Role of cholangioscopy as a rescue technique in the retrieval of proximally migrated biliary stents.

A 15-years-old boy with a medical history of an orthotopic liver transplant in 2021 due to methylmalonic aciduria, on a multi-stenting strategy for biliary anastomotic strictures, underwent a reassessment endoscopic retrograde cholangiopancreatography and the two previously placed biliary stents had migrated proximally, above the anastomosis. A digital single-operator cholangioscopy was performed (SpyGlass, Boston Scientific, Marlborough, Massachusetts) with direct visualization of the migrated stents, however, the accessories through the cholangiocope, such as the SpyBite forceps or the SpySnare (Boston Scientific), were not available in our department at that time. Nevertheless, the cholangioscopy allowed a successful guidewire advancement into the lumen of the stents and its subsequent removal to the duodenum using a Soehendra Stent Retriever (Cook Japan, Tokyo, Japan). Endoscopic removal of proximally migrated stents can be challenging and cholangioscopy has emerged as an additional tool in these cases. This case report represents a successful retrieval of two biliary stents guided by cholangioscopy, avoiding further invasive procedures or even surgery with significant morbidity and mortality.

Lysosomal Acid Lipase: Can it be a New Non-Invasive Serum Biomarker of Cryptogenic Liver Fibrosis and Cirrhosis?

INTRODUCTION AND AIM: The association between lysosomal acid lipase (LAL) activity and liver steatosis or fibrosis is poorly studied. The aim of our study was to determine the predictive power of LAL for cryptogenic liver steatosis and cryptogenic significant fibrosis/cirrhosis. MATERIAL AND METHODS: Cross-sectional observational study of 101 adult patients with unexplained elevated liver enzymes/hepatomegaly with or without dyslipidemia submitted to the determination of LAL activity and LIPA gene (E8SJM-C.894G^A) mutation. Seventy-one patients underwent liver biopsy or FibroScan®. Patients with an identifiable liver dysfunction cause and well-stablished NAFLD/NASH risk factors were excluded. Predictors for liver steatosis, significant fibrosis (> F2) or cirrhosis (F4) were evaluated. RESULTS: Liver steatosis and fibrosis were mainly assessed by liver biopsy (74.6%; n = 53). Steatosis was present in 62.0% (n = 44), significant fibrosis in 47.9% (n = 34) and cirrhosis in 39.4% (n = 28). The median LAL was 0.36 (0.21-0.46)nmol/spot/h (vs. 0.29 (0.20-0.47); p = 0.558) for liver steatosis, 0.22 (0.11-0.29) nmol/spot/h (vs. 0.40 (0.34-0.51); p <0.001) for significant fibrosis and 0.21 (0.11-0.27) nmol/spot/h (vs. 0.40 (0.32-0.52); p < 0.001) for cirrhosis. No LIPA gene mutations were found. LAL activity was the strongest predictor of significant fibrosis (AUROC: 0.833; p < 0.001) with a cut-off of 0.265 (sensitivity: 85.9%; specificity: 75.0%) and cirrhosis (AUROC: 0.859; p < 0.001) with a cut-off of 0.235 (sensitivity: 86.2%; specificity: 75.0%), being higher than FIB4, GUCI or APRI. However, LAL activity was not associated with liver steatosis (AUROC: 0.536; p =0.558). CONCLUSION: LAL activity can be considered a non-invasive new marker of cryptogenic liver fibrosis with higher accuracy than other known biomarkers. LAL activity < 0.265 nmol/spot/h was strongly associated with cryptogenic significant fibrosis and <0.235 nmol/spot/h with cryptogenic cirrhosis. LAL activity was not associated with cryptogenic liver steatosis.

Efficacy of Endoscopic Retrograde Cholangiopancreatography in the Treatment of Biliary Complications following Liver Transplant: 10 Years of a Single-Centre Experience.

BACKGROUND AND AIMS: Biliary tract complications following liver transplant remain an important source of morbidity and mortality. Endoscopic retrograde cholangiopancreatography (ERCP) has become a common therapeutic option before other invasive procedures. The aim of this study was to evaluate ERCP efficacy in managing this type of complications. METHODS: Retrospective study of all patients who underwent therapeutic ERCP due to post-liver transplant biliary complications between September 2005 and September 2015, at a deceased donor liver transplantation centre. RESULTS: Therapeutic ERCP was performed in 120 patients (64% men; mean age 46 ± 14 years). Biliary complications were anastomotic strictures (AS) in 70%, non-anastomotic strictures (NAS) in 14%, bile leaks (BL) in 5.8%, and bile duct stones (BDS) in 32%. The mean time between liver transplant and first ERCP was: 19 ± 30 months in AS, 17 ± 30 months in NAS, 61 ± 28 months in BDS, and 0.7 ± 0.6 months in BL (p < 0.001). The number of ERCP performed per patient was: 3.8 ± 2.4 in AS, 3.8 ± 2.1 in NAS, 1.9 ± 1 in BDS, and 1.9 ± 0.5 in BL (p = 0.003). The duration of the treatment was: 18 ± 19 months in AS, 21 ± 17 months in NAS, 10 ± 10 months in BDS, and 4 ± 3 months in BL (p = 0.064). Overall, biliary complications were successfully managed by ERCP in 46% of cases, either as an isolated procedure (43%) or rendez-vous ERCP (3%). Per complication, ERCP was effective in 39% of AS, in 12% of NAS, in 91% of BDS, and in 86% of BL. Globally, the mean follow-up of the successful cases was 43 ± 31 months. Percutaneous transhepatic cholangiography and/or surgery were performed in 48% of patients in whom ERCP was unsuccessful. The odds ratio for effective endoscopic treatment was 0.2 for NAS (0.057-0.815), 12.4 for BDS (1.535-100.9), and 6.9 for BL (0.798-58.95). No statistical significance was found for AS (p = 0.247). CONCLUSIONS: ERCP allowed the treatment of biliary complication in about half of patients, avoiding a more invasive procedure. Endoscopic treatment was more effective for BDS and BL.

INTRODUÇÃO: As complicações biliares após transplante hepático são uma fonte importante de morbilidade e mortalidade. A colangiopancreatografia retrógrada endoscópica (CPRE) é a primeira opção de tratamento em muitos casos, previamente a procedimentos mais invasivos. O objetivo deste trabalho foi avaliar a eficácia da CPRE no tratamento destas complicações. DOENTES E MÉTODOS: Estudo retrospetivo de todos os doentes submetidos a CPRE terapêutica devido a complicações biliares após transplante hepático, entre setembro de 2005 e setembro de 2015. RESULTADOS: Incluídos 120 doentes submetidos a CPRE terapêutica, sendo 64% do sexo masculino, com idade média de 46 ± 14 anos. Complicações biliares: estenose da anastomose (EA) em 70%, estenose não anastomótica (ENA) em 14%, coledocolitíase em 32% e fuga biliar (FB) em 5,8%. Tempo entre transplante e primeira CPRE (meses): 19 ± 30 nas EA, 17 ± 30 nas ENA, 61 ± 28 na coledocolitíase e 0,7 ± 0,6 na FB (p < 0,001). Número de CPRE por doente: 3,8 ± 2,4 nas EA, 3,8 ± 2,1 nas ENA, 1,9 ± 1 na coledocolitíase e 1,9 ± 0,5 na FB (p = 0,003). Duração do tratamento (meses): 18 ± 19 nas EA, 21 ± 17 nas ENA, 10 ± 10 na coledocolitíase e 4 ± 3 nas FB (p = 0,064). Globalmente, a CPRE terapêutica foi eficaz em 46% dos casos (como procedimento isolado em 43% e por rendez-vous em 3%). Eficácia por complicação: 39% nas EA, 12% nas ENA, 91% na coledocolitíase e 86% nas FB. O tempo médio de follow-up foi de 43 ± 31 meses. Em 48% dos doentes, foi realizada terapêutica por colangiografia percutânea e/ou cirurgia por ineficácia da CPRE. Odds ratio para um tratamento endoscópico eficaz: 0,2 para ENA (0,057­0,815), 12,4 para coledocolitíase (1,535­100,9) e 6.9 para FB (0,798­58,95). Não houve diferenças estatisticamente significativas para a presença de uma EA. CONCLUSÕES: A CPRE foi eficaz no tratamento de complicações biliares após transplante hepático em cerca de metade dos casos, evitando outros procedimentos invasivos. O tratamento endoscópico foi particularmente eficaz em casos de coledocolitíase e FB.

Clinical applicability of Tokyo guidelines 2018/2013 in diagnosis and severity evaluation of acute cholangitis and determination of a new severity model.

OBJECTIVE: To determine the diagnostic accuracy of Tokyo guidelines (TG) 2018/2013 (TG18/TG13) and predictors of poor prognosis in acute cholangitis. METHODS: Retrospective 1-year study of consecutive hospital admissions for acute cholangitis. Prognosis was defined in terms of 30 d in-hospital mortality. RESULTS: Of the 183 patients with acute cholangitis, diagnostic accuracy based on Charcot's triad, TG07 and TG18/TG13 was 67.8, 86.9 and 92.3% (p < .001), respectively. Regarding severity based on TG18/TG13, 30.6% of cases were severe. A poor prognosis was found in 10.9% of patients. After multivariate analysis, systolic blood pressure <90 mmHg (OR 11.010; p < .001), serum albumin <3 g/dL (OR 1.355; p = .006), active oncology disease (OR 3.818; p = .006) and malignant aetiology of obstructive jaundice (OR 2.224; p = .021) were independent predictors of poor prognosis. The discriminative ability of the model with these four variables was high (AUROC 0.842; p < .001), being superior to TG18/TG13 (AUROC 0.693; p = .005). CONCLUSIONS: TG18/TG13 showed high diagnostic accuracy in acute cholangitis. Compared with TG18/TG13, the simplified severity model ≥2 allows easy selection of patients who will benefit from admission to the intensive care unit and early biliary decompression.

The clinical extremes of autoimmune cholangitis.

Autoimmune cholangitis (AIC) was first described in 1987 as immunocholangitis in three women who presented with signs and symptoms of primary biliary cholangitis (PBC), but who were antimitochondrial (AMA) negative and antinuclear antibodies (ANA) positive, and responded to immunosuppressive therapy with azathioprine and prednisolone (1). AIC is a rare chronic cholestatic inflammatory disease characterized by the presence of high ANA or smooth muscle antibodies (SMA) but AMA seronegativity. Histologically, AIC exhibits bile duct injury (2). In terms of therapeutics, in addition to response to ursodeoxycholic acid, a prompt response to corticosteroids has also been reported in earlier stages, distinguishing it from PBC. Herein the authors describe two cases with mixed signs of PBC and autoimmune hepatitis (AIH). The diagnostic differentiation between these diseases (AIC, PBC and AIH) is essential because of the different therapeutic strategies. Our cases highlight the importance of clinician awareness of the autoimmune spectrum of liver diseases.

Groove Pancreatitis with Biliary and Duodenal Stricture: An Unusual Cause of Obstructive Jaundice.

INTRODUCTION: Groove pancreatitis is an uncommon cause of chronic pancreatitis that affects the groove anatomical area between the head of the pancreas, duodenum, and common bile duct. CLINICAL CASE: A 67-year-old man with frequent biliary colic and an alcohol consumption of 30-40 g/day was admitted to the hospital complaining of jaundice and pruritus. Laboratory analysis revealed cholestasis and the ultrasound scan showed intra-hepatic biliary ducts dilatation, middle third cystic dilatation of common bile duct, enlarged Wirsung and pancreatic atrophy. The magnetic resonance cholangiopancreatography showed imaging findings compatible with groove pancreatitis. An esophagogastroduodenoscopy later excluded duodenal neoplasia. He was submitted to a Roux-en-Y cholangiojejunostomy because of common bile duct stricture. Five months later a gastrojejunostomy was performed due to a duodenal stricture. The patient remains asymptomatic during follow-up. DISCUSSION: Groove pancreatitis is a benign cause of obstructive jaundice, whose main differential diagnosis is duodenal or pancreatic neoplasia. When this condition causes duodenal or biliary stricture, surgical treatment can be necessary.

INTRODUÇÃO: A pancreatite da goteira duodeno-pancreática é uma forma rara de pancreatite crónica, que afeta a área anatómica entre a cabeça do pâncreas, duodeno e ducto biliar comum. CASO CLÍNICO: Doente do sexo masculino, 67 anos, com antecedentes de cólicas biliares de repetição e consumo etílico de 30-40 g/dia, internado por icterícia e prurido. Analiticamente, apresentava colestase e, ecograficamente, dilatação moderada das vias biliares intra-hepáticas (VBIH), dilatação quística do 1/3 médio do colédoco, ectasia do Wirsung e atrofia pancreática. A colangiopancreatografia por ressonância demonstrou aspetos imagiológicos compatíveis com pancreatite paraduodenal. A endoscopia alta excluiu neoplasia duodenal. Foi submetido a colangiojejunostomia em Y Roux por estenose do colédoco e após 5 meses a gastrojejunostomia por estenose duodenal. O doente mantem seguimento, permanecendo assintomático. DISCUSSÃO: A pancreatite paraduodenal é uma forma benigna de icterícia obstrutiva, cujo principal diagnóstico diferencial é a neoplasia duodenal/pancreática. Quando esta condição causa estenose duodenal ou biliar, a terapêutica cirúrgica poderá ser necessária.

Portal Hypertensive Biliopathy: An Infrequent Cause of Biliary Obstruction.

INTRODUCTION: Biliary obstruction is usually caused by choledocholithiasis. However, in some circumstances, alternative or concurring unusual ethiologies such as portal hypertensive biliopathy (PHB) must be considered. CLINICAL CASE: We present the case of a 36-year-old female complaining of jaundice and pruritus. Liver function tests were compatible with biliary obstruction and the ultrasound scan of the abdomen showed dilatation of the intrahepatic biliary ducts, a dilated common bile duct (CBD) and biliary calculi. The computed tomography of the abdomen revealed a portal cavernoma encasing the CBD. DISCUSSION: Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.

INTRODUÇÃO: A causa mais comum de icterícia obstrutiva é a coledocolitíase. No entanto, no contexto clínico adequado, devem ser consideradas etiologias alternativas ou concomitantes, nomeadamente a biliopatia hipertensiva portal (BHP). CASO CLÍNICO: Apresentamos o caso de uma doente do sexo feminino com 36 anos de idade com icterícia e prurido. O estudo bioquímico era compatível com icterícia obstrutiva e o estudo ecográfico do abdómen revelou dilatação das vias biliares intra-hepáticas e do colédoco associados a litíase biliar. A tomografia computorizada abdominal realizada mostrou a existência um cavernoma da veia porta a envolver o colédoco. DISCUSSÃO: O cavernoma da porta, no contexto de obstrução portal venosa extra-hepática pode complicar-se com BHP. Quando sintomática manifesta-se por colestase crónica caso exista uma estenose dominante ou dor biliar e/ou colangite aguda quando predomina a litíase. O tratamento definitivo é individualizado, incluindo terapêutica endoscópica visando a litíase associada e uma derivação cirúrgica venosa porto-sistémica.

Left-Sided Portal Hypertension: A Sinister Entity.

INTRODUCTION: Sinistral, 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 METHODS: This 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. RESULTS: In 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). CONCLUSIONS: Acute 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.

INTRODUÇÃO: A hipertensão portal esquerda ou sinistra (HTPS) é uma entidade rara, que pode resultar de diferentes etiologias. A hemorragia gastrointestinal de origem varicosa e o hiperesplenismo são as principais manifestações clínicas. O principal objetivo do presente estudo consiste em estabelecer os achados clínicos mais relevantes num grupo de doentes com HTPS. DOENTES E MÉTODOS: Foi efetuada uma análise retrospetiva de um grupo consecutivo de doentes com HTPS diagnosticados ou acompanhados no serviço 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, manifestações clínicas, tratamentos e evolução. Também foram analisados potenciais fatores associados com hemorragia digestiva como forma de apresentação. RESULTADOS: Neste período foram incluídos 22 doentes (sexo masculino ­ 17; média etária ­ 59,6 ± 10,6 anos). As manifestações clínicas foram: assintomático/dor abdominal inespecífica (n = 14); hemorragia gastrointestinal (n = 8). A função hepática era normal em todos os doentes mas 11 (50%) apresentavam uma elevação 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 malformação 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 embolização da artéria esplénica e/ou esplenectomia. Não se verificaram casos de recidiva hemorrágica e ocorreram duas mortes. Os doentes sem alterações da enzimologia hepática foram os mais propensos a apresentar hemorragia gastrointestinal (87,5% vs. 28,6%; p = 0,024). CONCLUSÕES: A pancreatite aguda e a pancreatite crónica são as principais causas da HTPS. A hemorragia gastrointestinal é a manifestação clínica mais relevante e os doentes sem alterações da enzimologia hepática parecem apresentar um risco superior para desenvolver esta complicação. O tratamento específico raramente é necessário/realizado.

Risk factors for stricture development after caustic ingestion.

BACKGROUND/AIMS: Stricture of the upper digestive tract is the most feared sequelae of caustic ingestion. Determinant risk factors for its development are not entirely known. The aim of this study was to investigate the risk factors associated with the development of fibrotic strictures induced by caustic ingestion. METHODOLOGY: Clinical, laboratory, radiological and endoscopic data from 48 patients admitted to our department for caustic ingestion were reviewed. All cases were submitted to emergency endoscopy and caustic lesions were graded according to Zargar's classification. Twenty-three patients with severe endoscopic lesions (grade IIb/grade III) and/or acute severe complications (gastrointestinal bleeding, respiratory failure) were admitted to an intensive care unit. After hospital discharge, all patients were followed-up (mean follow-up time: 4.1 +/- 1.8 years). RESULTS: Twelve patients (25%) developed caustic strictures. Four patients (8%) were submitted to surgery for stricture resolution. Factors significantly associated with development of caustic stricture were hematemesis (p = 0.002), serum lactic dehydrogenase > 600 U/L (p = 0.008), grade III lesions (p = 0.0002) and involvement of the entire esophagus (p = 0.0003). CONCLUSIONS: Severe endoscopic lesions, involvement of the entire length of the esophagus, hematemesis and increased serum lactic dehydrogenase represent risk factors for the development of fibrotic strictures induced by caustic ingestion. Assessment of these parameters may contribute to prevent this complication.