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

Pregnancy in Patients with Non-cirrhotic Portal Hypertension: A Literature Review / Gravidez em pacientes com hipertensão portal não cirrótica: Uma revisão da literatura

Abstract Pregnancy in non-cirrhotic portal hypertension (NCPH) is an uncommon condition. Its management is challenging both to the obstetricians as well as to the gastroenterologists due to the lack of more extensive studies and standard clinical practice guidelines. These patients are at increased risk of portal hypertension (PTH) complications, especially variceal bleeding, and with an increased incidence of adverse maternal and fetal outcomes. Hence, a multidisciplinary approach is required for management of pregnancy in NCPH. This short review describes the different aspects of pregnancy with NCPH, emphasizing specific strategies for preventing and managing PTH from the preconceptional period to postpartum.

Resumo A gravidez na hipertensão portal não cirrótica (HPNC) é uma condição incomum. Seu manejo é desafiador tanto para os obstetras quanto para os gastroenterologistas devido à falta de estudos mais extensos e diretrizes de prática clínica padrão. Esses pacientes apresentam risco aumentado de complicações da hipertensão portal (PTH) especialmente sangramento por varizes e têm maior incidência de desfechos maternos e fetais adversos. Portanto uma abordagem multidisciplinar é necessária para o manejo da gravidez na NCPH. Esta breve revisão descreve os diferentes aspectos da gravidez com HPNC enfatizando estratégias específicas para prevenção e manejo do PTH desde o período pré-concepcional até o pós-parto.

Imaging Insights into EHPVO Induced Portal Biliopathy and its Grading: Retrospective Study Over A Decade at A Tertiary Care Hospital

Background and Aim: There is limited data on imaging features of extra hepatic portal venous obstruction (EHPVO) induced portal biliopathy. We describe a decade long experience of imaging spectrum of EHPVO induced portal biliopathy using MR/CT in a referral centre for liver diseases. Methods: Retrospective data of patients with primary EHPVO on contrast magnetic resonance imaging MRI/ computed tomography (CT) (n=120) and portal biliopathy (PB) on subsequent magnetic resonance cholangiopancreatography (MRCP) (n=80/120) between June 2009 - 2019 was collected. Categorisation of portal biliopathy was as per the Sarin classification and the corresponding, relevant imaging parameters were studied and analysed. Results: Sarin Type 1 biliopathy was present in 16.3 %, Type 2 in 13.8%, Type 3a in 8.8% and Type 3b in 61.3%patients. The median total serum bilirubin was 1.6 (0.9-3.4) mg/dl with a mean CBD diameter of 6.7 ± 2.9 mm. Bilobar and unilobar IHBRD were observed in 87.5% and 6.3% patients respectively. The mean CBD angle was 138.9 ± 18.8º. CBD showed smooth wall contours (10%), extrinsic indentations (83.8%) and smooth strictures (6.3%). The median CBD stricture length was 26 mm (1.25- 45 mm). Pre-stenotic dilatation was observed in 66.3% with stricture length >16 mm (sensitivity 81.1 % specificity 78%) predisposing to it. Statistically significant associations were tabulated according to the classification of PB. Conclusion: This study provides the decade long experience of imaging findings in EHPVO induced portal biliopathy according to its classification and its clinical implications.

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.