scholarly journals Variceal Bleed and Portal Hypertensive Gastropathy in a Noncirrhotic Patient with Isolated Splenomegaly

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
S. M. Mahmudul Hasan ◽  
Meghan Dmitriew ◽  
Jennifer Leonard
Keyword(s):  
Portal Hypertension ◽  
Portal Hypertensive Gastropathy ◽  
Esophageal Variceal ◽  
Ct Guided ◽  
Noncirrhotic Patient ◽  
Variceal Bleed ◽  
Life Threatening ◽  
Intrahepatic Portosystemic Shunt ◽  
Common Etiology

Portal hypertension caused by cirrhosis is the most common etiology of esophageal varices. However, abnormalities of the splenoportal axis in the absence of liver disease may also cause portal hypertension resulting in varices. We report a rare case of esophageal variceal bleed in a noncirrhotic patient with isolated splenomegaly secondary to chronic granulocyte colony stimulating factor (G-CSF) therapy. The patient is a 26-year-old male with Cohen syndrome who required long-term G-CSF treatment for chronic neutropenia. He presented with large volume hematemesis and pancytopenia in the setting of known splenomegaly with no evidence of cirrhosis. An urgent EGD revealed active variceal bleeding and portal hypertensive gastropathy. The patient was appropriately resuscitated and underwent a successful transjugular intrahepatic portosystemic shunt and CT-guided coil placement for the bleeding varices. We are the first to report variceal bleed as a complication of long-term G-CSF use, a life-threatening consequence that requires urgent intervention.

scholarly journals Portal Hypertension in Childhood Bilateral Wilms' Tumor Survivor: An Excellent Indication for TIPS

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Ghassan Nakib ◽  
Valeria Calcaterra ◽  
Marco Brunero ◽  
Ilaria Goruppi ◽  
Pietro Quaretti ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Wilms Tumor ◽  
Beta Blockers ◽  
Rare Complication ◽  
Paediatric Population ◽  
Portal Venous System ◽  
Additional Indication ◽  
Life Threatening ◽  
Intrahepatic Portosystemic Shunt

Introduction. Increased pressure in portal venous system is relatively a rare complication after chemoradiotherapy for Wilms' tumor (WT). In paediatric population, feasibility and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in portal hypertension nonresponsive to medical or endoscopic treatment have been recently advocated. We report a case of TIPS positioning in a 15-year-old girl with portal hypertension as a long-term sequel of multimodality therapy in bilateral WT.Case Report. Two-year-old girl was diagnosed for bilateral WT. Right nephrectomy with left heminephrectomy and chemoradiotherapy were performed. At 7 years of age, the first gastrointestinal bleeding appeared, followed by another episode two years later, both were treated successfully with beta-blockers. At 15 years of age, severe unresponsive life-threatening gastroesophageal bleeding without hepatosplenomegaly was managed by TIPS. Reduction of the portosystemic pressure gradient was obtained.Conclusion. TIPS positioning for portal hypertension in long-term tumors' sequel is feasible and could be considered as an additional indication in paediatric patients.

scholarly journals A72 VARICEAL BLEED & PORTAL HYPERTENSIVE GASTROPATHY IN A NON-CIRRHOTIC PATIENT WITH ISOLATED SPLENOMEGALY

2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 85-86
Author(s):  
S Hasan ◽  
M Dmitriew ◽  
J Leonard
Keyword(s):  
Intensive Care ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Cirrhotic Patient ◽  
Rare Case ◽  
Treatment Plan ◽  
Venous Pressure ◽  
Retinal Dystrophy ◽  
Ct Guided ◽  
Variceal Bleed

Abstract Background Portal hypertension caused by cirrhosis is the most common etiology of esophageal varices. However, abnormalities of the spleno-portal axis in the absence of liver disease may also cause portal hypertension resulting in varices. We report a rare case of esophageal variceal bleed in a non-cirrhotic patient with isolated splenomegaly secondary to chronic G-CSF therapy. Aims This report outlines the case of a patient with Cohen Syndrome (CS) who presented with an upper gastrointestinal (GI) bleed in the setting of previously documented splenomegaly and portal hypertension. We expand on the clinical investigations, diagnosis, treatment plan and hospital course of this patient. Methods Case report, review of literature. Results A 26-year old male with previously diagnosed CS presented with large volume hematemesis and pancytopenia. CS is a rare autosomal recessive disorder. In our patient this manifested with congenital neutropenia, microcephaly, retinal dystrophy and global developmental delay. He required long term G-CSF therapy to manage chronic neutropenia and subsequently developed splenomegaly, a known side effect. The most recent MRI identified stable splenomegaly with a craniocaudal length of 23 cm, normal liver size and no radiographic evidence of cirrhosis. The imaging was also significant for gastroesophageal and splenorenal varices but no ascites or recanalization of the umbilical vein. A recent liver biopsy had shown mild pericellular fibrosis with no active liver disease or cirrhosis. In the past, the patient had declined EGD, therapeutic splenectomy or assessment of hepatic venous pressure gradient through invasive venography. His liver enzymes, bilirubin and albumin had always been within normal limits. The patient had no history of GI bleeding. Previous investigations for hematologic malignancies or myelodysplastic syndrome had been negative. Upon admission, an urgent EGD revealed active variceal bleeding in the esophagus and portal gastropathy. Given the extent of his congenital orofacial abnormalities a variceal band ligator could not be passed for appropriate intervention. The patient was transferred to the Intensive Care Unit and managed with intravenous proton pump inhibitor, octreotide, as well as transfusions of packed red blood cells, platelets and fresh frozen plasma. Within the next 48 hours, the patient underwent successful transjugular intrahepatic portosystemic shunt and CT-guided coil placements for the bleeding varices. Conclusions This is a rare case of variceal bleed in a non-cirrhotic patient with portal hypertension from iatrogenic splenomegaly. While there are previous reports of spontaneous splenic rupture secondary to G-CSF therapy we are the first to report variceal bleed as a complication. This is a life-threatening consequence that requires urgent intervention and intensive care. Funding Agencies None

Portal Hypertension and Variceal Hemorrhage

2015 ◽  
Author(s):  
Amir Qamar
Keyword(s):  
Portal Hypertension ◽  
Natural History ◽  
Esophageal Varices ◽  
Variceal Hemorrhage ◽  
Portal Hypertensive Gastropathy ◽  
Gastric Antral Vascular Ectasia ◽  
Esophageal Variceal ◽  
Gastroesophageal Varices ◽  
Vascular Ectasia ◽  
History Of

Gastrointestinal bleeding in patients with cirrhosis can occur from a number of different causes, including portal hypertension, gastric antral vascular ectasia, and acute variceal hemorrhage. The management of these conditions involves a combined medical and endoscopic approach, with radiologic and surgical therapies restricted to refractory cases. This review covers the natural history of gastroesophageal varices, portal hypertensive gastropathy, and gastric antral vascular ectasia; diagnostic principles; primary and secondary prophylaxis relating to esophageal variceal hemorrhage; and treatment overviews for gastric variceal hemorrhage, portal hypertensive gastropathy, and gastric antral vascular ectasia. Figures show the pathophysiology of complications of cirrhosis, esophageal varices as seen during an upper endoscopic procedure, natural history of esophageal varices in patients with cirrhosis, portal hypertensive gastropathy, gastric antral vascular ectasia, and management principles for acute variceal hemorrhage, esophageal variceal ligation, and gastric varices. Tables list the prevalence of various etiologies of hemorrhage in patients with cirrhosis, current recommendations for follow-up screening and surveillance of varices, sensitivities and specificities of some noninvasive markers, and principles of initial management of acute variceal hemorrhage. This review contains 8 highly rendered figures, 4 tables, and 44 references.

scholarly journals Potential Pitfalls in Transjugular Portosystemic Shunt Placement for Bleeding Rectal Varices

2015 ◽  
Vol 9 (2) ◽  
pp. 296-301 ◽  
Author(s):  
S M Nazmus Sakib ◽  
Katsuhiro Kobayashi ◽  
Mohammed Jawed
Keyword(s):  
Portal Hypertension ◽  
Endoscopic Therapy ◽  
Alcoholic Cirrhosis ◽  
Massive Bleeding ◽  
Portosystemic Shunt ◽  
Early Mortality ◽  
Rectal Varices ◽  
Life Threatening ◽  
Intrahepatic Portosystemic Shunt ◽  
Transjugular Portosystemic Shunt

In patients with portal hypertension, bleeding from rectal varices is rare. However, it can be life-threatening. We report a case of massive bleeding from large rectal varices in a 59-year-old man with alcoholic cirrhosis. Emergent transjugular intrahepatic portosystemic shunt (TIPS) placement was performed following failed local endoscopic therapy. Despite normalization of the portosystemic pressure gradient, the patient had another episode of massive bleeding on the following day. Embolization of the rectal varices via TIPS successfully stopped the bleeding. After the procedure, rapid decompensation of the cirrhosis led to severe encephalopathy, and death was observed. Although TIPSs have been reported to be useful in controlling bleeding from rectal varices, our case illustrates the potential pitfalls in using this technique in the treatment of rectal variceal bleeding. TIPSs may not be always successful in controlling massive bleeding from large rectal varices, even after normalization of portal hypertension. TIPSs can also be associated with life-threatening complications that may lead to early mortality.

scholarly journals Outcome of Band Ligation in Esophageal Varices of Bangladeshi Children: A Tertiary Centre Experience

2017 ◽  
Vol 41 (1) ◽  
pp. 28-33 ◽  
Author(s):  
Salahuddin Mahmud ◽  
Syed Shafi Ahmed ◽  
Jahida Gulshan ◽  
Farhana Tasneem ◽  
Madhabi Baidya
Keyword(s):  
Portal Hypertension ◽  
Esophageal Varices ◽  
Variceal Bleeding ◽  
Clinical Situation ◽  
Gastric Varices ◽  
Band Ligation ◽  
Tertiary Centre ◽  
Life Threatening ◽  
Common Etiology ◽  
One Year

Background: Variceal bleeding is often a life threatening clinical situation in infants and children. Band ligation is the main endoscopic treatment for esophageal varices.Objective: To see the outcome of band ligation of esophageal varices in extra-hepatic and hepatic cases of portal hypertension.Methods: This prospective study was done in the Department of Pediatric Gastroenterology, Hepatology & Nutrition, Dhaka Shishu (Children) Hospital, Dhaka, Bangladesh on 40 consecutive cases of esophageal varices enrolled from April, 2014 to March 2016. Every case was treated with band ligation followed by tab. propranolol. Cases were followed up for a minimum period of one year after the band ligation.Results: Age of the children was 2-12 years with mean age of 7.2±4.3 years and male:female ratio was 1.5:1. Out of 40 children, 32 (80%) were pre-hepatic and 8 (20%) hepatic (chronic liver disease with portal hypertension) causes. Only 1 session required in 50% pre-hepatic cases and multiple (2-3) sessions required in hepatic (100%) cases. Almost same number of band (average 2-3) required in every session of both cases. Grade-II esophageal varices with red sign were more common in prehepatic cases & severity of grading much more (grade-III & IV) in hepatic cases. Gastric varices were more common in hepatic (50%) cases than extra-hepatic (12.5%) cases. Recurrence of bleeding occurred in all hepatic (100%) cases and half (50%) of the pre-hepatic cases. Early re-bleeding was more common in hepatic (75%) cases & late re-bleeding in both pre-hepatic (43.7%) & hepatic (100%) cases. Minimal side effect like discomfort (10%) & Nausea (10%) were present after the procedure.Conclusion: Pre-hepatic was the most common etiology of portal hypertension in studied children. Fewer sessions were required in pre-hepatic cases than in hepatic cases. Severity of grading, re-bleeding & associated gastric varices were more common in hepatic cases. Band ligation was found to be the treatment of choice for the control of acute variceal bleeding and prevention of re-bleeding with less complications.Bangladesh J Child Health 2017; VOL 41 (1) :28-33

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