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Background Extrahepatic portal vein obstruction (EHPVO) results in severe portal hypertension (PHT) leading to severely compromised quality of life. Often, pharmacological and endoscopic management are unable to solve this problem. Restoring hepatic portal flow using meso-Rex bypass (MRB) may solve it. This procedure, uncommon in adult patients, is considered the treatment of choice for EHPVO in children. Methods From 1997 to 2018, 8 male and 6 female adults, with a median age of 51 years (range 22–66) underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels, Belgium. Symptoms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings, sepsis due to portal biliopathy, and/or severe abdominal discomfort. The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein. Results Median operative time was 500 min (range 300–730). Median follow-up duration was 22 months (range 2–169). One patient died due to hemorrhagic shock following percutaneous transluminal intervention for early graft thrombosis. Major morbidity, defined as Clavien-Dindo score ≥ III, was 35.7% (5/14). Shunt patency at last follow-up was 64.3% (9/14): 85.7% (6/7) of pure venous grafts and only 42.9% (3/7) of prosthetic graft. Symptom relief was achieved in 85.7% (12/14) who became asymptomatic after MRB. Conclusions Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications. In such patients, MRB represents the only and last resort to restore physiological portal vein flow. Although successful in a majority of patients, this procedure is associated with major morbidity and mortality and should be done in tertiary centers experienced with vascular liver surgery to get the best results.

Background. Hepatic encephalopathy is a pathological change in the brain functions of non-inflammatory origin, which is manifested by a syndrome of secondary neurological and cognitive disorders caused by a set of deep metabolic disorders, as a result of acute hepatic insufficiency, chronic liver disease or portosystemic shunting of the blood. The initial stage of encephalopathy does not have clinical neurologic symptoms, but is defined in 20–80 % of patients with liver cirrhosis, and, unlike the final stages, is reversible. The purpose of the study was to evaluate the effectiveness of the number connection test, сritical flicker frequency test and an electroencephalography in the diagnosis of encephalopathy in patients with hepatic and extrahepatic form of portal hypertension. Materials and methods. Syndrome of hepatic encephalopathy was studied in 50 patients with hepatic form of portal hypertension, 30 — with extrahepatic form of portal hypertension and 25 persons without signs of portal hypertension. For the diagnosis, number connection test, сritical flicker frequency test and an electroencephalography were used. Results. A simple-to-use and number connection test made it possible to establish manifestations of hepatic encephalopathy in almost all patients, regardless of the presence of portal hypertension and its form. However, manifestations of hepatic encephalopathy prevailed in patients with hepatic portal hypertension in terms of incidence and severity. Critical flicker frequency test allowed to diagnose minimal hepatic encephalopathy in 96.0 % of patients with hepatic portal hypertension and 60.0 % of patients with extrahepatic portal hypertension. The electroencephalography established the presence of hepatic encephalopathy in 60.0 % of patients with hepatic portal hypertension, 50.0 % — with extrahepatic portal hypertension and 12.0 % of individuals without portal hypertension. Conclusions. Comparison of the effectiveness of diagnostic tests showed that am easy to use and accessible number connection test is most effective, however, other tests also have benefits in the clinical evaluation of hepatic encephalopathy in patients with portal hypertension.

The article deals with the clinical observation of extrahepatic portal hypertension as an outcome of portal vein thrombosis, which was first manifested by hemorrhage from varicose veins of the esophagus and stomach, in a young patient. Features of an anamnesis, a clinical picture pointed to the asymptomatic course of the disease until the moment of the catastrophe. The complexity of these patients is caused by the lack of informative routine methods of study, the need for special research methods for the diagnosis and non-standard methods of treatment. The data of laboratory-instrumental methods of study in this patient are analyzed, a modern diagnostic search for the causes of liver disease and portal hypertension syndrome in real conditions is presented. The absence of signs of hepatitis was revealed, and the evaluation of the liver with elastometry excluded the presence of fibrosis in this patient. The diagnosis of extrahepatic portal hypertension is made, and the patient is directed to surgical treatment. A successful shunt operation was performed at the Institute of Surgery and Transplantology. Further, a positive dynamics of clinical data, splenomegaly has been shown, which caused pain syndrome and hypersplenism. The conservative treatment of the syndrome of portal hypertension and symptomatic treatment of the patient are described. The necessity of an individual choice of motion regimen and drug management with inclusion of ursodeoxycholic acid is proved. For 3 years, the patient has a significant reduction in portal hypertension, with the preservation of the parenchyma of the liver, which gives hope for positive long-term results.

Актуальность. Значительная вариабельность количественных показателей портального кровотока при хроническом гепатите, циррозе печени, хронической сердечной недостаточности, болезни Бадда — Киари подтверждает необходимость дальнейшего поиска клинико-гемодинамических особенностей при различных формах портальной гипертензии (ПГ). Цель. Определение связи между допплерографическими показателями портальной системы, клиническими данными и основными маркерами цитолитического, холестатического и мезенхимально-воспалительного синдромов у больных с печеночной и внепеченочной ПГ. Материалы и методы. В исследование вошли 205 пациентов с ПГ и 35 больных без ПГ, проходивших обследование и лечение в гастроэнтерологическом отделении Николаевской областной клинической больницы. Всем больным проводили клиническое обследование, допплерографию сосудов портальной системы, биохимическое исследование крови. Результаты. У больных с печеночной ПГ были обнаружены наибольшие изменения допплерографических показателей как венозного, так и артериального кровотока одновременно со значительной выраженностью маркеров цитолиза и холестаза, а выявленные достоверные корреляционные связи свидетельствуют об их ассоциативном характере. Характерной чертой внепеченочной ПГ было отсутствие признаков печеночной недостаточности или их меньшая выраженность; не обнаружено параллелизма между биохимическими и гемодинамическими показателями при этой форме ПГ.

Мета: оцінити клінічні особливості хворих з портальною гіпертензією (ПГ) та синдромом надлишкового бактеріального росту (СНБР). Методи. Під спостереженням перебувало 67 хворих із синдромом портальної гіпертензії, віком від 43 до 81 року, які сформували 2 групи: І група — 60 пацієнтів із печінковою ПГ; ІІ група — 7 хворих із позапечінковою ПГ. Проводили оцінку СНБР за допомогою сучасного газоаналізатора Gastro + Gastolyzer та встановлювали взаємозв’язок отриманих результатів з клінічними даними. Результати. Встановлено, що для пацієнтів із печінковою ПГ СНБР зустрічається частіше (80 %), ніж з позапечінковою ПГ (57 %) (р

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.

Background. Making treatment / prevention decisions in bleeding from gastroesophageal varices in patients with subhepatic portal hypertension remains challenging and thus requires further research. Objective. To perform a preliminary analysis of the outcomes of selective devascularization of the gastric fundus in patients with chronic pancreatitis and subhepatic portal hypertension. Material and methods. Selective devascularization of the gastric fundus was performed in five patients (3 men and 2 women) aged 23 to 54 with chronic recurrent pancreatitis and subhepatic portal hypertension. In 2 patients, Roux-en-Y cystojejunostomy for pancreatic pseudocyst was performed as the main treatment, and in 3 patients - Frey's surgery. Selective devascularization of the gastric fundus was adjuvant to a draining surgery or a resection-draining one. Results. All patients underwent surgery successfully. The duration of the operation (Me [min; max]) was 205 [190; 255] min. The time spent in the Department of anesthesiology, resuscitation and intensive care comprised 1 [1; 2] days. Postoperative follow-up of these patients over the period of 17 - 58 months detected no recurrence of bleeding from gastric varices. Endoscopy performed a year after revealed a decrease in the size of esophageal varices in all patients. Conclusions. The proposed technique of selective devascularization of the gastric fundus is substantiated by numerous studies of the variant anatomy of venous outflow from the spleen in extrahepatic portal hypertension. This technique is regarded as adjuvant to a draining surgery or a resection-draining one in patients with chronic pancreatitis and subhepatic portal hypertension performed to prevent bleeding from gastric fundus varices. There should be noted the reproducibility of this surgical intervention, its effectiveness and safety. Further research on the efficiency of the technique using randomized controlled trials is required.

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.

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular lesion. It is a very rare cause of splenic artery aneurysm (SAA). An 18-year-old girl presented with hematemesis, melena, pancytopenia, and splenomegaly. Endoscopy showed esophageal varices. Computed tomography angiography showed splenic infarct and a giant splenic artery aneurysm. Portal vein showed cavernous transformation with enlarged periportal and lienorenal collaterals. The liver and pancreas were unremarkable. Microscopy of the SAA revealed intimal fibroplasia and medial dysplasia. Symptoms of extrahepatic portal hypertension were relieved by aneurysmectomy, thus proving SAA as the underlying cause. Pancytopenia was reversed post-splenectomy, thus proving hypersplenism. This is the first-ever report showing a quadruple association of FMD, splenic artery aneurysm, extrahepatic portal hypertension, and hypersplenism. Key messages Fibromuscular dysplasia can present as a giant aneurysm of the splenic artery. The resultant extrahepatic portal hypertension and splenomegaly can result in hypersplenism. Splenectomy and aneurysmectomy can reverse pancytopenia and portal hypertension. How to cite this article Shinde S. A Rare Quadruple Association: Fibromuscular Dysplasia, Giant Splenic Artery Aneurysm, Extrahepatic Portal Hypertension, and Hypersplenism. Indian J Crit Care Med 2021;25(1):100–103.

To assess the long-term results after Rex bypass (RB) shunt and Rex transposition (RT) shunt and determine the optimal approach. Between 2010 and 2019, traditional RB shunt was performed in 24 patients, and modified RT shunt was performed in 23 children with extrahepatic portal hypertension (pHTN). A retrospective study was conducted based on comparative symptoms, platelet counts, color Doppler ultrasonography and computed tomographic portography of the portal system, and gastroscopic gastroesophageal varices postoperatively. The portal venous pressure was evaluated intraoperatively. The operation in the RB group was notably more time-consuming than that in the RT group (P

Aim. To present the first Russian experience in the management of portal hypertension of various etiology by creating a selective portosystemic shunt between left gastric vein and inferior vena cava (leftgastric vein caval bypass).Material and methods. “Left gastric vein to inferior vena cava” bypass was performed in 6 patients with portal hypertension: 4 men and 2 women (mean age 40.4 years). The follow-up period ranged from 10 to 36 months.Results. “Left gastric vein to inferior vena cava” bypass was effective in the management of portal hypertension and its complications in 5 out of 6 patients. Complete eradication of esophagogastric varices and no variceal bleeding were observed in long-term period. These patients demonstrated no clinical or laboratory signs of hepatic encephalopathy and/or hepatic failure within the follow-up. One patient with thrombophilia and extrahepatic portal vein obstruction (factor V Leiden mutation) had thrombosis of the shunt and recurrent variceal bleeding in 6 months after surgery because of unauthorized abandonment of the anticoagulation.Conclusion. “Left gastric vein to inferior vena cava” bypass is an effective procedure for prevention of variceal bleeding (or recurrence) in patients with portal hypertension. The limitation of this technique is insufficient diameter of left gastric vein in many patients. This procedure has certain pathophysiological advantages over other types of portosystemic anastomoses due to highly selective nature of the shunt. Thus, this approach should be introduced into surgical treatment of these patients.

Purpose . The basic management task of children with extrahepatic portal hypertension (EHPH) is to prevent bleeding from intestinal and gastric protuberant varicosities. Materials and methods . Meso-portal shunting doesn’t only prevent bleedings from varicose veins, but also restores normal anatomical and physiological relations within the basin of the portal vein. The Clinical Hospital No. 13 has the largest experience of treating patients with EHPH. From 1989 to 2017 we observed 789 patients with portal hypertension aged 2.5 months to 17 years. 23.5% of them included children under 3 years old. Since 2000, meso-portal shunting was done in 261 children. 239 (91.6%) patients had an excellent result such as reduction of varicose veins and restored portal hepatic perfusion. Discussion . A clinical case of a 9-year-old child diagnosed with extrahepatic portal hypertension was presented. The girl underwent meso-portal shunting. However, no reduction of intestinal varicose veins was presented during the post-operative period. Meso-portal shunting stenosis was found during an angiographic study. The child had another operation; stenosis of meso-portal shunt was diagnosed, a narrowed section was resected with subsequent restoration of vascular insertion patency. During the postoperative period, varicose veins disappeared completely. Conclusion . The presented clinical example displays modern surgical possibilities of portal hypertension in children even in such complicated cases as stenosis of vascular venous anastomosis occurring following MPS approximately in 10% of cases.

We report the case of a 54-year-old male with alcoholic cirrhosis who presented several times to the emergency department (ED) with right upper quadrant abdominal pain. Ten days after his initial presentation, the patient represented with hypotension and anemia. An abdominal CT angiogram identified hemorrhage from an ectopic varix successfully treated with emergent glue embolization of mesenteric, omental, and periumbilical varices. Intraperitoneal bleeding from ectopic varices in cirrhosis patients is a rare, life-threatening condition. Consideration and recognition of ectopic variceal hemorrhage in patients with cirrhosis can facilitate prompt life-saving treatment in a population susceptible to significant morbidity and mortality.

A solid pseudopapillary tumor (SPT) of the pancreas is an uncommon neoplasm, characterized by a well-encapsulated mass, with low malignant potential. It occurs predominantly in young females. We present a case of SPT of the pancreas which presented with sinistral portal hypertension. Despite characteristic radiological findings due to its rarity, it may be missed to more common conditions like peptic ulcer disease. Delayed diagnosis can lead to complications like portal hypertension. To the best of our knowledge, in existing medical literature, SPT of the pancreas in males has rarely been described. In our case, we found that the tumor was causing extrahepatic portal hypertension which is also a very unique presentation of this tumor. Due to its vague clinical manifestations, definitive diagnosis is often a challenge hence requiring prompt investigations.

Objectives: Chronic pancreatitis produces disabling pain and loss of pancreatic endocrine/exocrine function. Almost half of the patients will need surgery during the course of the disease. Certain conditions, such as extrahepatic portal hypertension or cavernous transformation of the portal vein, can increase the risk of morbidity and mortality. These complications must be borne in mind in the design of the surgical treatment of chronic pancreatitis. This study is a systematic review on the coexistence of chronic pancreatitis and extrahepatic portal hypertension/cavernous transformation in patients undergoing pancreatic surgery. Methods: We conducted an unlimited search updated on 10 December 2017, which yielded 535 results. We selected 11 articles. Results: Main indication for surgery was intractable pain. Presence of extrahepatic portal hypertension and/or cavernous transformation increased intraoperative bleeding and general postoperative morbidity, though the increase in general morbidity was less when the different postoperative complications were analyzed individually. Case series showed a higher mortality in patients with extrahepatic portal hypertension. Conclusion: Little is known about the presence of extrahepatic portal hypertension in patients undergoing pancreatic surgery for chronic pancreatitis. More studies are needed in order to standardize criteria for vascular involvement in patients with chronic pancreatitis, in order to select the surgical technique and, if necessary, to establish contraindications, in this subgroup of patients.

BACKGROUND/AIM This study was concerned with whether vWF (von Willebrand factor) and a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 (ADAMTS13) has altered in patients with cirrhosis and extrahepatic portal hypertension (EPH). We aimed to investigate changes to vWF and ADAMTS13 in children with cirrhosis and EPH. PATIENTS AND METHODS This study was conducted between January and October 2019 with both cirrhosis and EPH patients and with healthy volunteers. The von Willebrand factor antigen (vWF:Ag), von Willebrand Ristocetin cofactor (vWF:RCo), and ADAMTS13 antigen and activity were studied. RESULTS Twenty-eight children with cirrhosis, 16 children with EPH, and 20 healthy controls were included in the study. vWF:Ag and vWF:RCo levels were higher in patients with cirrhosis than in healthy controls (171.65±101.67 vs. 85.86±30.58, P

Large portosystemic venous collaterals pathways can be found in any part of the gastrointestinal tract, but when these veins are not in the gastroesophageal region they are denominated ectopic varices (EcV). In addition, dilated veins in the abdominal wall and retroperitoneum are also classified as EcV. These veins can cause up to 5% of all variceal bleeding in patients with liver cirrhosis and even more in extrahepatic portal hypertension. When compared with esophageal varices, EcV tend to be larger, leading to significant hemorrhage and requiring more time to find the bleeding source during the endoscopic approach. Despite the advances in diagnostic modalities, the management of EcV is still under debate, requiring skilled doctors on a multidisciplinary team. The initial clinical support for treating gastroesophageal variceal hemorrhage can be extrapolated to EcV bleeding. EcV sites, stigmata of high risk of bleeding, hemodynamic profile, local expertise and resources, the patient’s conditions, and underlying diseases can influence the indication of the therapeutic modality. Endoscopic interventions, such as injection of thrombin, cyanoacrylate, sclerotherapy, and rubber band ligation, should be attempted before radiologic or surgical interventions.

The aim of the study was to test the feasibility of spleen stiffness measurement (SSM) by two-dimensional shear wave elastography (2D-SWE) and compare data on its diagnostic use with upper gastrointestinal endoscopy in children with extrahepatic portal hypertension (EHPH) before and after surgery. A total of 44 children were included in this study [34 children with EHPH (main group)] and 10 controls (7.57 ± 1.22 years), who underwent ultrasonography including SSM by 2D-SWE. Patients in the main group also underwent upper gastrointestinal endoscopy (UGE) and CT angiography. The main group was divided into three subgroups: Group A: patients with EHPH without large spontaneous portosystemic shunts (n = 15); Group B: patients with EHPH with large spontaneous portosystemic shunts (n = 9); Group C: patients with EHPVO and after surgical portosystemic shunts (n = 10). According to UGE, children in group A had significantly higher grades of esophageal varices (EV) (2.3 ± 0.14; p

Background The study was to investigate the role of color Doppler ultrasonography in the evaluation of the effect of Rex-bypass shunt on the cavernous transformation of the portal vein (CTPV) in children. Methods Fifty children with symptomatic extrahepatic portal hypertension who received Rex-bypass shunt were retrospectively reviewed, and they were diagnosed with CTPV by ultrasonography. The clinical characteristics were analyzed before and after operation. Results Forty-five patients received color Doppler ultrasonography at 6 months after surgery, and good patency in the bypass vessels was displayed. The platelet count significantly increased (P < 0.001) and the esophagogastric varices were improved significantly (p < 0.001). The patency of bypass vessels on color Doppler ultrasonography was consistent with the changes in the platelet count and the degree of esophagogastric varices on gastroscopy before and after operation. The diameter of bypass vessels at 6 months was slightly larger than that at 7 days after operation, and there was no significant difference in blood flow velocity between two time points (P = 0.507). Conclusions Color Doppler ultrasonography can display the patency, diameter, and flow velocity of bypass vessels. It plays an important role in evaluating the effect of Rex-bypass shunt on the CTPV.

Identification of endoscopic signs of concomitant pathology of the upper gastrointestinal tract allows to determine the tactics of managing patients with different forms of portal hypertension (PG). The analysis of macroscopic picture of esophagus, stomach and duodenum mucosa was carried out in 104 patients with portal hypertension (88 patients with hepatic portal hypertension, 46 – with extrahepatic portal hypertension, 16 – without portal hypertension). The frequency of changes in the esophagus, specific for gastroesophageal reflux disease did not differ significantly in the researched groups. The frequency and severity of varicose veins of the esophagus were diagnosed more often in patients with hepatic form of portal hypertension (OR=10.0, p

Abnormalities of the walls of the biliary tree with occasional obstruction in patients with mainly extrahepatic portal hypertension leads to an entity defined as portal biliopathy. These patients are generally young, asymptomatic and predominantly from Asian countries. Symptomatic patients with portal biliopathy may present with pain, jaundice and upper gastrointestinal (GI) bleeding. They are difficult to manage and may require both diagnostic and therapeutic endoscopic procedures followed, if necessary, by a lienorenal shunt. After a shunt, the portal biliopathy usually regresses in most patients, but in some, especially those who have undergone endoscopic intervention, the obstruction remains. The reversal of portal biliopathy after portal decompression has been described usually six months after surgery. We report a reversal of portal biliopathy after a splenorenal shunt as early as eight days after the procedure.

Since 2008, the modified Rex shunt has been used for the treatment of extrahepatic portal venous obstruction (EHPVO) in our center, and satisfactory results were initially achieved. However, the postoperative recurrence rate gradually increased. We therefore performed a retrospective study to evaluate the outcome and identify the optimal procedure.Between October 2008 and March 2016, 79 children with EHPVO underwent the Rex shunt in our hospital: 48 underwent the gastroportal shunt (GP), 26 underwent the portal cavernoma-Rex shunt with interposition of grafted portal vessel (PP), and 5 underwent a spleen-preserving splenoportal bypass (SP). The results of these three types of operation were compared.The portal pressure and splenic size were significantly reduced after surgery (P .05). The levels of platelet and hemoglobin were significantly increased after surgery (P .05). The operative time in the GP group was significantly longer than in the PP group (P = .045). The diameter of the bypass vein was significantly larger in the GP group than in the PP group (P = .029). Postoperative rebleeding occurred in 19%: 22.9% (11 of 48) in the GP group, 11.5% (3 of 26) in PP group, and 20% (1 of 5) in the SP group. There was no significant difference in the splenic size, postoperative portal pressure, hospital stay, blood flow velocity, or hemoglobin among the three groups (P.05).The modified Rex shunt is an effective treatment of EHPVO in children, and we believe that the PP procedure is the most suitable modified Rex shunt in our center.

Individuals with trisomy 21 have an 80% risk reduction of vascular anomalies compared with general population. However, an association of trisomy 21 and portal vascular and arteriovenous anomalies has been defined in the literature. The primary hemodynamic abnormality in portal hypertension is increased resistance to portal blood flow. In various case reports in adults and pediatric age group patients, transarterial coil embolization of hepatoportal fistula was described. One of the authors of this article has previously reported successful treatment of congenital arterioportal fistula (APF) with percutaneous transhepatic liquid embolization in a patient who previously had transarterial coil embolization. To date, eight patients with trisomy 21 (Down syndrome) and congenital portosystemic shunts were reported of which four were treated with embolization. Here, we describe a 3-month-old infant with trisomy 21 and intrahepatic APF associated with extrahepatic portal hypertension and massive ascites. In the current report, a rare case of a patient with a diagnosis of trisomy 21 is discussed who was attempted to be treated with transarterial coil embolization and percutaneous transhepatic liquid embolization of the congenital APF in a single session.

Gastrointestinal varices are a consequence of portal hypertension that can occur in the setting of cirrhosis or extrahepatic portal vein obstruction. Increased intrahepatic vascular resistance, a hyperdynamic circulation, and increased flow through the portal and collateral venous system lead to persistently elevated portal pressures that result in angiogenesis and formation of collaterals between the portal and systemic circulation. Despite this physiological attempt at decompression, portal hypertension persists as collateral vessels have higher resistance than the normal liver. Variceal wall tension is the main factor that determines vessel rupture and bleeding occurs when tension in the wall exceeds the limit of elasticity of the vessel. Progressive distension leads to increasing resistance to flow and hemorrhage ensues when the limits of resistance to further dilation are surpassed. Gastroesophageal varices are present in 50% of patients with cirrhosis and progress in size at a rate of 8%-10% per year. Hemorrhage occurs at a rate of approximately 12% per year and large esophageal varices carry a higher risk of rupture. Gastric varices occur in 20% of patients with portal hypertension and bleed less frequently, but more severely. Cardiofundal varices have a complex vascular anatomy that is important to consider as it pertains to the effectiveness of strategies used for management. Ectopic varices make up 2%-5% of all variceal bleeding, occur more frequently in patients with extrahepatic portal hypertension, and their identification should prompt assessment of the intra-abdominal vasculature. Varices in the setting of splenic vein thrombosis should be considered a distinct entity owing to their disparate etiologic basis and treatment approach.

Background: Portal hypertension is a clinical syndrome defined by a pathologic increase of portal venous pressure. The objectives of this study were to evaluate etiological and clinical presentation of portal hypertension admitted in a tertiary care centre of Bangladesh.Materials and Methods: This cross sectional study was done at the Department of Paediatric Gastroenterology & Nutrition, BSMMU on 100 consecutive cases admitted during the period from July 2013 through June 2015. Confirmation of the presence of portal hypertension was done by demonstration of oesophageal varices during upper GI endoscopic examination. The diagnosis of chronic liver disease was based on a combination of clinical, biochemical (abnormal liver function tests) and ultrasonographic (coarse echotexture of liver) features. Extrahepatic portal hypertension was diagnosed on the basis of clinical, normal liver function test and ultrasonographic evidence of portal or splenic vein thrombosis, with or without cavernous transformation. Doppler ultrasound showed increase portal venous pressure.Results: Patient's age group was 2 to 15 years. 69 cases were extra hepatic portal hypertension. Haematemesis and/or melaena were found in 42(60.9%) and splenomegaly was found in 68(98.6%) cases. Of extra hepatic 69 cases 10% had history of umbilical sepsis. Of the 31cases of CLD with portal hypertension, haematemesis and/or melaena was found in 20(64.5%) cases, splenomegaly in 30(96.8%), ascites 8(25.8%).Most (50%) of CLD cases were cryptogenic followed by Wilson’s disease 29%. Of the 100 cases, endoscopy revealed grade 3 esophageal varices in 45% cases. All the patients were treated with propranolol. EVL was done in 70% cases.Conclusion: In this study, most of the cases were extra hepatic portal hypertension. Gastrointestinal bleeding & splenomegaly were found in most of the cases. No risk factor was found in most of extra hepatic cases. Portal vein thrombosis & cryptogenic were the most common cause in extra-hepatic and intra-hepatic cases respectively.J Shaheed Suhrawardy Med Coll, June 2016, Vol.8(1); 26-29