Left-Sided Portal Hypertension: A Sinister Entity

摘要

Abstract IntroductionSinistral, or left-sided, portal hypertension (SPH) is a rare entity, with multiple potential causes. Gastrointestinal variceal bleeding and hypersplenism are its’ major clinical manifestations. The main aim of the present study is to summarize the clinical features of patients with SPH.Patients and methodsThis was a retrospective analysis of consecutive patients with present or previous diagnosis of SHP, observed in a Gastroenterology Department, in a period of 2 years. Patients with clinical, radiological or laboratory alterations suggestive of cirrhosis were excluded. Causes of SPH, clinical manifestations and outcomes were registered. Potential factors associated with gastrointestinal bleeding were analyzed.ResultsIn the study period a total of 22 patients (male – 17; mean age – 59.6?±?10.6 years) with SHP were included. Clinical manifestations were: asymptomatic/unspecific abdominal pain (n?=?14); gastrointestinal bleeding (n?=?8). Eleven (50%) patients had increased aminotransferases, GGT and/or alkaline phosphatase although liver function was normal in all of them. Causes of SPH were chronic pancreatitis (n?=?7), acute pancreatitis (n?=?7), pancreatic cancer (n?=?4), pancreatic surgery (n?=?3) and arteriovenous malformation (n?=?1). All patients had gastric and/or esophageal varices and seven had splenomegaly. Five (22.7%) had thrombocytopenia, associated with hypersplenism. Five patients (22.7%) were submitted to endoscopic treatment and eight were submitted to splenic artery embolization and/or splenectomy. There were no cases of variceal rebleeding and two patients died. Patients without liver enzymes elevation had a higher probability of gastrointestinal bleeding (87.5% vs. 28.6%; p =?0.024).ConclusionsAcute and chronic pancreatitis are the major causes of SHP. Gastrointestinal bleeding is the most important clinical manifestation and patients without liver enzyme elevation seem more prone to bleed. Specific treatment is seldom performed or needed. Resumo Introdu??oA hipertens?o portal esquerda ou sinistra (HTPS) é uma entidade rara, que pode resultar de diferentes etiologias. A hemorragia gastrointestinal de origem varicosa e o hiperesplenismo s?o as principais manifesta??es clínicas. O principal objetivo do presente estudo consiste em estabelecer os achados clínicos mais relevantes num grupo de doentes com HTPS.Doentes e métodosFoi efetuada uma análise retrospetiva de um grupo consecutivo de doentes com HTPS diagnosticados ou acompanhados no servi?o de Gastrenterologia durante o período de 2 anos. Os doentes com estigmas clínicos, radiológicos ou laboratoriais sugestivos de cirrose hepática foram excluídos. Foram registadas as etiologias, manifesta??es clínicas, tratamentos e evolu??o. Também foram analisados potenciais fatores associados com hemorragia digestiva como forma de apresenta??o.ResultadosNeste período foram incluídos 22 doentes (sexo masculino – 17; média etária – 59,6?±?10,6 anos). As manifesta??es clínicas foram: assintomático/dor abdominal inespecífica (n?=?14); hemorragia gastrointestinal (n?=?8). A fun??o hepática era normal em todos os doentes mas 11 (50%) apresentavam uma eleva??o da enzimologia hepática (aminotransferases, GGT e/ou fosfatase alcalina). As principais etiologias da HTPS foram a pancreatite crónica (n?=?7), a pancreatite aguda (n?=?7), os carcinomas pancreáticos (n?=?4), as cirurgias pancreáticas prévias (n?=?3) e uma malforma??o arterio-venosa (n?=?1). Foram identificadas varizes gástricas e/ou esofágicas em todos os doentes e 7 apresentavam esplenomegália. A trombocitopenia, associada ao hiperesplenismo, estava presente em 5 doentes (22,7%). Cinco doentes foram submetidos a tratamento endoscópico e oito foram sujeitos a emboliza??o da artéria esplénica e/ou esplenectomia. N?o se verificaram casos de recidiva hemorrágica e ocorreram duas mortes. Os doentes sem altera??es da enzimologia hepática foram os mais propensos a apresentar hemorragia gastrointestinal (87,5% vs. 28,6%; p?=?0,024).Conclus?esA pancreatite aguda e a pancreatite crónica s?o as principais causas da HTPS. A hemorragia gastrointestinal é a manifesta??o clínica mais relevante e os doentes sem altera??es da enzimologia hepática parecem apresentar um risco superior para desenvolver esta complica??o. O tratamento específico raramente é necessário/realizado. prs.rt("abs_end"); Keywords Hypertension Portal ; Esophageal and Gastric Varices ; Pancreatitis ; Pancreatic Neoplasms Palavras-chave Hipertens?o Portal ; Varizes Gastroesofágicas ; Pancreatite ; Neoplasias Pancreáticas 1. Introduction Sinistral portal hypertension (SPH) is also known as splenoportal, left-sided, segmental, regional, localized, compartmental or lineal portal hypertension. 1 and 2 Its’ pathophysiology was first outlined by Greenwald and Wasch in 1939. 3 It is a rare entity, accounting for less than 5% of all patients with portal hypertension, and results from splenic vein thrombosis o