Portal Hypertension In Children Research Articles

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.

Risk factors for portal hypertension in children with biliary atresia

Introduction: Biliary atresia is a hepatobiliary disease which frequently results in portal hypertension.Objectives: To analyse the risk factors for portal hypertension in children with biliary atresia.Method: A case-controlled study was performed on 96 children with biliary atresia. Subjects were enrolled based on the inclusion and exclusion criteria. Medical history, physical examination results, imaging data, and laboratory examination results were collected prospectively. Patients were divided into two groups based on the signs of portal hypertension clinically during the follow-up period. Risk factors for portal hypertension were analysed using SPSS. Univariate analysis was used first to identify possible risk factors. A multivariate analysis was performed using logistic regression with p significant <0.05.Results: The median age was 18.21 (3.14-128.86) weeks in the portal hypertension group and 9.07 (1.00-50.57) weeks in the non-portal hypertension group. Age, duration of illness, birth weight, gestational age, and laboratory examination [Haemoglobin (Hb), white blood cell (WBC) count, albumin, direct bilirubin, total bilirubin, prothrombin time (PT), gamma-glutamyl transferase (GGT), aspartate transaminase (AST), and AST: alanine transaminase (ALT) ratio] were significantly different in the 2 groups (p<0.05). For every one-week increase in the subject's age, the risk of portal hypertension increased by 1.127. For every one unit increase in Hb, PT, GGT, and the AST:ALT ratio, the risk of having portal hypertension was 0.746, 1.125, 1.00, and 2.862 in children with biliary atresia (p<0,05).Conclusions: The risk factors for portal hypertension in children with biliary atresia were age, Hb, PT, GGT levels, and the AST:ALT ratio.

Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Surgical Treatment of Portal Hypertension in Children.

Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.

Portal Hypertension in Children at the Department of Pediatric Gastroenterology & Nutrition, BSMMU, Dhaka, Bangladesh

Background: Portal hypertension is the hemodynamic abnormality frequently associated with serious liver disease, although it is recognized in a variety of extrahepatic diseases also. Portal hypertension is an important cause of morbidity and mortality in Bangladeshi children. Development of esophageal varices and bleeding is one of the major complications of CLD. The mortality from each episode of variceal bleeding is 30-50% depending on the clinical status of the patient. All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. Objective: To assess the portal hypertension in children at the Department of Pediatric Gastroenterology & Nutrition, BSMMU, Dhaka, Bangladesh. Methods: This cross-sectional descriptive study was conducted at the Department of Pediatric Gastroenterology & Nutrition, BSMMU during the period Jan 2018 to July 2019. 50 patients who were diagnosed as portal hypertension were determined by Upper GI Endoscopy. Doppler USG was also done for supporting the diagnosis of Portal HTN as well as to differentiate between extrahepatic Portal HTN and CLD with portal HTN. Demographic data and other related information regarding etiology and complications were recorded in a standard datasheet. Results: Results: A total of 50 cases were included in this study. Their age range was 1.5-16 years. It was observed that 21 (42.0%) patients belonged to age group 6-10 years. The mean age was 9.22±9.85 years with ranged from 2.5 to 16 years. It was observed that almost two third 31 (62.0%) patients were male and 19 (38.0%) were female. Among 50 patients 29 were diagnosed as extrahepatic portal hypertension and 21 were diagnosed as CLD with portal HTN. Shows the etiology of portal hypertension of studied patients. Extrahepatic portal hypertension was the most common etiology (58.0%). Among CLD patients Wilson disease was the most common (13; 26.0%). Two (4.0%) patients were cryptogenic CLD and two (4.0%) were Budd Chiari Syndrome. One patient was Biliary cirrhosis and one patient had Auto immune hepatitis. Conclusion: We concluded that Extrahepatic Portal HTN is the most common cause of portal hypertension in children in this center. Among the intrahepatic causes of portal HTN Wilson disease was the

Liver reportalization in extrahepatic portal hypertension

The study involved literature review of history and current state of surgical treatment in extrahepatic portal hypertension in children and adults. The author analyzed Russian and foreign publications on surgical repair of hepatopetal blood flow in extrahepatic portal hypertension. The issue is a high priority in Russia, which is confirmed by the fact that the author found out the pioneer studies of Russian authors, which were published earlier than the works of foreign researchers. It was found that the role of hepatopetal blood flow restoration in extrahepatic portal hypertension in adults had been much less studied than in children. More clinical material is to be gathered to determine the feasibility of such interventions in adults.

Vascular bypass surgery in the treatment of extrahepatic portal hypertension in children (review)

In children with extrahepatic portal hypertension (EPHT), the most frequent and life-threatening complication is bleeding from varicose veins of the esophagus and stomach. Therefore, the main task of HSV treatment is to prevent bleeding from the veins of the upper gastrointestinal tract. The most effective treatment is portosystemic bypass surgery, with effectiveness of 94%97%. However, the use of these operations is limited precisely by a decrease in portal liver perfusion and development of portosystemic encephalopathy. This study aimed to analyze domestic and foreign publications devoted to the surgical treatment of portal hypertension and the development of portosystemic encephalopathy during the postoperative period. A systematic search was carried out in PubMed, Web of Science, Scopus, MEDLINE, eLibrary, RSCI, and Cyberleninka; 345 references were analyzed, 110 articles were reviewed, and 97 publications on the surgical treatment of portal hypertension were selected for the review. The development of portosystemic encephalopathy during the postoperative period often occurs after the imposition of total portosystemic bypass surgery. To reduce the risk of developing portosystemic encephalopathy, selective shunts have been developed, which to some extent preserve the PPP. With distal splenorenal anastomosis, the frequency of portosystemic encephalopathy is 10%15%. An intermediate position is occupied by a side-to-side splenorenal anastomosis, which has signs of selective surgery. The results of the analysis of literature sources showed that discussions are still underway regarding the choice of the optimal treatment strategy for patients with EPHT, place and role of endoscopic methods for the prevention of gastrointestinal bleeding, and mesoportal shunt in the treatment of patients with EPHT. Nevertheless, the majority of world experts consider mesoportal shunt to be the most optimal operation for the primary and secondary prevention of varicose bleeding and other HSV complications. If it is impossible to perform, selective bypass surgery of the distal splenorenal anastomosis can be an alternative to a mesoportal shunt.

“Etiology of Portal Hypertension in Children at the Department of Pediatric Gastroenterology & Nutrition, Bangladesh Shishu Hospital & Institute, Dhaka, Bangladesh”

Background: Portal hypertension is an important cause of morbidity and mortality in Bangladeshi children. Development of esophageal varices and bleeding is one of the major complications of CLD. The mortality from each episode of variceal bleeding is 30-50% depending on the clinical status of the patient. All conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management of this disorder, it is important to determine the underlying cause. Objective: To assess the Etiology of Portal Hypertension in Children. Methods: This cross-sectional descriptive study was conducted at the Emergency, Observation and Referral Unit, Bangladesh Shishu Hospital and Institute, Dhaka, Bangladesh during the period Jan 2018 to July 2019. 47 patients who were diagnosed as portal hypertension were determined by liver biopsy, abdominal sonography, abdominal computed tomography scan, and liver doppler so¬nography. Demographic data and other related information regarding etiology and complications were recorded in a standard datasheet. Results: Total 47 cases were included in this study. Their age range was 1.5-16 years. It was observed that 20 (42.6%) patients belonged to age group 6-10 years. The mean age was 9.22±9.85 years with ranged from 2.5 to 16 years. It was observed that almost two third (67.7%) patients were male and 18 (38.3%) were female. Among 47 patients 27 were diagnosed as extrahepatic portal hypertension and 20 were diagnosed as CLD with portal HTN. Shows the etiology of portal hypertension of studied patients. Extrahepatic portal hypertension was the most common etiology (57.4%). Among CLD patients Wilson disease was the most common (12; 25.5%). Two (4.3%) patients were cryptogenic CLD and two (4.3%) were Budd Chiari Syndrome. One patient was Biliary cirrhosis and one patient had Auto immune hepatitis. Conclusion: We concluded that intrahepatic diseases are the most common causes of portal .............

A100 CAVERNOUS TRANSFORMATION OF THE PORTAL VEIN IN A PEDIATRIC PATIENT WITH GOLDENHAR SYNDROME: A CASE REPORT

Abstract Background Goldenhar syndrome is a rare congenital condition thought to arise from the first and second branchial arches and typically asymmetrically affects the eyes, ears, and spine. Portal vein thrombosis/cavernous transformation is one of the main causes of portal hypertension in children. It is often associated with risk factors such as catheterization of the umbilical vein in the neonatal period, omphalitis, intra-abdominal infections in the neonatal period and prothrombotic conditions. This is a challenging clinical scenario as it leads to portal hypertension and variceal bleed with no chronic liver disease stigmata and liver function is essentially normal. While previous European literature from the early-2000s reported associated portal vein anomalies in Goldenhar syndrome, we are not aware of any recent and/or Canadian pediatric cases. Purpose We report a Canadian case of cavernous transformation of the portal vein with resulting gastrointestinal bleeding in a child with Goldenhar syndrome. Method Case report Result(s) A pediatric patient with a postnatal suspicion of Goldenhar syndrome, with confirmation by 6 months of age, presented with an acute 3-day history of melena, on the context of a recent viral illness and ibuprofen use. Laboratory testing showed a normocytic anemia (Hb 68) with a normal INR (1.1). The rest of the workup was unremarkable. There was no history of catheterization of the umbilical vein on the neonatal period nor other hematological complications. To assess for potential duplication cyst as differential diagnosis, a computerized tomography of the chest, abdomen and pelvis was ordered and revealed suspected cavernous transformation of the portal vein with suspected varices near the gastroesophageal junction and within the abdomen, and hepatomegaly with spleen size at the upper limit of normal. Octreotide was started after the CT results. A gastroscopy was done 24h after presentation and found portal hypertensive gastropathy and esophageal varices (2 Grade II and 1 Grade I). Sclerotherapy was performed. Post-gastroscopy ultrasound supported earlier radiographic findings of cavernous portal vein transformation and hepatosplenomegaly. Moreover, the ultrasound noted normal flow direction in the portal and hepatic veins. The patient has not re-presented for further episodes of gastrointestinal bleeding. Conclusion(s) This case report supports the previously reported association between Goldenhar syndrome and portal venous anomalies. Early consideration may lead to prompt diagnosis and management of the potentially life-threatening complications of portal hypertension in this population. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

The presence of high-risk varices after sclerotherapy in biliary atresia.

Esophagogastric varices (EGVs) may develop as a result of portal hypertension in children with biliary atresia (BA). Although endoscopic injection sclerotherapy (EIS) with ethanolamine oleate (EO) is reported useful for children, risk factors associated with the presence of high-risk EGVs after treatment remain unknown. The subjects were BA patients under 15 years of age who underwent EO-EIS. We retrospectively reviewed a total of 28 treatment sessions of EGVs with red signs and those larger than F2, which were considered to be at high risk of bleeding. Survival analysis was performed for the presence of high-risk EGVs at the time of follow-up endoscopy as the occurrence of an event. Univariate analysis showed a significantly increased risk of the presence of high-risk EGVs post-EO-EIS in patients with increased liver stiffness (LS) and Mac-2 binding protein glycan isomer (M2BPGi), with hazard ratios of 1.48 and 1.15, respectively. The median presence-free period was significantly shorter in the LS ≥ 2.8m/s patients than in those with LS <2.8m/s (189 vs. 266 days). Similarly, the median presence-free period was significantly shorter in patients with M2BPGi ≥ 4.0 than in those with M2BPGi < 4.0 (182 vs. 203 days). The results of multivariate analysis revealed that the risk of the presence of high-risk EGVs was significantly higher only in the high-LS group, with a hazard ratio of 2.76. Increased LS is associated with risk of the presence of high-risk EGVs following EO-EIS in children with BA.

Portal Hypertension in Children: A Tertiary Center Experience in Turkey.

Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.

Liver cirrhosis in children

Liver cirrhosis in children and adolescents is a consequence of a variety of chronic liver diseases, metabolic disorders, and genetic diseases. In infants, cirrhosis is most often due to biliary atresia and genetic-metabolic diseases, while in older children it usually occurs as a result of autoimmune hepatitis, Wilson’s disease, alpha-1 antitrypsin deficiency, and primary sclerosing cholangitis. Symptoms of cirrhosis in children and adolescents are not specific. In pediatric patients, the first sign of cirrhosis may more often be low weight gain, weakness, loss of appetite, low-grade fever, as well as more specific signs: transient jaundice, mild pruritus, epistaxis, heaviness in the right hypochondrium, changes in liver density on palpation, hyperbilirubinemia, hyperenzymemia, etc. Complications of pediatric cirrhosis are similar to those observed in adult patients and include gastrointestinal bleeding caused by esophageal varices, ascites, and spontaneous bacterial peritonitis. In pediatric practice, special attention should be paid to nutritional correction in liver cirrhosis since children have higher requirements for protein and micronutrients for growth and development. Treatment of cirrhosis-induced portal hypertension in children and adolescents is mainly based on methods developed for adults. The article deals with diagnostic and differential diagnostic aspects of the terminal stage of liver disease in children. The therapeutic management of patients with cirrhosis of the liver and acute renal failure is based on support of various organ functions. Hemodialysis/ plasmapheresis may serve in some patients as an interim therapy before liver transplantation. The indications for liver transplantation and problems arising after surgery are considered.

Pediatric liver cirrhosis interventional procedures: from biopsy to transjugular intrahepatic portosystemic shunt

Cirrhosis is a complex diffuse process whereby the architecture of the liver is replaced by abnormal nodules because of the presence of fibrosis. Several pediatric diseases such as extrahepatic portal vein obstruction, biliary atresia, alpha-1-antitrypsin deficit and autoimmune hepatitis can lead to cirrhosis and portal hypertension in children. In this article the authors describe interventional radiology procedures that can facilitate the diagnosis and treatment of diseases associated with liver cirrhosis and portal hypertension in the pediatric population. These procedures include image-guided liver biopsy, mesenteric–intrahepatic left portal vein shunts, balloon-occluded retrograde transvenous obliteration, transjugular intrahepatic portosystemic shunts and splenic embolization.

Meso-Rex bypass shunt vs. transposition shunt for cavernous transformation of portal vein in children.

BackgroundCavernous transformation of the portal vein (CTPV) causes portal hypertension in children. Among Meso-Rex treatments, it is unclear whether the Meso-Rex bypass shunt (MRB) or the Meso-Rex transposition shunt (MRT) offers lower postoperative morbidity. Our objective was to evaluate postoperative outcomes, comparing MRB and MRT for children with CTPV.MethodsA retrospective study was conducted on children undergoing Meso-Rex for CTPV from January 2010 to December 2020. The primary outcome was shunt complications, including shunt stenosis and thrombus. The secondary outcome was re-operation.ResultsOf the 43 patients included, 21 underwent MRT and 22 underwent MRB. MRT was associated with a higher rate of shunt complications when compared to MRB (23.8 vs. 9.1%, p = 0.191). The patients exhibited a higher rate of re-operation under the MRT than under the MRB (19 vs. 4.5%, p = 0.138). The operative time in the MRT group was significantly shorter than in the MRB group. Compared to MRT, the reduction in the length and thickness of the spleen was significantly greater in the MRB group. The increases in platelets were significantly higher in the MRB group than in the MRT group. The postoperative shunt velocity of MRB was notably faster than MRT. There was no significant difference in postoperative portal pressure between the two groups (p > 0.05).ConclusionBoth MRB and MRT result in acceptable postoperative outcomes, but MRT is associated with higher post-shunt complications, which often increase the re-operation rate. This study suggests that MRB may offer advantages for children with CTPV.

Frequency of Causes of Portal Hypertension in Children.

IntroductionThe most common etiology of portal hypertension (PH) in children is obstruction at the presinusoidal or sinusoidal level. In addition, portal vein thrombosis (PVT) and biliary atresia are the most prevalent extrahepatic causes. This study aims to evaluate all the possible etiologies leading to PH in the pediatric population and provide the most common cause associated with this condition along with the age group most frequently affected by it.Material and MethodsFrom January 2018 to December 2020, a cross-sectional study was carried out in tertiary care hospitals in Pakistan. A total of 100 children, both male and female, aged one month to 15 years and diagnosed with PH, were enrolled for the evaluation for the causes of PH. The Statistical Package for the Social Sciences (SPSS), version 20, was used to analyze the data.ResultsThe mean age of enrolled participants was 9.01 ± 2.81 years. It was found that PVT (63%) was the most common cause of PH, followed by liver cirrhosis (19%) and biliary atresia (18%). Age of more than eight years was significantly associated with PVT (p-value: 0.007).ConclusionIn children, PH may be caused by a wide range of etiologies. It is imperative to understand the underlying etiologies contributing to PH for proper guidance and management, prevention, and overall outcomes.

Abstract No. 584 Recanalization of chronically occluded portal and mesenteric veins: a non-transplant pediatric cohort

Extrahepatic portal vein obstruction is the most frequent cause of portal hypertension in children. Patients may not be amenable to meso-Rex bypass and alternative surgical bypasses do not restore physiologic portal flow to the liver, a critical component of somatic growth in pediatric patients. Superior mesenteric and splenic vein occlusions, while far less common, are also problematic with regards to GI bleeding. The current literature is lacking in non-transplant patients with portal and other visceral occlusion.

Non-invasive diagnostics of extrahepatic portal hypertension in children

BACKGROUND: The primary method for diagnosing gastroesophageal bleeding in varicose veins of the esophagus and stomach in children is fibroesophagogastroduodenoscopy. This study investigates the possibilities of 2D shear wave elastography stiffness of the spleen to determine esophageal varicose veins in children with extrahepatic portal hypertension.&#x0D; MATERIALS AND METHODS: A retrospective analysis of the effectiveness of the method of two-dimensional elastography by shear wave stiffness of the spleen was conducted in children with extrahepatic portal hypertension in 39 children (main group) and 11 healthy children (control group). All patients initially underwent fibroesophagogastroduodenoscopy followed by ultrasound, including 2D shear wave stiffness elastography of the spleen. Spleen stiffness was then compared with clinical symptoms, the degree of esophageal varices, and other sonographic parameters.&#x0D; RESULTS: According to elastography data, the spleen stiffness index in children with extrahepatic portal hypertension was 43.98 3.8 kPa, significantly higher than in the control group children (p = 0.006). Spleen stiffness measurements significantly correlated with the degree of esophageal varices in children with extrahepatic portal hypertension (r = 0.57, p = 0.0002). According to the endoscopy results in seven patients after vascular bypass surgery, esophageal varicose veins were not detected. Nevertheless, spleen stiffness in these children remained significantly higher than in the control group (27 3.9 kPa and 18 1.2 kPa, respectively, p = 0.05).&#x0D; CONCLUSIONS: The study results indicate that 2D stiffness shear wave elastography of the spleen effectively assesses esophageal varices in children with extrahepatic portal hypertension. This method is also convenient to monitor the reduction of varicose veins after surgical treatment and is a possible alternative to endoscopy, especially in young children.

Abdominal CT and MRI Findings of Portal Hypertension in Children and Adults with Fontan Circulation.

Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9-55 years; 32 children) were evaluated (median age, 23 years; IQR, 17-30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12-23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); median follow-up duration was 0.4 year (IQR, 0.1-3.1 years). Compared with patients who had Fontan circulation without deterioration, patients with Fontan deterioration were more likely to have moderate or severe ventricular systolic dysfunction (P < .01), moderate or severe atrioventricular valve regurgitation (P < .01), higher Fontan pressure (P = .01), radiologic evidence of portal hypertension (P < .01), and VAST score of 2 or greater (P < .01). Conclusion Radiologic evidence of portal hypertension at abdominal imaging in children and adults with Fontan circulation was associated with higher venous pressures and an increased risk for Fontan deterioration. These characteristics may be used to identify patients who warrant comprehensive hemodynamic evaluation. © RSNA, 2022.

Extrahepatic Portal Vein Thrombosis in Childhood: Risk Factors, Clinical Manifestations, and Management

Objective: Extrahepatic portal vein thrombosis (EHPVT) is a common cause of portal hypertension in children. The aim of the present study was to identify the clinical manifestations and the risk factors for development of EHPVT in pediatric patients. Subjects and Methods: This was a single-center retrospective cohort study. A total of 12 children (6 boys and 6 girls) took part in the study. We noted the clinical presentations and the predisposing risk factors for development of EHPVT in all patients. In addition, as all of them had undergone an esophagogastroduodenoscopy for detection and grading of esophageal varices as part of the treatment algorithm, we analyzed the endoscopic findings and the therapeutic approach. Results: The median age of subjects at diagnosis was 3.5 years (range: 1–17 years). The most frequent initial clinical manifestation was upper gastrointestinal bleeding (6 cases, 50.0%) followed by splenomegaly (3 cases, 25.0%). The most frequent systemic risk factor for EHPVT was presence of inherited prothrombotic disorder (10 cases, 83.3%), and the most common local risk factor for EHPVT was umbilical vein catheterization (5 cases, 41.7%). Esophageal varices were revealed in all the study participants, and in the most cases, they were grade ≥2. Propranolol was used as primary or secondary prophylaxis in 7 children (58.3%), and in 5 children (41.7%), a shunt was performed (Meso-Rex bypass in 3 children and splenorenal shunt in 2 children). Conclusion: Patients with known systemic or local risk factors for EHPVT are indicated for proactive ultrasound screening for early diagnosis and timely management.

A Study on Duplex Doppler Ultrasonography as a Non-Invasive Test for Diagnosis of Portal Hypertension in Children Attending a Tertiary Care Hospital in Bangladesh

Background: Portal hypertension is the hemodynamic abnormality frequently associated with serious liver disease, although it is recognized in a variety of extrahepatic diseases also. Endoscopy of upper GIT is the gold standard for diagnosis of oesophageal varices but it is invasive, costly and not easily available. Doppler ultrasound is a non-invasive, relatively cheap and easily accessible imaging modality that helps in making the diagnosis of clinically significant portal hypertension.