Because abernethy malformation is a very rare condition, experience with liver transplantation is limited. The purpose of this study was to demonstrate manifestations of. Abernethy malformation is defined as congenital diversion of portal blood away from the liver by either endtoside or sidetoside shunt. Letters to the editor abernethy malformation crosssectional imaging or angiography performed before shunt closure. The abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. Abernethy type ii malformation occurs more frequently in adulthood and is more common in male patients, is associated with liver dysfunction and encephalopathy. Abernethy malformation, also called as congenital extrahepatic portosystemic. Abernethy malformation type ib ceps discussion abernethy malformation is a congenital extra hepatic portosystemic shunt ceps that develops between the portomesenteric vasculature and the systemic veins. A 17yearold girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone kawashima operation 10 years back. It comprises a group of vascular anomalies of the splanchnic venous system and is characterized.
Abernethy malformation radiology reference article. First reported in 1793, abernethy malformations are congenital extrahepatic pss ceps. Approximately 15% of ceps cases result in hepatic encephalopathy. Single stage endovascular treatment of a type 2 abernethy.
The area containing the village of nethy bridge formerly known as abernethy, highland, within the cairngorms national park. Type ii abernethy malformation lurie childrens hospital. Congenital extrahepatic portosystemic shunts abernethy. Only 11 cases have been reported in the literature so far, including this one and two of our own patients reported recently. The abernethy malformationmyriad imaging manifestations of a. Abernethy malformation chen guo, yumin zhong, qian wang, liwei hu, xiaohong gu, hong shao, wei wu, jianfeng cao and haisheng qiu abstract background. Only 80 cases have been described since the initial discovery, with most patients facebook page is dedicated to abernethy malformations. Abernethy malformations are a rare collection of congenital hepatic. Morgan and superina classified congenital extrahepatic portosystemic shunt cepsh into two types see table table1. Imaging examination is an important method for the diagnosis. The abernethy malformation, as the name suggests, was first described by abernethy and consists of a congenital extrahepatic portosystemic shunt ceps. Abernethy and kincardine, the former ecclesiastical and. Abernethy malformation is an abnormal portosystemic shunt that was first described in 1793 as a postmortem finding1.
Alomari, md, msc, portocaval shunts at imaging is paramount for division of vascular and interventional radiol proper classification and treatment. Discussion first reported in 1793 1, abernethy malformations are congenital extrahepatic pss ceps. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein, results from the malformation of the splanchnic venous system and was first described in 1793 by john abernethy. It is commonly associated with multiple congenital anomalies. Kylans journey with abernethy malformation global genes. Only 80 cases have been described since the initial discovery, with most patients abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end. Congenital extrahepatic portosystemic shunt ceps or abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt.
Diagnostic accuracy of multislice computed tomography in. Type i malformations are thought to occur only in females, while type ii have a male predominance 1 pathology subtypes. Children with abernathy malformation can develop hepatopulmonary. Our, now almost 17 month old, son was born with congenital heart defects or chd and at two weeks old we found out that he had a complex anatomy, but up until recently we didnt know just how complex it truly was. There are two main types of abernethy malformations that have been described initially devised by g morgan and r superina 6 type i. There are no current published guidelines for followup of ceps. Pdf abernethy malformation, also known as congenital extrahepatic portosystemic shunts ceps is a rare clinical. The significance of continued monitoring and longterm followup is clear.
Abernethy malformation in an adult with intermittent. Abernethy malformation type ii and concurrent nodular. Patients may develop hepatic encephalopathy he, pulmonary hypertension paht, or liver tumors, among other complications. Congenital extrahepatic portosystemic shunt abernethy. Abernethy nbr railway station, a former railway station in this village. We present a 33year old female presenting with a 20year history of intermittent hematochezia with multiple blood transfusion. It provides information about am and the opportunity to.
Exploiting phenotypic plasticity for the treatment of. The patient remains free of hps stigmata after 2 years. Rex shunt as a treatment of type ii abernethy malformation with severe dysplasia of portal vein annals of vascular surgery, vol. Pdf the abernethy malformationmyriad imaging manifestations. Abernethy malformation is defined as congenital diversion of portal blood away from the liver by either endtoside or sidetoside shunt definition 3. She had significant desaturation because of a large abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein.
Although overall a rare malformation, congenital extrahepatic ps shunts are being diagnosed more frequently with the advances in imaging techniques, especially in the perinatal periods following directed screening exercises. The malformation is further classified on the presence or absence mesenteric blood flow to the hepatic parenchyma. Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. Abernethy malformation is a very rare congenital vascular malformation defined by diversion. Abernethy malformation type ii and concurrent nodular hyperplasia in a rare female case. Empirical analysis of the vascular anatomy of wibke uller, md, ahmad i. Lukasz kwapisz, md, malcolm m wells, md frcpc, and bandar aljudaibi, md frcpc. It is also an extremely rare cause of chronic lower gastrointestinal bleeding. John abernethy first reported congenital absence of portal vein and congenital mesentericocaval shunt in 1793. Althoughcongenital extrahepatic portosystemic shunts are increasingly suspected and. Abernethy ps malformation in adulthood, is an even rarer occurrence and may represent a milder disease variant, especially because most of these are. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. The patient totally remains free of hps stigmata 2 years later.
Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease abernethy malformation. Abernethy malformation is a rare congenital abnormality. We have also described various associated anomalies. This is a retrospective analysis of data from nine. Abernethy malformation or congenital extrahepatic portosystemic shunt ceps as the. First, the legend for the t2weighted magnetic resonance mr image shown in figure 5b describes an endtoside congenital anastomosis between the portal vein and ivc inferior vena cava, a finding compatible with type 2 abernethy malformation. Nevertheless, we believe that the large shunt shown on the image is completely intrahepatic.
Abernethy forest, a forest and national nature reserve within the area. Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. Abernethy definition and meaning collins english dictionary. The abernethy malformation congenital extrahepatic portosystemic shunt ceps is a rare entity that most often presents in the pediatric population but may go unidentified until adulthood, as in this case.
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