The Abernethy malformation-myriad imaging manifestations of a single entity

Ghuman, Samarjit Singh; Gupta, Saumya; Buxi, T. B. S.; Rawat, Kishan Singh; Yadav, Anurag; Mehta, Naimish; Sud, Seema

doi:10.4103/0971-3026.190420

Cited by 35 publications

(28 citation statements)

References 53 publications

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“…Congenital portosystemic shunts are classified into 2 major categories: total shunting with complete absence of intrahepatic portal venous flow (extrahepatic) – type I; type 1 Abernethy malformation can be further divided into subtypes 1a and 1b. In subtype 1a, the superior mesenteric vein and the splenic vein do not connect, whereas in subtype 1b, the superior mesenteric vein and splenic vein connect to form a portal vein, which then drains into a systemic vein, as in our illustrated case; In type 2 Abernethy malformation, the portal vein is intact, but a side-to-side shunting with the inferior vena cava (intrahepatic) [2] .…”

Section: Case Reportmentioning

confidence: 69%

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An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia

Yam¹,

Cheung²,

Sim³

et al. 2020

Radiology Case Reports

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Congenital portosystemic venous shunts are developmental anomalies. They represent portal communication with the systemic circulation. The pathogenesis is linked to the complexity of the embryological development of the inferior vena cava and portal vein. We reported a case of an asymptomatic 14-year-old Chinese adolescent girl in Hong Kong with a confirmed congenital portosystemic shunt type 1b. The condition can be diagnosed using contrast-enhanced CT scans and MRIs. Early recognition of the condition is important due to elevated risks of developing hepatocellular tumours. Liver transplantation may be considered curative.

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Section: Case Reportmentioning

confidence: 69%

“…Most adult patients with portosystemic shunts present with bleeding or hepatic encephalopathy as first manifestations [1] . It has been hypothesized that patients become more symptomatic with advancing age as the tolerance for high ammonia levels diminishes [2] . Ultrasound may reveal drainage of portal vein into inferior vena cava.…”

Section: Introductionmentioning

confidence: 99%

An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia

Yam¹,

Cheung²,

Sim³

et al. 2020

Radiology Case Reports

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“…For the extrahepatic ones the shunt connects the portal trunk or its tributaries to a vessel belonging to the systemic circulation. [4] Type I portosystemic shunts are characterized by the complete absence of the portal vein, the portal blood draining entirely into the inferior vena cava. [5] Type I is further divided into IA—for which the splenic vein and the mesenteric vein separately drain into the inferior vena cava, and IB for which the portal vein itself drains into the inferior vena cava.…”

Section: Discussionmentioning

confidence: 99%

“…[5] Type I is further divided into IA—for which the splenic vein and the mesenteric vein separately drain into the inferior vena cava, and IB for which the portal vein itself drains into the inferior vena cava. [4] For type II portosystemic shunts the portal vein is unharmed, but there is an extrahepatic connection between the portal vein and the inferior vena cava. [5]…”

Section: Discussionmentioning

confidence: 99%

“…Congenital portosystemic shunt, also known as Abernethy malformation, is a rare vascular malformation in which the portal blood drains towards the systemic circulation eluding the liver. [1,3,4] These shunts arise through disturbances that intervene in the embryonic development of the splanchnic circulation, or when elements that are specific to the fetal circulation (especially those regarding the ductus venosus) persist beyond the intrauterine life. [1,3,5]…”

Section: Introductionmentioning

confidence: 99%

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A case report on an incidental discovery of congenital portosystemic shunt

Păcurar

Dijmărescu

et al. 2019

Medicine

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Rationale: Congenital portosystemic shunt (Abernethy malformation) is a rare entity causing the portal blood to drain directly into the systemic circulation, eluding the liver. These shunts arise through disturbances in the embryonic development. Patient concerns: A 9-year-old male patient was referred to our department for further evaluation of a vascular malformation which was diagnosed in another facility when the patient was 2 years old, following a routine abdominal ultrasound. The patient had no complaints and the physical examination was normal at all times. Diagnosis: Laboratory tests and esogastroduodenoscopy were normal. The abdominal ultrasound showed a side-to-side shunt between a short portal trunk and the inferior vena cava. A hepatic mass suggestive for focal noduar hyperplasia was seen in the left liver lobe. Abdominal angio-computed tomography (angio-CT) was performed and the ultrasonographic anomalies were confirmed. Multiple other vascular malformations were diagnosed—hepatic artery emerging from the superior mesenteric artery, with early division; hepatic veins forming a short common trunk before draining into the inferior vena cava; supranumerary right renal artery emerging from the aorta, tributary for the upper renal pole. Ecocardiography showed left superior vena cava persistence. The final diagnosis was Abernathy malformation type IB. In the meantime the patient was diagnosed with allergic asthma. Interventions: No surgical cure was pursued because the malformation was an incidental discovery. Outcomes: The patient was followed-up closely from the final diagnosis (when he was 9 years old) to present (he is currently 10 years old) with no change in his status—he remained asymptomatic. Lessons: Angio-CT should be the performed whenever a vascular malformation is suspected in order to establish a correct diagnosis, because portosystemic shunts carry a high risk of severe complications. Knowing that patients with portosystemic shunts may have pulmonary hypertension, respiratory complaints should be carefully evaluated—in this particular case, even though the most probable cause for the respiratory symptoms was pulmonary hypertension, it was ruled out by cardiac ultrasonography and further investigations confirmed the diagnosis of allergic asthma.

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Letter to the Editor: Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): A New Variant

et al. 2021

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We read with interest the article by Baiges et al., (1) which shed light on the rare entity of the Abernethy malformation and gives a working algorithm for extrahepatic shunts. We commend all the researchers for the effort to put together a large series and have a follow-up up to the age of 30 years, missed by previous series.The researchers have shown how shunt closure can be considered in therapeutic and prophylactic settings to reduce complications. However, the researchers have not given us details on the precise anatomy of the shunts they saw in the series. This is important in planning an intervention.The Abernethy malformation is commonly described as the shunting of splanchnic blood into the systemic venous network by abnormal communications. The Abernethy malformation is anatomically classified as a type 1 (Ia)-splenic and superior mesenteric vein draining separately in the inferior vena cava (IVC) and (Ib) draining though a common trunk. Type 2 is classified as a shunt from the portal vein (PV) to IVC. (1,2) The Kobayashi classification describes types A, B, and C of portal blood flowing directly into the IVC, renal vein, and iliac vein, respectively. Blanc et al. classified the congenital extrahepatic portosystemic shunt considering the starting as well as the end point of the shunt. (2,3) Based on our clinical observations, we believe the Abernathy malformation can also result in, portal blood being shunted directly into the right atrium (RA), although has not been described in any of the previous classifications. Hence, we believe this is a description of a porta-atrial shunt. This should perhaps be incorporated into the current classifications, and also also provides an opportunity to further understand hepatic vascular embryology, malformations, and their clinical spectrum. Moreover, such large shunts with direct communication with the RA may not be amenable to the usual radiological interventions.

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The Abernethy malformation-myriad imaging manifestations of a single entity

Cited by 35 publications

References 53 publications

An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia

An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia

A case report on an incidental discovery of congenital portosystemic shunt

Letter to the Editor: Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): A New Variant

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