| Introduction:Regional portal hypertension(RPH),which is left portal hypertension,is caused by obstruction of splenic venous blood reflux that was caused by various factors.The main pathophysiological feature of this syndrome is the increase of pressure in the spleen and stomach region of portal vein.The etiology of RPH contains pancreatic origin,splenic origin and retroperitoneal origin.Among them,pancreatic portal hypertension(PPH)is most common.Because the number of reported RPH cases is relatively small,the large sample data analysis on epidemiology,clinical manifestations,diagnosis,treatment and prognosis of RPH is lack.Therefore,we systematically summarized the case data of 3576 RPH patients in China in order to further improve the understanding and realization of RPH for clinicians.Data and methods:1.DataChinese literature databases from January 1980 to June 2019 were searched with the terms including "regional portal hypertension","left portal hypertension" or "pancreatic portal hypertension".By reading the full text or abstract manually,the relevant literature of all the original cases of RPH was included.After eliminating the unqualified literature,150 articles were finally included.The publication time was from 1992 to 2019.A total of 3576 RPH patients were enrolled.2.Methods2.1 Data extractionAfter reading the literature,relevant data were extracted which included area,total cases,male cases,female cases,age,etiology,clinical symptoms and signs,diagnostic methods,gastroscopy or barium meal results,treatment,follow-up time and prognosis.2.2 Prognostic criteriaCure or improvement of treatment: cure referred to the complete disappearance of portal hypertension through surgery,improvement of treatment referred that the patient did not recur gastrointestinal bleeding,or re-examination of gastroscopy,CT and other examinations suggested that gastric and/or esophageal varices were alleviated or disappeared.Ineffective treatment: gastrointestinal bleeding uncontrolled,or gastric and/or esophageal varices were not alleviated.Death: death during follow-up.2.3 Statistical methodsExcel tables were established to analyze the sex,age,geographical distribution,etiology,clinical symptoms,treatment and prognosis of the cases.Cases and percentages was used to describe counting data.Results:1.Gender,age and geographical distribution of RPHThere were 2340 males and 1236 females in all RPH patients.The ratio of males to females was 1.89:1.The age ranged from 3 to 85 years,with an average age of 50.23 years.According to the statistics of regional distribution,the morbidity of East and Southwest China is relatively high.In addition,the morbidity in the south is slightly higher than that in the north.2.Etiological distribution of RPHAmong the 3576 patients,3291 cases(92.03%)were pancreatic origin,60 cases(1.68%)were splenic origin,24 cases(0.67%)were retroperitoneal origin,and 35(0.98%)were other rare causes including gastric cancer,celiac lymphoma,bone marrow fibrosis and so on.In addition,the etiology of 166 patients(4.64%)were unknown or not explained in the literature.3.Clinical manifestations of RPHA total of 123 articles recorded the clinical manifestations of 2715 RPH patients.The main clinical symptoms were upper gastrointestinal bleeding which was manifested in 1491 cases(54.92%),upper abdominal discomfort or upper abdominal pain in 1047 cases(38.56%),and anemia in 82 cases(3.02%)and so on.The main signs were splenomegaly in 1859 cases(68.47%),abdominal mass in 46 cases(1.69%),ascites sign in 20 cases(0.74%)and so on.In addition,115 articles recorded the results of gastroscopy,barium meal or CT examination in 2423 patients,including 1731 cases of isolated gastric varices(71.44%),62 cases of isolated lower esophageal varices(2.56%),336 cases of gastric varices complicated with lower esophageal varices(13.87%),128 cases of esophageal or gastric varices(5.28%)(It is uncertain that whether gastric varices or esophageal varices in the literature.)No varicose veins was showed in 166 cases(6.85%).4.Examination methods of RPHA total of 140 articles described 3212 patients who were diagnosed as RPH by gastroscopy,color Doppler ultrasound,CT,MRI,ultrasound gastroscopy,barium meal,DSA or other examination methods.1219 patients(37.95%)received only one examination method to diagnose RPH,995 patients(30.98%)received two examination methods to diagnose RPH,850 patients(26.46%)received three examination methods to diagnose RPH,and 148 patients(4.61%)received more than four examination methods to diagnose RPH.Among all the patients,784(24.41%)were confirmed RPH by CT examination.5.Treatment and prognosis of RPHA total of 115 literatures recorded the treatment methods of 2345 patients,including that 1663 cases(70.92%)received splenectomy,265 cases(11.30%)received comprehensive medical treatment,176 cases(7.50%)received surgery without splenectomy,such as exploratory laparotomy,cholangioenterostomy and palliative surgery for tumors.Endoscopic varicose vein ligation or sclerotherapy was performed in 109 cases(4.65%),splenic artery embolization or ligation in 93 cases(3.97%),splenic artery embolization combined with splenectomy in 25 cases(1.07%)and abandonment of treatment in 14 cases(0.60%).A total of 100 articles recorded the prognosis of 2089 patients.The follow-up period ranged from 1 week to 18 years.1738 patients'(83.20%)condition were improved after treatment,40 patients'(1.91%)treatment were ineffective,182 patients died(8.71%)and 129 patients(6.18%)couldn't be followed up successfully or were not mentioned the prognosis.Discussion:The results showed that the ratio of male to female was 1.89:1,the age of onset ranged from 3 to 85 years old,and the average age was 50.23 years old.It can be seen that the incidence of RPH in males is significantly higher than that in females,and the middle-aged people are high risk population of RPH.The statistics of regional distribution showed that the incidence of this disease is relatively high in East,Southwest,North and Central China,which may be related to the medical level in this area and the attention of these doctors.The statistical results of etiological distribution in this study showed that PPH still accounts for the majority of RPH(92.03%).The main pathogenesis of PPH is that pancreatic tumors or pancreatic pseudocysts which can directly squeeze or infiltrate the splenic vein,resulting in slowing or obstruction of splenic vein blood flow and inducing splenic vein thrombosis.In addition,acute and chronic pancreatitis can injury the splenic vein,damage vascular endothelial cells,and form splenic vein thrombosis which lead to RPH.From the 3756 cases of RPH,we can see that the main clinical manifestations of RPH are the following aspects: first,the clinical manifestations of basic diseases that cause obstruction of splenic venous reflux,such as upper abdominal discomfort,pain and so on;second,the clinical manifestations of increased pressure in spleen and stomach region of portal vein,such as gastrointestinal bleeding,splenomegaly and so on;third,no cirrhosis and normal liver function test;fourth,the characteristically isolated gastric varices can be found in endoscopy examination.The diagnosis of RPH is mainly based on clinical manifestations,serological and imaging examinations.Endoscopic detection of isolated gastric varices is one of the most important characteristics of RPH which is different from general portal hypertension.And without liver cirrhosis,liver function tests are normal on the whole,which is one of the most important differences between RPH and other portal hypertension.The treatment of RPH includes correcting portal hypertension in spleen and stomach region of portal vein and treating primary diseases.Specific methods include medical treatment,endoscopic treatment,interventional treatment and surgical treatment.Surgical treatment is considered to be the most effective treatment.The prognosis of this disease is mostly good,mainly depending on the etiology of RPH. |
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