Abernethy malformation
Updates to Article Attributes
Abernethy malformations are rare vascular anomalies of the splanchnic venous system. They consist of congenital portosystemic shunts and result from persistence of the embryonic vessels.
Epidemiology
Type I malformations are thought to occur only in females, while type II have a male predominance 1.
Pathology
Subtypes
There are two main types of Abernethy malformations that have been described (initially devised by G Morgan and R Superina 6):
-
type I: end-to-side
shunt7shunt; superior mesenteric and splenic vein drain separately into inferior vena cava - type II: side-to-side shunts; superior mesenteric vein and splenic vein form a common trunk before draining into the inferior vena cava
In type I shunts (end-to-end), there is a congenital absence of the portal vein with a complete diversion of portal blood into systemic veins (inferior vena cava, renal veins, or iliac veins). These are further subdivided into:
- type Ia: separate drainage of the superior mesenteric vein and splenic vein into systemic veins
- type Ib: superior mesenteric vein and splenic vein join to form a short extra-hepatic portal vein which drains into a systemic vein (inferior vena cava, right atrium or iliac veins)
In type II shunts (side-to-side), there is a hypoplastic portal vein with portal blood diversion into the inferior vena cava through a side-to-side, extrahepatic communication.
Associations
- hepatic encephalopathy: from portosystemic shunting
- hepatic mass lesions
- focal nodular hyperplasia: thought to generate due to the absence of the portal vein
- hepatocellular carcinoma
- hepatoblastoma
- hepatopulmonary syndrome 2
-
pulmonary arteriovenous fistulae
- thought to arise due to hyperammonaemia
- may lead to systemic emboli
- other congenital abnormalities, particularly associated with type I 1 :
History and etymology
Named after John Abernethy, who gave the first account of an absent portal vein with accompanying congenital mesentericocaval shunt 1,5 in 1793.
-<strong>type I:</strong> end-to-side shunt7</li>- +<strong>type I:</strong> end-to-side shunt; superior mesenteric and splenic vein drain separately into inferior vena cava</li>
-<strong>type II:</strong> side-to-side shunts</li>- +<strong>type II:</strong> side-to-side shunts; superior mesenteric vein and splenic vein form a common trunk before draining into the inferior vena cava</li>
References changed:
- 8. Uller W & Alomari A. Abernethy Malformation. Radiographics. 2015;35(5):1623. <a href="https://doi.org/10.1148/rg.2015150089">doi:10.1148/rg.2015150089</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26371585">Pubmed</a>
- 9. Pathak A, Agarwal N, Mandliya J, Gehlot P, Dhaneria M. Abernethy Malformation: A Case Report. BMC Pediatr. 2012;12(1):57. <a href="https://doi.org/10.1186/1471-2431-12-57">doi:10.1186/1471-2431-12-57</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22642663">Pubmed</a>
- Wibke Uller, Ahmad I. Alomari. Abernethy Malformation. (2015) RadioGraphics. 35 (5): 1623. <a href="https://doi.org/10.1148/rg.2015150089">doi:10.1148/rg.2015150089</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26371585">Pubmed</a> <span class="ref_v4"></span>