Budd-Chiari Syndrome In Children Research Articles

Budd-Chiari syndrome in children: Challenges and outcome

Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study

Utility of Hepatic 2D Shear-Wave Elastography in Monitoring Response to Image-Guided Intervention in Children With Chronic Budd-Chiari Syndrome: A Prospective Study.

BACKGROUND. Image-guided interventions for Budd-Chiari syndrome (BCS) reduce hepatic congestion, thereby reducing liver stiffness. Liver stiffness measurement (LSM) by means of ultrasound may provide a noninvasive marker for assessing treatment response. OBJECTIVE. The purpose of this study was to assess, with attention to changes in LSMs in patients with disease recurrence, the utility of 2D shear-wave elastography (SWE) for monitoring response to image-guided intervention in children with BCS. METHODS. This prospective study included children with chronic BCS and planned image-guided intervention. Color Doppler ultrasound (CDUS) and 2D SWE were performed at baseline; 24 hours, 1 month, and 3 months after intervention; and every 3 months thereafter or when recurrence was clinically suspected. Eighteen children underwent liver biopsy at intervention for fibrosis staging according to the Metavir criteria. Disease recurrence was diagnosed with CDUS. Statistical evaluation was performed by means of nonparametric tests. RESULTS. A total of 32 children (28 boys, four girls; mean age, 9 years; range, 3-14 years) were included. The median LSM at baseline was 43.7 (interquartile range [IQR], 33.0-65.4) kPa, at 24 hours was 22.5 (IQR, 16.8-32.0) kPa, at 1 month was 18.7 (IQR, 14.2-32.0) kPa, and at 3 months was 16.7 (IQR, 11.5-22.5) kPa (p = .001 for all postintervention time points vs baseline). Nine (28.1%) patients had a recurrence at a mean of 4 months after intervention. In one patient with recurrence, LSM was higher at 24 hours (52.3 kPa) than at baseline (44.2 kPa). In the other eight patients with recurrence, LSM was increased at recurrence compared with the prior postintervention LSM (median absolute increase, 11.0 [IQR, 6.1-24.4] kPa). Fibrosis stage was not significantly correlated with baseline LSM (r = 0.11 [95% CI, -0.37 to 0.54]; p = .51) or LSM 24 hours after intervention (r = 0.39 [95% CI, -0.11, 0.73]; p = .11). CONCLUSION. LSMs decreased significantly after image-guided intervention for chronic BCS in children, exhibiting a maximal decrease 24 hours after intervention. Disease recurrence was typically associated with an increase in LSM compared with the patient's prior measurement. CLINICAL IMPACT. LSM obtained with 2D SWE may serve as a useful quantitative adjunct to CDUS in monitoring children with chronic BCS for disease recurrence after percutaneous interventional treatment.

Budd Chiari syndrome in children: experience from a single center

Background and Aim: Budd Chiari syndrome (BCS) is obstruction of venous outflow at any level from the small hepatic veins to right atrium. The etiologies of BCS are multifactorial, prothrombotic conditions are most common. Ultrasonography with Doppler, CT, and MR angiography are diagnostic. Treatment modalities are anticoagulants, angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), thrombectomy, and liver transplantation. We aimed to analyze demographic, clinical, therapeutic, and interventional details along with outcomes of BCS patients.

Budd Chiari Syndrome in Children: Experience From a Single Center

Background: Budd Chiari syndrome (BCS) is obstruction of venous outflow at any level from the small hepatic veins to the right atrium. The etiologies of the BCS are multifactorial, prothrombotic conditions are most common. Ultrasonography with doppler, CT and MR angiography are diagnostic. Treatment modalities are anticoagulants, angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), thrombectomy and liver transplantation.

Budd Chiari Syndrome in Children

Budd-Chiari syndrome (BCS) is a rare disorder presenting with hepatic venous outflow obstruction from the level of the hepatic veins to the junction of the inferior vena cava (IVC) with the right atrium. It is rare in infants and children so, delay in diagnosis or misdiagnoses are very commonly seen. It results from either primary or secondary cause. Hypercoagulable state can be identified in majority of the cases. The clinical presentations may vary from asymptomatic to fulminant liver failure. A high index of suspicion and imaging findings may help in early diagnosis of this condition.Doppler ultrasonography can be the initial diagnostic investigation of choice.The treatment options include medical therapy along with surgical measures including liver transplantation for end-stage liver disease.
 KYAMC Journal Vol. 11, No.-1, April 2020, Page 46-49

Promising Advances in Budd-Chiari Syndrome in Children

Promising Advances in Budd-Chiari Syndrome in Children

39. Budd-Chiari syndrome in children – outcome following radiological intervention

Background and Aims: Hepatic vein outflow tract obstruction (HVOTO) or Budd-Chiari syndrome is a vascular disorder of liver with occlusion of hepatic veins with or without inferior vena cava (IVC). HVOTO in children is described predominantly in the Asian children. There is limited data on clinical profile and outcome of these cases. We aim to describe the etiological spectrum, clinical profile and outcome of children with HVOTO. Methods: From June 2010 to April 2018, all children under-18 years of age with HVOTO were enrolled. Diagnosis was based on ultrasound Doppler with or without contrast enhanced CT. Results: There were 36 children (25 males, 69%). Median age of presentation and duration of symptoms were 10 years and 12 months, respectively. Abdominal distension (85%) and pain (58%) were the commonest symptoms. Seven (19%) presented with predominant portal hypertension in the form of variceal bleeding while 6 (17%) had encephalopathy. Common findings at admission were ascites (69%), edema (47%) and jaundice (42%). Median Child Pugh, PELD and MELD scores at admission were 8, 8 and 13, respectively. Radiologically, there was isolated involvement of all 3 hepatic veins in 30 (85%), 5 (15%) had associated IVC occlusion; only 1 patient had isolated IVC block. Inherited prothrombotic mutations were found in 9 out of 22 children – MTHFR (6 heterozygous, 1 homozygous), 1 factor-V Leiden and janus kinase (JAK)-2 mutation in 1. Two children died before intervention due to acute kidney injury and multiorgan dysfunction. Radiological interventions were performed in 23 children-direct intrahepatic portosystemic shunt (DIPSS) (n = 8), transjugular intrahepatic portosystemic shunt (TIPSS)(n = 4), and angioplasty of right hepatic vein (RHV) (n = 6), left hepatic vein (LHV) (n = 1) or IVC (n = 4). Two patients had reversal of fulminant presentation following a successful intervention (DIPSS 1, LHV angioplasty 1). There were 9 post procedure complications seen in 7 children – congestive cardiac failure, bleeding (hemoperitoneum 2, hemobilia 1), encephalopathy, stent infection, sepsis, liver dysfunction in 3, 3, 1, 1 and 1 child, respectively. One child died of hemoperitoneum, had liver dysfunction post-DIPSS. Immediate success was seen in 21 – however, 5 (24%) required revision of stent due to stent block (4 DIPSS and 1 LHV angioplasty), 2 were related to non-compliance to anti-coagulants. Eleven children were managed conservatively in view of development of good collateral circulation (n = 9) or spontaneous recanalization (n = 1) and refusal (n = 1). Combined pre and post procedure mortality rate of 8.3% (3/36) was seen in this cohort. Conclusions: HVOTO presents as acute or chronic liver dysfunction with features of portal hypertension. Hepatic vein involvement is the commonest site of obstruction. Establishment of porto-systemic blood flow is the treatment of choice. Radiological therapeutic interventions are feasible, safe and effective in children with HVOTO even with fulminant presentation. Post procedure recurrence is seen in one-fourth. The authors have none to declare.

Doppler ultrasound in the diagnosis of Budd-Chiari syndrome in children after split liver transplantation

To assess the capabilities of a velocity ratio>3 for the diagnosis of Budd-Chiari syndrome (BCS) in children after split liver transplantation using Doppler ultrasonography (DUS). A total of 28 children who underwent liver transplantation using a split procedure were included. There were 11boys and 17girls with a mean age of 3.8years (range: 0.7-12years). Velocity ratio between blood velocity upstream of the anastomosis and that at the level of the inferior vena cava anastomosis was calculated. Sensitivity, specificity and accuracy of DUS for the diagnosis of BCS were estimated using a velocity ratio>3. Eight children (8/28; 29%) had BCS and 20 (20/28; 71%) did not have BCS using the standard of reference. A velocity ratio>3 on DUS yielded 88% sensitivity (95% CI: 53-98%), 80% specificity (95% CI: 58-92%) and 82% accuracy (95% CI: 64-92%) for the diagnosis of BCS. A velocity ratio>3 on DUS is a reliable finding for the diagnosis of BCS in children after split liver transplantation.

Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children.

Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention. PSS analysed included Paediatric end-stage liver disease (PELD), Rotterdam, BCS-Transjuglar intrahepatic Portosystemic shunt (BCS-TIPS) index, Zeitoun, Child-Pugh and Model for end-stage liver disease. Of 113 BCS children, 48 children underwent 53 successful primary RI. Actuarial probability of vascular patency was 87% at 1year and 82% at 5years follow-up. Four groups (SI: n=40, PO: n=7, NU: n=13, DBI: n=6) were analysed. Univariate analysis showed pre-intervention PELD score [PO: 11 (-1-23) vs SI: 2 (-8-25), P=.009] with a cut-off of 4 (AUC: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (NU vs DBI), multivariate analysis demonstrated that Zeitoun score predicted death independently (OR 15.4, 95% CI: 1.17-203.56, P=.04) with a cut-off of 4.3 (AUC: 0.923, 83% sensitivity and 77% specificity). Children with BCS have a favourable long-term outcome. Among those undergoing RI, pre-intervention PELD score determines the outcome. Survival is determined by Zeitoun score in those unintervened.

Primary Budd-Chiari Syndrome in Children: King's College Hospital Experience.

Primary Budd-Chiari syndrome is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary Budd-Chiari syndrome presenting from January 2001 to November 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2, and 1 patients, respectively; 1 child underwent bone marrow transplantation following transjugular intrahepatic portosystemic shunts and 1 child was managed only medically. After liver transplantation, one child died 3 years later as a result of subarachnoid haemorrhage, whereas others remain well at a median follow-up of 6 years. Despite high morbidity, the disease can have a good long-term outcome with a multidisciplinary approach.

Prognostic Scoring Systems and Outcome of Radiological Intervention of Chronic Budd-Chiari Syndrome in Children: A First Time Application

Prognostic Scoring Systems and Outcome of Radiological Intervention of Chronic Budd-Chiari Syndrome in Children: A First Time Application

Ultrasound and Doppler features of Budd-Chiari syndrome in pediatric population.

Budd-Chiari syndrome (BCS) occurs as a result of hepatic venous outflow obstruction. In the pediatric population, the etiologies vary as compared with the adult population. Decompensation can occur faster in this set of patients. Ultrasound and Doppler represent important imaging modalities for diagnosing BCS in children. The imaging features differ depending upon the level of obstruction, acuteness of the condition, and secondary decompensation. Caudate lobe hypertrophy is a salient feature. Obstruction at the level of hepatic veins may be manifested by ostial narrowing, echogenic thrombus, and altered flow patterns in the form of turbulent flow, nonvisualization of the veins, or reversal of flow. Obstruction in the inferior vena cava may present as an echogenic web, membrane, or thrombus with turbulent flow to absent flow within depending upon the degree of luminal compromise. Collateral formation is an important distinctive feature of subacute and chronic BCS. Collaterals that develop may be of intrahepatic or extrahepatic type. Secondary signs of liver failure would be present in late stages. Understanding the clinical presentation and imaging features can help in achieving the correct diagnosis because an early diagnosis of the disease will impact patient management.

Budd-Chiari syndrome in children and adolescents: therapeutic radiological intervention

Due to its minimal-invasive approach, endovascular procedure had replaced surgery in treating Budd-Chiari syndrome (BCS). The interventional therapy was a safe and effective treatment in adults with BCS and the cure rate was high. However Budd-Chiari syndrome in children and adolescents is rare. Published literature on interventional procedure for Budd-Chiari syndrome in children and adolescents is scarce. The aim of the study was to present results of percutaneous transluminal angioplasty (PTA) and stents placement in children and adolescents with BCS and to evaluate the efficacy and safety in these patients of this approach. Twenty-five patients [16 boys and 9 girls; average age of (14.5 ± 3.4) years old; age ranged from 5 to 17 years] with Budd-Chiari syndrome who were hospitalized from December 1990 to August 2012 were presented. All of them were diagnosed by color Doppler ultrasound scan while 12 of them had magnetic resonance venography (MRV) scan. All of the patients had undergone angiographic examination. Four cases with membranous obstruction of the inferior vena cava (IVC) were treated with PTA. One case with segmental block of IVC was treated with PTA and stent placement. Five cases with membranous obstruction of IVC and hepatic vein (/and accessory hepatic vein) were treated with PTA. Among 8 cases with membranous obstruction of hepatic veins, 6 cases were treated with PTA and the others with PTA and stent placement. Among 4 cases with blocks of 3 hepatic veins (HVs), one was treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement and the other one was unsuccessful. Three cases with obstruction of HV and accessory HV (AHV) were treated with PTA. Totally, 24 patients were treated with interventional approach and followed up. The procedure was successful in 24 patients. The involved veins (hepatic veins or IVC) were patented after interventional procedure. The pressure of hepatic vein was (42.1 ± 4.2) cm H2O (37-50 cm H2O) (1 cm H2O = 0.098 kPa) before the interventional therapy, while it was (17.3 ± 3.3) cm H2O (14-26 cm H2O) after it. The pressure of IVC was (30.6 ± 2.9) cm H2O (26-36 cm H2O) before the interventional therapy, while it was (18.8 ± 4.2) cm H2O (15-26 cm H2O) after it. The symptoms and signs vanished instantly after interventional procedure. There were no procedure-related complications. The rate of overall initial cure was 96%. The patients were followed up for a mean of 25.8 months (range 6 months to 8 years). Seven cases developed restenosis after first procedure. Five of them were treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement. All of the involved veins were patented again. Clinical symptoms were relieved. There were no procedure-related complications as well. The interventional procedure in children and adolescents with BCS is the same as in adults. Radiological therapeutic intervention is efficacious and safe in children and adolescents with BCS.

Budd Chiari Syndrome in Children: Clinical Features, Radiological Intervention and Outcome Presidential Poster

Purpose: “Radiological intervention” is now the first line of therapy in adults with Budd-Chiari syndrome (BCS). Published literature on pediatric BCS is scarce. We evaluated the clinical profile and role of therapeutic radiological intervention in children with BCS. Methods: 46 children (29 boys, median age 10.5 [2-16] y) diagnosed as BCS were evaluated. Standard medical therapy was given to all patients. Radiological intervention {angioplasty (hepatic vein [HV]-3), stenting (HV-18, inferior vena cava [IVC]-5), transjugular intrahepatic portosystemic shunt [TIPS] (n-3)} was done in 25 cases. All patients subjected to radiological intervention were started on long-term anticoagulation. Clinical, biochemical and radiological follow-up was done. Results: Doppler ultrasonography was diagnostic in 96% cases. All patients had chronic BCS, with hepatomegaly in 84.8%, ascites in 82.6%, splenomegaly in 69.6%, prominent abdominal veins in 69.6%, variceal bleed in 34.8% and jaundice in 19.6% cases. Hepatic vein (all three) was the commonest site of block (n=33, 72%) followed by combined HV and IVC block (n=11, 24%) and isolated IVC block (n=2, 4%). 8/12 (75%) cases had abnormal procoagulant workup. There was no difference in the clinical presentation and liver functions of the patients given only medical therapy as compared to those who underwent radiological intervention except for higher bilirubin in the nonintervention group. Radiological intervention was technically successful in 100%. Clinical and biochemical improvement was seen in the intervention group. Complications included neck hematoma and haemorrhagic ascites in 1 patient each. One child in the intervention group (post TIPS sudden cardiac event) and 2 in non-intervention group (end stage liver disease-1, head injury-1) died. Stent was patent in 15/20 (75%) children in follow-up [median 6.5mo (15 days to 7.2 y)]. Two of the 5 cases with re-stenosis have undergone repeat radiological procedures succesfully. Only 2/21 (10%) patients in the nonintervention group showed improvement. Conclusion: Hepatic vein block is the commonest site of block seen in 72% cases. Therapeutic radiological intervention is a technically feasible, safe and effective therapeutic modality for children with BCS.

50 Years Ago in The Journal of Pediatrics: Budd-Chiari Syndrome

50 Years Ago in The Journal of Pediatrics: Budd-Chiari Syndrome

The Budd-Chiari syndrome in children: the spectrum of management

The Budd-Chiari syndrome (BCS), characterized by hepatic venous outflow occlusion, is rare in children. We describe the cases of 3 patients with BCS who presented at different stages of liver dysfunction that required tailored management. A 10-year-old boy who presented with a weeklong history of abdominal discomfort received an early diagnosis. Treatment with thrombolytic therapy and adjunctive percutaneous interventional radiologic management led to a favorable outcome in this patient. A 2-year-old girl referred with a 6-week illness not amenable to thrombolytic therapy was managed with a pericardial patch atriocavoplasty to restore hepatic venous outflow tract patency. The third patient, a 5-year-old girl, presented with established cirrhosis and massive ascites. After we controlled the ascites with an extracorporeal shunt system and nutritional resuscitation, we conducted an orthotopic liver transplantation on this patient. The stage of disease at diagnosis influences the management strategy, and an early diagnosis offers patients the best possible chance of cure without major surgery. However, a later presentation can still be successfully managed by more radical surgical interventions. The aims of this case report were to emphasize the importance of a high index of suspicion in the diagnosis of pediatric BCS and to highlight the importance of early referral to a specialized pediatric liver unit with the necessary facilities to deal with the medical and surgical aspects of management.

Surgical Therapy and Nursing of Budd-Chiari Syndrome in Children

目的 提高小儿布-加综合征(Budd-Chiari Syndrome,BCS)的治愈率,预防并发症的发生.方法 总结25例小儿BCS的外科治疗与护理.选择合适的治疗方法,术前给予心理支持和术前充分准备,术后严密地监测生命体征的变化,做好出院健康指导.结果 25例小儿BCS围手术期无并发症出现 ,均痊愈出院.结论 及时、全方位的护理是手术成功的重要保障。

LOBAR ATROPHY CAUSED BY COMBINED PORTAL AND HEPATIC VEIN THROMBOSIS IN CHILDREN

188 Structural and functional integrity of the liver parenchyma is dependent on intact vascular supply. Non-cirrhotic portal hypertension results from hepatic vascular injury: among its various etiologies, portal vein thrombosis with cavernous transformation of the portal vein is most common in children. Hepatic vein thrombosis causes severe parenchymal injury but is much less common in the pediatric age group. We hypothesize that combined portal vein and hepatic vein thrombosis would lead to significant macroscopic loss of liver parenchyma. We describe this condition in three children. Patient 1: 17 year-old male with eosinophilic vasculitis developed acute Budd-Chiari syndrome with liver failure followed by superior mesenteric arteritis with bowel infarction several days later. After recovery, sonography, showed persistent thrombosis of R hepatic vein and R portal vein branches, with very small R lobe of liver and an enlarged L lobe. No clinically significant portal hypertension in 7 years' follow-up. Patient 2: 4 year-old male had bilateral pleural effusions at birth requiring prolonged perinatal hospitalization with umbilical vein catheterization; a clot in the inferior vena cava (IVC) was found on day 11 of life. Subsequent sonography disclosed segmental thrombosis of L portal vein, small L hepatic vein, and small L lobe of liver and enlarged R lobe. Asymptomatic in 4 years' follow-up. No evidence of thrombotic tendency. Patient 3: 7 year-old female with congenital heart disease and asymmetric leg length was incidentally found to have abnormal hepatic findings on sonography. MR showed absent L portal vein, absent or markedly hypoplastic L lobe, and abnormal confluence and course of hepatic veins; IVC was patent. Asymptomatic before diagnosis and after 1 year's follow-up. Summary: Combined loss of vascular outflow and inflow to a single lobe leads to lobar atrophy. Clinically-significant portal hypertension may not develop. Since hepatic vein thrombosis causes parenchymal extinction, it is likely the dominant lesion. This may be a benign variant of Budd-Chiari syndrome in children.

Surgical repair in children with the Budd-Chiari syndrome

Membranous obstruction of the inferior vena cava at the level of the diaphragm is a rare cause of Budd-Chiari syndrome in children. Medical therapy usually fails. Surgical intervention aims at reestablishing patency of the inferior vena cava and hepatic venous outflow to the right atrium. We report on the management of this condition in 19 children of whom 7 were treated surgically. Indications for operation were persistent ascites, deteriorating liver function, and hepatic and inferior vena caval obstruction without significant collateral circulation. Three pathologic types were identified by ultrasonography and cavography and were confirmed at operation. These were type I (4 cases), with a thin membrane occluding the inferior vena cava at the level of the diaphragm; type II (12 cases), with segmental fibrotic obstruction of the inferior vena cava with variable involvement of hepatic veins; and type III (3 cases), with complete absence or nonvisualization of the inferior vena cava. All procedures were done with an extended midline sternotomy incision, cardiopulmonary bypass, core cooling to 16 degrees to 20 degrees C, and periods of circulatory arrest. Type I lesions necessitated membranectomy; type II lesions necessitated transcaval resection of the occluded confluence of the inferior vena cava and the hepatic vein with repair of the defect with an autogenous pericardial patch. One type II lesion, in addition, called for use of a 14 cm polytetrafluoroethylene tube graft to restore inferior vena caval flow. After the operation, marked clinical improvement was observed with an immediate reduction in liver and spleen size and resolution of ascites. Repeat cavography 10 to 30 days after the operation revealed complete patency in four cases and residual stenosis, which required transiliac balloon angioplasty to normalize the inferior vena cava/right atrial pressure gradient, in 3 cases. Thus eventual relief of hepatic venous outflow obstruction and inferior vena caval flow was restored in all cases. We advocate transcardiac membranectomy and pericardial patch grafting for symptomatic and deteriorating membranous obstruction of the inferior vena cava in children.