scholarly journals Phlebosclerotic Colitis in a Cirrhotic Patient with Portal Hypertension: The First Case in Korea

2009 ◽  
Vol 24 (6) ◽  
pp. 1195 ◽  
Author(s):  
Ha Yan Kang ◽  
Ran Noh ◽  
So Mi Kim ◽  
Hyun Deok Shin ◽  
Se Young Yun ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Cirrhotic Patient ◽  
First Case

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

scholarly journals Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia

2022 ◽  
Vol 18 (1) ◽  
Author(s):  
Mongkhon Sompornrattanaphan ◽  
Ranista Tongdee ◽  
Chamard Wongsa ◽  
Anupop Jitmuang ◽  
Torpong Thongngarm
Keyword(s):  
Portal Hypertension ◽  
Common Variable Immunodeficiency ◽  
Multiple Organ ◽  
Late Complications ◽  
Liver Mass ◽  
Cvid Patient ◽  
Hepatic Rupture ◽  
Fatal Liver ◽  
First Case ◽  
Common Variable

Abstract Background Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case presentation A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750–1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

scholarly journals Hemoperitoneum in a Cirrhotic Patient Due to Rupture of Retroperitoneal Varix

HPB Surgery ◽  
2009 ◽  
Vol 2009 ◽  
pp. 1-5 ◽  
Author(s):  
Igor Rafael Sincos ◽  
Grace Mulatti ◽  
Sheila Mulatti ◽  
Ilana Cristina Sincos ◽  
Sergio Q. Belczak ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Abdominal Pain ◽  
Portal Hypertension ◽  
Early Diagnosis ◽  
Hemorrhagic Shock ◽  
Effective Treatment ◽  
Cirrhotic Patient ◽  
Therapeutic Approach ◽  
Medical Literature ◽  
Management Algorithm

The rupture of retroperitoneal varices is a rare and catastrophic complication of portal hypertension. We describe a case of this nature, the first in Brazilian medical literature, and also reviewing all previous 34 cases. We systematically analyzed all therapeutic approach and propose a management algorithm for diagnosis and treatment of this lethal condition. The majority of the patients presented with abdominal pain, distention and hypotension, and developed hemorrhagic shock. Rupture of retroperitoneal varices can be properly managed if an early diagnosis is made and surgery is performed promptly, which is the only effective treatment. Arteriography should be used when the suspicion is of rupture of hepatocellular carcinoma.

Evaluation of portal hypertension in the cirrhotic patient: hepatic vein pressure gradient and beyond

2018 ◽  
Vol 53 (10-11) ◽  
pp. 1153-1164 ◽  
Author(s):  
D. S. Karagiannakis ◽  
T. Voulgaris ◽  
S. I. Siakavellas ◽  
G. V. Papatheodoridis ◽  
J. Vlachogiannakos
Keyword(s):  
Portal Hypertension ◽  
Pressure Gradient ◽  
Hepatic Vein ◽  
Cirrhotic Patient

The first case of Pasteurella canis bacteremia: a cirrhotic patient with an open leg wound

Infection ◽  
2010 ◽  
Vol 38 (6) ◽  
pp. 483-485 ◽  
Author(s):  
T. J. Albert ◽  
D. L. Stevens
Keyword(s):  
Cirrhotic Patient ◽  
First Case

scholarly journals Pancytopenia and Massive Gastroesophageal Bleeding Due to Hypersplenism Treated with Partial Splenic Embolization

2019 ◽  
Vol 1 (1) ◽  
pp. 42-46
Author(s):  
Akhmadu Muradi ◽  
Fadhli Waznan ◽  
Cynthia Jasirwan ◽  
Dedy Pratama ◽  
Raden Suhartono
Keyword(s):  
Portal Hypertension ◽  
Splenic Abscess ◽  
Antibiotic Administration ◽  
Partial Splenic Embolization ◽  
Adequate Treatment ◽  
Gastrointestinal Complications ◽  
First Case ◽  
Splenic Embolization ◽  
History Of ◽  
And Control

Introduction: Portal hypertension may cause gastrointestinal complications; one of the most serious is a ruptured esophageal varices. Portal hypertension is also the main cause of hypersplenism, which in turn could lead to pancytopenia. Despite adequate therapy, some cases of hypersplenism could not be resolved. Partial splenic embolization (PSE) is an effective alternative method to treat this condition. Method: We reported two cases of hypersplenism treated with PSE. The first case was a 10- year-old girl with pancytopenia and a history of recurrent esophageal ligation. The second case was a 32-year-old man with recurrent episodes of hematemesis for two years before admission. Results: After the PSE procedure, the first patient’s white blood cell and platelet doubled in one month after procedure and stable at follow-up three months later, with no complaint of hematemesis. The second patient’s platelet doubled five days after the procedure. The first patient developed a complication of a splenic abscess, but after antibiotic administration and pus drainage, the condition was resolved. Conclusion: PSE is an effective method to treat hypersplenism secondary to the hypertensive portal. Treatment goals successfully achieved include improvement in blood count and control of bleeding. There are risks following PSE, but with adequate treatment, it can be overcome. Keywords: hypersplenism, partial splenic embolization, portal hypertension, pancytopenia

Post liver biopsy hemobilia in a cirrhotic patient with portal hypertension and varices: the diagnostic role of ERCP

1986 ◽  
Vol 32 (3) ◽  
pp. 213-215 ◽  
Author(s):  
K. Rajender Reddy ◽  
Lennox J. Jeffers ◽  
Jeffrey B. Raskin ◽  
Alan S. Livingstone ◽  
Howard O. Manten ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Liver Biopsy ◽  
Cirrhotic Patient ◽  
Diagnostic Role

scholarly journals Misdiagnosed as pancreatic cancer seven years ago, a 29-year-old woman suffered from left-sided portal hypertension caused by peripancreatic lymph node tuberculosis: a case report and literature review

2020 ◽  
Author(s):  
Dajun Yu ◽  
Xiaolan Li ◽  
Jianping Gong ◽  
Jinzheng Li ◽  
Fei Xie ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Lymph Node ◽  
Portal Hypertension ◽  
Liver Function ◽  
Splenic Vein ◽  
Normal Liver ◽  
Wall Thickening ◽  
Normal Liver Function ◽  
First Case ◽  
Lymph Node Tuberculosis

Abstract Background: As the only curable portal hypertension, left-sided portal hypertension (LSPH) is a very rare clinical syndrome. With normal liver function, LSPH is mostly due to pancreatic disease and its complications that cause spleen vein compression, inflammatory wall thickening or lumen blockage, isolated splenic vein thrombosis, restricted splenic vein reflux, finally resulting in increased splenic vein pressure, opened collateral circulation, and bleeding from isolated gastric varices. With a quiet occurrence, LSPH often lacks specific symptoms, which finally leads to difficult diagnosis. Therefore, acuminous options of clinical examination are exceedingly crucial. Splenectomy is the prime treatment for cases complicated by variceal bleeding, but the effect of treatment depends mainly on the condition of the primary disease. Other than these, diseases resulting in LSPH often need to be distinguished from pancreatic cancer, so it is necessary for us to pay more attention to the diagnosis and treatment of LSPH. Case presentation: Here, we report a case of 29-year-old women who was admitted to the hospital for repeated hematemesis and black stool, with a differential diagnosis of pancreatic cancer seven years ago. Abdominal computed tomography (CT), CT angiography (CTA), portal phase three-dimensional vascular reconstruction, and gastroscopy indicated varicose gastric fundus veins, pancreatic mass, and enlarged peripancreatic lymph nodes. Erythrocyte, platelet, and leukocyte counts in decline, positive gamma interferon release assay, and normal liver function were given by laboratory examination. Abdominal exploration, splenectomy, varicose veins dissection, and lesions resection were performed by laparotomy. After surgery, the diagnosis of lymph node tuberculosis was confirmed by the technology of biopsy. Based on mention above, a diagnosis of LSPH caused by peripancreatic lymph node tuberculosis was confirmed. Postoperative evolution was steady, and the patient was in ideal clinical status at 3 months follow-up. Conclusions: We reported the first case of LSHP caused by peripancreatic lymph node tuberculosis. At the same time of resulting in left portal hypertension, the peripancreatic lymph node tuberculosis is often misdiagnosed as pancreatic cancer. Further studies were necessary to explore more favorable diagnosis method for pancreas mass and more advantageous therapy for LSPH, especially caused by mechanical compression.

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