Congenital Portosystemic Shunt: Our Experience

Timpanaro, Tiziana; Passanisi, Stefano; Sauna, Alessandra; Trombatore, Claudia; Pennisi, Monica; Petrillo, Giuseppe; Smilari, Pierluigi; Greco, Filippo

doi:10.1155/2015/691618

Cited by 21 publications

(39 citation statements)

References 20 publications

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“…The extremely hypoplastic portal veins distal to the shunt are better visualized by catheter angiography than conventional CT angiography; catheter angiography can distinguish a true Type I shunt having absolute lack of opacification of intrahepatic portal branches from a Type II shunt with very small portal vein branches. 5 …”

Section: Discussionmentioning

confidence: 99%

Role of ultrasonography in early diagnosis of congenital extrahepatic portosystemic shunt

Sadat¹

2016

BJR|case reports

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A 1-year-old male presented to our unit for ultrasonography of the abdomen with a history of fever and diarrhoea for 2 days. The clinical examination was normal. Ultrasound of the abdomen showed a small portal vein and splenomesenteric venous shunt into the left renal vein that was consistent with a Type II congenital extrahepatic portosystemic shunt (CEPS). Doppler sonography confirmed the flow towards the left renal vein. No abnormal findings were observed in the liver and rest of the abdominal organs. CEPS is an uncommon anomaly in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt. It is usually diagnosed incidentally during investigation for other causes. Morgan and Superina (Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994; 29: 1239–41) proposed a classification for these shunts in 1994 that divided these anomalies into two types. In Type I CEPS, the intrahepatic portal venous supply is absent, whereas in Type II, the intrahepatic portal venous supply is preserved. This case highlights the role of ultrasonography in the early diagnosis of CEPS.

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Section: Discussionmentioning

confidence: 99%

Role of ultrasonography in early diagnosis of congenital extrahepatic portosystemic shunt

Sadat¹

2016

BJR|case reports

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“…Congenital portosystemic shunts (CPSS) are a rare vascular consequence from the atypical formation of the splanchnic venous system, in which the portal blood drains entirely or partially into a systemic vein 1 2. They are the result of embryogenetic vascular alterations or the persistence of the fetal circulation elements, especially those related to the ductus venosus 3. The first description of CPSS was given by the London surgeon John Abernethy in 1793, known as the Abernethy malformation.…”

Section: Introductionmentioning

confidence: 99%

Abernethy malformation and hepatocellular carcinoma: a serious consequence of a rare disease

2020

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Congenital portosystemic shunts (CPSS) are a rare vascular consequence of embryogenetic vascular alterations or the persistence of the fetal circulation elements, first described by John Abernethy in 1793 and classified by Morgan and Superina, into complete and partial portosystemic shunts. Its prevalence to this day has not been defined. We present a patient series of a 44-year-old and 47-year-old man and woman, with this rare congenital malformation and underlining hepatocellular carcinoma (HCC) treatment strategies. Over half of the individuals with CPSS have benign or malignant liver tumours, ranging from nodular regenerative hyperplasia, focal nodular hyperplasia, adenomas, HCC and hepatoblastomas. Additionally, it is known that half of individuals with Abernethy malformation type Ib will develop one or multiple types of tumours. There seems to be a direct association with tumorigenesis and CPSS, which is the primary consequence of absent portal flow. Surgery is the treatment of choice, either as a curative resection or orthotopic liver transplantation if recommended as per the criteria, in which replacing the hepatic parenchyma in the setting of an Abernathy malformation will correct the underlining hyper-arterialisation.

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Section: Introductionmentioning

confidence: 99%

“…Congenital portosystemic shunts (PSS) are rare vascular abnormalities, well described in cats, dogs and humans, which result from the abnormal development of the hepatic vascular network during embryogenesis . PSS have been classified into intra‐ and extra‐hepatic, depending on whether the connection between the portal venous system and the systemic circulation is located inside or outside the liver, respectively . In either case, mesenteric blood bypasses the liver and is drained directly to the systemic circulation, hampering hepatic nutrition, development and function, and resulting in numerous ensuing complications, such as liver lesions, cardiac anomalies or hepatic encephalopathy (HE) (see reviews).…”

Section: Introductionmentioning

confidence: 99%

Non‐invasive diagnosis and metabolic consequences of congenital portosystemic shunts in C57BL/6 J mice

Soares

Lei

2017

NMR in Biomedicine

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This study demonstrates the suitability of magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) for the imaging of congenital portosystemic shunts (PSS) in mice, a vascular abnormality in which mesenteric blood bypasses the liver and is instead drained directly to the systemic circulation. The non-invasive diagnosis performed in tandem with other experimental assessments permits further characterization of liver, whole-body and brain metabolic defects associated with PSS. Magnetic resonance measurements were performed in a 26-cm, horizontal-bore, 14.1-T magnet. MRA was obtained with a three-dimensional gradient echo sequence (GRE; in-plane resolution, 234 × 250 × 234 μm ) using a birdcage coil. Two-dimensional GRE MRI with high spatial resolution (in-plane resolution, 100 × 130 μm ; slices, 30 × 0.3 mm) was performed using a surface coil. Brain- (dorsal hippocampus) and liver-localized H magnetic resonance spectroscopy (MRS) was also performed with the surface coil. Whole-body metabolic status was evaluated with an oral glucose tolerance test (OGTT). Both MRA and anatomical MRI allowed the identification of hepatic vessels and the diagnosis of PSS in mice. The incidence of PSS was about 10%. Hepatic lipid content was higher in PSS than in control mice (5.1 ± 2.8% versus 1.8 ± 0.6%, p = 0.02). PSS mice had higher brain glutamine concentration than controls (7.3 ± 1.0 μmol/g versus 2.7 ± 0.6 μmol/g, p< 0.0001) and, conversely, lower myo-inositol (4.2 ± 0.6 μmol/g versus 6.0 ± 0.4 μmol/g, p < 0.0001), taurine (9.7 ± 1.2 μmol/g versus 11.0 ± 0.4 μmol/g, p < 0.01) and total choline (0.9 ± 0.1 μmol/g versus 1.2 ± 0.1 μmol/g, p < 0.001) concentrations. Fasting blood glucose and plasma insulin were lower in PSS than in control mice (4.7 ± 0.5mM versus 8.8 ± 0.6mM, p < 0.0001; and 0.04 ± 0.03 μg/L versus 0.3 ± 0.2 μg/L, p = 0.02, respectively). Glucose clearance during OGTT was delayed and less efficient in PSS mice than in controls. Thus, given the non-negligible incidence of PSS in inbred mice, the undiagnosed presence of PSS will, importantly, have an impact on experimental outcomes, notably in studies addressing brain, liver or whole-body metabolism.

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Congenital Portosystemic Shunt: Our Experience

Cited by 21 publications

References 20 publications

Role of ultrasonography in early diagnosis of congenital extrahepatic portosystemic shunt

Role of ultrasonography in early diagnosis of congenital extrahepatic portosystemic shunt

Abernethy malformation and hepatocellular carcinoma: a serious consequence of a rare disease

Non‐invasive diagnosis and metabolic consequences of congenital portosystemic shunts in C57BL/6 J mice

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