Variceal Bleeding In Children Research Articles

Endoscopic variceal ligation combined with sclerotherapy for management of gastroesophageal variceal bleeding in pediatric patients: a single-center retrospective study.

Portal hypertension (PH) frequently gives rise to severe and life-threatening complications, including hemorrhage accompanied by the rupture of esophageal and gastric varices. In contrast to the guidelines for the management of PH in adults, the optimal endoscopic management of variceal bleeding for secondary prophylaxis in children remains unclear. The present study evaluated the efficacy and safety of endoscopic variceal ligation (EVL) and endoscopic sclerotherapy (EST) to control gastroesophageal variceal bleeding in children. This retrospective study included children with gastroesophageal variceal bleeding who underwent EST or EVL at Xinhua Hospital, Shanghai Jiaotong University School of Medicine, between February 2013 and March 2020. Short-term hemostasis rate and long-term rebleeding rate were evaluated. Adverse events related to the procedures, such as esophageal ulcer, esophageal stricture, abnormal embolization, pneumonia and perforation, were also recorded. EVL (n = 8) and EST (n = 13) were performed successfully in all pediatric patients diagnosed with moderate to severe esophageal varices concurrent with gastric varices. Hemostasis was achieved during episodes of upper gastrointestinal bleeding. The mean volume of each single aliquot of cyanoacrylate injected was 0.3 ± 0.1 ml (range: 0.1-0.5 ml). Varices were eradicated in six (75%) of the eight patients who underwent EVL after a median 2 (range: 1-4) procedures and a median time of 3.40 months (range: 1.10-13.33 months). Eleven (52.4%) of the 21 patients developed rebleeding events, with the mean duration of hemostasis being 11.1 ± 11.6 months (range 1.0-39.2 months). No treatment-related complications, for example, distal embolism, occurred except for abdominal pain in one patient (4.8%). EST, alone or in combination with EVL, is an effective and safe method of managing gastroesophageal variceal hemorrhage in children undergoing secondary prophylaxis.

Analysis of the effectiveness of the method of primary bleeding prevention in the treatment of portal hypertension in children

Objective. To evaluate the role of primary prevention of variceal bleeding in children with prehepatic portal vein obstruction. Materials and methods. The single–centre prospective cohort clinical trial involved 120 patients with extrahepatic obstruction of the hepatic portal vein who underwent primary or secondary prevention of variceal bleeding in 2016–2021. Laboratory, ultrasound, and endoscopic data were collected and evaluated before and after prophylactic treatment. Results. Episodes of variceal bleeding were observed in 3 (5.78%) patients who received primary prophylaxis, and rebleeding occurred in 19 (27.94%) patients who received secondary prophylaxis (p=0.013). Of the 53 (44.17%) patients who underwent endoscopic treatment, variceal bleeding occurred in 3 (5.66%), and 19 (28.36%) of the 67 (55.83%) patients who underwent surgery experienced rebleeding (p=0.001). Esophageal varices eradication was achieved in 83 (69.17%) patients: 44 (53.01%) patients who received primary prevention and 39 (46.99%) patients who received secondary prevention. Bleeding episodes occurred less frequently after eradication (p<0.001). After primary prevention, varicose veins disappeared more often than after secondary prevention (p=0.003). The incidence of varicose veins recurrence after achieving eradication did not differ in the groups (p=0.51). Conclusions. Primary prevention can reduce the high risk of bleeding in prehepatic portal vein obstruction. Prophylactic endoscopic ligation is important in the treatment of portal hypertension in children, which, together with portosystemic shunting, can potentially improve the results of preventive treatment.

The role of variceal bleeding primary prophylaxis in the management of extrahepatic portal vein obstruction in children

Extrahepatic portal vein obstruction is a major cause of pediatric symptomatic portal hypertension and can lead to profuse variceal hemorrhaging. Purpose - to evaluate the role of primary prophylaxis of the variceal bleeding in children with extrahepatic portal vein obstruction. Materials and methods. A one-center prospective cohort clinical study involved 120 patients with extrahepatic portal vein obstruction who underwent primary or secondary variceal bleeding prophylaxis in 2016-2021. The laboratory, ultrasound, and endoscopy data before and after treatment were collected and evaluated. Results. Variceal bleeding episodes occurred in 5.8% (n=3) of patients who underwent primary prophylaxis and rebleeding occurred in 27.9% (n=19) of those who underwent secondary prophylaxis. Bleeding episodes occurred less frequently in patients from primary prophylaxis group (p=0.013). In patients treated endoscopically (n=53, 44.17%) Variceal bleeding appeared less frequently (n=3, 5.66%) after treatment compared to patients who underwent surgery (n=67 (55.83%), 19 had rebleeding (28.35%)) (p=0.001). In 69.17% patients (n=83) esophageal varices eradication was achieved: in 53.01% (n=44) patients from the primary prophylaxis group and 46.99% (n=39) from the secondary prophylaxis group. Bleeding episodes occurred less frequently after the eradication achievement (p<0.001). The primary prophylaxis led to varices eradication more often than secondary (p=0.003). The varices recurrence episodes rates after the eradication achievement were not different between groups (p=0.51). Conclusions. Primary prophylaxis can reduce the bleeding risk in extrahepatic portal vein obstruction with high risk of variceal bleeding. The prophylaxis by endoscopic variceal banding is an important bridge in the treatment for pediatric patients with portal hypertension that along with portosystemic shunting could potentially improve prophylactic treatment results. The Committee on Clinical Investigation of Bogomolets National Medical University approved this study (Protocol No.141, 27.01.2021). All the studies were conducted according to implemented guidelines in consideration of GCP-ICH and Declaration of Helsinki. The written informed consent of all participants’ parents/guardians was achieved. No conflict of interests was declared by the authors.

S1476 Efficacy and Clinical Outcomes of Endoscopic Variceal Ligation With N-butyl-2-cyanoacrylate in Pediatric Patients in a Tertiary Care Center—A Retrospective Study

Introduction: Acute variceal bleeding (AVB) from gastroesophageal varices is a life-threatening complication in patients with portal hypertension. Bleeding from gastric varices is less common and treatment options are not standardized. Endoscopic variceal obliteration (EVO) with N-butyl-2-cyanoacrylate glue or balloon-occluded retrograde transvenous obliteration (BRTO) are the 2 most common treatment modalities and information on these are limited to adult series. There is paucity of information about gastric varices and its treatment in children. Methods: We conducted a retrospective cohort study among pediatric patients (1-18 years) who presented to our center with AVB from gastric varices. Between 2011-2021, we identified 538 among 4539 cases (12%) of portal hypertension with bleeding from gastric varices. Of these only 14 cases (2.6%) had a diagnosis of gastric variceal bleeding in children. Details of 13 patients were extracted with non-availability of records of 1 patient. Cyanoacrylate glue in 0.5-1 ml were injected in to the gastric varices. Patients were evaluated for efficacy, safety and complications associated with the treatment. Endoscopy was repeated after 3 weeks for assessment of variceal size and need for repeat glue injection. Results: The mean age of the patients was 13.3±5.2, with males (54%) and females (46%). The mean age at first presentation to hospital with gastrointestinal bleeding was 8.7±3.6, with hematemesis (62%) being the most common presentation. On endoscopy, 8 patients had type 1 gastro-esophageal varices and 3 patients had type 2 gastroesophageal varices. All 13 patients had stigmata of recent gastric variceal bleeding. The most common cause of portal hypertension was extra hepatic portal venous obstruction (EHPVO) (54%). The mean age at glue therapy treatment was 11.3±5.3. While immediate hemostasis was achieved in all 13 patients (100%) with gastric variceal bleeding, re-bleeding was seen among 3 patients, which occurred after 1 month of glue therapy. 1-3 sessions were required for obliteration of gastric varices. There was one case of death among the 13 children: a patient with decompensated Wilson’s disease. We did not encounter complications like anaphylactic shock, treatment-associated infection, gastric perforation, and distant emboli. Conclusion: The experience from our series suggests that endoscopic vascular obliteration using cyanoacrylate glue injection is safe and effective in children with gastric variceal bleeding.

Abstract No. 119 Transjugular intrahepatic portosystemic shunt for pediatric portal hypertension: a meta-analysis

The purpose of this study is to review and characterize the feasibility, safety and evidence supporting the use of TIPS shunt in the pediatric population by conducting a meta-analysis. Cochrane Database and PubMed were queried from establishment to April 2020 with the following keywords: “transjugular intrahepatic portosystemic shunt“ AND (“child” OR “pediatric”). The following criteria were adopted: 1. Patients< 18 years old received TIPS; 2. Sample size ≥5; 3. Studies with original data. The following data was extracted by two researchers: study name, year of publication, region, patient number, sex, age, indication of TIPS, portal HTN etiology, TIPS stent type, laboratory values, technical success, hemodynamic success, immediate clinical success, post-TIPS bleeding rate, survival/mortality, shunt patency, and complications. Statistical analysis was performed with STATA 15.1 (STATA Corp., College Station, TX). Pooled analysis was conducted. A random-effect model was used. Technical success, hemodynamic success, immediate clinical success, post-TIPS bleeding rate, survival, and shunt dysfunction were reported with 95% CI. Eleven observational studies totaling 210 subjects were included. The pooled technical success rate and hemodynamic success rate were 94% (95%CI: 86-99%) and 91% (95%CI: 82-97%), respectively; ongoing variceal bleeding resolved in 99.5% (95%CI: 97-100%); refractory ascites was improved in 96% (95%CI: 69-100%); post-TIPS bleeding rate was 14% (95%CI: 1-33%); 88% patients were alive or successfully received liver transplant (95%CI: 79-96%); Shunt dysfunction rate was 27% (95%CI: 17-38%). Hepatic encephalopathy occurred in 10.2% (19/187), though 84.2% (16/19) resolved with medical management. In conclusion, based on moderate level of evidence, TIPS is a safe and effective intervention that should be considered in pediatric patients with portal hypertensive complications. Our study demonstrates that TIPS is effective in achieving immediate clinical success and preventing future variceal bleeding in children. The risk of medically refractory hepatic encephalopathy is minimal. However, quality of published data is rather limited and future comparative studies are warranted.

Beta-blockers versus placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including haemorrhage from oesophageal and gastrointestinal varices. Variceal haemorrhage commonly occurs in children with chronic liver disease or portal vein thrombosis. Therefore, prevention is important. Band ligation, beta-blockers, and sclerotherapy have been proposed as alternatives for primary prophylaxis of oesophageal variceal bleeding in children. However, primary prophylaxis is not the current standard of care in paediatric patients because it is unknown whether those treatments are of benefit or harm when used for primary prophylaxis in children and adolescents. To determine the benefits and harms of beta-blockers compared with placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase, LILACS, and Science Citation Index Expanded (April 2020). We screened the reference lists of the retrieved publications and manually searched the main paediatric gastroenterology and hepatology conference (NASPGHAN and ESPGHAN) abstract books from 2008 to December 2019. We searched clinicaltrials.gov, the United States Food and Drug Administration (FDA), the European Medicines Agency (EMA), and the World Health Organization (WHO) for ongoing clinical trials. We imposed no language or document type restrictions on our search. We planned to include randomised clinical trials, irrespective of blinding, language, or publication status to assess benefits and harms. We included observational studies, retrieved with the searches for randomised clinical trials, for a narrative report of harm. We planned to summarise data from randomised clinical trials by standard Cochrane methodologies. We planned to asses risk of bias and use GRADE to assess the certainty of evidence. Our primary outcomes were all-cause mortality, serious adverse events and liver-related morbidity, and health-related quality of life. Our secondary outcomes were oesophageal variceal bleeding and adverse events not considered serious. We planned to use intention-to-treat principle. We planned to analyse data with RevMan Analysis. We found no randomised clinical trials that assessed beta-blockers compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We found four observational studies that reported on harms. As a systematic search for observational studies was not planned, we only listed the reported harms in a table. Randomised clinical trials assessing the benefits or harms of beta-blockers versus placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are lacking. Therefore, trials with adequate power and proper design, assessing the benefits and harms of beta-blockers versus placebo on patient-relevant clinical outcomes, such as mortality, quality of life, failure to control variceal bleeding, and adverse events are needed. Unless such trials are conducted and the results become published, we cannot make any conclusions regarding the benefits or harms of the two interventions.

Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.

Band ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.

Role of primary prophylaxis in preventing variceal bleeding in children with gastroesophageal varices

Primary endoscopic prophylaxis in pediatric gastroesophageal varices is not universally practiced. We aimed to determine the role of primary endoscopic prophylaxis in preventing variceal bleeding in gastroesophageal varices in children. We reviewed all children with gastroesophageal varices seen in our unit from 2000 to 2019. Primary prophylaxis was defined as endoscopic procedure without a preceding spontaneous bleeding and secondary prophylaxis as preceded by spontaneous bleeding. High-risk varices were defined as presence of grade III esophageal varices, cardia gastric varices or cherry red spots on the varices. Outcome measures (spontaneous rebleeding within 3 months after endoscopic procedure, number of additional procedures to eradicate varices, liver transplant [LT], death) were ascertained. Sixteen of 62 (26%) patients (median [± S.D.] age at diagnosis=5.0±4.3 years) with varices had primary prophylaxis, 38 (61%) had secondary prophylaxis while 8 (13%) had no prophylaxis. No difference in the proportion of patients with high-risk varices was observed between primary (88%) and secondary (92%; P=0.62) prophylaxis. As compared to secondary prophylaxis, children who had primary prophylaxis were significantly less likely to have spontaneous rebleeding (6% vs. 38%; P=0.022) and needed significantly fewer repeated endoscopic procedures (0.9±1.0 vs. 3.1±2.5; P=0.021). After 8.9±5.5 years of follow-up, overall survival was 85%; survival with native liver was 73%. No statistical difference was observed in the eventual outcome (alive with native liver) between primary and secondary (71% vs. 78%, P=0.78). Children with PHT who had primary prophylaxis had less subsequent spontaneous rebleeding and needed fewer additional endoscopic procedures as compared to secondary prophylaxis but did not have an improved eventual outcome. Screening endoscopy in all children who have signs of PHT and primary prophylaxis in high-risk esophageal varices should be considered before eventual LT.

Mortality, Risk Factors and Disparities Associated with Esophageal Variceal Bleeding in Children's Hospitals in the US

To use a large administrative database to determine the mortality, risk factors, and comorbidities of esophageal variceal bleeding in children. Retrospective cohort study using Pediatric Health Information System data from 50 tertiary children's hospitals in the US. International Classification of Diseases(ICD) codes (FY 2020 ICD-10 update and revision 10 of ICD-9) from 2004 through 2019 identified children 18years and younger with variceal bleeding and complications. Univariate analyses used the Student t -test for continuous variables (age) and the χ2 test for categorical variables (all others). A mixed-effects linear regression was performed for multiple variables. There were 1902 patients who had 3399 encounters for esophageal variceal bleeding. The mortality rate for variceal bleeding was 7.3%, increasing to 8.8% by 6weeks; any mortality during the study was 20.1%. Transfusion was required in 54.7% of encounters, and 42.6% were admitted to the intensive care unit. Variceal bleeding encounters were complicated by peptic ulcer disease (6.9%), bacteremia (11.4%), acute renal failure (5.1%), mechanical ventilation (18%), ascites (21.3%), and peritonitis (3.3%). Multivariable mixed-effects logistic regression showed that Black race (OR, 2.59; P<.001) or Hispanic ethnicity (OR, 2.31; P=.001), but not sex, household income, or insurance type, were associated with increased mortality. Bacteremia, peritonitis, mechanical ventilation, acute renal failure, and transfusion were associated with higher mortality (ORs of 2.29, 2.18, 1.93, 6.33, and 1.81, respectively; P<.001, .005, .011, <.001, and .005, respectively). The 6-week mortality rate for variceal bleeding in children is 8.8%. Black or Hispanic children are at higher risk of dying. Serious morbidities associated with variceal hemorrhage impact mortality. These data can inform consideration of prophylactic or therapeutic interventions for children at risk.

Band ligation versus sclerotherapy for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including haemorrhage from oesophageal and gastrointestinal varices. Variceal haemorrhage commonly occurs in children with chronic liver disease or portal vein obstruction. Prevention is therefore important. In adults, numerous randomised clinical trials have demonstrated benefits of non-selective beta-blockers and endoscopic variceal ligation as primary prevention in decreasing the risk of variceal haemorrhage. In children, band ligation, beta-blockers, and sclerotherapy have been proposed as alternatives for primary prophylaxis of oesophageal variceal bleeding. However, primary prophylaxis is not the current standard of care in children because it is unknown whether those treatments are of benefit or cause harm when used for primary prophylaxis of oesophageal variceal bleeding in children and adolescents. To determine the benefits and harms of band ligation versus sclerotherapy for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase, LILACS, and Science Citation Index Expanded (27 April 2020). We scrutinised the reference lists of retrieved publications, and performed a manual search from the main paediatric gastroenterology and hepatology conferences (NASPGHAN and ESPGHAN) abstract books from 2008 to 2019. We searched ClinicalTrials.gov, FDA, EMA, and WHO for ongoing clinical trials. There were no language or document type restrictions. We planned to include randomised clinical trials irrespective of blinding, language, or publication status for assessment of benefits and harms. If the search for randomised clinical trials retrieved quasi-randomised and observational studies, then we read them through to extract information on harms. We planned to summarise data from randomised clinical trials by standard Cochrane methodologies. We planned to assess risk of bias and use GRADE to assess the certainty of evidence per outcome. Our primary outcomes were all-cause mortality, serious adverse events and liver-related morbidity, and quality of life. Our secondary outcomes were oesophageal variceal bleeding and adverse events not considered serious. We planned to analyse data with intention-to-treat. We planned to use Review Manager 5 to analyse the data. We found no randomised clinical trials assessing band ligation versus sclerotherapy for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Randomised clinical trials assessing the benefits or harms of band ligation versus sclerotherapy for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are lacking. Therefore, trials with adequate power and proper design, assessing the benefits and harms of band ligation versus sclerotherapy on patient-relevant clinical outcomes such as mortality, quality of life, failure to control variceal bleeding, and adverse events are needed. Unless such trials are conducted and the results become published, we cannot make any conclusions regarding the benefits or harms of these two interventions.

Sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal and gastrointestinal varices. Variceal bleeding commonly occurs in children with chronic liver disease or portal vein obstruction. Therefore, prevention is important. Primary prophylaxis of variceal bleeding in adults is the established standard of care because of the results of numerous randomised clinical trials demonstrating the efficacy of non-selective beta-blockers or endoscopic variceal ligation in decreasing the incidence of variceal bleeding. In children, band ligation, beta-blockers, and sclerotherapy have been proposed as alternatives for primary prophylaxis of oesophageal variceal bleeding. However, it is unknown whether those treatments are of benefit or harm when used for primary prophylaxis in children. To assess the benefits and harms of sclerotherapy compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase Elsevier, and two other registers in February 2019. We scrutinised the reference lists of the retrieved publications, and performed a manual search of the main paediatric gastroenterology and hepatology conference (NASPGHAN and ESPGHAN) abstracts from January 2008 to December 2018. We searched four registries for ongoing clinical trials. There were no language or document type restrictions. We included randomised clinical trials irrespective of blinding, language, or publication status assessing sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We used standard Cochrane methodology to perform this systematic review. We used the intention-to-treat principle to analyse outcome data, and GRADE to assess the certainty of evidence per outcome. We found only one randomised clinical trial that fulfilled our inclusion criteria. The trial was at high risk of bias. The trial included 108 Brazilian children with median age of 4.3 years (range 11 months to 13 years). Fifty-six children were randomised to prophylactic sclerotherapy (ethanolamine oleate 2%) and 52 children to no intervention (control). Children were followed up for a median of 4.5 years. Eight children (six from the sclerotherapy group versus two from the control group) dropped out before the end of the trial. The follow-up was from 18 months to eight years. Mortality was 16% (9/56 children) in the sclerotherapy group versus 15% (8/52 children) in the control group (risk ration (RR) 1.04, 95% confidence interval (CI) 0.44 to 2.50; very low-certainty evidence). Upper gastrointestinal bleeding occurred in 21% (12/56) of the children in the sclerotherapy group versus 46% (24/52) in the control group (RR 0.46, 95% CI 0.26 to 0.83; very low-certainty evidence). There were more children with congestive hypertensive gastropathy in the sclerotherapy group than in the control group (14% (8/56) versus 6% (3/52); RR 2.48, 95% CI 0.69 to 8.84; very low-certainty evidence). The incidence of gastric varices was similar between the sclerotherapy group and the control group (11% (6/56) versus 10% (5/52); RR 1.11, 95% CI 0.36 to 3.43; very low-certainty evidence). The incidence of bleeding from gastric varices was higher in the sclerotherapy group than in the control group (4% (3/56) versus 0% (0/52); RR 6.51, 95% CI 0.34 to 123.06; very low-certainty evidence). The study did not assess health-related quality of life. Oesophageal variceal bleeding occurred in 5% (3/56) of the children in the sclerotherapy group versus 40% (21/52) of the children in the control group (RR 0.13, 95% CI 0.04 to 0.42; very low-certainty evidence). The most prevalent complications (defined as non-serious) were pain and fever after the procedure, which promptly resolved with analgesics. However, numerical data on the frequency of these adverse events and their occurrences in the two groups were lacking. No funding information was provided. We found no ongoing trials. The evidence, obtained from one randomised clinical trial at high risk of bias, is very uncertain on whether sclerotherapy has an influence on mortality and if it may decrease first upper gastrointestinal or oesophageal variceal bleeding in children. The evidence is very uncertain on whether sclerotherapy has an influence on congestive hypertensive gastropathy, incidence on gastric varices, and incidence of bleeding from gastric varices. Health-related quality of life was not measured. There were no serious events caused by sclerotherapy, and analysis of non-serious adverse events could not be performed due to lack of numerical data. The GRADE assessment of each outcome showed a very low-certainty evidence. The results of the trial need to be interpreted with caution. Larger randomised clinical trials, following the SPIRIT and CONSORT statements, assessing the benefits and harms of sclerotherapy compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are needed. The trials should include important clinical outcomes such as death, failure to control bleeding, and adverse events.

Endoscopic variceal ligation as primary prophylaxis for upper GI bleeding in children

Variceal hemorrhage can be a life-threatening adverse event of chronic liver disease. In contrast to the well-described guidelines for the management of portal hypertension (PH) in adults, there is limited evidence about the optimal prophylactic management of variceal bleeding in children. This study was carried out to assess the efficacy of endoscopic variceal ligation (EVL) as primary prophylaxis to prevent upper GI bleeding in children with PH. From January 2014 to April 2018, all pediatric patients with PH disease and medium to large esophageal varices or reddish spots, regardless of the grade of the varix, were prospectively included in the protocol of primary prophylaxis with EVL. A second retrospective group of patients was made after reviewing medical records of 32 pediatric patients with PH that presented esophageal varices in the upper endoscopy and had received propranolol as primary prophylaxis. Twenty-four patients (75%) reached varices eradication in the EVL group, with a median of 2 procedures (range, 1-4) before eradication and a median time to eradication of 3.40 months (range, 1.10-13.33). No EVL-related adverse events were observed. Statistically significant differences were observed in the bleeding rate at 3 years between propranolol and EVL groups (6/32 [21.9%] vs 1/32 [3.2%], P< .02). The hazard ratio for bleeding for patients treated with propranolol compared with those treated with EVL was 2.6 (95% confidence interval, 1.53-3.67). EVL is a safe and effective treatment to prevent upper GI bleeding in pediatric patients with PH. (Clinical trial registration number: NCT03943784.).

Sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal and gastrointestinal varices. Variceal bleeding commonly occurs in children with chronic liver disease or portal vein obstruction. Prevention is therefore important. Primary prophylaxis of variceal bleeding in adults is the established standard of care because of the results of numerous randomised clinical trials demonstrating the efficacy of non-selective beta-blockers or endoscopic variceal ligation in decreasing the incidence of variceal bleeding. However, sclerotherapy is the only endoscopic prophylactic option currently available in infants weighing less than 10 kg of bodyweight due to the size of the endoscopic ligator. To assess the benefits and harms of sclerotherapy versus any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase Elsevier, LILACS (Bireme), and Science Citation Index Expanded (Web of Science) in February 2019. We scrutinised the reference lists of the retrieved publications and performed a manual search from the main paediatric gastroenterology and hepatology conferences (NASPGHAN and ESPGHAN) abstract books from January 2008 to December 2018. We searched ClinicalTrials.gov, FDA, EMA, and WHO, for ongoing clinical trials. There were no language or document type restrictions. We planned to include randomised clinical trials irrespective of blinding, language, or publication status, assessing sclerotherapy versus any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. We planned to include quasi-randomised and other observational studies retrieved with the searches for randomised clinical trials for report of harm. We planned to collect and summarise data from randomised clinical trials as described in our protocol, using standard Cochrane methodologies. We found no randomised clinical trials assessing sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. Randomised clinical trials assessing the benefits or harms of sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis are lacking. Therefore, trials with adequate power and proper design, assessing the benefits and harms of sclerotherapy versus beta-blockers on patient-relevant clinical outcomes such as mortality, failure to control bleeding, and adverse events are needed. Unless such trials are conducted and the results become published, we cannot make any conclusions regarding the benefits or harms of the two interventions.

Band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including haemorrhage from oesophageal and gastrointestinal varices. Variceal haemorrhage commonly occurs in children with chronic liver disease or portal vein obstruction. Prevention is therefore important. Following numerous randomised clinical trials demonstrating efficacy of non-selective beta-blockers and endoscopic variceal ligation in decreasing the incidence of variceal haemorrhage, primary prophylaxis of variceal haemorrhage in adults has become the established standard of care. Hence, band ligation and beta-blockers have been proposed to be used as primary prophylaxis of oesophageal variceal bleeding in children. To determine the benefits and harms of band ligation compared with any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register (February 2019), CENTRAL (December 2018), PubMed (December 2018), Embase Ovid (December 2018), LILACS (Bireme; January 2019), and Science Citation Index Expanded (Web of Science; December 2018). We scrutinised the reference lists of the retrieved publications and performed a manual search from the main paediatric gastroenterology and hepatology conferences (NASPGHAN and ESPGHAN) abstract books from 2009 to 2018. We searched ClinicalTrials.gov for ongoing clinical trials. There were no language or document type restrictions. We planned to include randomised clinical trials irrespective of blinding, language, or publication status for assessment of benefits and harms. We planned to also include quasi-randomised and other observational studies retrieved with the searches for randomised clinical trials for report of harm. We planned to summarise data from randomised clinical trials using standard Cochrane methodologies. We found no randomised clinical trials assessing band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Randomised clinical trials assessing the benefits or harms of band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are lacking. There is a need for well-designed, adequately powered randomised clinical trials to assess the benefits and harms of band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Those randomised clinical trials should include patient-relevant clinical outcomes such as mortality, failure to control bleeding, and adverse events.

Acute Variceal Bleeding Causes Significant Morbidity.

The need for primary prophylaxis of variceal bleeding in children is unclear due to insufficient evidence of the efficacy of prophylactic therapy and the mortality and morbidity associated with the first bleeding event. Previous studies have provided estimates of mortality. We aimed to investigate the morbidity associated with acute variceal bleeding (AVB) in children and to identify contributing factors. We retrospectively reviewed children with chronic liver disease or portal vein thrombosis admitted with acute upper gastrointestinal bleeding between 2000 and 2015. Seventy AVB episodes in 57 children (median age 6 years, 52% girls) were included, 58% with cirrhosis and 30% portal vein thrombosis. Approximately 67% were the patient's first bleed. Post-AVB morbidity was present in 57% of all episodes and in 64% of first bleeds and included: ascites (34%), infection (30%), respiratory complications (24%), intensive care unit admission (20%), rebleed (11%), encephalopathy (7%), acute kidney injury (6%), and failure to control bleed (4%). Two patients died (4% of first bleeds, 8% of cirrhotics' first bleeds) within 6 weeks of bleeding. Median length of stay was 7 days. Overall morbidity was associated with total bilirubin (P = 0.001). Ascites after AVB was associated with pediatric end-stage liver disease (P = 0.0007), total bilirubin (P = 0.001), and cirrhosis (P = 0.006). Median length of stay was longer in patients with morbidities (18 vs 4 days, P < 0.0001). Children with AVB suffer significant morbidity but have a low risk of death. Morbidity should therefore be considered in future studies measuring the risks and benefits of primary prophylaxis of first AVB in children.

Anaesthetic management of extrahepatic portal venous obstruction

Extrahepatic portal venous obstruction (EHPVO) is the commonest cause of portal hypertension and variceal bleeding in children. Major concerns are growth retardation, decrease lean body mass and loss of muscle that may lead to postoperative respiratory failure. Upper abdominal surgery with severe pain may compound the problem of pulmonary atelectasis. Poor preoperative nutritional status and anemia may lead to delayed wound healing, delayed ambulation, and respiratory complications. To avoid postoperative respiratory complications and surgical site infection we inserted epidural catheter despite the possibility of intraepidural space bleed due to low platelet counts. The patient had an excellent recovery profile in term of analgesia, decrease postoperative nausea and vomiting due to decrease consumption of opioids, increase sedation-free period, early ambulation and parent's satisfaction. The intraoperative epidural also provides better abdominal muscle relaxation, wider surgical bloodless field and optimal pressure in anastomosis vessels. Regional techniques are avoided in thrombocytopenia, but risk and benefits must be assessed in each case.

Effect of endoscopic injection of cyanoacrylate in treatment of acute esophageal and gastric variceal bleeding in children

Effect of endoscopic injection of cyanoacrylate in treatment of acute esophageal and gastric variceal bleeding in children

Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium.

Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized. There are few pediatric data to indicate the efficacy and safety of pharmacologic or endoscopic therapies as primary prophylaxis or that prevention of a sentinel variceal bleed will ultimately improve survival; therefore, no recommendation for primary prophylaxis with endoscopic variceal ligation, sclerotherapy, or nonspecific beta-blockade in children was proposed.

Sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children

Sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children

Banding ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children

Banding ligation versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children