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