Laparoscopic Double Banding/ligation Of Hepatic Artery In The Treatment Of Two Cases Of Hepatic Hereditary Hemorrhagic Telangiectasia And Literature Analysis

Background:Hereditary Hemorrhagic Telangiectasia(HHT)is an autosomal dominant genetic disease characterized by systemic vascular development abnormalities.The effect of pathogenic mutations on the transforming growth factor β(TGF-β)signaling pathway in vascular endothelial cells has caused abnormal development from microvessels to large arteries and veins in the patient’s body.The shunt caused by vascular malformations has caused complications such as acute and chronic bleeding,leading to clinical symptoms such as epistaxis,gastrointestinal bleeding and anemia.The clinical symptoms of patients with hepatic hereditary hemorrhagic telangiectasia are mainly caused by arteriovenous shunt.The clinical diagnostic criteria of HHT need to meet the following three or more clinical manifestations:recurrent epistaxis;skin and mucosal capillaries and bleeding;internal organs(lung,liver,gastrointestinal tract,brain,etc.)involvement;family history of HHT.Those who meet 3 or more can be diagnosed as HHT,those who meet 2 are suspected of HHT,and HHT is not considered if the patients meet less than 2 items.Because the clinical manifestations of HHT patients are mostly age-related,this diagnostic criterion is not sensitive to the application of young people and children.At present,the treatment of HHT is mainly based on symptoms.When the hemostatic drugs such as tranexamic acid or endoscopic hemostasis are ineffective to treat epistaxis and gastrointestinal bleeding,the systemic anti-angiogenic drugs are applied.Patients with severe anemia should be given iron and appropriate blood transfusion treatment.Orthotopic liver transplantation is still the most effective treatment for HHT patients with symptomatic liver vascular malformations.Interventional embolization of the liver is not recommended treatment option except the biliary bleeding is occurred.Bevacizumab may provide a potential bridging effect for liver transplantation.It has a certain effect on improving the related complications caused by liver vascular malformations.Objective:At present,there is no clear standard for the treatment of hepatic hereditary hemorrhagic telangiectasia.In addition,the lack of understanding of its surgical treatment options has caused certain limitations in the diagnosis and treatment of the disease.Our research center has more than ten years of experience in the treatment of hepatic hereditary hemorrhagic telangiectasia,and has a comprehensive understanding of the diagnosis and treatment of the disease.We innovatively carried out hepatic artery banding/ligation combined with binding and ligation of multiple blood vessels at the beginning of the hepatic artery and its branches.This procedure has treated a large number of patients with severe hepatic hereditary hemorrhagic telangiectasia after ten years.The surgery has a definite effect.The purpose of this study is to summarize the clinical data of two recent patients with hepatic hereditary hemorrhagic telangiectasia who underwent laparoscopic double banding/ligation of the hepatic artery in Qilu Hospital of Shandong University.The feasibility of laparoscopic surgery is proposed through its clinical manifestations,laboratory examinations and imaging characteristics.Methods:Case 1 was a 48-year-old female who had epistaxis for more than 3 years and was admitted to the hospital for more than 1 month after the discovery of liver deformity.During the hospitalization,laparoscopic double banding/ligation of the common hepatic artery+cholecystectomy+liver biopsy was performed.During the operation,the starting part and main trunk of the common hepatic artery of the patient were ligated and constricted separately with No.10 thread.The mutated artery supplying the right liver and gastroduodenal artery were double-clamped.She was discharged from the hospital after hepatoprotective and acid-suppressing nutrition treatments after the operation.After discharge from the hospital,the patient’s chest tightness was significantly relieved during follow-up.The echocardiography showed that the pulmonary artery pressure was lower than before the operation.The abdominal enhanced CT showed significant improvement of intrahepatic arteriovenous fistula.Case 2 was a 39-year-old male who was admitted to the hospital due to repeated nosebleeds for more than 20 years and aggravation for more than 1 year.Laparoscopic double banding/ligation of hepatic artery and liver biopsy were performed during hospitalization.During the operation,the patient’s variant celiac trunk and common hepatic artery were double ligated and the left hepatic artery was ligated.Considering that there are more blood spots in the patient’s stomach,the left gastric artery is ligated.The lymphatic fatty tissue around the hepatoduodenal ligament and the perihepatic ligament are preserved.After the operation,he was given liver-protecting and acid-suppressing nutrition treatment and was discharged from the hospital.Reexamination of the abdominal enhanced CT showed that the intrahepatic arteriovenous fistula was significantly improved after the patient was discharged from the hospital.We studied the clinical data of two cases of hepatic hereditary hemorrhagic telangiectasia.It also compares its inspection results and laboratory tests,combined with previous related research and literature analysis.It is suggested that the hepatic hereditary hemorrhagic telangiectasia can be treated by double banding/ligation of the hepatic artery.Laparoscopic surgery can greatly reduce the surgical trauma to the patient.This surgical method has a significant effect on the improvement of the quality of life of the patient.Results:Laparoscopic surgery has improved the clinical symptoms,laboratory indexes and imaging findings of the patients with double ligation of the hepatic artery at the beginning and into the liver.The double ligation of the beginning of the hepatic artery and the place where it enters the liver can effectively reduce the amount of blood entering the liver.In particular,ligation was given to the branches of the hepatic artery that were significantly expanded,and the relatively normal branches and the beginning of the hepatic artery were retained and shrunk.There were no deaths related to surgery after the operation.The clinical symptoms of all postoperative patients were improved and the quality of daily life scores were improved.The pulmonary artery systolic blood pressure was reduced by performing cardiac color Doppler ultrasound to evaluate the cardiac function after the operation of the patient.Laboratory test results showed that the patient’s glutamyl transpeptidase and alkaline phosphatase levels significantly decreased.Discussion:The hepatic hereditary hemorrhagic telangiectasia can eventually develop into heart failure,severe heart insufficiency,refractory portal hypertension,biliary lesions and even liver failure.For patients with surgical indications,laparoscopic double banding/ligation of the hepatic artery can significantly improve the clinical symptoms of patients with liver involvement,and can significantly improve their prognosis.It helps us to provide a basis for clinical diagnosis and treatment of different patients in the clinic,which can make better targeted personalized treatment.