Waheed Nadia, Cheema Huma Arshad, Suleman Hassan, Mushtaq Iqra, Fayyaz Zafar, Anjum Nadeem
Department of Pediatric Gastroenterology and Hepatology, The Children Hospital, Lahore.
J Coll Physicians Surg Pak. 2018 May;28(5):344-347. doi: 10.29271/jcpsp.2018.05.344.
To differentiate between clinical and demographic spectrum, and outcome in hepatovenocaval syndrome (HVCS) and Budd-Chiari syndrome (BCS).
Descriptive study.
Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital, Lahore, from January 2014 to January 2017.
All children less than 18 years of age, presenting with ascites and visible veins over abdomen, flanks and back were enrolled in the study. Real time Doppler Ultrasonogram was performed in all children for documentation of intra- hepatic part of IVC obstruction along with or without hepatic venous obstruction. Children meeting inclusion criteria underwent liver profile, coagulation profile, diagnostic paracentesis for SAAG gradient, and Gadolinium enhanced multiphasic MR scan. Liver biopsy and venography was performed in selected patients.
A total of 92 children presented with ascites, among them 58 children met our inclusion criteria. Intrahepatic IVC obliteration, i.e. HVCS, found in 67% (n=39) and hepatic venous outflow obstruction, i.e. BCS was found in 33% (n=19) children. Children with BCS were older than HVCS with mean age of 9.5 ±2.58 versus 4.12 ±0.977 years. HVCS group had 14 boys and 24 girls with a ratio of 1:1.8, while BCS had a ratio of 1:0.9 with 10 boys and 9 girls. No etiological factor was found for HVCS, while most of patients with BCS had a procoagulant disorder. Caudate lobe hypertrophy was a consistent feature in BCS, while IVC obstruction was found in HVCS persistently. Orthotopic liver transplant was needed in three cases (7.6%) of HVCS and four (20.96%) of BCS cases. Antibiotic therapy has a good role in HVCS, while anticoagulation and diuretics had good result in BCS.
Hepatovenocaval syndrome (HVCS) mostly affected younger children, especially girls. BCS usually affected older age groups with pro-coagulant disorders who responded to anticoagulation and diuretic. Further studies are needed to compare both conditions.
鉴别肝腔静脉综合征（HVCS）和布加综合征（BCS）的临床及人口统计学特征谱以及预后情况。
描述性研究。
2014年1月至2017年1月，拉合尔儿童医院小儿胃肠病学、肝病学与营养科。
纳入所有18岁以下出现腹水且腹部、侧腹及背部可见静脉的儿童。对所有儿童进行实时多普勒超声检查，以记录下腔静脉肝内段梗阻情况以及有无肝静脉梗阻。符合纳入标准的儿童接受肝功能检查、凝血功能检查、诊断性腹腔穿刺以测定血清腹水白蛋白梯度（SAAG），并进行钆增强多期磁共振扫描。对部分患者进行肝活检及静脉造影。
共有92名出现腹水的儿童，其中58名符合我们的纳入标准。67%（n = 39）的儿童发现肝内下腔静脉闭塞，即HVCS；33%（n = 19）的儿童发现肝静脉流出道梗阻，即BCS。BCS患儿的年龄大于HVCS患儿，平均年龄分别为9.5±2.58岁和4.12±0.977岁。HVCS组有14名男孩和24名女孩，比例为1:1.8，而BCS组男孩与女孩的比例为1:0.9，分别为10名男孩和9名女孩。未发现HVCS的病因，而大多数BCS患者存在促凝障碍。尾状叶肥大是BCS的一个持续特征，而HVCS持续存在下腔静脉梗阻。3例（7.6%）HVCS患者和4例（20.96%）BCS患者需要进行原位肝移植。抗生素治疗对HVCS有较好作用，而抗凝和利尿剂对BCS效果良好。
肝腔静脉综合征（HVCS）主要影响年幼儿童，尤其是女孩。BCS通常影响年龄较大且患有促凝障碍的人群，这些人对抗凝和利尿剂有反应。需要进一步研究以比较这两种情况。
