scholarly journals Case Report of Gastrointestinal Bleeding in an Adult with Chronic Visceral Acid Sphingomyelinase Deficiency

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
David Cassiman ◽  
Louis Libbrecht ◽  
Wouter Meersseman ◽  
Alexander Wilmer
Keyword(s):  
Portal Hypertension ◽  
Gastrointestinal Bleeding ◽  
Variceal Bleeding ◽  
Significant Risk ◽  
Acid Sphingomyelinase ◽  
Intestinal Bleeding ◽  
Intestinal Loop ◽  
Gastroesophageal Varices ◽  
Sphingomyelinase Deficiency ◽  
Venous Collaterals

Introduction. Acid sphingomyelinase deficiency (ASMD, also known as Niemann-Pick Type A and Type B disease) is a rare, inherited metabolic disorder. Liver-related issues, including cirrhosis and variceal haemorrhage, are a leading cause of early mortality in individuals with chronic forms of ASMD. Due to the rarity of this lysosomal storage disorder, there can be a lack of awareness that adults with chronic ASMD disease are at significant risk of cirrhosis, portal hypertension, and variceal bleeding. This case highlights an unusual presentation of recurrent variceal bleeding in an adult with cirrhosis and portal hypertension due to chronic visceral ASMD. Case Presentation. A patient with severe splenomegaly was diagnosed with ASMD at age of 25. At age 64 they had multiple hospital admissions for hematochezia (originally diagnosed as ischemic colitis) accompanied by hypotension (blood pressure 91/45 mmHg), anemia (hemoglobin 8.5g/dL, ref 12-16; INR 1.4, ref ≤1.2), and mild renal insufficiency (creatinine 1.33mg/dL, ref 0.51-0.95). Colonoscopy did not reveal a source of bleeding. Computerized tomography scanning imaging showed diffuse venous collaterals and ascites. Arteriographies during subsequent episodes of bleeding were negative for active arterial intestinal bleeding. Recurrent gastrointestinal bleeding was found to originate from a varicose vein cluster connected to the right iliac vein and the superior mesenteric vein, located in the submucosa of a small intestinal loop. Multiple varices were secondary to portal hypertension in the context of cirrhosis. The patient died from recurrent variceal bleeding that exacerbated liver failure worsened by pneumonia and hypovolemic and septic shock. Conclusions. The variceal bleeding in this patient was atypical in that it originated from venous collaterals bleeding into the small intestine rather than the more typical gastroesophageal varices observed in ASMD. With long standing liver dysfunction and gradual development of portal hypertension, intestinal varices rather than occult intestinal bleeding due to ischemia should be considered in ASMD patients presenting with either hematochezia or hematemesis.

scholarly journals A174 RARE BUT DEADLY: AN APPROACH TO MANAGEMENT OF DUODENAL VARICEAL HEMORRHAGE

2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 39-41
Author(s):  
M Dahiya ◽  
M Tomaszewski ◽  
G Ou ◽  
A Ramji
Keyword(s):  
Interventional Radiology ◽  
Case Report ◽  
Portal Hypertension ◽  
Literature Review ◽  
Gastrointestinal Bleeding ◽  
Variceal Bleeding ◽  
Variceal Hemorrhage ◽  
Cyanoacrylate Glue ◽  
Duodenal Varices ◽  
Ectopic Varices

Abstract Background Common etiologies of upper gastrointestinal bleeding (UGIB) in cirrhotic patients with portal hypertension include gastroesophageal varices (GOV), portal hypertensive gastropathy (PHG) and gastric antral vascular ectasia (GAVE). Less commonly, patients with portal hypertension develop varices in ectopic sites, including the rectum, biliary tree and duodenum. Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Aims To describe the management of duodenal variceal hemorrhage. Methods A case report and literature review was performed. Results Case report: We present a case of recurrent UGIB in a 59-year-old male with decompensated cirrhosis due to non-alcoholic steatohepatitis (CP-C; Meld 14). Initial endoscopy was negative for GOV, peptic ulcer, PHG, and GAVE, but an erosion over a mucosal bulge in the third segment of the duodenum was identified, raising possibility of vascular malformation versus ectopic varix. There was active bleeding after water provocation, so clips were deployed for hemostasis. CT imaging showed mesenteric venous collaterals abutting the duodenum, again raising possibility of duodenal varix, which was ultimately confirmed by endoscopic ultrasound (EUS). Patient had recurrent overt bleeding despite beta-blockage for prophylaxis, endoscopic clipping on four separate occasions, attempted angioembolization by interventional radiology, and cyanoacrylate glue. Transjugular intrahepatic porto-systemic shunt (TIPS) was not possible due to portal vein occlusion, so he underwent EUS-guided cyanoacrylate glue a second time. Literature review: Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Duodenal variceal hemorrhage can lead to hemorrhagic shock, and is potentially life threatening, with quoted mortality rates of 40%. Unfortunately, duodenal varices can be difficult to identify. Diagnosis is often delayed due to a combination of lower awareness and endoscopic challenges given the unusual serosal and submucosal location. Evidence-based guidelines for the management of ectopic varices are limited. For this reason, our current management strategies rely heavily on local expertise. Splanchnic vasoconstrictor medication, endoscopic ligation, EUS guided gluing, interventional radiology guided embolization, TIPS, balloon retrograde transvenous obliteration and surgical shunts are potential therapeutic options to manage the acutely bleeding varix. Following a variceal bleed, liver transplantation should be considered in eligible patients with no other contraindications. Conclusions Duodenal varices are a rare, potentially fatal, and underrecognized cause of gastrointestinal bleeding in patients with portal hypertension. Definitive therapy currently relies upon local expertise in the absence of clear guideline-based therapy. Funding Agencies None

scholarly journals Efficacy of long-acting somatostatin analogs in recurrent variceal bleeding in a patient with pre-hepatic portal vein thrombosis

2015 ◽  
Vol 72 (3) ◽  
pp. 283-286
Author(s):  
Tamara Alempijevic ◽  
Ana Balovic ◽  
Aleksandra Pavlovic-Markovic ◽  
Dino Tarabar ◽  
Miodrag Krstic ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Surgical Treatment ◽  
High Risk ◽  
General Condition ◽  
Variceal Bleeding ◽  
Somatostatin Analogue ◽  
Variceal Hemorrhage ◽  
Gastroesophageal Varices ◽  
Vein Thrombosis ◽  
Long Acting

Introduction. Bleeding from esophageal varices is a serious medical problem because of the risk of recurrent bleeding and high mortality rate (17-54%). Gastroesophageal varices develop in 50% of cirrhotic patients with portal hypertension, but can also develop in other pre- or post-hepatic causes of portal hypertension. Case report. We reported a 48-year-old female patient with portal hypertension caused by mesenterial vein thrombosis due to congenital thrombophilia. The patient was hospitalized several times because of recurrent gastroesophageal bleeding. Thrombosis of portal, lienal and mesenteric veins was diagnosed using multislice computed tomography (MSCT) angiography. Sclerotherapy and/or variceal ligation could not be used due to variceal size and distribution. Beta blockers were ineffective. Balloon tamponade and octreotide were used in each massive bleeding episode. Carvedilol therapy was introduced but rebleeding occured. Surgical treatment was considered a high risk procedure due to massive thrombosis of mesenterial veins, patient's general condition and high risk of postoperative thrombotic events. Thus, long-acting somatostatin analogue - Sandostatin? LAR was initiated at a dose of 30 mg im/month. The patient responded to the therapy well and variceal bleeding did not occur for the following 3 months. After 3 months another episode of gastric variceal hemorrhage occurred and surgical treatment was reconsidered. Total gastrectomy was performed in order to prevent repeated bleeding from large gastric varices and the patient recovered successfully, and after 1 year is symptom-free. Conclusion. Long-lasting somatostatin analogue was used for the first time in treatment of gastroesophageal variceal hemorrhage in the patient with prehepatic portal hyperten-sion. It was effective as temporary therapeutic option allowing the improvement of the patients general condition and adequate planning of elective surgical procedure. Futher reports are needed in order to compare efficacy in treatment of patients with variceal bleeding, where poor outcome is expected.

scholarly journals Prognostic criteria of variceal bleeding in patients with portal hypertension

2019 ◽  
Vol 23 (4) ◽  
pp. 76-85
Author(s):  
S. B. Zhigalova ◽  
G. V. Manukiyan ◽  
A. G. Shertsinger ◽  
E. E. Fandeyev ◽  
T. S. Semenova ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Portal Hypertension ◽  
Variceal Bleeding ◽  
Meld Score ◽  
Gastroesophageal Varices ◽  
Bleeding Rate ◽  
Prognostic Criteria ◽  
Class B ◽  
Pugh Class ◽  
Grade 3

Aim. To determine prognostic criteria of variceal bleeding in patients with portal hypertension.Material and methods. There were 53 patients with portal hypertension and the threat of variceal bleeding from the esophagus and stomach without previous hemorrhage. Univariate and multivariate analysis was applied to determine prognostic criteria of the first bleeding. Patients with esophageal and gastroesophageal varices were analyzed separately. The following prognostic criteria were considered: dimensions of the veins, vasculopathy and gastropathy, liver function.Results. Incidence of hemorrhage was 72.7% in patients with esophageal varices grade 3, in case of vasculopathy — 80%. Child-Pugh class A was followed by incidence of hemorrhage near 12.5%, class B — 44%, C — 100%. MELD score < 9 was associated with bleeding rate 28.6%, 10—19 — 39.3%, over 20 scores — 100%. According to multivariate analysis consisting of Child—Pugh classification, in patients with < 11 scores 1-year incidence of bleeding was 13.3%, in case of MELD classification — 16.7%. The same values in patients with 11—16 scores were 77.8% and 31.3%, respectively. Analysis including both classifications (overall score over 16) was associated with incidence of hemorrhage up to 100%. Gastroesophageal varices grade 3 were associated with the risk of hemorrhage near 44.4% within 12 months, in case of vasculopathy — 60%, gastropathy — 37.5%. Incidence of hemorrhage in patients with Child-Pugh class A was 12.5%, class B — 44%, class C — 100%. In patients with MELD score < 9 bleeding rate was 28.6%, 10—19 scores — 39.3%, over 20 scores — 100%. According to multivariate analysis consisting of Child—Pugh classification, patients with gastroesophageal varices and less than 11 scores have the probability of hemorrhage near 15.4% within 12 months, in case of MELD score — 23%. The same values were 55.6% and 50%, respectively in case of 11—18 scores. In patients with more than 18 scores incidence of hemorrhage was 75% considering Child-Pugh classification and 100% for the MELD classification.Conclusion. The group of patients with threat of bleeding within 12 months was determined that makes possible a differentiated approach to patients who for need primary prevention of bleeding.

scholarly journals Management of acute variceal hemorrhage

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 966 ◽  
Author(s):  
Alberto Zanetto ◽  
Guadalupe Garcia-Tsao
Keyword(s):  
Clinical Practice ◽  
Gastrointestinal Bleeding ◽  
Medical Care ◽  
Variceal Bleeding ◽  
Multidisciplinary Approach ◽  
Causes Of Death ◽  
Variceal Hemorrhage ◽  
Esophageal Variceal ◽  
Gastroesophageal Varices ◽  
Esophageal Variceal Bleeding

Gastrointestinal bleeding is one of the major causes of death in patients with cirrhosis, and gastroesophageal varices represent the main source of hemorrhage. Even though in the last decades survival has been improved because of the widespread adoption of effective treatments and optimization of general medical care, mortality is still significantly high, and decompensated patients pose a complex challenge requiring a multidisciplinary approach that is crucial to improve survival. The aims of this commentary are to review the most recent advances in the management of esophageal variceal bleeding and to highlight useful information to aid hepatologists in clinical practice.

Endoscopic treatment of gastroesophageal variceal bleeding after oxaliplatin-based chemotherapy in patients with colorectal cancer

Endoscopy ◽  
2020 ◽  
Vol 52 (09) ◽  
pp. 727-735 ◽  
Author(s):  
Xiaoquan Huang ◽  
Feng Li ◽  
Lifen Wang ◽  
Mei Xiao ◽  
Liyuan Ni ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
Portal Hypertension ◽  
Hepatitis B ◽  
Variceal Bleeding ◽  
Endoscopic Treatment ◽  
Cox Regression ◽  
Endoscopic Management ◽  
Gastroesophageal Varices ◽  
Cox Regression Analysis ◽  
First Choice

Abstract Background Oxaliplatin, used as first-choice treatment for colorectal cancer (CRC), induces sinusoidal endothelial injury and portal hypertension. This study investigated the characteristics of oxaliplatin-induced portal hypertension and evaluated the efficacy of endoscopic management of gastroesophageal variceal bleeding. Methods We performed a retrospective, multicenter, case-control study between January 2010 and December 2018. Patients who received oxaliplatin-based chemotherapy after CRC surgery and presented with portal hypertension and gastroesophageal varices were compared with consecutive patients with hepatitis B-related cirrhotic portal hypertension receiving endoscopic treatment for variceal bleeding. Results 39 patients with oxaliplatin-induced portal hypertension were identified, 35 of whom had a history of variceal bleeding. The median period between start of oxaliplatin-based chemotherapy and the occurrence of varices was 50.4 months (n = 39). A total of 26 patients with oxaliplatin-related portal hypertension and 230 patients with hepatitis B-related portal hypertension underwent endoscopic treatment. Kaplan-Meier analysis revealed that the 1-year rebleeding rate was significantly higher in the oxaliplatin group than in the hepatitis B group (43.3 % vs. 19.0 %, P = 0.001). Multivariable Cox regression analysis showed that oxaliplatin-based chemotherapy was an independent factor for 3-year rebleeding (hazard ratio [HR] 2.46, 95 % confidence interval [CI] 1.24–4.87; P = 0.01) and 3-year overall mortality (HR 9.43, 95 %CI 2.32–38.31; P = 0.002). Conclusions Oxaliplatin-related portal hypertension was characterized by massive ascites, splenomegaly, gastric varices, concomitant arterioportal fistula, and relatively normal liver function. Endoscopic treatment to prevent variceal rebleeding in these patients was unsatisfactory compared with endoscopic treatment for hepatitis B-related portal hypertension.

scholarly journals Emergency management of upper gastrointestinal bleeding

2018 ◽  
Vol 5 (9) ◽  
pp. 3647
Author(s):  
Badr Abulhamail ◽  
Ali Alamer ◽  
Khalid Asiri ◽  
Abdullah Alasmari ◽  
Ahmed Al Hamad ◽  
...  
Keyword(s):  
Emergency Department ◽  
Peptic Ulcer ◽  
Gastrointestinal Bleeding ◽  
Variceal Bleeding ◽  
Upper Gastrointestinal Bleeding ◽  
Morbidity And Mortality ◽  
Intestinal Bleeding ◽  
Gi Bleeding ◽  
Common Causes ◽  
Upper Gastrointestinal

Upper gastro intestinal bleeding is one of the most common reasons of emergency department visits, totaling up to 400,000 annual admissions in the United States. Peptic ulcer disease and variceal bleeding are two of the most common causes of GI bleeding. Several studies have been done, and major advancements were made in its management leading to significant drop in morbidity and mortality. Our aim is to study the common causes of upper gastrointestinal bleeding that come to the emergency department and understand the latest guidelines to manage them. We conducted this review using a comprehensive search of PubMed, MEDLINE, and EMBASE from March 1981, through November 2017. The following search terms were used: upper gastro intestinal bleeding, management of upper GI bleeding, variceal bleeding, peptic ulcer bleeding, hemorrhage in the emergency department. Acute upper gastrointestinal bleeding is one of the most common cases encountered in the emergency department and leading to significant morbidity and mortality. Clearing airway and breathing and stabilizing the vitals of the patient by achieving hemodynamic stability and bleeding control is the primary goal in the emergency department. 

scholarly journals Foam sclerotherapy during shunt surgery for portal hypertension and varices

Open Medicine ◽  
2017 ◽  
Vol 12 (1) ◽  
pp. 384-390 ◽  
Author(s):  
Zhe Zhang ◽  
Xueming Chen ◽  
Chenyu Li ◽  
Hai Feng ◽  
Hongzhi Yu ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Variceal Bleeding ◽  
Gastric Varices ◽  
Ulcer Formation ◽  
Inferior Mesenteric Vein ◽  
Shunt Surgery ◽  
Foam Sclerotherapy ◽  
Clinical Safety ◽  
Gastroesophageal Varices ◽  
Mesocaval Shunt

AbstractObjectiveThis preliminary study investigated the clinical safety and efficacy of foam sclerotherapy during shunt surgery to treat portal hypertension and gastroesophageal varices.MethodsSeven patients with confirmed portal hypertension and a variceal bleeding history underwent mesocaval shunt with simultaneous polidocanol foam injection into the varices. Computed tomography and endoscopic reviews were conducted within two weeks following the procedures and around six months later.ResultsSix patients underwent side-to-side mesocaval shunt. One received a prosthetic mesocaval shunt. Polidocanol foam was injected into the gastric varices or the inferior mesenteric vein during the surgery. Surgical success and survival was achieved in all patients. Gastric ulcer formation and thrombocytopenia occurred in one patient respectively, which were ameliorated by conservative treatment. During 12 to 24 months’ follow-up, three patients had obvious decrease or eradication of gastroesophageal varices; four patients had obvious decrease of gastric varices but residual esophageal varices; and all patents had unobstructed shunts. Encephalopathy occurred in one patient two months postoperatively. No sclerosant-related complications were observed and no postoperative recurrent variceal bleeding occurred.ConclusionsFoam sclerotherapy during shunt surgery is safe and effective for portal hypertension and varices treatment.

UPPER GASTROINTESTINAL BLEEDING IN PATIENTS WITH CIRRHOSIS: BEYOND VARICEAL BLEEDING

2020 ◽  
Author(s):  
G Demetriou ◽  
A Veniamin ◽  
E Orfanudaki ◽  
V Valatas ◽  
M Koulendaki ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Variceal Bleeding ◽  
Upper Gastrointestinal Bleeding ◽  
Upper Gastrointestinal

PROGNOSTIC VALUE OF COMBINATION AIMS65 AND MELD SCORES IN CIRRHOTIC PATIENTS WITH ACUTE VARICEAL BLEEDING

2019 ◽  
pp. 35-40
Author(s):  
Thi Nhung Nguyen ◽  
Trung Nam Phan ◽  
Van Huy Tran
Keyword(s):  
Clinical Practice ◽  
Portal Hypertension ◽  
Key Words ◽  
Variceal Bleeding ◽  
Prognostic Value ◽  
High Rate ◽  
Central Hospital ◽  
Acute Variceal Bleeding ◽  
Cirrhotic Patients ◽  
Sensitivity Specificity

Bacground: Variceal bleeding is a severe complication of portal hypertension due to cirrhosis with high rate of motality, hence, predicting early rebleeding and mortality in cirrhotic patients with acute variceal bleeding is vital in clinical practice. Objectives: To evaluate the prognostic value of the combination of AIMS65 and MELD scores in predicting first 5 days in-hospital rebleeding and mortality in cirrhotic patients with acute variceal bleeding. Materials and Methods: 44 cirrhotic patients with acute variceal bleeding hospitalized at Hue Central Hospital. MELD and AIMS65 scores were calculated within the first 24 hours and monitoring rebleeding and mortality in the first 5 days in these patients. Results: AIMS65, MELD scores can predict first 5 days rebleeding and mortality with AUROC are 0.81, 0.69 and 0.92, 0.95, respectively. Combination of AIMS65 and MELD scores can predict first 5 days in hospital rebleeding with AUROC is 0.84, sensitivity 83.3%, specificity 81.6% (p<0.001) and mortality with AUROC is 0.96, sensitivity 100%, specificity 92.7% (p<0.001). Conclusions: The combination of AIMS65 and MELD scores increased the sensitivity, specificity and prognostic value in predicting first 5 days in-hospital rebleeding and mortality in cirrhotic patients with acute variceal bleeding in compare to each single scores. Key words: AiMS65 score, MELd, acute variceal bleeding

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