic in origin, the rare occurrence of an acquired intrahepatic arterioportal fistula presents a therapeutic dilemma. Case presentation We report the case of a 57-year-old female with refractory variceal bleeding that occurred six years after a percutaneous liver biopsy. As part of the workup for placement of Transjugular Intrahepatic Portosystemic Shunt, a computed tomography hepatic arteriography was performed. This revealed a large arterioportal fistula in left lobe of liver. Variceal bleeding was controlled following successful embolisation of the arterioportal fistula. Clinical discussion Persistent intrahepatic arterioportal fistula can result in portal hypertension and variceal bleeding. This is a rare complication of percutaneous liver biopsy that warrants consideration as an aetiology of portal hypertension with variceal bleeding. The therapeutic strategy for refractory bleeding due to intrahepatic arterioportal fistula is different from cirrhotic portal hypertension and requires trans-arterial embolisation of the fistula. Conclusion This case highlights the need to consider arterioportal fistula as an aetiology of portal hypertension as therapeutic strategy in refractory variceal bleeding is different from cirrhotic portal hypertension.

Arterioportal fistula (APF) is a rare and potentially fatal cause of portal hypertension. In this case report, we present an unwonted case of a Nigerian with this disorder. As well, this article documents details of successful therapy for such a large APF with transcatheter arterial embolization which was carried out locally. This study is thus a first-of-its-kind for our African subregion. We hope that this article will help to highlight the role and significance interventional radiology services and expertise in an emerging health-care system as the one seen in West Africa – particularly as it relates to its benefits in the field of hepatology.

We present a rare case of an arterioportal fistula that formed between the superior mesenteric artery and portal vein 30 days following a pancreaticoduodenectomy, which was successfully managed with endovascular procedures.

Transjugular intrahepatic portosystemic shunt (TIPS) occlusion is a common occurrence that can be managed based on the nature and acuity of shunt failure. Arterioportal fistulas (APFs) are rare communications between portal venous and systemic arterial vasculature that can present with symptomatic portal hypertension. In this case, we describe the management of a patient with variceal bleeding due to TIPS dysfunction complicated by the presence of an APF.

Traumatic hepatic arterioportal fistula is an abnormal communication between the hepatic artery and portal vein and is a rare cause of non-cirrhotic portal hypertension with delayed presentation, usually after a remote history of abdominal trauma or an interventional procedure. This case report is of one such rare presentation, wherein a 59-year-old gentleman presented with unexplained ascites and complications of portal hypertension, eventually diagnosed with an arterioportal fistula on a computed tomography scan and managed by angioembolization. There was a remarkable improvement in the complications of portal hypertension after the coil embolization.

Background Few cases of postoperative arterioportal fistula (APF) have been documented. APF after hepatectomy is a very rare surgery-related complication. Case presentation A 62-year-old man was diagnosed with hepatocellular carcinoma in segments 5 and 8, respectively. Anterior segmentectomy was performed as a curative surgery. Each branch of the hepatic artery, portal vein, and biliary duct for the anterior segment was ligated together as the Glissonean bundle. The patient was discharged on postoperative day 14. Three months later, dynamic magnetic resonance imaging showed an arterioportal fistula and portal vein aneurysm. Surprisingly, the patient did not have subtle symptoms. Although a perfect angiographic evaluation could not be ensured, we performed angiography with subsequent interventional radiology to avoid sudden rupture. Arteriography was immediately performed to create a portogram via the APF from the stump of the anterior hepatic artery, and portography clearly revealed hepatofugal portal vein flow. Portography also showed that the stump of the anterior portal vein had developed a 40-mm-diameter portal vein aneurysm. Selective embolization of the anterior hepatic artery was accomplished in the whole length of the stump of the anterior hepatic artery, and abnormal blood flow through the APF was drastically reduced. The portal vein aneurysm disappeared, and portal flow was normalized. Dynamic computed tomography after embolization clearly demonstrated perfect interruption of the APF. The patient maintained good health thereafter. Conclusions Post-hepatectomy APFs are very rare, and some appear to be cryptogenic. Our thought-provoking case may help to provide a possible explanation of the causes of post-hepatectomy APF.

Rationale: Hepaticarterioportal fistula (APF) is a rare cause of portal hypertension and gastrointestinal hemorrhage, and presents as abnormal communication between the hepatic artery and portal vein. Percutaneous liver biopsy is a main iatrogenic cause of AFP. However, non-iatrogenic, abdominal, trauma-related APF is rarely reported. Patient concerns: A 29-year-old man presenting with severe, watery diarrhea was transferred to our hospital, and his condition was suspected to be acute gastroenteritis because he ate expired food and suffered a penetrating abdominal stab wound 5 years ago. After admission, the patient suffered from hematemesis, hematochezia, ascites, anuria, and kidney failure, and he developed shock. Diagnoses: The patient was finally diagnosed as a traumatic hepatic artery pseudoaneurysm and APF. Interventions: This patient was treated with emergency transarterial embolization using coils. Since a secondary feeding vessel was exposed after the first embolization of the main feeding artery, a less-selective embolization was performed again. Outcomes: During the 6-month follow-up period, the patient remained asymptomatic. Lessons: A penetrating abdominal stab wound is a rare cause of hepatic APFs, and occasionally leads to portal hypertension, the medical history and physical examination are the most important cornerstones of clinical diagnosis. Interventional radiology is essential for the diagnosis and treatment of an APF.

Radiofrequency ablation (RFA) is commonly used as a treatment for small hepatocellular carcinoma (HCC). Although several complications such as intraperitoneal bleeding are often observed after RFA, hepatic arterioportal fistula (APF) is a less frequently occurring complication. In this study, we describe two cases of APF caused by RFA, which was successfully occluded by an interventional approach. Case 1 involved a 68-year-old man with solitary HCC in segment VIII of the liver. Both contrast-enhanced computed tomography and color Doppler sonography indicated an APF between the anterosuperior branch of the right hepatic artery (A8) and the portal branch (P8). Concordant with these findings, digital subtraction angiography (DSA) revealed an APF in segment VIII of the liver. Subsequently, the APF was successfully occluded by transarterial embolization (TAE) using gelatin sponge particles. Case 2 involved a 67-year-old man with solitary HCC in segment VII of the liver. Although he developed obstructive jaundice because of hemobilia after RFA, it was improved by endoscopic nasobiliary drainage and the systemic administration of antibiotics. In addition, color Doppler sonography revealed a disturbed flow of the right branch of the portal vein. Similar to case 1, DSA showed an APF between A8 and P8. The APF was successfully embolized by TAE using microcoils. In conclusion, it appears that the formation of APF should be checked after RFA. It is preferable to treat RFA-induced APF promptly by an interventional approach to avoid secondary complications such as portal hypertension and liver dysfunction.

Arterio-portal fistulas (APFs) are characterized by anomalous communication between arteries and the portal vein (PV) system. Treatment of APF is imperative as an emergency or if there is development of portal hypertension/heart failure in chronic cases. Both endovascular and surgical managements can be attempted, however since endovascular management carries comparatively low intra and post procedural morbidity it is mostly preferred. This is a case report on endovascular management of post-traumatic pseudoaneurysm arising from bifurcation of common hepatic artery with complete disruption of the gastroduodenal artery and high-flow APF. This report describes the intraprocedure challenges in exclusion of fistula from the circulation, without disruption of portal system and anticipation of recruitment of new collateral feeders to the fistula immediate post exclusion with its embolization, which needs appropriate positioning of the catheter prior to exclusion of the fistula.

The use of the patent ductus venosus via a transjugular approach to access the portal system for endovascular treatment of hepatic vascular anomalies in three infants is reported. Two patients had an arterioportal fistula, and one had a rapidly involuting congenital hemangioma. All patients underwent arteriography followed by embolization of the vascular anomalies without complications. This alternative route is technically simpler and likely safer than transarterial and transhepatic approaches.

Arterioportal fistulas (APFs) are a group of vascular disorders, in which systemic arteries communicate with the portal circulation, presenting as a congenital syndrome or more commonly acquired from iatrogenic instrumentation or abdominal trauma. We report the case of a 58-year-old man who developed ascites without underlying risk factors for portal hypertension, which was attributed to an APF found on imaging, manifesting 43 years after sustaining a liver laceration. After angiographic embolization of the APF, the patient's ascites resolved completely. The prolonged latent period between the patient's abdominal trauma and eventual presentation with ascites highlights the need to consider vascular malformations in the differential diagnosis of unexplained noncirrhotic portal hypertension.

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.

Hepatic arteriovenous fistulae (AVF) are rare but increasingly encountered clinical entities. Due to their poorly understood natural history and multiple underlying etiologies, treatment currently represents a clinical quandary. There are no definitive studies regarding management, but rather, only small published case series. A better understanding of the underlying mechanisms of AVF formation, factors affecting clinical significance, and knowledge of the risks and benefits of treatment can help guide physicians in their management of this complex clinical dilemma.

Extrahepatic arterioportal fistulas are a rare cause of portal hypertension in infants and adults. Most cases of arterioportal fistulas in adults are due to trauma, liver biopsy, surgery or tumours. The authors present a case of arterioportal fistula diagnosed in adult life without any of the above aetiologies. The patient became acutely symptomatic with diarrhoea and ascites at the age of 66 years and was successfully treated with a single endovascular embolisation procedure after failed medical therapy.

We describe a child who presented with a traumatic hepatic artery pseudoaneurysm and arterioportal fistula, which were subsequently managed with an endovascular stent graft and coil embolization using flow control with balloon remodeling. This case demonstrates a rarely seen condition in the pediatric population and a novel management strategy, which should be considered in the management of this complex injury.

Congenital intrahepatic arterioportal fistula (IAPF) is a rare condition and there is limited experience of transcatheter embolization. We report here the transcatheter coil embolization of an incidentally found, asymptomatic congenital IAPF in a 16-month-old patient. After demonstrating that the IAPF was fed by two branches of a dilated left hepatic artery and drained into an aneurysm of the left portal vein, the feeding arteries were superselectively catheterized and occluded by microcoils in a single session. Follow-up was uneventful for 1 year. With respect to this and previously reported cases, we believe that embolization of feeding arteries is a safe and effective method that should be the primary choice of treatment; however, further cases should be reported to confirm this belief.

An 80-year-old woman with hepatic encephalopathy and esophagogastric varices was admitted. Doppler sonography and arteriography demonstrated an arterioportal fistula in the right anterior superior segment of the liver. Two attempts at transcatheter arterial embolization failed to manage the fistula and portacaval collaterals. We carried out hepatectomy and balloon-occluded retrograde transvenous obliteration, by which the portal flow changed from hepatofugal to hepatopetal. The resected specimen demonstrated multiple small holes in the right portal vein, which are arterioportal fistula. The underlying liver was normal. After treatment, esophagogastric varices and hepatic encephalopathy were markedly improved. Hepatectomy is useful as a reliable therapy for arterioportal fistula in case of failure of transarterial embolization.

Intrahepatic arterioportal fistula (IAPF) can be caused by many secondary factors. We report four cases of portal hypertension that were eventually determined to be caused by congenital hepatic arterioportal fistula. The clinical manifestations included ascites, variceal hemorrhage and hepatic encephalopathy. Computed tomography scans from all of the patients revealed the early enhancement of the portal branches in the hepatic arterial phase. All patients were diagnosed using digital subtraction angiography (DSA). DSA before embolization revealed an arteriovenous fistula with immediate filling of the portal venous radicles. All four patients were treated with interventional embolization. The four patients remained in good condition throughout follow-up and at the time of publication. IAPF is frequently misdiagnosed due to its rarity; therefore, clinicians should consider IAPF as a potential cause of non-cirrhotic portal hypertension.