Treatment Of Hepatic Hereditary Hemorrhagic Telangiectesia

Objective:To investigate the treatment and curative effect of double ligation/constriction of hepatic artery in hereditary hepatic telangiectasia.The purpose of this thesis is multidimensional.A review of the English literature was carried out for good understand and define the outcome of surgical procedure involving hepatic arteries in HHT.A retrospective investigation of patients identified at the Shandong University Qilu Hospital was done,including clinical characteristics,intervention and results analysis.Study was done mainly to evaluate the effectiveness of double banding/ligation of hepatic arteries in curing patients with hepatic hereditary hemorrhagic telangiectasia(HHHT).Visceral Arteriovenous abnormalities are also seen in many sick persons,and these are very commonly observed in the gastrointestinal tract,brain,lungs and in this special case in kidneys as well.Methods:The clinical data of 13 patients with hereditary hemorrhagic telangiectasia who underwent double ligation/constriction of hepatic artery from July 2005 to February 2011 were analyzed retrospectively,and the prognosis of the patients who underwent surgical treatment was evaluated.Additionally,a case study was done on a 40-year-old woman with a history of bilateral nasal cavity and gingival bleeding without obvious cause for 20 years,with bleeding averaging once a day occasionally,lip bleeding spotted as well.The patient was presented with complains of chest tightness,fatigue and dizziness during intense activity.Hereditary Hemorrhagic Telangiectasia is a rare autosomal dominant vascular condition.The pathophysiology of this condition constitutes shortage of capillary beds linking arterioles and venules,resulting in through contact between the vessels.This leads to telangiectasia on specific places such as the fingers,face,nasal mucosa and mouth.Results:After 5-14 years of follow-up,the clinical manifestations,laboratory indicators and imaging symptoms of illnesses with double ligation/constriction of hepatic artery were all improved.Visceral Arteriovenous abnormalities are also seen in many sick persons,and these are so often seen in the gastrointestinal tract,brain,lungs and in this special case in kidneys as well.CT and CT Angiography(CTA)demonstrated arteriovenous shunting in this case.Hepatic AVMs are also present in numerous patients with hereditary hemorrhagic telangiectasia,though these individuals are often without symptoms,however,hepatic arteriovenous malformations(AVMs)could lead to serious problems,such as high output cardiac failure,other complications include bleeding,iron deficiency and anemia.Conclusion:The double ligation/constriction at the origin and entrance of hepatic artery in patients with hepatic HHT can effectively reduce pulmonary artery pressure and reduce liver injury.Double ligation/constriction of hepatic artery is an effective method to treat hereditary hemorrhagic telangiectasia(HHT)of the liver.Moreover,Hereditary hemorrhagic telangiectasia is identified by vascular abnormalities it can also involve multiple organs such as the lungs,skin,brain and the gastrointestinal tract.In gastrointestinal tract,the most common cases of hereditary hemorrhagic telangiectasia are found with the liver involvement but in our study HHT is also involved with the kidneys which leads to disturbance of kidneys electrolytes.One of our study reports the HHT with kidney involvement that has never been previously reported in the literature.Diagnosis of hereditary hemorrhagic telangiectasia(HHT)relies on fulfilling 3/4 criteria:Family chronicle of the condition,mucocutaneous telangiectasia,spontaneous and periodic episodes of bleed(epistaxis),and visceral arteriovenous malformations.Management is conducted by global agreement guidelines,which targets sick persons' specific arteriovenous malformations.Newly emergent therapies both surgical and medicinal are being surveyed in clinical trials;pazopanib and bevacizumab retards angiogenesis,while thalidomide reduces blood vessel maturation.Additionally,double ligation/constriction of hepatic artery in HHT as well.