Abernethy malformation
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- updated 6 Dec 2022:
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Abernethy malformations are rare vascular anomalies of the splanchnic venous system. They consist of congenital portosystemic shunts and resultresulting from persistence of the embryonic vessels' persistence.
Epidemiology
Type I malformations are thought to occur only in females, while type II havehas a male predominance 1.
Pathology
Subtypes
There are twoTwo main types of Abernethy malformations that have been described 6:
type I: end-to-side shunt; 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
-
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.
-<p><strong>Abernethy malformations</strong> are rare vascular anomalies of the splanchnic venous system. They consist of <a href="/articles/congenital-portosystemic-shunt">congenital portosystemic shunts</a> and result from persistence of the embryonic vessels. </p><h4>Epidemiology</h4><p>Type I malformations are thought to occur only in females, while type II have a male predominance <sup>1</sup>.</p><h4>Pathology</h4><h5>Subtypes</h5><p>There are two main types of Abernethy malformations that have been described <sup>6</sup>:</p><ul>-<li>-<strong>type I:</strong> end-to-side shunt; superior mesenteric and splenic vein drain separately into inferior vena cava</li>-<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>- +<p><strong>Abernethy malformations</strong> are rare vascular anomalies of the splanchnic venous system. They consist of <a href="/articles/congenital-portosystemic-shunt">congenital portosystemic shunts</a> resulting from the embryonic vessels' persistence. </p><h4>Epidemiology</h4><p>Type I malformations are thought to occur only in females, while type II has a male predominance <sup>1</sup>.</p><h4>Pathology</h4><h5>Subtypes</h5><p>Two main types of Abernethy malformations have been described <sup>6</sup>:</p><ul>
- +<li><p><strong>type I</strong>: end-to-side shunt; superior mesenteric and splenic vein drain separately into inferior vena cava</p></li>
- +<li><p><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</p></li>
-<li>-<strong>type Ia:</strong> separate drainage of the superior mesenteric vein and splenic vein into systemic veins</li>-<li>-<strong>type Ib:</strong> 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)</li>- +<li><p><strong>type Ia</strong>: separate drainage of the superior mesenteric vein and splenic vein into systemic veins</p></li>
- +<li><p><strong>type Ib</strong>: 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)</p></li>
- +<li><p><a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a>: from portosystemic shunting</p></li>
-<a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a>: from portosystemic shunting</li>-<li>hepatic mass lesions <ul>-<li>-<a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia</a>: thought to generate due to the absence of the portal vein</li>-<li><a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a></li>-<li><a href="/articles/hepatoblastoma">hepatoblastoma</a></li>- +<p>hepatic mass lesions </p>
- +<ul>
- +<li><p><a href="/articles/focal-nodular-hyperplasia">focal nodular hyperplasia</a>: thought to generate due to the absence of the portal vein</p></li>
- +<li><p><a href="/articles/hepatocellular-carcinoma">hepatocellular carcinoma</a></p></li>
- +<li><p><a href="/articles/hepatoblastoma">hepatoblastoma</a></p></li>
- +<li><p><a href="/articles/hepatopulmonary-syndrome">hepatopulmonary syndrome</a> <sup>2</sup></p></li>
-<a href="/articles/hepatopulmonary-syndrome">hepatopulmonary syndrome</a> <sup>2</sup>-</li>-<li>-<a href="/articles/pulmonary-arteriovenous-fistulas">pulmonary arteriovenous fistulae</a><ul>-<li>thought to arise due to hyperammonaemia</li>-<li>may lead to systemic emboli</li>- +<p><a href="/articles/pulmonary-arteriovenous-fistulas">pulmonary arteriovenous fistulae</a></p>
- +<ul>
- +<li><p>thought to arise due to hyperammonaemia</p></li>
- +<li><p>may lead to systemic emboli</p></li>
-<li>other congenital abnormalities, particularly associated with type I <sup>1</sup> :<ul>-<li><a href="/articles/congenital-cardiovascular-anomalies">congenital cardiac anomalies</a></li>-<li><a href="/articles/biliary-atresia">biliary atresia</a></li>-<li><a href="/articles/polysplenia-syndrome-1">polysplenia</a></li>- +<li>
- +<p>other congenital abnormalities, particularly associated with type I <sup>1</sup>:</p>
- +<ul>
- +<li><p><a href="/articles/congenital-cardiovascular-anomalies">congenital cardiac anomalies</a></p></li>
- +<li><p><a href="/articles/biliary-atresia">biliary atresia</a></p></li>
- +<li><p><a href="/articles/polysplenia-syndrome-1">polysplenia</a></p></li>
References changed:
- 1. Howard E & Davenport M. Congenital Extrahepatic Portocaval Shunts—The Abernethy Malformation. J Pediatr Surg. 1997;32(3):494-7. <a href="https://doi.org/10.1016/s0022-3468(97)90614-x">doi:10.1016/s0022-3468(97)90614-x</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9094026">Pubmed</a>
- 2. Alvarez A, Ribeiro A, Hessel G, Baracat J, Ribeiro J. Abernethy Malformation: One of the Etiologies of Hepatopulmonary Syndrome. Pediatr Pulmonol. 2002;34(5):391-4. <a href="https://doi.org/10.1002/ppul.10182">doi:10.1002/ppul.10182</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12357487">Pubmed</a>
- 3. Gallego C, Miralles M, Marín C, Muyor P, González G, García-Hidalgo E. Congenital Hepatic Shunts. Radiographics. 2004;24(3):755-72. <a href="https://doi.org/10.1148/rg.243035046">doi:10.1148/rg.243035046</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15143226">Pubmed</a>
- 4. Chandrashekhara S, Bhalla A, Gupta A, Vikash C, Kabra S. Abernethy Malformation with Portal Vein Aneurysm in a Child. J Indian Assoc Pediatr Surg. 2011;16(1):21-3. <a href="https://doi.org/10.4103/0971-9261.74517">doi:10.4103/0971-9261.74517</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/21430844">Pubmed</a>
- 6. Morgan G & Superina R. Congenital Absence of the Portal Vein: Two Cases and a Proposed Classification System for Portasystemic Vascular Anomalies. J Pediatr Surg. 1994;29(9):1239-41. <a href="https://doi.org/10.1016/0022-3468(94)90812-5">doi:10.1016/0022-3468(94)90812-5</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/7807356">Pubmed</a>
- 7. Ringe K, Schirg E, Melter M et al. [Congenital Absence of the Portal Vein (CAPV). Two Cases of Abernethy Malformation Type 1 and Review of the Literature]. Radiologe. 2008;48(5):493-502. <a href="https://doi.org/10.1007/s00117-007-1561-1">doi:10.1007/s00117-007-1561-1</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/17879079">Pubmed</a>
- 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>
- 1. Howard E & Davenport M. Congenital Extrahepatic Portocaval Shunts--The Abernethy Malformation. J Pediatr Surg. 1997;32(3):494-7. <a href="https://doi.org/10.1016/s0022-3468(97)90614-x">doi:10.1016/s0022-3468(97)90614-x</a>
- 2. Alvarez A, Ribeiro A, Hessel G, Baracat J, Ribeiro J. Abernethy Malformation: One of the Etiologies of Hepatopulmonary Syndrome. Pediatr Pulmonol. 2002;34(5):391-394. <a href="https://doi.org/10.1002/ppul.10182">doi:10.1002/ppul.10182</a>
- 3. Gallego C, Miralles M, Marín C, Muyor P, González G, García-Hidalgo E. Congenital Hepatic Shunts. Radiographics. 2004;24(3):755-772. <a href="https://doi.org/10.1148/rg.243035046">doi:10.1148/rg.243035046</a>
- 4. Chandrashekhara SH, Bhalla AS, Gupta AK et-al. Abernethy malformation with portal vein aneurysm in a child. J Indian Assoc Pediatr Surg. 2011;16 (1): 21-3. <a href="http://dx.doi.org/10.4103/0971-9261.74517">doi:10.4103/0971-9261.74517</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3047770">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/21430844">Pubmed citation</a><div class="ref_v2"></div>
- 6. Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J. Pediatr. Surg. 1994;29 (9): 1239-41. <a href="http://linkinghub.elsevier.com/retrieve/pii/0022-3468(94)90812-5">J. Pediatr. Surg. (link)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/7807356">Pubmed citation</a><div class="ref_v2"></div>
- 7. Ringe K, Schirg E, Melter M et-al. [Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature]. Radiologe. 2008;48 (5): 493-502. <a href="http://dx.doi.org/10.1007/s00117-007-1561-1">doi:10.1007/s00117-007-1561-1</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17879079">Pubmed citation</a><div class="ref_v2"></div>
- 8. 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>
- 9. Pathak A, Agarwal N, Mandliya J, Gehlot P, Dhaneria M. Abernethy malformation: a case report. (2012) BMC pediatrics. 12: 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> <span class="ref_v4"></span>
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