Abstract:
Objective:To investigate the application value of clinical typing in the treatment of BuddChiari syndrome (BCS).
Methods:The retrospective corsssectional study was adopted. The clinical data of 95 patients with BCS who were admitted to the First Affiliated Hospital of Zhengzhou University from January 2012 to September 2015 were collected. Based on patients′ compensation and clinical symptoms, 3 clinical typing and 8 subtypes of BCS were proposed, and each subtype was treated with corresponding strategies. Observation indices included (1) the clinical typing of BCS, (2) selection of treatment, (3) treatment effect, (4) followup situations. Followup using telephone interview and outpatient examination was performed once within 3 months after the first treatment and then once every 6 months up to December 2015 or death, loss to followup and experienced decompensation. During followup, color Doppler ultrasound and blood biochemistry test were performed regularly, and CT angiography was also conducted when necessary. Count data were presented as the case or percentage. The survival rate was calculated using Kaplan-Meier method and the survival curve was drawn.
Results:(1) BCS clinical typing of 95 patients: 4 were detected in typeⅠ(3 in typeⅠa and 1 in typeⅠb), 7 in typeⅡ (4 in type Ⅱa and 3 in type Ⅱb) , and 84 in type Ⅲ(43 in type Ⅲa, 4 in type Ⅲb, 32 in type Ⅲc, and 5 in type Ⅲd). (2) Selection of treatment in 95 patients: ① among the 3 patients with typeⅠa, 2 of them received inferior vena cava balloon angioplasty while 1 patient had to give up the operation due to failure in opening the occlusion. This patient underwent close observation and followup afterwards. ② The patient with type Ⅰb underwent cavityantrum artificial blood vessel bypass operation due to failure in opening the occlusion. ③Among the 4 patients with type Ⅱa, one of them underwent hepatic vein balloon angioplasty. The other 3 patients underwent close observation and followup because of failure in intervention therapy, such as segmental occlusion of hepatic vein or difficulty in finding the hepatic vein. ④ Among the 3 patients with type Ⅱb, due to the history of upper gastrointestinal bleeding, 2 patients received modified spleenlung fixation and intestinecavity blood vessels bypass, respectively, and 1 patient received intestinecavity artificial blood vessels bypass due to severe peritoneal effusion. ⑤ Among the 43 patients with type Ⅲa, 35 patients underwent inferior vena cava balloon angioplasty due to failure in hepatic vein intervention therapy (6 of them received firstly thrombolysis treatment due to combined thrombosis). Four patients received inferior vena cava and hepatic vein balloon angioplasties. Another 4 patients received close observation and followup due to failure in both inferior vena cava and hepatic vein intervention therapy. ⑥Among the 4 patients with type Ⅲb, 2 underwent inferior vena cava balloon angioplasty and intestinecavity artificial blood vessel bypass. The other 2 patients only received modified spleenlung fixation because of failure in inferior vena cava intervention therapy. ⑦ Among the 32 patients with type Ⅲc, 3 underwent inferior vena cava and hepatic vein balloon angioplasties, and 27 patients underwent only inferior vena cava balloon angioplasty due to failure in hepatic vein intervention therapy (7 of them received balloon angioplasty following thrombolysis treatment due to combined thrombosis). On account of failure in both inferior vena cava and hepatic vein intervention therapy, 2 patients underwent resection of lesion membranes and cavityantrum artificial blood vessel bypass, respectively. ⑧ Among the 5 patients with type Ⅲ d, 1 underwent inferior vena cava balloon angioplasty and intestinecavity artificial blood vessel bypass, and 4 underwent only modified spleenlung fixation due to failure ininferior vena cava intervention therapy. (3) Treatment efficacy: of 95 patients, 8 received followup observation, and 87 patients recovered to varied extent after interventional therapies and operations, with symptomatic relief of leg edema, ulcer, peritoneal effusion and esophageal varicosity. Eightyseven patients went through the perioperative period safely, and no death occurred. The incidence of postoperative complications was 10.3%(9/87). The complications mainly include venous thrombosis in lower limbs during catheterdirected thrombolysis therapy, pleural effusion, pneumatosis, and peritoneal effusion after surgery, all of which were cured after symptomatic treatment. (4) Followup results: 87 were followed up for 3-42 months with an average time of 19 months. During the followup, 5 patients (1 in type Ⅰa and 4 in type Ⅲa) received recanalization surgery because of the reocclusion after the inferior vena cava balloon angioplasty, and no decompensation occurred. However, decompensation was found in 11 patients (disease progression in 4 patients and symptom relapse in 7 patients). The survival rates of patients without decompensation at 0.5, 1.0, 2.0 and 3.0 years after the first treatment were 96.5%, 95.0%, 83.4% and 80.5%, respectively.
Conclusion:According to patients′ compensation and clinical symptoms, clinical typing of BCS and treatment strategiesis are determined, and it will provide a satisfactory clinical efficacy.