Extrahepatic Portal Hypertension In Children Research Articles

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.

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.
 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.
 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).
 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.

Assessment of the patency of the left portal vein during mesoportal shunting in children with extrahepatic portal hypertension

BACKGROUND: One of the most common causes of extrahepatic portal hypertension in children is portal vein thrombosis. The causes of this disease are different and, in most cases, remain unrecognized. Along with this, the mesoportal shunt (Rex shunt) proved itself and today is considered the gold standard to treat extrahepatic portal hypertension in children. The restoration of hepatopetal blood flow eliminates gastroesophageal bleeding, splenomegaly, hypersplenism, and many other complications. For the results of mesoportal shunt to be successful, several conditions must be met, one of which is the patency of the umbilical portion of the left portal vein. Despite the importance of preoperative diagnostics of the patency of this area, the most optimal instrumental research method has not yet been found.
 AIM: This literature review aims to highlight the main issues of extrahepatic portal hypertension etiopathogenesis, surgical treatment methods, and the most effective preoperative diagnostic methods to assess the patency of the left portal vein.
 RESULTS: The authors analyzed the sources of domestic and foreign literature on the etiology, pathogenesis of HSV in children, and laboratory and instrumental diagnostic methods to assess the patency of the PVI to plan the mesoportal shunting operation.
 CONCLUSIONS: Extrahepatic portal hypertension is a polyetiological disease with a possible hereditary predisposition to a thrombotic process under the influence of various triggers. The most common causes of portal vein thrombosis are omphalitis and umbilical vein catheterization in the neonatal period. Unfortunately, to date, none of the existing instrumental diagnostic methods can reliably answer the question about left portal vein patency. Due to the small number of works, the lack of a unified view on the problem of preoperative diagnosis of patients with extrahepatic portal hypertension, we could not reliably determine the specificity, sensitivity, and accuracy of each instrumental method. Therefore, we could not identify the gold standard method. Nevertheless, with further improvement of the methods for preoperative assessment of the left portal vein patency, surgeons will be more likely to predict the successful outcome of mesoportal shunting, which will generally affect the surgical treatment quality of extrahepatic portal hypertension in children.

Anesthesia in Children with Thrombocytopenia During Bypass Surgery for Extrahepatic Portal Hypertension (Review)

Portal hypertension syndrome is one of the most severe pediatric conditions causing gastroesophageal bleeding which can be fatal. The main challenge in the management of portal hypertension is prevention of bleeding from the portal vein system, which is achieved by vascular surgery, particularly portosystemic shunting. Epidural anesthesia, despite its advantages over the opioid one, is not always performed in children with extrahepatic portal hypertension, and still remains unsolved issue in pediatric anesthesiology.Aim of the review: to evaluate the scope of limitations of general anesthesia in bypass surgery for extrahepatic portal hypertension in children with thrombocytopenia. We searched PubMed, Medline, Elibrary.ru and other databases and used original clinical observations while performing anesthesiologic support of surgical correction of extrahepatic portal hypertension in children.We found that general anesthesia for portosystemic bypass surgery in children with thrombocytopenia requires the anesthetic support that does not include epidural anesthesia and high-dose opioid administration. This issue can be resolved by including the central selective alpha-2-adrenergic agonist dexmedetomidine with both analgesic and hypnotic effects in the anesthesia support. Due to its additive effects, this drug significantly reduces the need for hypnotics and opioid analgesics while maintaining a high level of neurovege-tative protection.Conclusion. In our opinion, optimizing anesthesia in children with thrombocytopenia during bypass surgery for extrahepatic portal hypertension is essential to minimize surgical stress and side effects of drugs.

WITHDRAWN: The optimal procedure of modified Rex shunt for the treatment of extrahepatic portal hypertension in children

WITHDRAWN: The optimal procedure of modified Rex shunt for the treatment of extrahepatic portal hypertension in children

Modified splenorenal shunt for extrahepatic portal hypertension in children: A technique to spare splenic vein-pancreatic disconnection

Background: Â Patients with extrahepatic portal hypertension may have to undergo splenorenal shunt surgery which requires a patent splenic vein of adequate calibre with a normal renal vein . This study reports a modification of splenorenal shunt for portal hypertension in children obviating the need for complex splenic vein-pancreatic disconnection.

The Prevalence of Portal Vein Thrombosis in Children under 3 Years Old with History of Neonatal Umbilical Vein Catheterization in the Neonatal Intensive Care Unit

Background Portal vein thrombosis (PVT) is one of the most common causes of extrahepatic portal hypertension in children, which may follow neonatal umbilical vein catheterization. The incidence rates of catheter-related PVT, in infants and children vary in different studies. This study aimed to determine PVT incidence in the children under 3 years old with a history of neonatal umbilical vein catheterization in the NICU of Ghaem Hospital, Mashhad- Iran. Materials and Methods This cross-sectional study included 38 children with a history of hospitalization in the NICU of Ghaem Hospital, Mashhad-Iran, during 2012 to 2013 for whom umbilical vein catheterization had been performed. The Children’s histories were taken and they were examined. Color Doppler ultrasound was performed on them. Data analysis was carried out using SPSS-13 and descriptive statistics, t-test, Fisher's exact test. Results Fourteen (36.8%) and 24 (63.2%) of the infants were males and females, respectively. Mean age of the infants was 33.1±3.55 months. PVT evidence in a child raises a 2.6% PVT incidence. No statistically significant relationship was observed among gender, age, catheter type, and catheterization duration and PVT. A statistically significant relationship was observed between spleen size and portal vein size in ultrasound and PVT (P<0.05). The liver and spleen examination was only abnormal, in the patient with thrombosis. Conclusion The study findings suggest a 2.6% incidence for PVT. In addition, neonatal umbilical catheterization causes PVT during childhood.

A New Procedure for the Treatment of Extrahepatic Portal Hypertension in Children: Portal Cavernoma-Rex Shunt with Interposition of Grafted Portal Vessel

A New Procedure for the Treatment of Extrahepatic Portal Hypertension in Children: Portal Cavernoma-Rex Shunt with Interposition of Grafted Portal Vessel

Blocked mesocaval shunt with protein C deficiency in a child with extrahepatic portal hypertension

Portal vein thrombosis (PVT) is an important cause of extrahepatic portal hypertension in children. A majority of children with PVT of unknown etiology have functional protein C deficiency or abnormally elevated levels of anti-cardiolipin antibodies. We present an 8-year-old boy with portal hypertension with protein C deficiency, who underwent mesocaval shunt surgery due to recurrent episodes of bleeding esophageal varices and subsequently developed a block in the mesocaval shunt due to a thrombotic tendency.

Spleen-preserving proximal splenic-left intrahepatic portal shunt for the treatment of extrahepatic portal hypertension in children

PurposeThe Rex shunt has been employed successfully to treat patients with extrahepatic portal hypertension. In the conventional Rex shunt, the internal jugular vein is used as a venous graft. Inevitably, such a procedure requires neck exploration and sacrifice of the internal jugular vein. The authors describe a novel adaptation of spleen-preserving spleno-Rex bypass, successfully carried out in children with extrahepatic portal hypertension. MethodsThe mean age of the four patients (1 boy, 3 girls) was 46months at the time of operation. All children had a history of upper gastrointestinal bleeding, and suffered from splenomegaly and hypersplenism. Spleen-preserving proximal splenic-left intrahepatic portal shunt was performed in all patients. The splenic artery and vein were ligated at the splenic hilum, and the splenic vein was completely separated from the bed of the pancreas to its junction with the inferior mesenteric vein. The freed splenic vein was anastomosed to left portal vein. The short gastric and left gastroepiploic vessels were kept intact to supply and drain the spleen. All patients were followed-up for 7–33months (median: 21.5months). ResultsThe spleen-preserving spleno-Rex bypass was successfully performed in all 4 patients. The median operative time was 225min (range: 215–260min). One patient received blood transfusion, and the postoperative length of hospital stay varied from 4 to 6days (median: 4.5days). Intraoperative portal venous angiography demonstrated the patency of the shunt in all patients. Postoperatively, the complete blood count normalized and the biochemistry tests were within normal range. Postoperative ultrasound confirmed shunt patency and satisfactory flow in the proximal splenic-portal shunt in each patient. The size of the spleen decreased and there was no recurrence of variceal bleeding. ConclusionsThe spleen-preserving spleno-Rex bypass is a viable option to treat EHPVO in children.

PD-051 Long-term outcome of extrahepatic portal hypertension in children(Prehepatic portal hypertention, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)

PD-051 Long-term outcome of extrahepatic portal hypertension in children(Prehepatic portal hypertention, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)

Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography: a new etiology of extrahepatic portal hypertension

Background/Purpose The cause of extrahepatic portal hypertension in children has not been clarified. Our aim was to determine the morphological features of the extrahepatic portal vein in children with extrahepatic portal hypertension by 3-dimensional computed tomographic portography and to clarify the etiology of this disorder. Materials and Methods Six patients ranging in age from 10 to 18 years (median age, 12.8 years) who had portal hypertension presented with hematemesis. They underwent intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction. Results The extrahepatic portal vein was visualized in all patients by 3-dimensional computed tomographic portography. None of the patients showed extrahepatic portal vein obstruction or cavernous transformation. All patients had a tortuous η-shaped extrahepatic portal vein, and a line could be drawn through the flexures of the portal vein to the hepatic hilum. Conclusion In children, extrahepatic portal hypertension is not caused by extrahepatic portal vein obstruction and may be of embryological origin.

Erratum to Management of extrahepatic portal hypertension in children

Erratum to Management of extrahepatic portal hypertension in children

Management of extrahepatic portal hypertension in children

Management of extrahepatic portal hypertension in children

Portosystemic shunts for extrahepatic portal hypertension in children: J. Tocornal and F. Cruz. Surg Gynecol Obstet 153:53–56, (July), 1981

Portosystemic shunts for extrahepatic portal hypertension in children: J. Tocornal and F. Cruz. Surg Gynecol Obstet 153:53–56, (July), 1981

Extrahepatic portal hypertension in children

In children the principal cause of extrahepatic portal hypertension is a “cavernomatous transformation” of the portal vein. In 54 children seen over a 20-yr period, hemorrhage was by far the most common presenting symptom, occurring in 30 patients. Occasionally these children presented with a symptomless splenomegaly. Liver function studies were always normal. The patients were classified into four groups, depending on the particular primary surgical treatment given. One-third of patients received no surgical treatment and all are well. Of the patients who have had a surgical procedure, postoperative bleeding has been frequent and further surgery has often been necessary. There have been seven deaths. Operation is not recommended in the child under 4 yr or during acute bleeding episodes. For recurrent life-threatening hemorrhage, a porto-systemic shunt is indicated.

Extrahepatic portal hypertension in children

<h2>Summary</h2> The details of eighteen children with extrahepatic portal hypertension seen during the last twenty years at the Childrens Hospital of Los Angeles are presented. All had bleeding from esophagogastric varices, but in several this was not the presenting symptom or finding. Three initially presented with splenomegaly and five with ascites. Eleven of the patients manifested ascites at some stage of their disease, more commonly during infancy or early childhood. Three older patients currently have ascites which has persisted for two, six, and seven years, respectively. An etiologic mechanism such as that usually ascribed to the development of occlusion of the portal vein was observed in only three of the eighteen children. It is suggested that neonatal omphalitis or peritonitis is an unlikely cause and that the etiologic mechanism is, in fact, unknown. Fever, usually due to a respiratory infection, preceded varical hemorrhage in a number of patients. The implication of this feature is discussed. The results of operative management were disappointing. The ineffectiveness of shunt procedures in eliminating recurrent varical hemorrhage was probably due in large part to the young age of the patients at the time of operation. Of great interest was the observation that in four children varical hemorrhage ceased spontaneously. In two of them, varices are no longer demonstrable on roentgenogram. In the other two, this examination had not been carried out. Six of the eighteen patients are currently over the age of twenty years; only one still has bleeding. These features are consonant with the rarity with which an adult with extrahepatic portal hypertension provides a history of bleeding episodes extending from childhood.