Abernethy Type Research Articles

A case of Abernethy malformation type I combined with hepatoblastoma.

This article encountered an extremely rare case of a 2-year-old male with Abernethy malformation Type I combined with hepatoblastoma. Furthermore, the medical history was characterized by several other abnormalities: gross facial asymmetry and cardiac defects，thus, diagnosis of Goldenhar syndrome in the setting of Abernethy type I was made. In this article, we exhibit the typical clinical presentation and Pathology imaging features of this disease.

Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis.

Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Cardiomegaly with prevalence of pulmonary vascular network. Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

The first report of living donor liver transplantation for abernethy malformation (congenital absence of the portal vein) in Vietnam

Abstract Abernethy malformation, also known as Congenital absence of the portal vein (CAPV), is a very rare malformation. There are some reports of liver transplantation as a treatment for CAPV, and our report is the first case in Vietnam. A 10-year-old boy, diagnosed with Abernethy type 1b malformation (portal vein draining directly into the inferior vena cava) and liver tumor. Living donor liver transplantation was performed using a right liver graft from his mother. Histopathological examination of the native liver showed a huge mass lesions within the right liver and absence of portal vein in portal space. Postoperatively, the patient attained optimal liver function and at a 13-month follow-up has returned to normal life.

Diagnostic accuracy of multi-slice computed tomography in children with Abernethy malformation

BackgroundAbernethy malformation is a rare congenital abnormality. Imaging examination is an important method for the diagnosis. The purpose of this study was to demonstrate manifestations of multi-slice computed tomography (MSCT) in Abernethy malformation and its diagnostic accuracy.MethodsFourteen children with Abernethy malformation were admitted to our center in China between July 2011 and January 2018. All 14 patients (eight males and six females) received MSCT and digital subtraction angiography (DSA) while eight patients also received ultrasound. The patients’ age ranged from 1 to 14 (median age 8 years old). The clinical records of the patients were retrospectively reviewed. MSCT raw data were transferred to an Advantage Windows 4.2 or 4.6 workstation (General Electric Medical Systems, Waukesha, WI). We compared the findings of MSCT with DSA and surgical results in order to ascertain diagnostic accuracy.ResultsThree cases had type Ib Abernethy malformation and eleven cases had type II. Two cases of type II Abernethy malformation were misdiagnosed as type Ib using MSCT. Comparing the findings of MSCT with DSA and surgical results, the accuracy of MSCT was 85.7% (12/14), in which 100.0% (3/3) were type Ib and 81.8% (9/11) were type II. Clinical information included congenital heart disease, pulmonary hypertension, diffuse pulmonary arteriovenous fistula, abnormal liver function, hepatic nodules, elevated blood ammonia, and hepatic encephalopathy. Eleven cases were treated after diagnosis. One patient with Abernethy malformation type Ib (1/3) underwent liver transplantation. Seven patients with Abernethy malformation type II (7/11) were treated by shunt occlusion, received laparoscopy, or were treated with open surgical ligation. Another three patients (3/11) with Abernethy malformation type II were treated by interventional portocaval shunt occlusion under DSA.ConclusionMSCT attains excellent capability in diagnosing type II Abernethy malformation and further shows the location of the portocaval shunt. DSA can help when it is hard to determine diagnosis between Abernethy type Ib and II using MSCT.

Malformation of Abernethy type 2: presentation of a clinical case and review of literature

The extrahepatic congenital porto systemic derivation is a very rare pathology of the venous vascular system of the abdomen described by John Abernethy in In this malformation there is a passage of substances not metabolized from the liver to the systemic circulation and dilation of the pulmonary capillary bed It is divided into groups according to the presence type II or total absence type I of portal flow We present the case of a year old child a twin product of the first pregnancy of a year old mother attended at Vicente Corral Moscoso Hospital in the city of Cuenca with respiratory symptoms without definitive diagnosis The presence of a portal vascular abnormality was found in a casual abdominal ultrasound study diagnosed as Abernethy type II malformation by abdominal angiography and nuclear magnetic resonance A thorough bibliographical review and a subsequent discussion of the case were carried out finding as important data the existence of cases reported until worldwide In Latin America there is the report of a case in Venezuela in and in Argentina in We conclude by emphasizing the importance of a correct anamnesis physical examination and the use of different diagnostic techniques in terms of respiratory symptoms due to there are rare pathologies that once an early diagnosed they can be treated successfully thus giving the patient a normal life The present case is the only one reported in Ecuador with a successful approach and treatment

Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report

BackgroundCongenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension.Case presentationWe report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient’s condition improved in the first year after discharge. However, one year later, the patient’s conditions start to deteriorate.ConclusionThis article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.

ABERNETHY MALFORMATION AS A RARE INDICATION FOR LIVER TRANSPLANTATION: CASE REPORT

Abernethy malformation or congenital agenesis of the portal vein (CAPV) is a rare malformation of the abdominal splanchnic venous system. This malformation is commonly found in children and is often associated with other malformations such as congenital cardiac anomalies and skeletal system disorders, as well as liver tumors. There are two types of Abernethy malformation. In type I, portal blood bypasses the liver completely, with the superior mesenteric vein and the splenic vein draining into the inferior vena cava separately (type Ia), or together (type Ib). There are no intrahepatic portal vein branches in the liver. Type II is a partial portocaval shunt in which portal blood partially supplies the liver. There is no unified therapeutic approach for all patients with Abernethy malformation, however, liver transplantation is recommended in patients with liver disease (encephalopathy, poor liver function) and those with liver tumors. In this case report we present a case of Abernethy type Ib malformation in a 17-year-old patient with chronic malaise and uper abdominal pain. During diagnostic work-up, an unresectable liver tumor was found and the patient was successfully treated with orthotopic liver transplantation.

Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation

In this report, we describe a living donor liver transplantation (LDLT) in a patient (7-year-old boy) with Abernethy type 2 congenital extrahepatic portocaval shunts (CEPS). This patient underwent a surgical shunt ligation as the first treatment for pulmonary hypertension; pulmonary hypertension was improved and controlled successfully 4 years after the first operation. However, pulmonary hypertension recurred gradually because of multiple intrahepatic portosystemic shunts; therefore, LDLT was performed as a radical treatment of intrahepatic portosystemic shunts. His pulmonary arterial pressure was also controlled 22 months after LDLT, the postoperative continuous intravenous prostaglandin I(2) (PGI(2)) treatment could be withdrawn successfully. We suggest that clinicians carefully follow up the recurrent portosystemic shunt and cardiopulmonary disorders secondary to Abernethy type 2 CEPS.

Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in abernethy malformation. A case report

Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9-year-old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation. HPS in Abernethy syndrome is a manifestation of normal portal blood flow bypassing the liver and can be ameliorated by redirecting blood flow to a segment of liver with normal portal anatomy. APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS.

Congenital extrahepatic portocaval shunt (Abernethy Type 2), huge liver mass, and patent ductus arteriosus-a case report of its rare clinical presentation in a young girl

The authors report a case of congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This type of shunt is called an Abernethy type 2 shunt, where the portal vein and inferior vena cava are connected side to side, and a hypoplastic portal flow exists in the liver parenchyma. This case of a young girl is complicated with a cardiac anomaly and a large hyperplastic nodule in the liver. This phenotype usually is seen in the Abernethy type 1 shunt, which is a side-to-end shunt with the complete absence of a portal vein. In this context, the current case is very unique in its clinical manifestation, and this phenotype has never been reported. Therefore, it is important to speculate the etiology and treatment of this kind of abnormality and its associated hepatic mass.