Basic Study
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World J Gastrointest Surg. Jan 27, 2020; 12(1): 1-8
Published online Jan 27, 2020. doi: 10.4240/wjgs.v12.i1.1
Pathological abnormalities in splenic vasculature in non-cirrhotic portal hypertension: Its relevance in the management of portal hypertension
Shahana Gupta, Biju Pottakkat, Surendra Kumar Verma, Raja Kalayarasan, Sandip Chandrasekar A, Ajith Ananthakrishna Pillai
Shahana Gupta, Biju Pottakkat, Raja Kalayarasan, Sandip Chandrasekar A, Department of Surgical Gastroenterology, JIPMER, Pondicherry 605006, India
Surendra Kumar Verma, Department of Pathology, JIPMER, Pondicherry 605006, India
Ajith Ananthakrishna Pillai, Department of Cardiology, JIPMER, Pondicherry 605006, India
Author contributions: All authors have made substantial contributions to conception and design of study, acquisition, analysis and interpretation of data; They have contributed equally to drafting of the manuscript and made critical revisions related to important intellectual content of the manuscript; All authors have approved the final version of the manuscript.
Institutional review board statement: The study has been approved by Post graduate Research Committee (PGRMC) and Institute Ethics Committee (IEC) of Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondycherry, India, an Institute of National Importance under Government Of India.
Conflict-of-interest statement: None of the authors have any conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Biju Pottakkat, MD, Professor, Additional Professor and Head, Department of Surgical Gastroenterology, JIPMER, Super-speciality Block 4th Floor, Pondicherry 605006, India.
Received: August 5, 2019
Peer-review started: August 5, 2019
First decision: August 31, 2019
Revised: September 25, 2019
Accepted: November 7, 2019
Article in press: November 7, 2019
Published online: January 27, 2020

Portal hypertension (PH) is associated with changes in vascular structure and function of the portosplenomesenteric system (PSMS). This is referred to as portal hypertensive vasculopathy. Pathological abnormalities of PSMS has been described in the literature for cirrhotic patients. Raised portal pressure and hyperdynamic circulation are thought to be the underlying cause of this vasculopathy. In view of this, it is expected that pathological changes in splenic and portal vein similar to those reported in cirrhotic patients with PH may also be present in patients with non-cirrhotic PH (NCPH).


To investigate pathological abnormalities of splenic vein in patients with NCPH, and suggest its possible implications in the management of PH.


A prospective observational study was performed on 116 patients with NCPH [Extrahepatic portal vein obstruction (EHPVO): 53 and non-cirrhotic portal fibrosis (NCPF): 63] who underwent proximal splenorenal shunt (PSRS), interposition shunt or splenectomy with devascularization in JIPMER, Pondicherry, India, a tertiary level referral center, between 2011-2016. All patients were evaluated by Doppler study of PSMS, computed tomography porto-venogram and upper gastrointestinal endoscopy. An acoustic resonance forced impulse (ARFI) scan and abdomen ultrasound were done for all cases to exclude cirrhosis. Intraoperative and histopathological assessment of the harvested splenic vein was performed in all. The study group was divided into delayed and early presentation based on the median duration of symptoms (i.e. 108 mo).


The study group comprising of 116 patients [77 (66%) females and 39 (34%) males] with NCPH had a median age of 22 years. Median duration of symptoms was 108 mo. The most common presentation in both EHPVO and NCPF patients was upper gastrointestinal bleeding (hematemesis and melena). The ARFI scan revealed a median score of 1.2 (1.0-1.8) m/s for EHPVO and 1.5 (0.9-2.8) m/s for NCPF. PSRS was performed in 84 patients (two of whom underwent interposition PSRS using a 10 mm Dacron graft); splenoadrenal shunt in 9; interposition mesocaval shunt in 5; interposition 1st jejunal to caval shunt in 1 patient and devascularization with splenectomy in 17 patients. Median pre-splenectomy portal pressure was 25 (range: 15-51) mm Hg. In 77% cases, the splenic vein was abnormal upon intraoperative assessment. Under macroscopic examination, wall thickening was observed in 108 (93%), venous thrombosis in 32 (28%) and vein wall calcification in 27 (23%) cases. Upon examination under a surgical magnification loupe, 21 (18%) patients had intimal defects in the splenic vein. Histopathological examination of veins was abnormal in all cases. Medial hypertrophy was noted in nearly all patients (107/116), while intimal fibrosis was seen in 30%. Ninety one percent of patients with intimal fibrosis also had venous thrombosis. Vein wall calcification was found in 22%, all of whom had intimal fibrosis and venous thrombosis. The proportion of patients with pathological abnormalities in the splenic vein were significantly greater in the delayed presentation group as compared to the early presentation group.


Pathological changes in the splenic vein similar to those in cirrhotic patients with PH are noted in NCPH. We recommend that PH in NCPH be treated as systemic and pulmonary hypertension equivalent in the gastrointestinal tract, and that early aggressive therapy be initiated to reduce portal pressure and hemodynamic stress to avoid potential lethal effects.

Keywords: Portal hypertensive vasculopathy, Non-cirrhotic portal hypertension, Splenic vasculature, Hyperdynamic circulation, Shunt surgery

Core tip: Portal hypertensive vasculopathy is well-investigated in cirrhotics. Raised portal pressure and hyperdynamic circulation are thought to be the underlying cause. Pathological changes in the splenic vein are similar in cirrhotic and non-cirrhotic portal hypertension (NCPH). They are not primarily due to venous degenerative changes, and are similar to those observed in the pulmonary vasculature in pulmonary hypertension. Portal hypertension in NCPH should be viewed as a systemic and pulmonary hypertension equivalent in the gastrointestinal tract. We show that these pathological venous changes in NCPH are observed in a greater proportion of patients in the delayed presentation group (P < 0.003). Interventions to reduce portal pressure should therefore be initiated at diagnosis of NCPH. Damage to the vasculature starts early and can be prevented from progressing to venous thrombosis and its sequelae if early surgical intervention is initiated to reduce portal pressure.