Extra hepatic portal vein obstruction due to combined protein ‘C’ and ‘S’ deficiency - A case report

Extra-hepatic portal vein obstruction (EHPVO) is an important cause of non-cirrhotic portal hypertension, in Third World countries like India. In this disorder, it results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intra-hepatic portal vein, splenic vein, or superior mesenteric vein resulting in portal hypertension and esophagogastric varices. Extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins which results in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, patients may have symptoms of portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Patients with extra-hepatic portal vein obstruction are usually young and belong to India and other Asian countries. The variceal bleeding in EHPVO can be managed by endoscopic obliteration of varices, or by portosystemic shunt surgery. In this case report, we present a case of 15 year old male, with extra-hepatic portal vein obstruction due to combined deficiency of Protein C and Protein S recanalized by short-term low molecular heparin plus oral Rivaroxaban therapy

Surgical Management of Portal Hypertension: Experience at a Medical College Hospital in South India

Introduction: Portal hypertension in the presence of cirrhosisof liver carries poor prognosis. The medical managementalong with endoscopic therapy helps to reduce bleeding.Surgery is reserved for patients who fail medical therapy.Patients with portal hypertension with good functioning liverbenefit from surgery. Study aimed to evaluate the resultsof surgical treatment for portal hypertension at our centerKarnataka Institute of Medical Sciences Hubli. Karnataka.Material and methods. This was a prospective observationalstudy. There were 34 patients undergoing surgical treatmentfor various presentations of portal hypertension during theperiod of 2015 to 2019.They were analyzed for demographics,etiology, presentation, various surgeries and outcome. Thedata was entered intoMicrosoft excel sheet and analyzed.Results: Of the 34 patients males were most common.Variceal bleeding was most common presentation followedby painful splenomegaly and anemia. ‘Extrahepatic portalvein obstruction’ was the leading cause of non-cirrhoticportal hypertension followed by ‘non cirrhotic portal fibrosis’and ‘left sided or sinistral portal hypertension’. Proximallinorenal shunt was the most common procedure followedby splenectomy with esophagogastric devascularization. Themorbidity and mortality were very low and yielded durablesatisfactory outcome.Conclusion: The surgery for non-cirrhotic portalhypertension has durable and satisfactory results and canbe done with minimal morbidity and mortality at trainedhands. For few selected cirrhotic patients surgery in the formof devascularization or shunt offers immediate relief frombleeding and gives time for future transplant if any.

Proximal Splenorenal Shunts (PSRS) for Extrahepatic Portal Venous Obstruction (EHPVO) in a Tertiary Care Hospital in Odisha

EHPVO more commonly involves children from the lower socioeconomic strata in developing countries. Variceal bleeding is the most common presentation.. Management with endoscopic means provide temporary palliation. It is believed that surgery carries high mortality and rebleeding rates and is followed by portosystemic encephalopathy and postsplenectomy sepsis. However, a proximal splenorenal shunt is a definitive procedure that may be more suitable for children, particularly in those who have limited access to medical facilities.Our aim was to evaluate the results of proximal splenorenal shunts done in children with extrahepatic portal venous obstruction. Methods: Between Aug 2017 & Jan 2019, we performed 20 elective proximal splenorenal shunts for EHPVO in the Department of Surgical Gastroenterology, SCB Medical College, Cuttack. Outcome was evaluated in term of rebleeding, encephalopathy, and pneumococcal infection. Results: Rebleeding occurred in 4 cases, pneumococcal infection & encephalopathy was detected in one patient & one patient died during follow up. Conclusion: A proximal splenorenal shunt, a one-time procedure with a low mortality rate and good long-term results, is an effective treatment for children in India with extrahepatic portal venous obstruction.

Pathogenesis and multidisciplinary diagnosis and treatment of portal vein embolization / 临床肝胆病杂志

Portal vein embolization involves different pathophysiological processes such as primary or secondary portal vein thrombosis and extrahepatic portal vein occlusion. After clarifying the connotation of portal vein embolization, this article briefly introduces the etiology and grading system of portal vein embolization, as well as its pathogenesis and multidisciplinary collaborative diagnosis and treatment methods, and emphasizes the importance of multidisciplinary collaboration.

Portal Vein Stenting for Delayed Jejunal Varix Bleeding Associated with Portal Venous Occlusion after Hepatobiliary and Pancreatic Surgery

OBJECTIVE: The study aimed to describe portal stenting for postoperative portal occlusion with delayed (≥ 3 months) variceal bleeding in the afferent jejunal loop. MATERIALS AND METHODS: Eleven consecutive patients (age range, 2–79 years; eight men and three women) who underwent portal stenting between April 2009 and December 2015 were included in the study. Preoperative medical history and the postoperative clinical course were reviewed. Characteristics of portal occlusion and details of procedures were also investigated. Technical success, treatment efficacy (defined as disappearance of jejunal varix on follow-up CT), and clinical success were analyzed. Primary stent patency rate was plotted using the Kaplan-Meier method. RESULTS: All patients underwent hepatobiliary-pancreatic cancer surgery except two children with liver transplantation for biliary atresia. Portal occlusion was caused by benign postoperative change (n = 6) and local tumor recurrence (n = 5). Variceal bleeding occurred at 27 months (4 to 72 months) and portal stenting was performed at 37 months (4 to 121 months), on average, postoperatively. Technical success, treatment efficacy, and clinical success rates were 90.9, 100, and 81.8%, respectively. The primary patency rate of portal stent was 88.9% during the mean follow-up period of 9 months. Neither procedure-related complication nor mortality occurred. CONCLUSION: Interventional portal stenting is an effective treatment for delayed jejunal variceal bleeding due to portal occlusion after hepatobiliary-pancreatic surgery.

Research advances in non-cirrhotic portal hypertension / 临床肝胆病杂志

Although liver cirrhosis is the most common cause of portal hypertension (PH), about 20% of PH cases are caused by non-cirrhotic reasons, which are referred to as non-cirrhotic portal hypertension (NCPH), with a high incidence rate in developing countries. NCPH is a group of heterogeneous hepatic vascular diseases, including idiopathic portal hypertension (IPH) and extrahepatic portal vein obstruction (EHPVO), as well as the rare diseases in clinical practice such as Budd-Chiari syndrome, congenital hepatic fibrosis, and nodular regenerative hyperplasia. The patients with NCPH usually have the symptoms of portal hypertension, such as recurrent variceal bleeding and splenomegaly, but liver function is well preserved in these patients. At present, the diagnosis of NCPH lacks a universally accepted standard and remains a challenge. In clinical practice, the method of exclusion is usually applied for the diagnosis of HCPH, and liver biopsy is performed when necessary to make a confirmed diagnosis. This paper introduces the pathogenesis and pathological manifestations of IPH and EHPVO, as well as the selection of diagnostic methods and therapeutic strategies. If upper gastrointestinal bleeding can be effectively controlled, NCPH is considered to have a relatively good prognosis.

Portal hypertensive biliopathy: review of pathophysiology and management.

Portal hypertensive biliopathy is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. In literature it has also been named as “Cholangiopathy associated with portal hypertension”, “Portal biliopathy” and “Portal cavernoma associated cholangiopathy”. It is usually asymptomatic and found incidentally, but rarely patients do present with symptoms of abdominal pain, jaundice, asthenia and fever. Treatment is warranted in symptomatic cases only, and is dictated by the clinical manifestations and complications of the disease process. Due to presence of underlying severe portal hypertension, endoscopic biliary intervention is usually the first line of management, and is relatively safe and often sufficient. When surgery is resorted to, a porto-systemic shunt prior to biliary bypass procedure provides early relief of obstructive biliary symptoms and often precludes the need for a biliary bypass surgery. This review describes the pathophysiology, presentation, progression and management approaches to portal biliopathy.