Background: Abernethy 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.
Methods: Fourteen 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.
Results: Three 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.
Conclusion: MSCT 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.
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http://dx.doi.org/10.1186/s12880-019-0396-3 | DOI Listing |
Exp Clin Transplant
October 2024
From the Department of General Surgery, Gazi University, Ankara, Türkiye.
Abernethy syndrome is a rare congenital vascular anomaly. In this condition, blood from the portal system is diverted to the caval system through a portocaval shunt, entirely or partially bypassing the liver. Prevalence figures in the literature range from 1 per 30000 population up to 1 per 50000 population.
View Article and Find Full Text PDFRadiol Case Rep
December 2024
Baylor University Medical Center, Department of Radiology, 3500 Gaston Avenue, Dallas, Texas, 75246, USA.
I review a case of a 30-year-old woman who presented with diarrhea, nausea and vomiting for several days. She was ultimately diagnosed with acute gastroenteritis but was incidentally found to have a congenital extrahepatic portosystemic shunt, also known as the Abernethy malformation. The Abernethy malformation, first described by Dr John Abernethy in the year 1793, is an exceptionally rare clinical entity.
View Article and Find Full Text PDFVasc Endovascular Surg
February 2025
Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein.
View Article and Find Full Text PDFAsian J Surg
September 2024
Shanghai Fengxian District Central Hospital, Shanghai, 201499, China. Electronic address:
J Pediatr Surg
October 2024
Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA; Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Background: Congenital portosystemic shunts (CPSS) are rare congenital abnormalities causing abnormal blood flow between the portal vein and systemic circulation. This study reports on the peri-operative anticoagulation management of CPSS patients post closure, focusing on the incidence of thrombotic and bleeding complications.
Methods: This is a single-center retrospective analysis of CPSS patients who underwent surgery or endovascular intervention between 2005 and 2021.
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