简介 Budd-Chiari's 综合症的相关因素 Budd-Chiari's综合症的病理生理 Budd-Chiari's综合症的临床表现 Budd-Chiari's综合症的影像学检查Budd-Chiari's综合症的治疗选择
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BCS 治疗的选择(2)

时间:2014-09-19 20:18来源:未知 作者:Mr.Editor
在外科搭桥手术,包括肠-房或腔-房旁路移植,或所谓肝静脉根治术无疾而终后,目前外科对于BCS的处置包括门腔分流术和肝移植。 门腔静脉分流术 尽管抗凝、血管成形术和肝静脉支撑架,很多病人仍然持续性恶化。门体分


       在外科搭桥手术,包括肠-房或腔-房旁路移植,或所谓肝静脉根治术无疾而终后,目前外科对于BCS的处置包括门腔分流术和肝移植。       


门腔静脉分流术
 
        尽管抗凝、血管成形术和肝静脉支撑架,很多病人仍然持续性恶化。门体分流是内科治疗或阻塞不能进行球囊扩张或支撑架病人的选择。
 
        外科分流手术已经应用近30年【28】。门腔侧侧吻合术是常见的手术,其次是肠腔分流术。尾叶肥大影响肝段下腔静脉,当足够严重的时候,通过肠腔分流防止肝的压迫导致更坏的情况,包括术后早期死亡。如果肝段下腔静脉被阻塞,分流的应用受到限制,外科手术分流通道并发功能不全发生率高达32%【29】。外科手术分流同样面临着较高的外科并发症和死亡率。几项研究显示外科分流对生存率的影响是不一样的【30,31】,从57%到94%不等[31,32]。外科分流术的研究缺乏外科选择的标准,时间过程信息和以前外科治疗的数据。最新研究提示越严重的病人越获益[33]。其它取得较好长期生存率的研究主要归功于仔细地选择病人和BCS早期进行外科手术治疗【34】。这一手术的外科经验正在逐渐减少,目前已经罕有这类手术报告,大部分为TIPSS所替代。
 
         BCS应用裸金属支撑架首次报告是在1992年和1993年【35-37】。TIPS并不是没有并发症。TIPSS术后发生肝性脑病将近1/3的病例。5% 的病例内科治疗无效【38】。但是BCS患者TIPSS术后肝性脑病的风险一直以来报告是低的【39,40】。颈动脉、右心房、IVC、肝包膜、门静脉、肝动脉和胆管损伤是可能的。门静脉血流转流到下腔静脉可能导致血流动力学并发症和爆发性肝功能衰竭。
 
         然而,TIPSS比手术创伤小,并且绕过阻塞的肝段下腔静脉将门静脉血分流到肝上的下腔静脉,通过减低门静脉压力是BCS患者获益。为在门静脉和下腔静脉之间提供理想的压力梯度,TIPSS可以容易地获得和方便调节分流通道的直径,从而确保所获得的经肝压力梯度充分地维持肝窦的灌注。当用于肝硬化门静脉高压时,裸支撑架1年血栓形成或狭窄率为50%【41,42】。支撑架内血栓可以发生在植入后任何时间可以通过支撑架内扩张得到纠正。为减少支撑架阻塞率建议门静脉穿刺后应用肝素。
 
        聚四氟乙烯(PTFE)覆膜支撑架可以进一步减少支撑架功能不全的发生率。两组系列病例报告显示PTFE覆膜支撑架比较裸支撑架可以改善TIPSS分流通道的开通率和减少功能不良的情况【43,44】。一项随机研究比较了PTFE与裸金属支撑架治疗食道静脉曲张和顽固性腹水的不同,结果显示中期随访300天,支撑架功能不良发生率40% vs 13%【45】 。与裸金属支撑架比较覆膜支撑架临床恶化较低,与为29% vs 8%。PTFE支撑架的应用被扩展应用到BCS,获得较好的开放率已经得到证实【44,46】。TIPSS的应用也可作为肝移植的桥梁,其内的支撑架并不干扰肝移植手术的实施。它也可用于更复杂的临床情况,如怀孕期间低剂量的X线并不影响胎儿的宫内发育。溶栓或不溶栓的TIPSS可以用于BCS和同时伴发PVT的情况【47】。
 
        由于罕有BCS研究,有关TIPSS对BCS结果影响的报告有限。没有前瞻性或随机对照研究报告。2008年,N=124例,欧洲多中心试验报告显示1-,5-年原位肝移植的存活率分别是88%和78%【40-67】。最近的BCS应用TIPSS研究见下表。
 

 

 

Studies using transjugular intrahepatic portosystemic stent-shunt for Budd–Chiari syndrome.
Study (year) Description Patients (n)
undergoing TIPSS
Patient outcome Conclusions Ref.
Attwell et al.
(2004)
Retrospective study
assessing role of TIPSS
and OLT in a single unit
17 14 improved, three died within
1 month. At a mean of 3 years,
eight patients remained well and
four died. Five underwent liver
transplant
TIPSS can be used as a
bridge to liver transplant
and offers excellent
short-term survival
【55】
Hernàdez-
Guerra et al.
(2004)
Retrospective study
comparing use of PTFE
(n = 16) and bare metal
(n = 9) stents in BCS
25 At median follow-up of
20.4 months, 16 (87%) bare
metal stents experienced
dysfunction vs 3 (33%) of
PTFE stents
PTFE stents have a lower
dysfunction rate, lower
number of reinterventions
than bare metal stents and
are preferable
【43】
Kavanagh et al.
(2004)
Retrospective study of
patients with acute BCS
and liver failure
5 4 underwent TIPSS. 1 died
unexpectedly 2 weeks post-
TIPSS. TIPSS was not possible in
final patient and they underwent
OLT. 80% survival at mean
follow up of 30 months
Initial TIPSS with OLT for
treatment failures may be
an effective treatment for
BCS
【56】
Rössle et al.
(2004)
Retrospective analysis of
patients with severe BCS
not responding to
medical therapy who
were selected for TIPSS
25 1-year and 5-year survival rate
without transplantation in all
patients was 93 and 74%,
respectively. Three patients died
and two required OLT
TIPSS results in an excellent
outcome in patients with
severe fulminant/acute,
subacute and chronic BCS
【57】
Khuroo et al.
(2005)
Retrospective analysis of
consecutive patients with
BCS receiving TIPSS
8   TIPSS recipients had
significantly better survival
than those treated with
medical therapy alone
(p = 0.04)
【58】
Eapen et al.
(2006)
Retrospective analysis of
61 patients treated with
radiological intervention
alone at a single center
26 1-year and 5-year survival rate of
whole cohort was 94 and 87%,
respectively
Excellent medium-term
survival for patients in all
categories of disease
severity
【59】
Gandini et al.
(2006)
Retrospective comparison
of bare (n = 6) or PTFE
(n = 7) stents
13 Mean primary patency duration
was 4.46 months ± 3.40 in bare
metal and 22.29 months ± 8.15
in PTFE group
PTFE-covered stent-grafts
significantly increased
primary patency rate of
TIPSS in BCS, with reduced
reintervention rate and
prolonged clinical
improvement
【13】
Corso et al.
(2008)
Retrospective analysis of
TIPSS in BCS where
medical treatment failed
15 TIPSS was very effective in
decreasing the portosystemic
pressure gradient. Two patients
died during follow up (median
follow up 29 months), did not
require OLT
TIPSS is an effective and
safe treatment for BCS and
may be considered a
valuable alternative to
traditional surgical
portosystemic shunting or
liver transplantation
【60】
Darwish Murad
et al. (2008)
Retrospective comparison
of patency rates between
bare metal and PTFE
stents
16 Primary patency rate at 2 years
was 12% using bare and 56%
using covered stents (p = 0.09)
PTFE stents have superior
patency rates in BCS
【49】
Garcai-Pagàn
et al. (2008)
Retrospective analysis of
outcome and prognostic
factors. Multicenter
European trial
124 1-year and 5-year OLT-free
survival were 88 and 78%,
respectively
Long-term outcome for
patients with severe BCS
treated with TIPSS is
excellent even in high-risk
patients, suggesting that
TIPSS may improve survival
【9】
Zahn et al.
(2010)
Retrospective analysis of
patients undergoing
TIPSS (n = 13) or OLT
(n = 4) for BCS
13 Survival in the TIPSS group was
92.3% and 75% in the OLT
group during a median follow-up
of 4 and 11.5 years, respectively
OLT should be limited to
patients who cannot be
managed by TIPSS
【61】
Al-Hilou et al.(2011) Retrospective analysis of
patients undergoing
TIPSS (PTFE) between
2001 and 2010
30 Mean follow-up 38.2 months.
Overall mortality was 23%.
TIPSS thrombosis occurred in
3 patients. 6, 12, 24 and 60
month survival was 96, 90, 86
and 66% respectively. Hepatic
encephalopathy occurred in 13%
Long-term outcomes
following PTFE covered
TIPSS for BCS are very good
with high rates of primary
patency and clinical
response, good overall
survival and low rates of
hepatic encephalopathy
【39】

 

BCS肝移植

       尽管进行内科治疗和TIPSS,BCS患者由于临床恶化需要进行肝移植占10%~40%[48]。在欧洲由于BCS需要进行原位肝移植约为1%。移植伴随感染、恶性肿瘤和肾功能的损害,因此肝移植应该保留给那些TIPSS术后或爆发性肝功能衰竭临床情况恶化的患者。移植前必须考虑肿瘤发生(由于使用免疫抑制剂可能增加发生率)和潜在血液性疾病的自然病史。

       外科手术可能并发广泛的血栓形成和纤维化,而且尾叶肥大可能干扰肝移植“背驮技术”的实施。肝移植的结果与其它疾病进行的肝移植类似[49]。抗凝治疗一般术后不久开始并持续终生。BCS的早期复发归因于此期间的可能不足。是否终生抗凝,特别是肝移植术后被认为此疾病被治愈(蛋白C或蛋白S缺乏症)的情况下,仍然不清楚。

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