Abstract
Objective: To summarize the clinical features of special portal hypertension-Abernethy malformation reported at home and abroad. Methods: The relevant literature on Abernethy malformation published at home and abroad from January 1989 to August 2021 was collected. Patients'clinical features, imaging and laboratory test results, diagnosis, treatment, and prognosis were analyzed. Results: A total of 380 cases were included from 60 and 202 domestic and foreign literatures. Among them, there were 200 cases of type I, with 86 males and 114 females, and the average age was (17.08±19.42) years, while there were 180 cases of type II, with 106 males and 74 females, and the average was (14.85±19.60) years. The most common reason for the first visit of an Abernethy malformation patient's was gastrointestinal system symptoms such as hematemesis and hematochezia caused by portal hypertension (70.56%). Multiple malformations were present in 45.00% of type Ⅰ and 37.80% of type Ⅱ patients. The most prevalent condition was congenital heart disease (62.22%, and 73.53%). Complications related to Abernethy malformation was occurred in 127 and 105 cases with type I and type II, respectively, with liver lesions in 74.02% (94/127) and 39.05% (42/105) and hepatopulmonary syndrome of 33.07% (42/127) and 39.05% (41/105), respectively. The imaging diagnosis of type I and type II Abernethy malformations were mainly based on abdominal computed tomography (59.00%, and 76.11%). Liver pathology was performed in 27.10% of patients. Blood ammonia increased by 89.06% and 87.50%, and AFP increased by 29.63% and 40.00% in laboratory findings. 9.76% (8/82) and 6.92% (9/130) died, while 84.15% (61/82) and 88.46% (115/130) had improved conditions after medical conservative, or surgical treatment. Conclusion: Abernethy malformation is a rare disease in which congenital portal vein development abnormalities lead to significant portal hypertension and portasystemtic shunt. Patients often seek medical treatment for gastrointestinal bleeding and abdominal pain. Type Ⅰ is more common in women, often associated with multiple malformations, and prone to secondary intrahepatic tumors. Liver transplantation is the main treatment method. Type Ⅱ is more prevalent in males, and shunt vessel occlusion is the first treatment choice. Overall, type Ⅱ has a better therapeutic impact than type Ⅰ.