Extrahepatic Portal Hypertension Research Articles

Angiographic patterns of portal venous system in children with extrahepatic portal hypertension and its etiological and clinical relevance.

To study the relationship between the angiographic pattern of extrahepatic portal vein obstruction (EHPVO) and its etiology and clinical manifestations. Clinical, etiological, and angiographic findings in 155 children with EHPVO were reviewed. Anatomy of extrahepatic portal venous system (EPVS) was categorized into five imaging patterns. Assessment of the severity of esophageal and gastric varices (EV and GV) was performed by upper gastrointestinal endoscopy. Based on multislice CT angiography, most commonly observed pattern of EHPVO was type I (48.4%) and type II (29%). According to anamnesis, 68 (43.8%) children had pathological conditions in neonatal period. Of these, 35 (22.6%) had an umbilical vein catheterization, 11 (7.1%) had a history of omphalitis, and 9 (5.8%) had prolonged jaundice. Thirteen (8.4%) patients had various septic conditions in neonatal period and it was more common associated with widespread thrombosis throughout the EPVS (type 5)-28% of observations. Significantly lower risk of bleeding from EV (p = 0.01) was noted in children with type IV pattern, whereas children with type III and V patterns had higher grades of EV. Angiographic pattern of portomesenteric occlusion may provide a clue to its etiology, and clinical manifestation, especially in children with widespread thrombosis throughout the EPVS.

Rare sources of bleeding in extrahepatic portal hypertension developed after surgery on the organs of the gepatopancreatobiliary zone

Rare sources of bleeding in extrahepatic portal hypertension developed after surgery on the organs of the gepatopancreatobiliary zone

Analysis of recurrent bleeding rate after various surgical interventions in extrahepatic portal hypertension

Aim. To compare the incidence and timing of recurrent bleeding from esophageal and gastric varices against the background of extrahepatic portal hypertension after various interventions.Materials and methods. The research methodology involved analyzing the treatment results of 131 patients with extrahepatic portal hypertension who underwent various surgical interventions from 2006 to 2020. Total dissociation of the gastroesophageal collector was carried out in 57 patients (43.5%), portosystemic shunting – in 51 (38.9%), endoscopic interventions – in 23 (17.6%).Results. When comparing the recurrent bleeding rate, it was found out that the most effective intervention is portosystemic shunting. After endoscopic interventions the bleeding rate is 65.2%, and rebleeding occurs in 13.5 ± 2.9 months. After total dissociation of the gastroesophageal collector, a recurrence-free course was observed in 35.1% of cases (p = 0.014), with timing of 25.9 ± 4.4 months (p < 0.05). In terms of timing, this did not differ from the recurrence after portosystemic shunt surgery (41.4 ± 8.2 months), but was significantly less in the bleeding rate after decompressive surgery (17.6%; p < 0.001). Conclusion. Portosystemic shunt surgery proves to be the most effective intervention in extrahepatic portal hypertension, with a five-year remission in 75% of cases. After dissociative surgeries, 35.1% of patients experienced recurrent bleeding, with average timing of 19.6 months.

25, 50 & 75 years ago.

25, 50 & 75 years ago.

SAT299 - Clinical significance of three-dimensional printing of the portal venous system in patients with extrahepatic portal hypertension

SAT299 - Clinical significance of three-dimensional printing of the portal venous system in patients with extrahepatic portal hypertension

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.

Anesthetic management of a repeatedly operated patient with extrahepatic portal hypertension: Сase report

BACKGROUND: The use of high doses of opioids and general anesthetics can cause unwanted side effects that directly affect the patient's recovery and his psycho-emotional and social well-being. In this regard, the general anesthesia method enables the dose reduction of general anesthetics and opioid analgesics. This is relevant, especially in children with concomitant thrombocytopenia, in whom epidural blockade is contraindicated.
 CASE REPORT: This article describes using the alpha-2-adrenergic agonist, dexmedetomidine, for anesthesia in a 16-year-old girl during corrective surgery for extrahepatic portal hypertension after several previous operations. Using the selective alpha-2-adrenergic agonist, dexmedetomidine, for general anesthesia produced effective and stable pain relief intraoperatively and rapid recovery of spontaneous breathing and consciousness after anesthesia. The total dose of intraoperative consumption of fentanyl compared with previously performed anesthesia was less than two times, and sevoflurane by 30%, while maintaining adequate anesthetic protection.
 CONCLUSION: During surgical interventions, it is impossible to perform epidural anesthesia in patients with thrombocytopenia with extrahepatic portal hypertension. The centrally acting alpha-2-agonist, dexmedetomidine, may be used to reduce the doses of anesthetics and opioid analgesics used.

The clinical applicability of percutaneous splenic vein stent implantation for pancreatic portal hypertension

BackgroundPancreatic portal hypertension (PPH) is a type of extrahepatic portal hypertension. We compared the clinical efficacy of different treatment methods for PPH caused by splenic vein stenosis in chronic pancreatitis.MethodsThis article retrospectively analyzed the PPH cases that were caused by splenic vein stenosis after chronic pancreatitis. Patients were divided into three groups according to the different treatments: splenic vein stent implantation (stent group), splenectomy, and only medications (conservative group). The treatment effects from each group were compared.ResultsA total of 33 patients were retrospectively analyzed in this study (9, 12, and 12 patients in each group respectively). All the procedures were successful in the stent and splenectomy groups. During the follow-up, no patient had gastrointestinal bleeding recurrence in the stent and splenectomy groups. However, in the conservative group, the incidence of portal hypertensive gastropathy and upper gastrointestinal bleeding were 50% and 25%. In the stent group, all the varicose veins at the base of the stomach had shrunk by varying degrees, and the red color signs regressed. The stent patency rate was 100%. No major complication occurred. The average platelet count at 1, 3, 6-months postoperatively were all significantly higher than the preoperative value (P < 0.05). The average postoperative hospital stay duration was significantly shorter than that of the splenectomy group (3.1 ± 1.4 days vs. 16.1 ± 8.1 days; P < 0.05). In the splenectomy group, postoperative fever occurred in 4 patients. Postoperative infection occurred in 2 patients (one with abdominal cavity infection and the other with incision infection). Delayed abdominal bleeding occurred in one patient. Portal vein thrombosis occurred in 2 patients during follow up.ConclusionPercutaneous splenic vein stent implantation for PPH treatment reduces the risk of gastrointestinal bleeding with minimal invasive. It has a high safety and reliable efficacy and is worthy of further clinical promotion.

Refractory ascites of complex genesis in patients with a severe combined form of extrahepatic portal hypertension

This study demonstrates a successful clinical outcome in a female patient with severe refractory ascites associated with a complex combined form of extrahepatic portal hypertension. The study emphasizes the need and particular importance of comprehensive diagnostics and assessment of the angioarchitectonics in the portal vein system, thorough study of the porto–hepatic circulation and central hemodynamics, as well as of other major homeokinesis disorders. Understanding the pathogenesis of the disease allows for the development of a therapeutic plan with a strictly personalized approach toward the selection and staging of various surgical interventions.

Splenic artery aneurysms and extrahepatic portal hypertension during pregnancy

Clinical case demonstrates successful treatment of a pregnant woman, who had multiple giant splenic artery aneurysms with a high risk of spontaneous rupture. Pregnancy proceeded along with hereditary thrombophilia, thrombosis and cavernous transformation of the vena cava, extrahepatic portal hypertension, esophageal and gastric varices II–III. The surgical and obstetric tactics, surgical intervention, appropriate salvage and multidisciplinary pregnancy follow-up ensured a successful Cesarean delivery at 38 weeks, saving the life of the child and mother.

S1618 The Need for Vigilance: A Rare Association of Pancreatic Atrophy With Hermansky-Pudlak Syndrome

Introduction: Hermansky-Pudlak Syndrome (HPS) is an autosomal recessive disorder, which is characterized by oculocutaneous albinism, bleeding disorder, and immunodefiecencies. HPS can involve multiple organs of the body and can cause serious complications in the later part of the disease. Major complications associated with this genetic disorder are granulomatous colitis and pulmonary fibrosis. Here in, we report a case of a young male with classic symptoms of HPS with renal, pulmonary, and pancreatic complications. Case Description/Methods: A 32-year-old man presented with a dry cough and shortness of breath. On clinical examination, he was in respiratory distress with diffuse bilateral inspiratory crackles. Hypopigmentation of hair and skin, clubbing in all fingers, were evident. The eye examination revealed nystagmus of both eyes. Hypo pigmented fundus and hypoplastic fovea were evident on fundoscopic examination. Chest Imaging was suggestive of interstitial lung disease with a restrictive pattern in spirometry. Blood chemistry was significant for elevated blood urea and serum creatinine. On further reviewing of his medical records, we found that his symptoms were ongoing since 15 years with worsening photophobia, decreased vision, recurrent urinary tract and ear infections, respiratory distress and worsening kidney function. Another interesting finding in the CT scan of the abdomen was extrahepatic portal hypertension and atrophy of the body and tail of the pancreas. He was diagnosed with HPS based on the above findings. Discussion: Pathogenesis of HPS can be attributed to mutations in the genes responsible for lysosome-related organelle(LRO) function in melanocytes and platelets, leading to albinism and bleeding disorder respectively. Besides typical clinical features of HPS with respiratory and renal complications, our patient also had portal hypertension and atrophy of the head and neck of the pancreas. As per our literature review, ours is the first case reporting pancreatic involvement in HPS. The mechanism behind pancreatic atrophy is unknown. A possible explanation can be the accumulation of ceroid lipofuscin, an amorphous lipid-protein complex, in the biliary or pancreatic duct or within the pancreatic parenchyma, which could cause pancreatic atrophy in the same way as ceroid lipofuscin causing pulmonary fibrosis. The purpose of our case report is to emphasize the possibility of unusual complications of HPS to facilitate the timely intervention to halt the progression of complications.

S2314 Management of Pregnancy in a Patient With Extrahepatic Portal Hypertension Complicated by Gastric Fundal Varices, Splenomegaly and Thrombocytopenia

S2314 Management of Pregnancy in a Patient With Extrahepatic Portal Hypertension Complicated by Gastric Fundal Varices, Splenomegaly and Thrombocytopenia

Особливості діагностики енцефалопатії у хворих з синдромом портальної гіпертензії

Актуальність. Печінкова енцефалопатія — патологічні зміни функцій головного мозку незапального генезу, що проявляються синдромом вторинних неврологічних і когнітивних розладів, спричинених комплексом глибоких метаболічних порушень, в результаті гострої печінкової недостатності, хронічних захворювань печінки або портосистемного шунтування крові. Початкова стадія енцефалопатії не має клінічних неврологічних симптомів, але визначається у 20–80 % хворих на цироз печінки і на відміну від кінцевих стадій є оборотною. Мета дослідження: оцінити ефективність тесту зв’язку чисел, флікер-тесту та електроенцефалографії в діагностиці енцефалопатії у хворих з печінковою та позапечінковою формою портальної гіпертензії. Матеріали та методи. Синдром печінкової енцефалопатії вивчено у 50 хворих з печінковою формою портальної гіпертензії, 30 хворих з позапечінковою формою портальної гіпертензії та 25 осіб без ознак портальної гіпертензії. Для діагностики використовували тест зв’язку чисел, флікер-тест та електроенцефалографію. Результати. Тест зв’язку чисел дозволив встановити ознаки печінкової енцефалопатії майже в усіх хворих незалежно від наявності та форми портальної гіпертензії. Однак прояви печінкової енцефалопатії за частотою і за вираженістю переважали у хворих з печінковою портальною гіпертензією. Проведення флікер-тесту дозволило діагностувати мінімальну печінкову енцефалопатію у 96 % хворих з печінковою портальною гіпертензією та 60 % хворих з позапечінковою портальною гіпертензією. Виконання електроенцефалографії визначило наявність печінкової енцефалопатії у 60 % хворих з печінковою портальною гіпертензією, 50 % хворих з позапечінковою портальною гіпертензією та 12 % хворих без портальної гіпертензії. Висновки. Зіставлення ефективності діагностичних тестів показало, що простий у виконанні і доступний тест зв’язку чисел є найбільш ефективним, однак і інші тести мають свої позитивні сторони в клінічній оцінці печінкової енцефалопатії у хворих з портальною гіпертензією.

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.&#x0D; 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.&#x0D; 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.&#x0D; 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.

СЕЛЕКТИВНАЯ ДЕВАСКУЛЯРИЗАЦИЯ ДНА ЖЕЛУДКА У ПАЦИЕНТОВ С ХРОНИЧЕСКИМ ПАНКРЕАТИТОМ И ПОДПЕЧЁНОЧНОЙ ПОРТАЛЬНОЙ ГИПЕРТЕНЗИЕЙ. Предварительное сообщение о серии случаев

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.

Multidetector-Row Computed Tomography and Colour Doppler Imaging in the Evaluation of Patients with Extrahepatic Portal Hypertension: A Prospective Study

Background and aims: Evaluating patients of extrahepatic portal hypertension with Colour Doppler and MDCT to study the morphology of splenoportal axis, determining the site of obstruction, the anatomy of portosystemic collaterals, any associated vascular aneurysms, and features of portal biliopathy. Materials and methods: We studied 45 patients (aged 6-55 years) with clinical features of portal hypertension with no identifiable hepatic cause or extrahepatic portal venous occlusion on sonography. All cases underwent Doppler ultrasound and MDCT. The data was compiled and analyzed. A comparative evaluation of Doppler imaging and MDCT was performed with the help of Cohen's kappa test to assess the agreement between the two modalities. Results: MDCT provides better details about the morphology of the splenoportal axis, the site, and cause of block, as well as the extent and distribution of collateral groups. Colour Doppler has an advantage over MDCT in determining the direction and pattern of blood flow within the splenoportal axis, various collaterals, and spontaneous shunts, along with a better depiction of features of portal biliopathy. Colour Doppler is inadequate in the visualization of gastroesophageal and retroperitoneal varices. Identification of spontaneous spleno-adreno-renal and gastrorenal shunts is achieved with the high agreement between Colour Doppler and MDCT. Conclusion: MDCT should be preferred for preoperative evaluation of patients being contemplated for shunt surgery. Colour Doppler imaging of portal biliopathy is sufficiently characteristic to avoid other invasive modalities. The two modalities provide complementary information in the evaluation of patients with extrahepatic portal hypertension.

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.

Optimal Rex shunt procedures as a treatment for pediatric extrahepatic portal hypertension.

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 < 0.05). Compared to RT shunt, the reduction in gastroesophageal varix grading, the increases in platelets, and the caliber of the bypass were greater in the RB group (P < 0.05). Although not statistically significant, higher morbidity of surgical complications was found after RT shunt (17.4%) compared with RB shunt (8.3%) with patency rates of 82.6 and 91.7%, respectively. Additionally, patients exhibited a lower rate of rebleeding under the RB procedure (12.5%) than under the RT procedure (21.7%). The RT procedure is an alternative option for the treatment of pediatric extrahepatic pHTN, and RB shunt is the preferred procedure in our center.

Antiphospholipid Syndrome (APS) With Chronic Thrombosis of Portovenous System and Extra Hepatic Portal Hypertension - Role of Anticoagulation

Antiphospholipid Syndrome (APS) With Chronic Thrombosis of Portovenous System and Extra Hepatic Portal Hypertension - Role of Anticoagulation

Оптимизация тактики ведения беременных с портальной гипертензией различного генеза

Objective. To optimize the management strategies in pregnant women with portal hypertension (PH) of different genesis. Patients and methods. Forty-four women with PH aged 19 to 44 years were examined and divided into two groups depending on the pathogenesis of PH. Group I (n = 24) included women with extrahepatic PH, group II (n = 20) – women with hepatic PH. Esophagogastroduodenoscopy, ultrasound, doppler ultrasound, fetal cardiotocography, and clinical and biochemical laboratory examinations were used as research methods. Results. Thrombophilia, anemia of varying severity, and liver cirrhosis have an impact on the development of pre-eclampsia, threatened preterm labor, placental insufficiency, and low birth weight in newborns. PH during pregnancy is a risk factor for bleeding from esophageal varices. Careful monitoring of patients using instrumental methods and management of pregnancy by obstetrician-gynecologists and surgeons create conditions for favorable perinatal outcomes. Conclusion. The risk of bleeding from esophageal varices during pregnancy, regardless of gestational age, and in the postpartum period is significantly more common in patients in group I compared to group II; the method of prevention is anticoagulant therapy. Key words: pregnancy, placental insufficiency, portal hypertension, preterm birth, cirrhosis