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

Teplisky, Darío; Tincani, Eliana Urueña; Lipsich, José; Sierre, Sergio

doi:10.1007/s00247-012-2443-x

Cited by 16 publications

(7 citation statements)

References 19 publications

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“…The most common cause is trauma, typically iatrogenic. Intrahepatic congenital arterioportal fistulas have also been reported and are typically characterized by several arterial feeding vessels with a portal vein varix (36,37). These may be isolated or seen with underlying connective tissue disorders such as Ehlers-Danlos syndrome (36).…”

Section: Arterioportal Shuntsmentioning

confidence: 99%

Vascular Anomalies of the Pediatric Liver

Albers

Khanna

2019

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The liver is a unique organ as it receives afferent blood supply from the umbilical vein, portal vein, and hepatic artery in the developing embryo but has only one efferent drainage method, through the hepatic veins. In the postnatal period, about 70% of the afferent blood flow into the liver is from the portal venous system, unique vessels that begin and end in a capillary system. Vascular anomalies of the hepatic artery, hepatic veins, portal vein, and/or umbilical vein can be congenital or acquired secondary to inflammation and/or infection, trauma, systemic disorders, or iatrogenic causes. The vascular anomalies can be incidental findings at imaging, or the infant or child can present with symptoms such as abdominal pain and ascites, be diagnosed with gastrointestinal bleeding, and have abnormal liver function test results. Imaging can demonstrate vascular findings such as shunts, thrombosis, or collaterals; secondary parenchymal findings such as diffuse or focal abnormal enhancement patterns; and parenchymal lesions such as regenerative nodules. This article discusses and illustrates vascular disorders of the liver that may be encountered in the pediatric population. These include (a) normal vascular variants; (b) congenital anomalies (preduodenal portal vein and infradiaphragmatic total anomalous pulmonary venous return); (c) acquired thromboses (extrahepatic portal venous thrombosis); (d) inflammatory vascular conditions, which can result in hepatic artery aneurysms or pseudoaneurysms; (e) hepatic venous outflow disorders (veno-occlusive disease); and shunt lesions. Liver transplantation and associated vascular complications are a large topic and will not be reviewed in this article. Knowledge of the vascular and parenchymal changes seen with these entities can aid imaging diagnosis and guide appropriate management. ©

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Section: Arterioportal Shuntsmentioning

confidence: 99%

Vascular Anomalies of the Pediatric Liver

Albers

Khanna

2019

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“…The ductus venosus typically closes within hours of birth, but are generally solitary connections (79). On gray-scale and color Doppler US images, APF manifests as an enlarged hepatic artery and dilatation of the portal vein segment involving the fistula, with pulsatile or arterialized hepatofugal portal vein flow (65,77) (Fig 15). If severe, there may also be increased flow and pulsatility of the hepatic veins.…”

Section: State Of the Art: Hepatic Imaging In Neonates And Young Infantsmentioning

confidence: 99%

“…Contrastenhanced MR imaging can be used to confirm the findings. Transcatheter embolization is the preferred method of treatment (77).…”

Section: State Of the Art: Hepatic Imaging In Neonates And Young Infantsmentioning

confidence: 99%

“…Congenital arterioportal fistula (APF) is an intrahepatic connection between the hepatic artery and portal venous system, without systemic venous communication and with no known acquired cause. It may cause portal hypertension and manifest in the newborn period with failure to thrive, abdominal distention, splenomegaly, gastrointestinal bleeding, and diarrhea (77,78). Whereas AVM contains a complex vascular nidus, APFs…”

Section: Arterioportal Fistulamentioning

confidence: 99%

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Hepatic Imaging in Neonates and Young Infants: State of the Art

et al. 2017

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Neonatal liver disease is an important source of morbidity in the pediatric population. The manifestation of liver disease in young infants may be different than in older patients, and there are a number of diagnoses that are unique to this age group. Familiarity with these entities is important as imaging plays a key role in the diagnostic workup, and prompt diagnosis is necessary to prevent complications. This article reviews the spectrum of liver pathologies that can manifest in the first 6 months of life and is intended to educate the general radiologist who may be faced with interpretation of neonatal liver imaging. Categories of disease that will be reviewed include cholestatic diseases, tumors, vascular anomalies, and acquired diseases. The authors will also review optimization of ultrasonography (US) and magnetic resonance imaging of the liver and present a systematic method for interpretation of neonatal liver US findings in the context of clinical and laboratory findings. RSNA, 2017.

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“…Ultrasonography is now widely used to investigate IAPF because it is convenient, reliable, economical and noninvasive in detecting blood flow from small targets in all directions in the liver [26][27][28] . Even some small asymptomatic arterioportal fistulas that are easily overlooked can be detected by ultrasound.…”

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confidence: 99%

Portal hypertension induced by congenital hepatic arterioportal fistula: Report of four clinical cases and review of the literature

Zhang

Weng

Dong

et al. 2015

WJG

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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.

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

Abstract: 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.

Cited by 16 publications

References 19 publications

Vascular Anomalies of the Pediatric Liver

Vascular Anomalies of the Pediatric Liver

Hepatic Imaging in Neonates and Young Infants: State of the Art

Portal hypertension induced by congenital hepatic arterioportal fistula: Report of four clinical cases and review of the literature

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