Surgery For Portal Hypertension Research Articles

Comparison of surgical outcomes between shunt surgery and devascularization in non-cirrhotic portal hypertension

Objective: Non-cirrhotic portal hypertension (NCPH) is the most common cause of portal hypertension and upper gastro-intestinal bleeding in children and adolescents in developing nations. It is characterized by features of portal hypertension with preserved liver function. Proximal splenorenal shunt (PSRS) and esophagogastric devascularization are the two most commonly performed surgeries for its management. The present study is aimed at comparison of surgical outcomes between these two procedures. Material and Methods: Between April 2018 and March 2022, prospectively maintained data of consecutive NCPH cases who underwent surgical intervention was reviewed retrospectively. Cases were categorized into two groups- shunt surgery and devascularization. The pre-operative characteristics, peri-operative morbidity and long-term outcomes were compared between the groups. Results: Of 112 cases who were treated during the study period, 54 cases which underwent surgery were included in the study. Of these, 20 cases underwent PSRS, and splenectomy and devascularization was performed in 34 cases. There was no difference in pre-operative variables between the two groups. Patients undergoing PSRS experienced longer duration of surgery (260 vs. 200 minutes, p< 0.001), and those in the devascularization group had significantly greater operative blood loss (350 vs. 455 ml, p< 0.001). Post-operative morbidity was comparable between the two groups. Hypersplenism was corrected in all cases and no cases reported rebleeding after median follow-up of 30 months. Three cases in each group developed features of portal biliopathy in follow up period. Conclusion: Both PSRS and devascularization procedures have comparable efficacy and safety in the management of NCPH.

Early mobilization and delayed arterial ligation (EMDAL) as a surgical technique for splenectomy and shunt surgery in portal hypertension.

Splenectomy is the most frequently performed procedure as definitive management or as part of shunt surgery or devascularization in portal hypertension. Splenectomy is technically challenging because of the frequent coexistence of multiple collateral varices, splenomegaly, poor liver function, and thrombocytopenia. Early arterial ligation and late mobilization (EALDEM) is the traditional method for splenectomy in portal hypertension. Early spleen mobilization offers good control of the hilum. We aim to compare the effect of the early mobilization and delayed arterial ligation (EMDAL) technique with that of the conventional splenectomy technique in patients with portal hypertension. During the study period from September 2011 to September 2022, 173 patients underwent surgical intervention for portal hypertension at our institution. Among these patients, 114 underwent the conventional method of splenectomy (early arterial ligation and late splenic mobilization) while 59 underwent splenectomy with the EMDAL technique. Demographics were compared between the two groups. Intraoperative and postoperative outcomes were analyzed using the Mann-Whitney test in each group. A minimum follow-up of 12 months was performed in each group. Demographics and type of surgical procedure were comparable in the two surgical method groups. Median blood loss was higher in the conventional group than in the EMDAL method. The median duration of surgery was comparable in the two surgical procedures. Clavien-Dindo grade III/IV complications were reported more frequently in the conventional group. The splenic hilum can be controlled well and bleeding can be minimised with early mobilization and delayed arterial ligation.

Historical aspects of portal hypertension surgery: evolution of thinking

The paper presents the evolution of views on the surgery of portal hypertension, as well as some historical aspects of its development. The authors considered the main types of surgical interventions to be performed in this pathology. It is noted that in the middle of the 20th century, the direct portacaval anastomosis gained the widest acceptance of all the proposed types of vascular anastomoses. A number of surgeons considered it a classical method of treatment in portal hypertension. Thereafter, the indirect portacaval anastomosis proved to have the best outcomes. The advantage of portacaval anastomoses in comparison with other modifications of surgeries was substantiated by a significant decrease in portal pressure and disappearance of oesophageal varices after developing the anastomosis. Surgical treatment of patients with liver cirrhosis and portal system thrombosis reasonably requires closer attention of doctors in various fields. Such patients should be managed in specialized clinics and well-equipped medical centers.

Comparison of prevention and treatment of portal vein thrombosis between proximal splenic vein ligation followed by postoperative transcatheter anticoagulant therapy and systemic anticoagulant therapy in patients with cirrhotic portal hypertension

AbstractAimsSplenectomy combined with pericardial devascularization is one of the important methods to treat hypersplenism, gastrointestinal bleeding, and other complications caused by liver cirrhosis; however, it is accompanied by a high risk of portal vein thrombosis (PVT). This study aimed to explore the preventive and therapeutic effects of proximal splenic vein ligation (PSVL) with postoperative transcatheter anticoagulant therapy (TCAT) on PVT.MethodsThis study retrospectively selected 143 patients with liver cirrhosis and portal hypertension, who received splenectomy combined with pericardial devascularization from June 30, 2018 to June 30, 2021. According to computed tomography photography, within 1 week before the operation, the patients were divided into a prevention group (without preoperative PVT, n = 112) and a treatment group (preoperative PVT, n = 31). Then, each group was subdivided based on the treatment and prevention measures into PSVL + TCAT (n = 70) and systemic anticoagulant therapy (SAT) subgroups (n = 73). The preventive and therapeutic effects of PSVL followed by TCAT on PVT were analyzed.ResultsThe operation time in the PSVL + TCAT subgroups was longer than that in the SAT subgroups (185 ± 76 min vs. 161 ± 55 min; p < 0.01). There was no difference between the two subgroups in terms of operative bleeding (345 ± 82 mL vs. 336 ± 65 mL; p > 0.50). There was no operative death, and all patients recovered uneventfully. In the prevention group, procedure‐related complications occurred in two patients in the PSVL + TCAT subgroup (3.7% [2/54]), including one patient with slight pancreatitis and one patient with chylous leakage, owing to mobilization of the pancreas. The PVT incidence in the prevention group was significantly different between the two subgroups at postoperative 7th day, 3rd month, and 6th month (PSVL + TCAT: 0%, 11.1%, and 5.6% vs. SAT: 39.7%, 31.0%, and 20.7%, respectively; all p < 0.05). In the treatment group, the thrombus regression rate at postoperative 7th day and disappearance rates at the 3rd month and the 6th month of the PSVL + TCAT subgroup were significantly higher than those in the SAT subgroup after anticoagulant and thrombolysis therapy (PSVL + TCAT: 75.0%, 68.8%, and 87.5% vs. SAT: 20.0%, 26.7%, and 40.0%; all p < 0.05).ConclusionsPSVL + TCAT reduces the risk of PVT after splenectomy and is safe and effective in treating PVT during surgery for portal hypertension.

The effectiveness of splenorenal shunt in the correction of portal hypertension and hypersplenism in the long-term period

The aim is to evaluate the long-term results of splenorenal anastomosis (SRA) in the treatment of portal hypertension a year or more after surgery. Materials and methods. The long-term results of the imposition of CPA after one to ten years have been studied. Life expectancy, the number of bleeding recurrences, and the severity of thrombocytopenia were assessed based on the results of fibrogastroscopy, duplex scanning (DS), and computed tomography with bolus enhancement. Results. One-year survival rate was 96.77%, three-year - 87.09%, five-year - 74.19%, ten-year - 22.58%. Within three years after bypass surgery, no recurrence of bleeding was observed, while in half of the patients (54.28%) the varicose veins of the esophagus disappeared, in the rest (45.72%) they decreased to grade I. Recurrence of bleeding occurred in six patients (17.14%) after 5-13 years, in one case massive, which was accompanied by shunt thrombosis (2.85%) according to the results of DS. For the rest, its function was preserved. The median platelet count one year and three after CP did not differ and was 110 x109 and 105 x109. It was found that the larger the diameter of the portal vein and the lower the volume velocity of blood flow in it, the smaller was the increase in platelets in the long-term period (r=0.90, p=0.037; r=0.87, p=0.01). Conclusions. After performing splenorenal bypass surgery for portal hypertension for the purpose of primary and secondary prevention of gastric bleeding, its recurrence occurred in 17.14% 5-13 years after surgery. After the correction of portal hypertension, there was a tendency to increase the number of platelets in the long-term postoperative period, which was dependent on the volume velocity of blood flow in the portal vein and the degree of its expansion before surgical treatment.

Unconventional shunt surgery for non-cirrhotic portal hypertension in patients not suitable for proximal splenorenal shunt.

Proximal splenorenal shunt (PSRS) is a commonly performed procedure to decompress portal hypertension, in patients with refractory variceal bleed, especially in non-cirrhotic portal hypertension (NCPH). If conventional methods are hindered by any technical or pathological factors, alternative surgical techniques may be required. This study analyzes the effectiveness of various unconventional shunt surgeries performed for NCPH. A retrospective analysis of NCPH patients who underwent unconventional shunt surgeries during the period July 2011 to June 2022 was conducted. All patients were followed up for a minimum of 12 months with doppler study of the shunt to assess shunt patency, and upper gastrointestinal endoscopy to evaluate the regression of varices. During the study period, 130 patients underwent shunt surgery; among these, 31 underwent unconventional shunts (splenoadrenal shunt [SAS], 12; interposition mesocaval shunt [iMCS], 8; interposition PSRS [iPSRS], 6; jejunal vein-cava shunt [JCS], 3; left gastroepiploic-renal shunt [LGERS], 2). The main indications for unconventional shunts were left renal vein aberration (SAS, 8/12), splenic vein narrowing (iMCS, 5/8), portalhypertensive vascular changes (iPSRS, 6/6), and portomesenteric thrombosis (JCS, 3/3). The median fall in portal pressure was more in SAS (12.1 mm Hg), and operative time more in JCS, 8.4 hours (range, 5-9 hours). During a median follow-up of 36 months (6-54 months), shunt thrombosis had been reported in all cases of LGERS, and less in SAS (3/12). Variceal regression rate was high in SAS, and least in LGERS. Hypersplenism had reversed in all patients, and 6/31 patients had a recurrent bleed. Unconventional shunt surgery is effective in patients unsuited for other shunts, especially PSRS, and it achieves the desired effects in a significant proportion of patients.

Easy to say, hard to do. To the anniversary of two fundamental discoveries. Part 3

On the eve of the anniversaries of two historical events: the 145th anniversary of the experiments in which Eck's fistula was performed and the 55th anniversary of the successful clinical approbation of the selective distal splenorenal anastomosis, a retrospective analysis of the key historical stages in the development of portal hypertension surgery was performed: from the first attempts to describe the anatomy of the hepatic vascular system, explaining its purpose in the body, the development of direct portacaval anastomosis, and the widespread use of selective splenorenal anastomoses, to the Transjugular Intrahepatic Portosystemic Shunt procedure and orthotopic liver transplantation. Meantime, the emphasis has been focused on the most colorful characters of researchers and clinicians who passed this path. The expediency of an integrated approach in solving the problems of portal hypertension with the development of both fundamental and applied clinical and organizational aspects has been substantiated. It is shown that the discoveries born from summarizing the results achieved by numerous researchers have contributed to a better understanding of this field of medicine, have become a solid foundation for what we have today and are a reliable platform for a successful start into the future.

Assessment of risk in portal hypertension surgery in patients with liver cirrhosis (literature review)

Severity of liver dysfunction, comorbidities, type and complexity of surgery, including elective or emergency surgery, are determinants of perioperative mortality and morbidity in patients with cirrhosis. Risk stratification is a powerful decision-making tool and should be a mandatory part of surgical practice. In this paper we briefly describe and define perioperative risk scores commonly used for portal hypertension surgery in terms of their predictive values and clinical applicability to predict morbi-, mortality postoperative.

Easy to say, hard to do. To the anniversary of two fundamental discoveries. Part 2

On the eve of the anniversaries of two historical events: the 145 th anniversary of the experiments in which Eck's fistula was performed and the 55th anniversary of the successful clinical approbation of the selective distal splenorenal anastomosis, a retrospective analysis of the key historical stages in the development of portal hypertension surgery was performed: from the first attempts to describe the anatomy of the hepatic vascular system, explaining its purpose in the body, the development of direct portacaval anastomosis, and the widespread use of selective splenorenal anastomoses, to the Transjugular Intrahepatic Portosystemic Shunt procedure and orthotopic liver transplantation. Meantime, the emphasis has been focused on the most colorful characters of researchers and clinicians who passed this path. The expediency of an integrated approach in solving the problems of portal hypertension with the development of both fundamental and applied clinical and organizational aspects has been substantiated. It is shown that the discoveries born from summarizing the results achieved by numerous researchers have contributed to a better understanding of this field of medicine, have become a solid foundation for what we have today and are a reliable platform for a successful start into the future.

Easy to say, hard to do. To the anniversary of two fundamental discoveries. Part 1

On the eve of the anniversaries of two historical events: the 145th anniversary of the experiments in which Eck's fistula was performed and the 55th anniversary of the successful clinical approbation of the selective distal splenorenal anastomosis, a retrospective analysis of the key historical stages in the development of portal hypertension surgery from the first attempts to describe the anatomy of the hepatic vascular system, explaining its purpose in the body, the development of direct portocaval anastomosis, the widespread use of selective splenorenal anastomoses, to the Transjugular Intrahepatic Portosystemic Shunt procedure and orthotopic liver transplantation. At the same time, the emphasis is focused on the most colorful characters of researchers and clinicians who passed this path. The expediency of an integrated approach in solving the problems of portal hypertension with the development of both fundamental and applied clinical and organizational aspects is substantiated. It is shown that the discoveries born from the generalization of the results achieved by numerous researchers have contributed to a better understanding of this field of medicine, have become a solid foundation for what we have today and are a reliable platform for a successful start into the future.

Chinese expert consensus for laparoscopic esophagogastric devascularization(2021)

Laparoscopic esophagogastric devascularization has the advantages of less trauma, less bleeding and faster recovery.In order to promote this technology in a scientific and standardized manner and to guide clinical practice, the Chinese Society of Spleen and Portal Hypertension Surgery, Chinese Medical Association, discussed and composed the "Chinese expert consensus for laparoscopic esophagogastric devascularization（2021）".This consensus adheres to the standardization and minimally invasive principles of surgical treatment of portal hypertension, and elaborates the surgical indications and contraindications, preoperative evaluation, surgical technical points and precautions, postoperative management and common complications, especially the prevention and treatment of portal vein thrombosis.

Umbilical hernia repair in patients with cirrhosis: who, when and how to treat.

Hernia management in patients with cirrhosis is a challenging problem, where indication, timing and type of surgery have been a subject of debate. Given the high risk of morbidity and mortality following surgery, together with increased risk of recurrence, a wait and see approach was often advocated in the past. The purpose of this review was to provide an overview of crucial elements in the treatment of patients with cirrhosis and umbilical hernia. Perioperative ascites control is regarded as the major factor in timing of hernia repair and is considered the most important factor governing outcome. This can be accomplished by either medical treatment, ascites drainage prior to surgery or reduction of portal hypertension by means of a transjugular intrahepatic portosystemic shunt (TIPS). The high incidence of perioperative complications and inferior outcomes of emergency surgery strongly favor elective surgery, instead of a "wait and see" approach, allowing for adequate patient selection, scheduled timing of elective surgery and dedicated perioperative care. The Child-Pugh-Turcotte and MELD score remain strong prognostic parameters and furthermore aid in identifying patients who fulfill criteria for liver transplantation. Such patients should be evaluated for early listing as potential candidates for transplantation and simultaneous hernia repair, especially in case of umbilical vein recanalization and uncontrolled refractory preoperative ascites. Considering surgical techniques, low-quality evidence suggests mesh implantation might reduce hernia recurrence without dramatically increasing morbidity, at least in elective circumstances. Preventing emergency surgery and optimizing perioperative care are crucial factors in reducing morbidity and mortality in patients with umbilical hernia and cirrhosis.

Chinese expert surgical procedure consensus on open pericardial devascularization(2021)

Although the treatment strategy of esophageal and gastric varices bleeding in portal hypertension has been diversified and multidisciplinary now,the surgical treatment represented by pericardial devascularization operation will still play an important and irreplaceable role in China. In order to standardize the surgical procedure,guide clinical practice and improve the level of surgical treatment of portal hypertension,Chinese Society of Spleen and Portal Hypertension Surgery,Chinese Surgical Society,Chinese Medical Association organized Chinese experts to formulate this consensus. The main contents include:the position of surgical treatment,surgical indications and contraindications,preoperative evaluation,key points and precautions of surgical procedure,perioperative treatment,prevention and treatment of postoperative complications. The consensus emphasizes the standardization of surgical treatment of portal hypertension,pay attention to the prevention and treatment of postoperative portal vein thrombosis,and expect to provide surgeons with clinical guidance.

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.

Expert consensus on clinical diagnosis and treatment of portal hypertension with hepatocellular carcinoma (2022)

At present, there is no uniform standard for diagnosis and treatment of portal hypertension complicated with hepatocellular carcinoma internationally. Although in recent years, with the significant advances of surgical technique and the positive progress of targeted and immunotherapy in the field of hepatocellular carcinoma, the survival of hepatocellular carcinoma patients has improved, but the risk of surgery in patients with portal hypertension complicated with hepatocellular carcinoma remains high, and surgical treatment is still controversial. Therefore, based on the existing evidence, the Chinese Society of Spleen and Portal Hypertension Surgery, Chinese Society of Surgery, Chinese Medical Association has organized relevant experts to develop the consensus on clinical diagnosis and treatment of portal hypertension with hepatocellular carcinoma (2022) after full discussion. This consensus aims to provide the latest guidance for the standardized diagnosis and treatment of portal hypertension with hepatocellular carcinoma in China. Given that most portal hypertension originates from cirrhosis, this consensus only addresses the diagnosis and treatment of cirrhosis-related portal hypertension with hepatocellular carcinoma.

Evaluating the Value of the Genetic Risk Variants of Haem-Oxygenase-1, Vascular Endothelial Growth Factor and Vascular Endothelial Growth Factor Receptor-2 in Prediction of the Presence of Esophageal Varices in Patients with Liver Cirrhosis

Abstract Background: Current guidelines recommend screening for Esophageal Varices (EV) by Esophagogastroduodenoscopy (EGD) for all cirrhotic patients. The cost and invasive nature of endoscopic screening mean that there is an interest in developing noninvasive predictors for EV that would decrease the number of EGDs performed. The genetic factors that are involved in the development and maintenance of porto-systemic collateral circulation have been rarely investigated. Variation at the genes that encode proteins involved in the systemic and splanchnic vasodilation, which include angi-otensin-converting enzyme and endothelial/constitutive nitric oxide synthase, have been found to be involved in the EV risk among patients withcirrhosis. In cirrhosis, Vascular Endothelial Growth Factor (VEGF) receptor 2/VEGF and carbon monoxide (CO) activity are significantly increased and are closely correlated with porto-systemic collateral vasodilations. Haem-Oxygenase-1 (HO-1) is the main rate-limiting enzyme involved in CO production. Aim of Study: This study aimed at investigating the value of the genetic risk variants of HO-1, VEGF and VEGF Re-ceptor-2 (VEGFR-2) in prediction of the presence of EV in patients with liver cirrhosis. Patients and Methods: Our study included 300 Egyptian patients with liver cirrhosis, aged 18 years or older, with no history of gastrointestinal bleeding, hepatic schistosomiasis, hepatocellular carcinoma, injection sclerotherapy or band ligation for EV, surgery for portal hypertension or portal or splenic vein thrombosis by ultrasonography. Patients underwent EGD to evaluate the presence and degree of EV, fibroscan for detection of liver stiffness and genetic investigation of HO-1, VEGF and VEGFR-2. Demographic, biochemical and endoscopic data were collected. Patients were divided into Group I (no varices), Group II A (small varices), Group IIB (large varices) and Group III (100 healthy control subjects).Results: All groups were age & BMI matched, platelet count and serum albumin were significantly lower in group II B compared to groups I & II A (p-value <0.001), while serum bilirubin level was significantly higher in group II B compared to groups I & II A (p-value <0.001). TT genotype SNP of HO-1 was associated with the presence of EV, while low grade varices were more detected in patients who carry AT genotype. Other genetic risk variants of VEGF and VEGFR-2 were not helpful in the prediction of EV or in the differen-tiation between small and large varices. Conclusion: Genetic risk variants of HO-1 genotype are helpful in prediction of EV as well as in differentiation between small and large varices. Combining these genetic variants to other noninvasive parameters will be a more sensitive and specific tool to predict EV in cirrhotic patients; it may help to select patients for endoscopic screening and decrease the number of EGDs. However, further studies will be needed from the economic point of view to determine the applicability of using these noninvasive tools as predictors of varices instead of EGD.

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.

The advances in surgery for cirrhotic portal hypertension in China

The cirrhotic portal hypertension is very common worldwide and poses a serious threat to the health of patients.Over past three decades, the surgical treatment for cirrhotic portal hypertension was strongly challenged by the drugs, endoscopy, interventional therapy and liver transplantation.However, under the multidisciplinary team(MDT) cooperative diagnosis and treatment mode, the surgical treatment still plays a unique and irreplaceable role.Laparoscopic pericardial vascular devascularization is characterized by less injury and bleeding, rapid postoperative recovery, which will coexist with open surgery for portal hypertension. It is important to focus on the development and application of new methods, new technologies and new concepts under the MDT cooperative diagnosis and treatment mode, giving full play to the advantages of each discipline and advocate standardized, individualized and precise treatment should be emphasized to maximize patient clinical benefits.

Expert consensus on diagnosis and treatment of esophagogastric variceal bleeding in cirrhotic portal hypertension (2019 edition)

Portal hypertension is a clinical syndrome which is a consequence of a pathological increase in portal vein pressure due to various causes, among which, cirrhosis being the most common cause. The most basic pathophysiological features of portal hypertension in cirrhosis are increased portal vein pressure due to blocked portal vein blood flow and open collateral circulation. Among the clinical manifestations of portal hypertension in cirrhosis, esophageal and gastric fundus varicose bleeding is the most urgent and the mortality rate is the highest. In order to standardize the diagnosis and treatment plan of esophagogastric variceal bleeding in cirrhotic portal hypertension, the Chinese Society of Spleen and Portal Hypertension Surgery, Chinese Society of Surgery, have renewed and revised this consensus on diagnosis and treatment of esophagogastric variceal bleeding in cirrhotic portal hypertension (2015 edition) hope to provide references for the clinical practices.

Pediatric non-cirrhotic portal fibrosis: role of endoscopic management in determining long-term outcome.

Non-cirrhotic portal fibrosis (NCPF) is a rare cause of pediatric portal hypertension. There is abundant literature in adults but paucity of data in children. We aimed to evaluate and compare the endoscopic and long-term outcomes of children with NCPF. Consecutive children (≤ 18years) diagnosed with NCPF evaluated for clinical and endoscopic profile and outcome. The cohort underwent 3weekly endoscopic sessions until esophageal variceal eradication followed by 6-12monthly endoscopic surveillance. Non-bleeders and bleeders were compared for endoscopic outcome. Forty-five NCPF children with median age of 14.5 (6-18) years and symptom duration 12 (1-120) months presented with spleen-related issues (78%), esophageal varices (96%), primary gastric varices (56%), and portal hypertensive gastropathy (89%). Thirty-three patients undergoing endotherapy (secondary prophylaxis n = 22, primary prophylaxis n = 11) showed primary eradication of varices after 5 (2-12) sessions. 36% showed recurrence of esophageal varices in 11 (6-42) months and secondary gastric varices developed in 12%. Overall 87% patients required endoscopic intervention at onset or follow-up. Poor outcome was observed in ten patients (n = 9 bleeders). Three children died of variceal bleeding before endoscopic eradication of esophageal varices. Three developed decompensation after a median follow-up of 48 (3-120) months and referred for liver transplantation. Four patients required surgery for portal hypertension. Overall, endoscopic outcome of NCPF is favorable. One-third patients have recurrence of esophageal varices. Small proportion of bleeders have poor outcome.