Budd-Chiari syndrome in youth: clinical features and interventional therapy
10.3760/cma.j.issn.1007-631X.2013.09.011
- VernacularTitle:青少年布加综合征临床特点及介入治疗
- Author:
Lei WANG
;
Maoheng ZU
;
Fei TENG
;
Qianjin HUA
;
Xiaoyu WANG
- Publication Type:Journal Article
- Keywords:
Budd-Chiari syndrome;
Radiology,interventional;
Hypertension,portal
- From:
Chinese Journal of General Surgery
2013;28(9):686-689
- CountryChina
- Language:Chinese
-
Abstract:
Objective To present the clinical features and to evaluate interventional therapy for Budd-Chiari syndrome in Chinese youth.Methods From January 1990 to April 2012,227 cases who hospitalized at the age ＜ 29 underwent color Doppler ultrasound scan and digital subtraction angiography (DSA).87 cases were with occlusive inferior vena cava (IVC type),105 cases with occlusive hepatic veins (HV type) and 35 cases with occlusive inferior vena cava and hepatic veins (MIX type).The occlusive veins were opened by percutaneous transluminal angioplasty (PTA),endovascular stent placement,intravenous catheter thrombolysis or combination.Postoperative anticoagulation was given to all patients.Results The symptoms and signs of portal hypertension disappeared or were alleviated in successful cases.Technical success was achieved in 210 patients.The success rate was 100％ in IVC type,85.7％ in HV type and 94.3％ in MIX type.IVC pressure decreased from (26.52 ± 8.16) cm H2O to (14.28 ±4.08) cmH2O(P ＜ 0.05) and HV pressure dropped from(35.70 ± 13.26) cm H2O to(18.36 ±8.16) cm H2O (P ＜0.05).Restenosis or occlusion was found in 21.4％ (45/210) patients after a follow-up of 1 month to 15 years.The rate was 13.8％ (12/87) in IVC type,31.1％ (28/90) in HV type and 15.2％ (5/33) in MIX type.These patients were managed by interventional procedures.Technical successwas achieved in 44 cases with restenosis.Conclusions Hepatic vein occlusion was the most common type of BCS in Chinese youth.The symptoms and signs of portal hypertension were the initial clinical manifestations.Postoperative recurrence rate in HV type was higher than that in the other two types.
