布加综合征临床分型在其治疗中的应用价值

Application value of clinical typing in the treatment of Budd-Chiari syndrome

  • 摘要: 目的:探讨依据布加综合征临床分型在其治疗中的应用价值。
    方法:采用回顾性横断面研究方法。收集2012年1月至2015年9月郑州大学第一附属医院收治的95例布加综合征患者的临床资料。根据患者的自身代偿情况及临床症状,提出了布加综合征的临床分型(3型、8个亚型),并针对每个亚型制订相应的治疗策略。观察指标:(1)布加综合征的临床分型。(2)治疗方法的选择。(3)治疗效果。(4)随访情况。随访自治疗结束后开始,采用电话和门诊方式进行随访,首次治疗后3个月内随访1次,此后每6个月随访1次。随访期间定期行彩色多普勒超声、血生化检查,必要时行CT血管造影检查。随访时间截至2015年12月,或至患者死亡、失访、临床症状发生失代偿。计数资料用例数或百分比表示。采用Kaplan-Meier法计算生存率并绘制生存曲线。
    结果: (1)95例布加综合征患者临床分型结果:Ⅰ型4例(Ⅰa型3例、Ⅰb型1例),Ⅱ型7例(Ⅱa型4例、Ⅱb型3例),Ⅲ型84例(Ⅲa型43例、Ⅲb型4例、 Ⅲc型32例、Ⅲd型5例)。(2)95例布加综合征患者治疗方式的选择:①-3例Ⅰa型患者中,2例行下腔静脉球囊扩张成形术,1例因介入开通闭塞段失败予以随访观察。②-1例Ⅰb型患者,因介入开通闭塞段失败行腔房人工血管转流术。③-4例Ⅱa型患者中,1例行肝静脉球囊扩张成形术,3例因肝静脉节段闭塞、无法寻找肝静脉等原因未行介入治疗,予以随访观察。④-3例Ⅱb型患者中,2例因上消化道出血史分别行改良脾肺固定术、肠腔人工血管分流术,1例因严重腹腔积液行肠腔人工血管分流术。⑤-43例Ⅲa型患者中,35例因肝静脉介入治疗失败仅行下腔静脉球囊扩张成形术(其中6例因合并血栓形成先行溶栓治疗,再行球囊扩张成形术),4例行下腔静脉球囊扩张成形+肝静脉球囊扩张成形术,4例因下腔静脉及肝静脉介入治疗均失败予以随访观察。⑥4例Ⅲb型患者中,2例行下腔静脉球囊扩张成形+肠腔人工血管分流术,2例因下腔静脉介入治疗失败仅行改良脾肺固定术。⑦-32例Ⅲc型患者中,3例行下腔静脉球囊扩张成形+肝静脉成形术,27例因肝静脉介入治疗失败仅行下腔静脉球囊扩张成形术(其中7例因合并血栓形成先行溶栓治疗,再行球囊扩张成形术),2例因下腔静脉及肝静脉介入治疗失败分别行病变隔膜切除术、腔房人工血管转流术。⑧-5例Ⅲd型患者中,1例行下腔静脉球囊扩张成形+肠腔人工血管分流术,4例因下腔静脉介入治疗失败仅行改良脾肺固定术。(3)治疗效果:95例患者中,8例随访观察;87例经介入及手术治疗后的患者症状均得到不同程度改善,表现为下肢水肿缓解、溃疡逐渐愈合、腹腔积液消退、食管曲张静脉缓解等。87例患者均安全度过围术期,无死亡病例。术后并发症发生率为10.3%(9/87),主要为置管溶栓期间并发下肢静脉血栓形成,以及外科手术后发生胸腔积液、积气、腹腔积液形成等,经对症治疗后症状消失。(4)随访情况:87例患者术后获得随访,随访时间为3~42个月,平均随访时间为19个月。随访期间,5例患者(Ⅰa型1例、Ⅲa型4例)因下腔静脉球囊扩张成形术治疗后再次闭塞接受再通手术,均未出现失代偿表现。11例患者出现失代偿表现(4例病情进展、7例症状复发)。患者首次治疗后0.5、1.0、2.0、3.0年无失代偿生存率为96.5%、95.0%、83.4%、80.5%。
    结论:依据患者的自身代偿情况及临床症状制订布加综合征临床分型并选择对应的治疗方案,可获得满意的临床效果。

     

    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.

     

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