Nevertheless, we believe that the large shunt shown on the image is completely intrahepatic. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. In abernethy malformation there is complete or partial shunting of hepatic venous supply of the portomesenteric blood, which drains into a systemic vein. Jun 01, 2014 abernethy malformation is defined as congenital diversion of portal blood away from the liver by either endtoside or sidetoside shunt definition 3. The purpose of this study was to demonstrate manifestations of. The term abernethy malformation was coined by howard and was classified into type 1 and type 2 by morgan, depending on the presence of intrahepatic pv. Download fulltext pdf download fulltext pdf abernethy malformation. Imaging examination is an important method for the diagnosis. A 45yearold man with incidentally discovered, unresectable hcc were treated with tace to the left hepatic lobe and tare to the right hepatic lobe. Patients may develop hepatic encephalopathy he, pulmonary hypertension paht, or liver tumors, among other complications. Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. In case you want to submit further pictures, please add these radiograph, ultrasound, ct or mr images and schematic drawing of the developmental process if applicable by clicking on the symbols within the boxes below.
Buddchiari syndrome bcs is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Diagnostic accuracy of multislice computed tomography in. Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Abernethy malformation or congenital extrahepatic portosystemic shunt ceps is an uncommon entity and has been classified into two types. The abernethy malformationmyriad imaging manifestations of a. Abernethy malformation with portal vein aneurysm in a child. Abernethy malformation and hepatocellular carcinoma. Capv abernethy malformation was first reported by john abernethy in 1793. The abernethy malformation is characterised by congenital extrahepatic portosystemic shunts and is divided.
There are no current published guidelines for followup of ceps. It was reported for the first time in 1793 by john abernethy. Abernethy malformation associated with carolis syndrome in a. Account of two instances of uncommon formation in the viscera of the human body.
The abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end. Alagille syndrome is an autosomal dominant multisystem disorder, usually involving hepatic, cardiac, ophthalmic, skeletal, or renal dysplasia. His pv flowed directly into the confluence of the cclmhv and the ivc. Children with type 2 abernethy who had had been operated between 20 and 2017 were included in the study. Abernethy malformation, also known as congenital extrahepatic portosystemic shunts ceps is a rare clinical entity and manifests with different clinical symptoms. Nowadays, with the evolution of medical imaging, diagnosis can be made more easily, but management of patients with an abernethy malformation is still open for discussion. This is a retrospective analysis of data from nine patients with abernethy malformation at a.
Abernethy malformation radiology reference article. Abernethy malformation is an extremely rare anomaly of the splanchnic venous system. Gallego c, miralles m, marin c, muyor p, gonzalez g, garciahidalgo e. Abernethy malformation is a very rare congenital vascular malformation. It is frequently associated with other congenital abnormalities, including the absence of a portal vein andor congenital mesentericocaval shunt, cardiac andor pulmonary malformation. Abernethy malformation is a rare congenital abnormality. An association with factor vii deficiency has not been reported before. Clinically, the signs and symptoms of the disease include. It is a rare vascular malformation in which the portal vein drains. Carolis disease combined with congenital hepatic fibrosis andor renal cystic. Abernethy malformation congenital extrahepatic portosystemic. The abernethy malformation, as the name suggests, was first described by abernethy and consists of a congenital extrahepatic portosystemic shunt ceps. The abernethy malformationmyriad imaging manifestations. A curious case of refractory hematochezia gastroenterology.
Multidetector computed tomography mdct is a fast and. Congenital extrahepatic portosystemic shunt genetic and. Abernethy malformation is a very rare congenital vascular malformation defined by diversion. Ceps are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Type i abernethy shunt discussion abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is a rare abnormality of the splanchnic venous system whereby blood from the mesentery and spleen bypass the liver through an anomalous shunt via the portal vein or equivalent and the ivc. The abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. Pdf abernethy malformation, also known as congenital extrahepatic portosystemic shunts ceps is a rare clinical entity and. There are two main types of abernethy malformations that have been described initially devised by g morgan and r superina 6 type i. The diagnosis of type 1b abernethy malformation was made.
In this case study, we describe a 34yearold caucasian man who presented with a large. Abernethy malformation a rare but important diagnosis. Congenital extrahepatic portosystemic shunts abernethy. The abernethy malformation is a rare anomaly with a widely variable clinical presentation. A case report article pdf available in journal of medical case reports 61.
In 2003, only 31 cases of abernethy malformation, mostly in children 80% were detected in the world. Download fulltext pdf download fulltext pdf management strategy after diagnosis of abernethy malformation. Abernethy malformation is a rare splanchnic vascular abnormality characterizing extrahepatic abnormal shunts that is classified into types i and ii. Abernethy malformations are a rare collection of congenital hepatic portosystemic shunts. Type i abernethy malformations are associated with hepatocellular carcinoma and hepatoblastoma 3,5,8,11. Approximately 15% of ceps cases result in hepatic encephalopathy. Significance of ct scan and color doppler duplex ultrasound. It comprises a group of vascular anomalies of the splanchnic venous system and is characterized. 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. Capillary malformationarteriovenous malformation cmavm is an autosomal dominant disorder characterized by cms, often in association with fastflow vascular malformations. Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. 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.
It is frequently associated with other congenital abnormalities, including the absence of a portal vein andor congenital mesentericocaval shunt, cardiac and. 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. Sep 18, 2015 abernethy malformation is a rare congenital vascular abnormality in which the portal vein bypasses the liver and drains directly into the inferior vena cava. Abernethy malformation is defined as congenital diversion of portal blood away from the liver by either endtoside or sidetoside shunt definition 3. It is characterized by a shunt between the portal vein and systemic circulation 1, 2. Fourteen children with abernethy malformation were admitted to our center in china between july 2011 and january 2018. Toxins, that are normally removed by the liver, build up in the blood stream and can lead to symptoms such as decreased oxygen to the brain hypoxia and loss of brain function hepatic encephalopathy. An abernethy malformation is a rare anomaly of the splanchnic venous system characterized by the presence of congenital extrahepatic portosystemic shunts, whereby portal blood bypasses the liver and drains directly into the systemic circulation. Dec 17, 2019 abernethy malformation is a rare congenital abnormality. Abernethy malformation with portal vein aneurysm in a. The first report of living donor liver transplantation for abernethy malformation congenital absence of the portal vein in vietnam. Surgical ligation for the treatment of an unusual presentation of. 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. Pdf download for congenital extrahepatic portosystemic shunt abernethy.
In this study we highlight the significance of the. This case report discusses variant splanchnic and portal. Abernethy malformation is a congenital vascular malformation defined by diversion of portal blood away from the liver. Type i malformations are thought to occur only in females, while type ii have a male predominance 1 pathology subtypes. Pdf the abernethy malformationmyriad imaging manifestations. Pdf abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. The first report of living donor liver transplantation for. The significance of continued monitoring and longterm followup is clear. Radiographics in the marchapril 2015 issue, we read with 2004.
The abernethy malformation is characterised by congenital extrahepatic portosystemic shunts and is divided into two groups according to the type of anastomosis. Congenital extrahepatic portosystemic shunt abernethy. This report details a single stage endovascular technique wherein shunt closure was achieved immediately by placement of an aortic endograft. Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly. This is a retrospective analysis of data from nine. Abernethy malformations are rare vascular anomalies of the splanchnic venous system. Abernethy malformation associated with carolis syndrome. Case based diagnosis training underlying step in embryological development. Type ii abernethy malformation in a patient with primary budd. Jun 28, 2012 the abernethy malformation is a rare anomaly with a widely variable clinical presentation. It provides information about am and the opportunity to in touch with other patients.
Sep 30, 2002 the abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end. This congenital anomaly may present in children or in adults and are often found following abdominal. Figure 1 contrastenhanced coronal magentic resonance image of the abdomen revealing an abnormal shunt between the main portal vein and inferior vena cava arrow. Presentation mode open print download current view. Jun 29, 2014 the morphology suggested a type ib abernethy malformation. Abernethy malformation, also termed congenital portosystemic shunt and. The purpose of this study was to demonstrate manifestations of multislice computed tomography msct in abernethy malformation and its diagnostic accuracy. Pdf management strategy after diagnosis of abernethy. They consist of congenital portosystemic shunts and result from persistence of the embryonic vessels.
Abernethy malformation often, as in the presented case, is detected incidentally while investigating cardiac anomalies or liver dysfunction. Diagnosis is complex and requires good quality imaging methods to identify details in systemic and portal circulation in order to establish diagnostic confirmation and treatment strategy. Type ii abernethy malformation lurie childrens hospital. Abernethy malformation type ii and concurrent nodular.
This congenital anomaly may present in children or in adults and are often found following abdominal imaging that was. Left the splenic and superior mesenteric smv veins draining into a shunt blue arrowheads to the left renal vein that drains into the inferior vena cava ivc. Sep 15, 2015 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. Author links open overlay panel thanh le van a dung dao duc b nam hoang duc b quang vu van. Congenital extrahepatic portosystemic shunt ceps occurs when the blood vessels that go to the liver do not form correctly during fetal development.
In type 1, all portal venous blood is discharged into the inferior vena cava and there is no intrahepatic portal vein. This facebook page is dedicated to abernethy malformations. Empirical analysis of the vascular anatomy of wibke uller, md, ahmad i. Since abernethy malformation can be without symptoms, the number of undiagnosed cases is not known. Only 80 cases have been described since the initial discovery, with most patients case. To study the varied presentations and the outcomes in children with type 2 abernethy malformation following shunt ligation. Alomari, md, msc, portocaval shunts at imaging is paramount for division of vascular and interventional radiol proper classification and treatment.
Fourteen children with abernethy malformation were admitted to our. Management strategy after diagnosis of abernethy malformation. Mar 05, 2019 congenital extrahepatic portosystemic shunt ceps occurs when the blood vessels that go to the liver do not form correctly during fetal development. He was an english anatomist, physiologist, and surgeon. Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is a rare malformation in which the intestinal and splenic venous blood bypasses the liver and drains into systemic veins through a complete or partial shunt. Single stage endovascular treatment of a type 2 abernethy. The constellation of symptoms portosystemic shunt, absence of intraheptic portal vein, benign liver lesions, and congenital cardiac defect is consistent with type i abernethy malformation am. In type 2, the portal vein is partially discharged to the inferior vena cava via sidebyside anastomoses. Letters to the editor abernethy malformation crosssectional imaging or angiography performed before shunt closure. Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Abernethy malformation a rare but important diagnosis prior. Our 19yearold patient is with a type 2 abernethy malformation elected permanent shunt closure following worsening dyspnea. Most of 34 reported cases of ams are diagnosed in juveniles.
This case report discusses variant splanchnic and portal anatomy in the setting of rare. Rex shunt as a treatment of type ii abernethy malformation with severe dysplasia of portal vein annals of vascular surgery, vol. Abernethy malformation is the congenital absence of the portal vein with secondary cavalmesenteric shunt. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Abernethy malformation am is a rare congenital abnormality described by john abernethy in 1793. We describe multidetector computed tomography findings of an incidentally detected abernethy malformation with portal vein aneurysm in a twoandhalfyear old child. Epidemiology type i malformations are thought to occur o. Jul 21, 2019 capv abernethy malformation was first reported by john abernethy in 1793. Aug 16, 2017 abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Abernethy malformation is the congenital absence of the portal vein, resulting in blood flow bypassing the liver to drain directly into the systemic veins, such as the inferior vena cava. Congenital extrahepatic portosystemic shunt is a rare congenital anomaly that was first described by john abernethy in 1793 at autopsy of a 10monthold infant who died of unknown cause. First reported in 1793, abernethy malformations are congenital extrahepatic pss ceps.
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