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Ozturk H, Sari S, Gurkan OE, Dalgic B. Prevalence of portal vein thrombosis in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension. Dig Liver Dis 2025:S1590-8658(25)00747-9. [PMID: 40350344 DOI: 10.1016/j.dld.2025.04.038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/17/2024] [Revised: 04/08/2025] [Accepted: 04/21/2025] [Indexed: 05/14/2025]
Abstract
INTRODUCTION Portal vein thrombosis (PVT) has been increasingly diagnosed in pediatric patients owing to the widespread use of non-invasive radiological techniques. Although the prevalence of PVT in adults with cirrhosis and intrahepatic non-cirrhotic portal hypertension ranges from 0.6 to 26 % and 13 to 46 %, respectively, no available data exist in the pediatric population. The prevalence of PVT in children with cirrhotic and intrahepatic non-cirrhotic portal hypertension was evaluated in this study. METHODS This retrospective study included children with cirrhosis and intrahepatic non-cirrhotic portal hypertension, which consisted of congenital hepatic fibrosis (CHF) and idiopathic noncirrhotic portal hypertension (INCPH). Patients with extrahepatic portal venous obstruction were excluded from the study. The presence of PVT was evaluated using abdominal Doppler ultrasonography and/or CT. Etiological, clinical, and laboratory findings were compared between the groups. RESULTS One hundred and forty-two patients with cirrhosis (mean admission age: 64.6 months ± 66.4, mean follow-up duration: 46.8 months ± 45.6) and 41 patients with non-cirrhotic patients (CHF=16, INCPH = 25, mean admission age: 126 months ± 64.2) were enrolled in this study. The prevalence of PVT was not significantly different between cirrhotic (8.5 %) and non-cirrhotic (9.7 %) patients. The incidence of PVT was significantly higher in patients with biliary atresia than in those with other etiologies in the cirrhotic group (p = 0.022). The frequency of PVT was higher in patients who had Child-Pugh score ≥7 in the cirrhotic group, but the difference was not statistically significant (p = 0066). The PVT group required more liver transplantations than the non-PVT group (p = 0.038). CONCLUSION The prevalence of PVT was similar in pediatric patients with cirrhosis and intrahepatic non-cirrhotic portal hypertension in our cohort, which is compatible with adult studies. Biliary atresia is found to be an important risk factor for PVT in our pediatric population. It might be associated with rapid progression of the disease, ascending cholangitis, and embryological abnormalities. These patients should be routinely evaluated to identify portal vein complications and early warning signs during follow-up.
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Affiliation(s)
- Hakan Ozturk
- Gazi University, Pediatric Gastroenterology, Hepatology, and Nutrition, Ankara, Turkey.
| | - Sinan Sari
- Gazi University, Pediatric Gastroenterology, Hepatology, and Nutrition, Ankara, Turkey
| | - Odul Egritas Gurkan
- Gazi University, Pediatric Gastroenterology, Hepatology, and Nutrition, Ankara, Turkey
| | - Buket Dalgic
- Gazi University, Pediatric Gastroenterology, Hepatology, and Nutrition, Ankara, Turkey
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Gulyaeva K, Nadinskaia M, Maslennikov R, Aleshina Y, Goptar I, Lukashev A, Poluektova E, Ivashkin V. Gut microbiota analysis in cirrhosis and non-cirrhotic portal hypertension suggests that portal hypertension can be main factor of cirrhosis-specific dysbiosis. Sci Rep 2025; 15:8394. [PMID: 40069378 PMCID: PMC11897210 DOI: 10.1038/s41598-025-92618-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2024] [Accepted: 03/03/2025] [Indexed: 03/15/2025] Open
Abstract
Gut dysbiosis plays an important role in cirrhosis, but the mechanism of its development was not established. The aim of the study was to test the hypothesis that portal hypertension can be the main factor in the development of gut dysbiosis in cirrhosis. This cross-sectional study included 25 patients with chronic non-cirrhotic portal hypertension due to extrahepatic portal vein obstruction after portal vein thrombosis (PVT) (NCPVT group), 29 cirrhotic patients without PVT (CirNoPVT), 15 cirrhotic patients with chronic PVT (CPVT), and 22 healthy controls. The fecal microbiota was assessed using 16S rRNA gene sequencing. The CirNoPVT and CPVT groups had largely similar differences in gut microbiota composition from the control group. Patients with NCPVT, as well as patients with cirrhosis, had a higher abundance of Streptococcus, Escherichia, Enterococcus, Enterobacteriaceae, Enterococcaceae, Streptococcaceae, Bacilli, Gammaproteobacteria, Proteobacteria, and a lower abundance of Roseburia, Faecalibacterium, Methanobrevibacter, Ruminococcaceae, Methanobacteriaceae, Clostridia, Methanobacteria, and Euryarchaeota as they were compared with healthy individuals. Patients with NCPVT had a higher abundance of Bifidobacterium, Bifidobacteriaceae, Actinobacteria, and a lower abundance of Gemmiger and Catenibacterium compared to healthy individuals, which was not observed in the cirrhosis groups. The abundance of Porphyromonadaceae with the genus Parabacteroides was reduced in both groups with PVT, but not in CirNoPVT. There were no significant differences in gut microbiota beta-diversity among the CirNoPVT, CPVT and NCPVT groups. All these groups had significant differences in beta-diversity from the control group. Portal hypertension seems be the main factor in the development of gut dysbiosis in cirrhosis.
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Affiliation(s)
- Kseniya Gulyaeva
- Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Pogodinskaya str., 1, bld. 1, Moscow, 119435, Russian Federation
| | - Maria Nadinskaia
- Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Pogodinskaya str., 1, bld. 1, Moscow, 119435, Russian Federation
| | - Roman Maslennikov
- Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Pogodinskaya str., 1, bld. 1, Moscow, 119435, Russian Federation.
| | - Yulia Aleshina
- Martsinovsky Institute of Medical Parasitology, Tropical and Vector Borne Diseases, Sechenov University, Moscow, Russian Federation, 19991
| | - Irina Goptar
- Martsinovsky Institute of Medical Parasitology, Tropical and Vector Borne Diseases, Sechenov University, Moscow, Russian Federation, 19991
| | - Alexander Lukashev
- Martsinovsky Institute of Medical Parasitology, Tropical and Vector Borne Diseases, Sechenov University, Moscow, Russian Federation, 19991
- Research Institute for Systems Biology and Medicine, Moscow, Russian Federation, 117246
| | - Elena Poluektova
- Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Pogodinskaya str., 1, bld. 1, Moscow, 119435, Russian Federation
- The Interregional Public Organization "Scientific Community for the Promotion of the Clinical Study of the Human Microbiome", Moscow, Russian Federation, 19991
| | - Vladimir Ivashkin
- Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Pogodinskaya str., 1, bld. 1, Moscow, 119435, Russian Federation
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Lucas IC, Lopes EP, Filgueira NA, Pereira CLD, Lucas TC, Domingues ALC. Prevalence of hepatic encephalopathy in non-cirrhotic portal hypertension: A systematic review and meta-analysis. Ann Hepatol 2025; 30:101902. [PMID: 40081810 DOI: 10.1016/j.aohep.2025.101902] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2024] [Revised: 12/28/2024] [Accepted: 01/20/2025] [Indexed: 03/16/2025]
Abstract
INTRODUCTION AND OBJECTIVES The prevalence of hepatic encephalopathy (HE) in non-cirrhotic portal hypertension (NCPH) is not well established, despite evidence of its occurrence in both minimal (MHE) and overt forms (OHE). Accurate diagnosis and management of HE can reduce morbidity and improve patients' and their families' quality of life. This study aimed to systematically review the prevalence of HE in NCPH. MATERIALS AND METHODS Systematic research in five databases (MEDLINE, LILACS, MEDRXIV, SCIELO and Scopus) was carried out from January to April 2024 to detect studies that address the prevalence of MHE and OHE in patients with NCPH, using the terms: "Hepatic Encephalopathy" or "Psychometrics" or "Cognition Disorders" or "Cognition" and "Noncirrhotic Portal Hypertension". RESULTS Twelve studies were included, including 575 patients. The prevalence of HE in patients with NCPH is 12 % (95 % CI: 6-24; I2 83 %, p<0.01), with high heterogeneity, with the pooled prevalence in studies evaluating MHE being 21 % (95 % CI: 11-38; I2 73 %, p<0.01) and the prevalence of OHE being 4 % (95 %CI: 1-15; I2 83 %, p<0.01). The prevalence of HE by etiology of NCPH is as follows: EHPVO is 25 % (95 %CI: 11-45; I2 0 %; p=1), PVT 2 % (95 %CI: 0-15; I2 0 %, p=1), in PSVD 8 % (95 %CI: 5-14; I2 87 %; p<0.01) and idiopathic NCPH 9 % (95 %CI: 5-14; I2 68 %, p<0.01), with the last two analyzes showing high heterogeneity. CONCLUSIONS HE occurs in approximately 12 % of patients with NCPH, with MHE being more common than OHE. Etiology plays a significant role in HE prevalence.
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Affiliation(s)
- Iris Campos Lucas
- Departamento de Medicina Tropical, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil.
| | - Edmundo Pessoa Lopes
- Departamento de Medicina Tropical, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil
| | - Norma Arteiro Filgueira
- Departamento de Medicina Tropical, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil
| | - Caroline Louise Diniz Pereira
- Departamento de Medicina Tropical, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil
| | - Thais Campos Lucas
- Departamento de Engenharia de Produção, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil
| | - Ana Lúcia Coutinho Domingues
- Departamento de Medicina Tropical, Universidade Federal de Pernambuco, Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife, Brazil
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Li P, Schiano TD, Thung SN, Ward SC, Fiel MI. Shared Features of Obliterative Portal Venopathy, Normal Liver, and Chronic Liver Disease: A Histologic and Morphometric Analysis. Mod Pathol 2025; 38:100739. [PMID: 39986468 DOI: 10.1016/j.modpat.2025.100739] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2024] [Revised: 01/27/2025] [Accepted: 02/06/2025] [Indexed: 02/24/2025]
Abstract
Obliterative portal venopathy (OPV) is a cause of noncirrhotic portal hypertension, and its diagnosis is challenging, as the features are heterogeneous, subtle, and may be mistaken as "normal." We sought to compare OPV cases (n = 72; 326 total portal tracts [PT]) with 2 control groups: control group 1 comprised of normal liver (n = 40; 192 PTs) and control group 2 comprised of liver biopsies with chronic liver disease with OPV features (n = 40; 200 PTs). Morphometry was applied to determine the overall PT area and the luminal area of dystrophic portal veins (PVs). The frequency of absent native PVs was determined. Using trichrome-stained slides, approximately 5 PTs were randomly selected for morphometry utilizing Philips IntelliSite Pathology Solution 3.3. Clinical data were extracted from electronic health records. Of the 326 PTs in the OPV cases, phlebosclerosis was found in 31.6%, densely fibrotic PTs in 12.7%, dystrophic PVs in 31.4%, and absent native PVs in 44.5%. When comparing the OPV group with control group 1, dystrophic PVs, absent native PVs, phlebosclerosis, fibrotic PTs, greater luminal area of dystrophic PV, and a higher ratio of dystrophic PV area to PT area were more frequently found in the OPV group. No significant difference in overall PT area was found. When comparing control group 2 with OPV cases, densely fibrotic PTs were more frequent when compared with OPV cases. This study shows that absent native PVs are the most frequent feature in OPV. Other features that are less frequent but still significantly different from normal liver include dystrophic PVs, greater luminal area of dystrophic PVs, phlebosclerosis, and PT fibrosis. Except for densely fibrotic PTs in control group 2, all other features showed similar frequency as OPV. Pathologists should be aware that OPV features may be present in liver biopsies from both normal and chronic liver diseases.
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Affiliation(s)
- Peizi Li
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Thomas D Schiano
- Division of Liver Diseases, Recanati/Miller Transplantation Institute, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Swan N Thung
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - Stephen C Ward
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York
| | - M Isabel Fiel
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, New York.
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Shimizu T, Yoshioka M, Matsushita A, Ueda J, Kawashima M, Ono T, Kawano Y, Yoshida H. Esophagogastric Varix Caused by Extrahepatic Portal Vein Obstruction with Essential Thrombocythemia: A Case Report. J NIPPON MED SCH 2024; 91:541-547. [PMID: 37558426 DOI: 10.1272/jnms.jnms.2024_91-601] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/11/2023]
Abstract
Extrahepatic portal vein obstruction (EHPVO) is a rare disease-causing form of portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is a reported risk factor for EHPVO due to underlying persistent thrombophilia. A Japanese woman in her 40s was referred to our hospital with a 1-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation showed thrombocytosis despite portal hypertension. She had a mutation in clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist. A bone marrow biopsy revealed megakaryocyte lineage proliferation, which confirmed a diagnosis of ET. Esophagogastroduodenoscopy revealed esophagogastric varices (LsF2CbRC2, Lg-cF1RC1), and abdominal computed tomography and angiography revealed splenomegaly and portal vein thrombosis with cavernous transformation, which suggested EHPVO. The patient had a history of ruptured esophagogastric varices and required prophylaxis against further variceal bleeding before antithrombotic therapy for EHPVO with ET. We performed laparoscopic Hassab's operation followed by endoscopic variceal ligation (EVL) and hematological cytoreduction therapy. Laparoscopic Hassab's operation and three bi-monthly EVL procedures improved the esophagogastric varix (LmF0RC0, Lg-f F0RC0) at 6 months after surgery. Cytoreduction therapy reduced platelet count to 60.1 × 104/uL, and the patient was very healthy at 7 months after surgery. Patients with EHPVO are traditionally referred to a gastroenterologist for abdominal pain, intestinal bleeding, or refractory ascites; however, hypercoagulative disease may be occult in such patients and require the attention of a hematologist. When treating patients with EHPVO, gastroenterologists should screen for hematological disease, including MPN.
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Affiliation(s)
- Tetsuya Shimizu
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Masato Yoshioka
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Akira Matsushita
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Junji Ueda
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Mampei Kawashima
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Takashi Ono
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Yoichi Kawano
- Department of Gastroenterological Surgery, Nippon Medical School
| | - Hiroshi Yoshida
- Department of Gastroenterological Surgery, Nippon Medical School
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Nie Q, Liang Q, Li M, Zhu R, Ren J, Jiang K, Li J. Idiopathic non-cirrhotic portal hypertension: A case report. Medicine (Baltimore) 2024; 103:e40642. [PMID: 39705493 PMCID: PMC11666171 DOI: 10.1097/md.0000000000040642] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2024] [Revised: 10/24/2024] [Accepted: 11/05/2024] [Indexed: 12/22/2024] Open
Abstract
RATIONALE Idiopathic noncirrhotic portal hypertension (INCPH) is a rare liver disorder with elevated portal pressure without cirrhosis, making diagnosis challenging. This case report presents a 46-year-old Chinese male with INCPH, highlighting the crucial role of liver biopsy. PATIENT CONCERNS A 46-year-old male presented with persistent fatigue that lasted for 2 months and significantly worsened over the last 3 days. The patient described his fatigue as a profound lack of energy that persisted throughout the day, which progressively impaired his ability to perform daily activities and maintain his usual work responsibilities. He reported feeling exhausted even after light physical exertion, such as walking or standing for short periods. The severity of the fatigue also led to frequent short rests during the day, and he experienced difficulty concentrating and carrying out routine tasks. In addition, he noted a loss of appetite and mild discomfort in the upper abdomen. Given his previous history of abnormal liver function tests and a liver biopsy showing mild chronic liver damage, the patient was initially diagnosed with cirrhosis at a local hospital. This initial diagnosis caused significant emotional distress, as the patient experienced a state of panic and anxiety over the implications of having a progressive liver disease. The psychological burden was evident in his reported difficulty sleeping and persistent worry about his health and future. DIAGNOSES Initial imaging suggested portal hypertension and cirrhosis, but a liver biopsy ruled out cirrhotic changes, confirming INCPH by excluding other causes such as chronic hepatitis. INTERVENTIONS The patient received symptomatic treatment (acid suppression, gastric and liver protection) and underwent a liver biopsy. Histological analysis confirmed INCPH, ruling out cirrhosis. OUTCOMES After the definitive diagnosis, the patient's anxiety lessened. Fatigue and weakness improved with ongoing symptomatic treatment, and psychological support enhanced his overall well-being. His follow-up plan includes regular liver function monitoring, imaging for portal pressure changes, and potential anticoagulation therapy for thrombosis risks. LESSONS This case highlights the diagnostic difficulty of INCPH and underscores the importance of liver biopsy. Further research is needed to develop specific diagnostic tools and treatments for INCPH.
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Affiliation(s)
- Qilong Nie
- The Eighth Clinical Medical College, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Qiuyan Liang
- The Eighth Clinical Medical College, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Mingyang Li
- The Eighth Clinical Medical College, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Ronghuo Zhu
- Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Jian Ren
- Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Kaiping Jiang
- Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
| | - Jianhong Li
- Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China
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Yorulmaz P, Hakalmaz AE, Kalyoncu Ucar A, Kendigelen P, Senyuz OF, Emre S. The effectiveness of endoscopic sclerotherapy in the treatment of esophageal varices in children with prehepatic portal hypertension. Pediatr Surg Int 2024; 41:36. [PMID: 39704839 DOI: 10.1007/s00383-024-05938-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 12/11/2024] [Indexed: 12/21/2024]
Abstract
AIM This study aims to evaluate the outcomes of endoscopic sclerotherapy (EST) in the treatment of esophagogastric varices in cases of extrahepatic portal hypertension (EHPH) secondary to portal vein thrombosis. MATERIALS AND METHODS Records of cases that underwent endoscopic sclerotherapy for esophagogastric varices between 1990 and 2022 in our clinic were retrospectively reviewed. The age, gender, symptomatology, etiology, clinical, laboratory, and radiological data of the patients, as well as treatment outcomes, were evaluated. Results were compared based on age groups, time periods, and etiological factors. The classification of the Japanese Portal Hypertension Research Society was used for variceal assessment. The absence of bleeding or bleeding not requiring transfusion within one year was considered a positive outcome. RESULTS Of the 126 cases that underwent endoscopy with a diagnosis of EHPH, 41 had varices at the F1-F2 level and were not subjected to sclerotherapy. In 21 cases, due to advanced varices, gastropathy findings, frequent bleeding episodes, and hypersplenism, surgical indication was established after the first endoscopy. The remaining 64 cases were included in the study. Positive outcomes were achieved in 44 cases with an average of 4.1 sessions. Desired outcomes were not achieved in 20 cases, and surgical procedures were performed. When the results were evaluated based on the periods 1990-2000, 2001-2010, and 2011-2022, the success rates were 60%, 44%, and 88.2%, respectively. Statistically significant more favorable results were obtained in the last decade. CONCLUSION EST is a safe and effective minimally invasive method for the elective treatment of esophagocardial varices in children with EHPH. More effective results have been achieved with increased experience and advancements in imaging technology.
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Affiliation(s)
- Poyraz Yorulmaz
- Cerrahpasa Faculty of Medicine, Department of Pediatric Surgery, Istanbul University-Cerrahpasa, Istanbul, Turkey
| | - Ali Ekber Hakalmaz
- Cerrahpasa Faculty of Medicine, Department of Pediatric Surgery, Istanbul University-Cerrahpasa, Istanbul, Turkey
| | - Ayse Kalyoncu Ucar
- Cerrahpasa Faculty of Medicine, Department of Radiology, Istanbul University-Cerrahpasa, Istanbul, Turkey
| | - Pinar Kendigelen
- Cerrahpasa Faculty of Medicine, Department of Anestesiology and Reanimation, Istanbul University-Cerrahpasa, Istanbul, Turkey
| | - Osman Faruk Senyuz
- Cerrahpasa Faculty of Medicine, Department of Pediatric Surgery, Istanbul University-Cerrahpasa, Istanbul, Turkey
| | - Senol Emre
- Cerrahpasa Faculty of Medicine, Department of Pediatric Surgery, Istanbul University-Cerrahpasa, Istanbul, Turkey.
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Gökçay B, Öztürk H, Sarı S, Eğritaş Gürkan Ö, Dalgıç B. Extrahepatic Portal Vein Obstruction in Children. EXP CLIN TRANSPLANT 2024; 22:117-123. [PMID: 39498932 DOI: 10.6002/ect.pedsymp2024.p6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2024]
Abstract
OBJECTIVES Extrahepatic portal vein obstruction is the most common cause of portal hypertension in children. This study aimed to evaluate the causes, clinical, laboratory and endoscopic findings, treatment approaches, long-term results, and prognosis of extrahepatic portal vein obstruction in children. MATERIALS AND METHODS We retrospectively evaluated 82 patients who were followed up with the diagnosis of extrahepatic portal vein obstruction at Gazi University Pediatric Gastroenterology clinic between January 1, 2011, and October 31, 2021. RESULTS Among our patients, 58.5% were male, median age at presentation was 36 months (range, 1 month to 6 years), and the follow-up period was 2 years (range, 1-14 years). The most common reasons for admission were gastrointestinal bleeding and splenomegaly. All patients diagnosed with incidentally detected obstruction were children under 5 years of age. The most frequently detected risk factors were history of umbilical vein catheterization (50%) and presence of procoagulant status (31%). Esophageal varices (94.8%), variceal bleeding (73.2%), hypersplenism (64.6%), and growth failure (13.4%) were the most common complications. Weight and/or height z-scores were lower in children with esophageal varices, variceal bleeding, and hypersplenism. Endoscopic treatments were more frequently applied to those with splenomegaly, thrombocytopenia, red color sign, and esophageal variceal bleeding. Rates of splenomegaly, hypersplenism, esophageal variceal bleeding, advanced varicose veins, and portal biliopathy were higher in patients who underwent portosystemic shunt surgery than in patients who did not undergo it. CONCLUSIONS Morbidity and mortality rates of variceal bleeding in extrahepatic portal vein obstruction were lower than bleeding seen in cirrhotic prehepatic portal hypertension. In our study, no patients died from extrahepatic portal vein obstruction-related complications. One patient died from tuberculous meningitis.
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Affiliation(s)
- Başak Gökçay
- From the Department of Pediatrics, Gazi University, Ankara, Turkey
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Zhang Y, Liu H, Xiong Q, Zhong Y, Liu D, Chen W, Yang Y. Application of liver biopsy in etiological diagnosis of unexplained portal hypertension: Porto-sinusoidal vascular disease should not be ignored. Medicine (Baltimore) 2024; 103:e39819. [PMID: 39331914 PMCID: PMC11441879 DOI: 10.1097/md.0000000000039819] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/29/2023] [Accepted: 09/02/2024] [Indexed: 09/29/2024] Open
Abstract
The diagnostic value of liver biopsy has been confirmed in patients with abnormal liver test results; however, little data are available on its application in patients with portal hypertension. This study aimed to investigate the utility of liver biopsy for the etiological diagnosis of unexplained portal hypertension, and explore the clinical and pathological characteristics of each etiology. A retrospective observational analysis was conducted on 1367 patients who underwent liver biopsy at the Second Hospital of Nanjing from 2017 to 2019. Of these, 188 patients with unexplained portal hypertension were enrolled. The clinical and pathological characteristics were collected and reassessed in a multidisciplinary team meeting. Among these patients, 174 (92.6%, 174/188) had a definite etiological diagnosis through liver biopsy. The main etiologies were autoimmune hepatitis in 47 patients (25%, 47/188), autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in 41 patients (21.8%, 41/188), and porto-sinusoidal vascular disease (PSVD) in 40 patients (21.3%, 40/188). Compared to liver cirrhosis, PSVD patients were younger and the liver function damage of which was subtler. The widths of portal vein diameter were widest in PSVD but the liver stiffness measurement were almost normal. Splenomegaly was common in PSVD, but ascites were less frequent than in autoimmune hepatitis (25.0% vs 51.1%, P = .013). Based on the histological patterns, we found that cholestatic liver diseases such as primary biliary cirrhosis, autoimmune hepatitis-primary biliary cirrhosis overlap syndrome, and progressive familial intrahepatic cholestasis could lead to non-cirrhotic portal hypertension, while vascular liver diseases such as PSVD and Budd-Chiari syndrome could also show fibrous proliferation as the disease progresses. Liver biopsy is safe and valuable for etiological diagnosis of unexplained portal hypertension. Cirrhosis is the leading cause of portal hypertension, and porto-sinusoidal vascular diseases should also be considered. Clinical features may be helpful in suggesting the cause; however, pathological examination is still indispensable for disease diagnosis and progression assessment.
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Affiliation(s)
- Yu Zhang
- Department of Medicine, Southeast University, Nanjing, China
- Department of Infectious and Liver Diseases, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
| | - Hongli Liu
- Department of Medicine, Southeast University, Nanjing, China
| | - Qingfang Xiong
- Department of Infectious and Liver Diseases, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
| | - Yandan Zhong
- Department of Infectious and Liver Diseases, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
| | - Duxian Liu
- Department of Pathology, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
| | - Wei Chen
- Clinical Research Center, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
| | - Yongfeng Yang
- Department of Infectious and Liver Diseases, The Second Hospital of Nanjing, Nanjing University of Chinese Medicine, Nanjing, China
- The Clinical Infectious Disease Center of Nanjing, Nanjing, China
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10
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Kaur P, Khanna R, Sood V, Lal BB, Mukund A, Kilambi R, Alam S. Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr 2024; 79:213-221. [PMID: 38847238 DOI: 10.1002/jpn3.12282] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2023] [Revised: 05/17/2024] [Accepted: 05/29/2024] [Indexed: 08/03/2024]
Abstract
BACKGROUND Meso-Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%. OBJECTIVES We aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency. METHODS Transjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A-E), and factors associated with patency of Rex vein were studied. RESULTS The patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso-Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under-5-year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65-90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity. CONCLUSION Less than one-third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.
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Affiliation(s)
- Prabhsaran Kaur
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Rajeev Khanna
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Vikrant Sood
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Bikrant Bihari Lal
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Amar Mukund
- Department of Interventional Radiology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Ragini Kilambi
- Department of Hepatopancreatobiliary Surgery, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Seema Alam
- Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
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11
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Gul MI, Umer W, Nawaz AD, Elhissi MJH, Zahid M. Management of extensive portal vein thrombosis via thrombolysis and thrombectomy without underlying liver disease: A case report. Clin Case Rep 2024; 12:e8920. [PMID: 38952464 PMCID: PMC11215528 DOI: 10.1002/ccr3.8920] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2024] [Revised: 04/17/2024] [Accepted: 04/24/2024] [Indexed: 07/03/2024] Open
Abstract
Portal vein thrombosis (PVT) is a rare condition, particularly in non-cirrhotic patients. Anticoagulation remains the mainstay of the treatment. Extensive PVT can lead to variceal bleeding, ascites, bowel ischemia, and hypersplenism. The role of thrombolysis and thrombectomy in these patients remains unclear. However, there is evidence that local thrombolysis and thrombectomy should be considered in those who remain symptomatic on anticoagulation and are at risk of complications with acute PVT.
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Affiliation(s)
| | - Waseem Umer
- Internal Medicine DepartmentHamad Medical CorporationDohaQatar
| | | | | | - Muhammad Zahid
- Internal Medicine DepartmentHamad Medical CorporationDohaQatar
- Qatar UniversityDohaQatar
- Weill Cornell MedicineDohaQatar
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12
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Giri S, Anand AC. Non-cirrhotic intrahepatic portal hypertension: Is the gun loaded? Indian J Gastroenterol 2024; 43:285-287. [PMID: 37940774 DOI: 10.1007/s12664-023-01466-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/10/2023]
Affiliation(s)
- Suprabhat Giri
- Department of Gastroenterology and Hepatology, Kalinga Institute of Medical Sciences, Patia, Bhubaneswar, 751 024, India
| | - Anil Chandra Anand
- Department of Gastroenterology and Hepatology, Kalinga Institute of Medical Sciences, Patia, Bhubaneswar, 751 024, India.
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13
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Shimizu T, Yoshida H, Taniai N, Yoshioka M, Kawano Y, Matsushita A, Ueda J, Iwai T, Murokawa T, Ono T, Hamaguchi A. Clinical features of extrahepatic portal vein obstruction: Myeloproliferative neoplasms eliminate hypersplenic hematologic changes in extrahepatic portal vein obstruction. Intractable Rare Dis Res 2024; 13:63-68. [PMID: 38404733 PMCID: PMC10883843 DOI: 10.5582/irdr.2023.01106] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2023] [Revised: 01/10/2024] [Accepted: 01/12/2024] [Indexed: 02/27/2024] Open
Abstract
Extrahepatic portal vein obstruction (EHPVO) is a rare disease. Most EHPVO patients are usually referred to a gastroenterologist for intestinal bleeding and hypersplenic thrombocytopenia; however, hypercoagulative diseases may be occult in these patients and require anticoagulation. The purpose of this study was to elucidate the clinical characteristics of EHPVO. We conducted a retrospective analysis of the hospital database, evaluating the medical records of 15 patients (7 males, 8 females, mean age of onset 42.0 years, range 5-74 years). Thirteen of 15 EHPVO patients (86.7%) had intestinal varices. These included 10 esophageal (66.7%), 12 gastric (80.0%), and 6 ectopic varices (40.0%). Nine (60.0%) of 15 had a history of intestinal bleeding. Regarding comorbidities, 5 of 15 (33.3%) suffered from vascular diseases, including acute myocardial infarction, cerebral infarction, pulmonary embolism, Budd-Chiari syndrome, and mesenteric vein thrombosis. The former 3 vascular commodities manifested at less than 32 years of age. Four patients (26.7%) with JAK2V617F mutation were diagnosed as myeloproliferative neoplasm (MPN). 72.3% of EHPVO patients without MPN experienced thrombocytopenic state. No EHPVO patients with MPN experienced thrombo-leukocytopenia. The elevation of white blood cell and platelet counts, and decrease of protein S were seen in EHPVO with MPN, compared with EHPVO without MPN. EHPVO is frequently associated with underlying hypercoagulative factors, causing a dilemma between thrombotic complications and portal hypertensive bleeding. Most EHPVO patients experience an evident thrombocytopenic state due to severe hypersplenism; however, hypersplenic hematologic changes are eliminated in EHPVO with MPN. MPN should be suspected in EHPVO patients negative for thrombo-leukocytopenia.
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Affiliation(s)
- Tetsuya Shimizu
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Hiroshi Yoshida
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Nobuhiko Taniai
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Masato Yoshioka
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Yoichi Kawano
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Akira Matsushita
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Junji Ueda
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takuma Iwai
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takahiro Murokawa
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Takashi Ono
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
| | - Akira Hamaguchi
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo, Japan
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14
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Philips CA, Sarin SK. Noncirrhotic portal hypertension-Historical perspectives bring clarity to the entity and its management. Clin Liver Dis (Hoboken) 2024; 23:e0232. [PMID: 38881719 PMCID: PMC11177835 DOI: 10.1097/cld.0000000000000232] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2024] [Accepted: 03/29/2024] [Indexed: 06/18/2024] Open
Affiliation(s)
- Cyriac Abby Philips
- Department of Clinical and Translational Hepatology, The Liver Institute, Center of Excellence in GI Sciences, Rajagiri Hospital, Aluva, Kerala, India
| | - Shiv K. Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, India
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Alkhasov A, Komina E, Ratnikov S, Saveleva M, Romanova E, Gusev A, Lochmatov M, Yatsyk S, Dyakonova E. Surgical Treatment of Portal Hypertension in Children. J Laparoendosc Adv Surg Tech A 2023; 33:1231-1235. [PMID: 37844079 DOI: 10.1089/lap.2022.0404] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2023] Open
Abstract
Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.
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Affiliation(s)
- Abdumanap Alkhasov
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Elena Komina
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Sergey Ratnikov
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Maria Saveleva
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Ekaterina Romanova
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Aleksey Gusev
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
- Peoples Friendship University of Russia (RUDN University), Moscow, Russia
| | - Maksim Lochmatov
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Sergey Yatsyk
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
| | - Elena Dyakonova
- "National Medical Research Center for Children's Health" Federal State Autonomous Institution of the Russian Federation Ministry of Health, Moscow, Russia
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16
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Ghibes P, Grözinger G, Hartleif S, Sturm E, Hefferman GM, Nadalin S, Tsiflikas I, Schäfer JF, Artzner C. Balloon angioplasty versus stent placement for the treatment of portal vein stenosis in children: a single center experience. Pediatr Radiol 2023; 53:1885-1893. [PMID: 37147428 DOI: 10.1007/s00247-023-05674-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Revised: 04/10/2023] [Accepted: 04/11/2023] [Indexed: 05/07/2023]
Abstract
PURPOSE To evaluate the safety and efficacy of percutaneous interventional treatment of portal vein stenosis in children. MATERIAL AND METHODS A retrospective analysis of all interventional treatments for portal vein stenosis in pediatric patients at a single institution from 2010 to 2021 was conducted. Platelet count, spleen size and portal vein flow velocity were assessed during the follow-up period. Primary and primary assisted patency time were determined. RESULTS A total of ten children (median age 28.5 months, interquartile range (IQR): 2.75-52.5 months) with portal vein stenosis after Mesorex-Shunt (n = 4), liver transplantation (n = 3) and other etiologies (n = 3) underwent 15 interventional procedures. There were five reinterventions and one discontinued intervention. The technical success rate was 93.3% (14/15) and clinical success of treated patients was 100% (14/14). Median follow-up was 18 months (IQR: 13.5-81 months). The median primary patency time for stent placement was 70 months (IQR: 13.5-127.25 months). For balloon angioplasty, the median primary patency time was 9 months (IQR 7.25-11.5 months), while the median assisted primary patency time was 14 months (IQR: 12 to 15 months). Platelet count, spleen size and portal vein flow velocity reliably corresponded to recurrence of portal vein stenosis in asymptomatic patients during follow-up. CONCLUSION Interventional treatment is a safe and efficient method to treat portal vein stenosis with long patency times, regardless of etiology. Primary stent placement shows a higher primary patency time than balloon angioplasty. Implementation of stent placement as the primary interventional method may improve patency times and reduce the need for repeat reinterventions in pediatric patients.
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Affiliation(s)
- Patrick Ghibes
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Staße 3, 72076, Tuebingen, Germany.
| | - Gerd Grözinger
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Staße 3, 72076, Tuebingen, Germany
| | - Steffen Hartleif
- Department of Pediatric Gastroenterology and Hepatology, University Children's Hospital Tuebingen, Tuebingen, Germany
| | - Ekkehard Sturm
- Department of Pediatric Gastroenterology and Hepatology, University Children's Hospital Tuebingen, Tuebingen, Germany
| | - Gerald M Hefferman
- Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Silvio Nadalin
- Department of General, Visceral, and Transplant Surgery, University Hospital Tuebingen, Tuebingen, Germany
| | - Ilias Tsiflikas
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Staße 3, 72076, Tuebingen, Germany
| | - Jürgen F Schäfer
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Staße 3, 72076, Tuebingen, Germany
| | - Christoph Artzner
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Staße 3, 72076, Tuebingen, Germany
- Department of Radiology, Diakonie-Klinikum Stuttgart, Stuttgart, Germany
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17
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Ghibes P, Artzner C, Partovi S, Hagen F, Nadalin S, Grözinger G. Endovascular treatment of symptomatic hepatic venous outflow obstruction post major liver resection. BMC Gastroenterol 2023; 23:241. [PMID: 37460992 DOI: 10.1186/s12876-023-02876-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/02/2023] [Accepted: 07/08/2023] [Indexed: 07/20/2023] Open
Abstract
PURPOSE To evaluate efficacy, safety, and outcomes of endovascular treatment of hepatic vein stenosis post major liver resection. METHODS A retrospective data analysis was performed including all interventional treatments of hepatic vein stenosis post major liver resection since 2010. Post procedural course and clinical parameters including amount of ascites accumulation and relevant laboratory values were assessed during the follow-up period. Primary and primary assisted hepatic venous patency time were calculated. RESULTS Twelve patients (median age 55.5, IQR 49.75 to 61.5 years) undergoing a total of 16 interventions were included. Interventions were primary stent placement (n = 3), primary balloon angioplasty (n = 8), three re-interventions and two aborted interventions (no significant pressure gradient). Technical success was 100% (16/16). Permanent reduction and / or complete resolution of ascites was achieved in 72% (8/11). Laboratory parameters related to liver function did not show significant improvement after intervention. Median follow-up period was 6 months (IQR: 1.5 to 18 months). The median primary patency time for patients with balloon angioplasty was 11 months (IQR: 1.375 to 22.25 months) and assisted patency time was 13.25 months (IQR: 4.5 to 22.25 months). The median primary patency time for patients with angioplasty and stent placement was 1 months (IQR: 1.0 to 1.5 months) and assisted patency time was 2.0 months (IQR: 1.5 to 2.5months). CONCLUSION An endovascular approach for the treatment of hepatic venous stenosis post major liver resection is safe and efficient to reduce and / or resolve refractory ascites. However, liver function parameters seem not to be improved by the procedure. Stent placement can be a reasonable option in patients with significant residual stenotic disease post angioplasty.
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Affiliation(s)
- Patrick Ghibes
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, Tuebingen, 72076, Germany.
| | - Christoph Artzner
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, Tuebingen, 72076, Germany
- Department for Radiology, Diakonieklinikum Stuttgart, Stuttgart, Germany
| | - Sasan Partovi
- Interventional Radiology Section, Imaging Institute, Cleveland Clinic Main Campus, Cleveland, OH, USA
| | - Florian Hagen
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, Tuebingen, 72076, Germany
| | - Silvio Nadalin
- Department of General, Visceral, and Transplant Surgery, University Hospital Tübingen, Tübingen, Germany
| | - Gerd Grözinger
- Department for Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, Tuebingen, 72076, Germany
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18
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Madhusudhan KS. Focal Nodular Hyperplasia-like Lesions in Patients with Extrahepatic Portal Vein Obstruction. Radiographics 2023; 43:e220164. [PMID: 36490209 DOI: 10.1148/rg.220164] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Affiliation(s)
- Kumble Seetharama Madhusudhan
- Department of Radiodiagnosis and Interventional Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
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19
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Kameyama M, Sato T, Nakano S, Maeda Y, Yamada Y, Ishii T, Hasegawa T. A girl with type 1A diabetes mellitus and extrahepatic portal vein obstruction. Pediatr Int 2023; 65:e15693. [PMID: 37968903 DOI: 10.1111/ped.15693] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2023] [Revised: 08/26/2023] [Accepted: 09/20/2023] [Indexed: 11/17/2023]
Affiliation(s)
- Madoka Kameyama
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Takeshi Sato
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Satsuki Nakano
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Yutaro Maeda
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
- Department of Surgery, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan
| | - Yohei Yamada
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Tomohiro Ishii
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Tomonobu Hasegawa
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
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20
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Muacevic A, Adler JR, Omar ZTO, Mungu EB, Aiyadurai S, Una IS, Sunday GC, Ajekigbe O, Chaudhry HA, Khan A. A Rare Case of Extrahepatic Portal Venous Obstruction in a Nine-Year-Old Female and Its Management: A Case Report. Cureus 2022; 14:e32150. [PMID: 36601204 PMCID: PMC9806945 DOI: 10.7759/cureus.32150] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/03/2022] [Indexed: 12/07/2022] Open
Abstract
Extrahepatic portal vein obstruction (EHPVO) is a kind of liver vascular disease that causes structural abnormalities in the portal veins, including cavernomatous metamorphosis and obstruction. It is the most common cause of esophageal varices-related hematemesis in youngsters. Significant risk factors include congenital abnormalities, dehydration, sepsis, trauma, hypercoagulable conditions, and multiple transfusions. Acute extrahepatic portal vein blockage is often ignored because patients are usually asymptomatic. Subacute and chronic stages can cause symptoms including splenomegaly and hematemesis without hepatic decompensation. Imaging studies aid in the diagnosis; Doppler imaging is added to ultrasonography to visualize portal vein blood flow. MRI and CT scans are used to visualize portal vein blockage. Prevention of acute bleeding is the cornerstone in the management. Studies have shown that transhepatic thrombolysis is the preferred choice to avoid systemic side effects. Transjugular intrahepatic portosystemic shunt (TIPS) treats extrahepatic portal venous thrombosis and is typically followed by conservative variceal hemorrhage treatment. Liver transplantation is performed when other management measures fail. Here, we present a rare case of EHPVO in a nine-year-old female who was lost to follow-up for a long time and later showed signs of portal biliopathy and non-visualization of a surgically created splenorenal shunt. Re-shunting was performed after detailed conservative management, and the patient responded well to the treatment given.
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21
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Interventional Management of Portal Hypertension in Cancer Patients. Curr Oncol Rep 2022; 24:1461-1475. [PMID: 35953600 DOI: 10.1007/s11912-022-01319-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/09/2022] [Indexed: 01/27/2023]
Abstract
PURPOSE OF REVIEW To provide an overview of the classifications and clinical hallmarks of common cancer-related conditions that contribute to the high incidence of portal hypertension in this population and provide an update on currently available interventional radiology therapeutic approaches. RECENT FINDINGS In the last few decades, there have been significant advancements in understanding the pathophysiology of portal hypertension. This knowledge has led to the development of safer and more effective minimally invasive approaches. The main objective is to provide alternatives to prevent life-threatening complications from clinically significant portal hypertension and to allow the continuation of cancer treatment interventions that would otherwise be stopped. Clinicians involved in cancer care should be aware of risk factors, associated complications, and management of portal hypertension in cancer patients. Interventional radiology offers minimally invasive alternatives that play a central role in improving clinical outcomes and survival of these patients, allowing the continuation of cancer treatments.
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22
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Semalti K, Kilambi R, Pal S, Srivastava DN, Sahni P, Madhusudhan KS. Benign Hepatic Nodules in Patients With Primary Extrahepatic Portal Vein Obstruction: Clinical and Magnetic Resonance Imaging Features. J Clin Exp Hepatol 2022; 12:1301-1309. [PMID: 36157149 PMCID: PMC9499837 DOI: 10.1016/j.jceh.2022.04.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2022] [Accepted: 04/15/2022] [Indexed: 12/12/2022] Open
Abstract
Background/objective To retrospectively evaluate the magnetic resonance imaging (MRI) features of benign hepatic nodules in patients with extrahepatic portal vein obstruction (EHPVO) and assess predictable features for their development. Methods This retrospective observational study included 18 diagnosed patients of EHPVO who underwent contrast enhanced abdominal MRI at our Institute between June 2016 and May 2017, and who could be followed up for at least two years. The patients with liver nodules formed the study group (n = 8; 4 males, 4 females; mean age: 26.1 ± 10.9 years) and patients without liver nodules were controls (n = 10; 3 males, 7 females; mean age: 24.2 ± 15.1 years). Liver nodules were confirmed as benign by either biopsy or stability on follow up imaging. MRI features of liver nodules were assessed. Clinical details and imaging data of the study group were compared with controls to assess predictable features. Results There was no statistically significant difference in age, gender, clinical characteristics and upper gastrointestinal endoscopic findings between the study and control groups. The size of the lienorenal collateral, left renal vein and superior mesenteric vein were significantly larger in the study group (P < 0.05). In the study group, the majority had multiple hepatic nodules with most of them being isointense on T1 (18/35; 51.4%) and T2-weighted images (16/35; 45.7%) and showing restriction of diffusion (21/35; 60%). All (n = 35) lesions showed arterial phase hyperenhancement and none showed washout in the venous phase. The patients in the control group did not develop any liver nodules during the follow-up period. Conclusion Liver nodules in patients with EHPVO are likely to be benign and have characteristic MRI features. Significantly larger lienorenal collateral, left renal vein and superior mesenteric vein were associated with the development of these nodules.
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Affiliation(s)
- Kapil Semalti
- Department of Radiodiagnosis and Interventional Radiology. All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
| | - Ragini Kilambi
- Department of Gastrointestinal Surgery, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi, 110070, India
| | - Sujoy Pal
- Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
| | - Deep N. Srivastava
- Department of Radiodiagnosis and Interventional Radiology. All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
| | - Peush Sahni
- Department of Gastrointestinal Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
| | - Kumble S. Madhusudhan
- Department of Radiodiagnosis and Interventional Radiology. All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India
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Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults (with video). Hepatobiliary Pancreat Dis Int 2022; 21:25-32. [PMID: 34426078 DOI: 10.1016/j.hbpd.2021.08.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2021] [Accepted: 08/02/2021] [Indexed: 02/07/2023]
Abstract
BACKGROUND Extrahepatic portal vein obstruction (EHPVO) results in severe portal hypertension (PHT) leading to severely compromised quality of life. Often, pharmacological and endoscopic management is unable to solve this problem. Restoring hepatic portal flow using meso-Rex bypass (MRB) may solve it. This procedure, uncommon in adult patients, is considered the treatment of choice for EHPVO in children. METHODS From 1997 to 2018, 8 male and 6 female adults, with a median age of 51 years (range 22-66) underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels, Belgium. Symptoms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings, sepsis due to portal biliopathy, and/or severe abdominal discomfort. The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein. RESULTS Median operative time was 500 min (range 300-730). Median follow-up duration was 22 months (range 2-169). One patient died due to hemorrhagic shock following percutaneous transluminal intervention for early graft thrombosis. Major morbidity, defined as Clavien-Dindo score ≥ III, was 35.7% (5/14). Shunt patency at last follow-up was 64.3% (9/14): 85.7% (6/7) of pure venous grafts and only 42.9% (3/7) of prosthetic graft. Symptom relief was achieved in 85.7% (12/14) who became asymptomatic after MRB. CONCLUSIONS Adult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications. In such patients, MRB represents the only and last resort to restore physiological portal vein flow. Although successful in a majority of patients, this procedure is associated with major morbidity and mortality and should be done in tertiary centers experienced with vascular liver surgery to get the best results.
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24
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De Bruyne R, De Bruyne P. Vascular Disorders of the Liver. TEXTBOOK OF PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION 2022:931-951. [DOI: 10.1007/978-3-030-80068-0_70] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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25
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Ferreira-Silva J, Gaspar R, Liberal R, Cardoso H, Macedo G. Transient splenic elastography predicts high-risk esophageal varices in patients with non-cirrhotic portal hypertension. Scand J Gastroenterol 2021; 56:1462-1466. [PMID: 34428123 DOI: 10.1080/00365521.2021.1968485] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND AND AIMS Non-cirrhotic portal hypertension (NCPH) comprise a group of diseases that cause portal hypertension without cirrhosis, leading to a high risk of hemorrhage from esophageal varices. There are no non-invasive predictors of high-risk varices (HRV) described in the literature for NCPH. This study aimed to evaluate whether transient splenic elastography (TSE) can predict HRV in patients with NCPH. METHODS Prospective study of patients with NCPH who underwent a single timepoint evaluation with transient liver and spleen elastography, ultrasonography, upper endoscopy, and laboratory tests. The study was performed from January to September 2020. Patients were divided into two groups based on the presence of HRV. The relation between TSE, transient liver elastography (TLE), spleen size, and platelet count to the presence of HRV was evaluated. RESULTS Of 42 patients with NCPH, 50% (21/42) presented HRV. In univariate analysis, TSE (median, 58.4 vs. 28.3, p = 0.009) and spleen size (median, 17.5 vs. 14.5 cm, p = 0.013) were associated with HRV. No statistically significant relationship was found between the presence of HRV and platelet count or TLE. In multivariate analysis, TSE was the only variable related to HRV (OR 1.21, 95% CI 1.02-1.38). TSE had a good performance in predicting HRV in our population (AUROC 0.878; 95% CI 0.751-1000). TSE > 35.4 kPa presents 93.3% sensitivity, 60.0% specificity, and 90.9% negative predictive value. CONCLUSION In our population of patients with NCPH, TSE is useful in predicting HRV. TLE, spleen size, and platelet count were not related to HRV.
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Affiliation(s)
- Joel Ferreira-Silva
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal
| | - Rui Gaspar
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Rodrigo Liberal
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Hélder Cardoso
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
| | - Guilherme Macedo
- Gastroenterology Department, Centro Hospitalar Universitário de São João, Porto, Portugal.,Faculty of Medicine, University of Porto, Porto, Portugal
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Barreto JCC, Sandy NS, Hessel G, de Alcantara RV, De Tommaso AMA, Yamada RM, Bellomo-Brandão MA. Increased Prevalence of Left Lobe Atrophy in Long-standing Extrahepatic Portal Vein Obstruction. J Pediatr Gastroenterol Nutr 2021; 73:642-646. [PMID: 34259650 DOI: 10.1097/mpg.0000000000003235] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVES The aim of this study was to describe long-term changes in standard blood tests and ultrasound (US) findings in pediatric patients with Extrahepatic Portal Vein Obstruction (EHPVO) who have not undergone Meso-Rex Bypass (MRB) surgery. METHODS US, laboratory, and endoscopic data of 77 patients were analyzed and compared at 2 different points in time: at initial workup and at most recent follow-up. Differences were assessed using McNemar and Wilcoxon tests, while correlations were evaluated using generalized estimating equations. RESULTS Upper gastrointestinal bleeding was the most frequent initial manifestation of EHPVO, in 45.4% of subjects. The mean age at diagnosis was 4.3 years. Seventy-four percentage had a previous history of umbilical catheterization and/or neonatal sepsis. Over time, there was a significant increase in the prevalence of leukopenia, thrombocytopenia, and, interestingly, of left lobe atrophy (LLA), even though the number of esophageal varices and the need for endoscopic interventions reduced. A significant correlation was found between history of umbilical catheterization and LLA, splenomegaly and LLA, and gallbladder wall thickening and LLA. Overall, the number of patients with cholelithiasis at initial workup was low (and therefore, not analyzed with inferential statistics); however, we observed a relative increase over time. CONCLUSIONS In this cohort of patients with EHPVO followed over more than 8 years without treatment with MRB, we report the novel finding of a significant increase in the prevalence of LLA over time. LLA correlated with past medical history of umbilical catheterization, and findings of splenomegaly and gallbladder wall thickening. We propose that that LLA should be further explored as a marker of portal hypertension, particularly in the context of a history of umbilical catheterization.
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Affiliation(s)
- Juliana C C Barreto
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
| | - Natascha S Sandy
- Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hospital for Sick Children, University of Toronto. Toronto, Ontario, Canada
| | - Gabriel Hessel
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
| | - Roberta V de Alcantara
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
| | - Adriana Maria A De Tommaso
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
| | - Roberto M Yamada
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
| | - Maria Angela Bellomo-Brandão
- Department of Pediatrics, Faculty of Medical Sciences, State University of Campinas (UNICAMP). Campinas, São Paulo, Brazil
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Li H, Sun PM, Sun HW, Cui Y. Progress in clinical diagnosis and treatment of cavernous transformation of the portal vein. Shijie Huaren Xiaohua Zazhi 2021; 29:662-669. [DOI: 10.11569/wcjd.v29.i12.662] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Cavernous transformation of the portal vein (CTPV) refers to the compensatory neoformation of venous collaterals around the hepatic portal after the main portal vein or its branches are blocked, in order to maintain liver blood perfusion. This disease is relatively rare, and in clinical practice, its etiology, diagnosis, and treatment are not well understood. Patients with portal hypertension-related syndromes need to receive active treatment. The main goal of treatment is to prevent upper gastrointestinal hemorrhage and hypersplenism caused by portal hypertension, and to restore hepatopetal portal blood perfusion. It is necessary to fully assess the patient's condition and understand the best indications for each treatment. On this basis, we should adopt individualized comprehensive treatment strategies. This article reviews the latest advances in the understanding of the etiology, diagnosis, classification, and treatment of CTPV.
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Affiliation(s)
- Hao Li
- Department of General Surgery, Strategic Support Force Medical Center, Beijing 100101, China
| | - Pei-Ming Sun
- Department of General Surgery, Strategic Support Force Medical Center, Beijing 100101, China
| | - Hong-Wei Sun
- Department of General Surgery, Strategic Support Force Medical Center, Beijing 100101, China
| | - Yan Cui
- Department of General Surgery, Strategic Support Force Medical Center, Beijing 100101, China
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28
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Current Indications and Long-Term Outcomes of Surgical Portosystemic Shunts in Adults. J Gastrointest Surg 2021; 25:1437-1444. [PMID: 32424687 DOI: 10.1007/s11605-020-04643-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2020] [Accepted: 05/03/2020] [Indexed: 01/31/2023]
Abstract
BACKGROUND Surgical portosystemic shunts are rare. We reviewed indications, operative details, and outcomes of patients undergoing surgical portosystemic shunt procedures. METHODS We retrospectively reviewed clinical data of consecutive patients between 1997 and 2018 from a single institution. Clinical characteristics and outcomes were compared between two groups: patients with portomesenteric venous thrombosis (PMVT) vs those with cirrhosis. Endpoints included 30-day mortality, shunt-related complications, patency, and survival. RESULTS There were 99 patients, 45 male and 54 female, with a mean age of 46 ± 18 years, enrolled in the study. There were 63 patients (63%) with PMVT and 36 patients (36%) with cirrhosis. Both groups had similar demographics, cardiovascular risk factors, and aneurysm extent, except for more diabetes among those with cirrhosis (p < 0.05). There were no significant differences in procedural metrics and intra-procedure complications between groups, except that patients with PMVT underwent more non-selective shunts than those with cirrhosis (63% vs. 30%, p < 0.001). There were two 30-day deaths (2%), with no difference in mortality and MAEs between groups. On univariate analysis, cholangiopathy and PMVT were associated with graft thrombosis (HR = 9.22, 95% CI 1.22-70.27) while race, smoking, cardiac comorbidity, type of operative shunt, configuration of the shunt, and use of conduit were not (p > 0.05). Patients with PMVT had significantly lower 1-, 5-, and 10-year primary (77%, 71%, and 71% vs. 97%, p = 0.009) and secondary patency (88%, 76%, and 72% vs. 96%, p = 0.027) compared with those with cirrhosis. The 1-, 5-, and 10-year survival rates were 94%, 84%, and 61% for patients with PMVT compared with 88%, 58%, and 26% for those with cirrhosis (non-adjusted HR 0.40, 95% CI 0.19-0.84, p = 0.01, age-adjusted HR 0.51, 95% CI 0.24-1.09, p = 0.08). The survival of patients with PMVT without liver disease trended higher than those with liver disease; however, when adjusted for age, the survival gap narrowed, and the difference was not statistically significant (p = 0.19), survival being lowest for those with PMVT and liver disease. CONCLUSIONS Surgical portosystemic shunts are safe and effective for symptom relief in selected patients with portal hypertension. The odds of graft thrombosis is 9 times higher in patients with PMVT. Overall survival is similar in patients with PMVT or cirrhosis.
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ChKurdia K, Aggarwal A, Tandup C, Dahiya D, Podder S, Behera A. Management of a rare case of extra hepatic portal vein obstruction with temporomandibular joint ankylosis and review of literature. Ann Hepatobiliary Pancreat Surg 2021; 25:283-286. [PMID: 34053933 PMCID: PMC8180409 DOI: 10.14701/ahbps.2021.25.2.283] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2020] [Revised: 09/16/2020] [Accepted: 09/20/2020] [Indexed: 11/17/2022] Open
Abstract
Extrahepatic portal venous obstruction (EHPVO) and temporomandibular joint (TMJ) ankylosisis are significant problems in Asian countries. Both EHPVO and bilateral TMJ ankylosis may have rare association due to protein C and S deficiency which may cause hypercoagulability as well as reduced fibrinolytic activity. Ankylosis arising in early childhood is associated with facial asymmetry, feeding difficulty and speech development alterations. It is also associated with great challenges of endoscopic management in extra hepatic portal vein obstruction (EHPVO) with variceal bleed as well as air way management during surgical management and post-operative recovery. Recently a case series had shown association of TMJ ankylosis with EHPVO due to protein C deficiency which might be an etiological factor for both EHPVO as well as TMJ ankylosis. This case report documents a case of 14 year young girl who had TMJ ankylosis due to ear infection and EHPVO with esophageal varices had multiple episodes of upper GI bleed with mild deficiency of protein C and S, successfully managed with proximal splenorenal shunt to prevent further episodes of upper GI bleed, as endoscopic management is not feasible due to TMJ ankylosis.
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Affiliation(s)
- Kailash ChKurdia
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Ambuj Aggarwal
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Cherring Tandup
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Divya Dahiya
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Subrata Podder
- Department of Anesthesia and Critical Care, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Arunanshu Behera
- Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Costache RS, Dragomirică AS, Dumitraș EA, Mariana J, Căruntu A, Popescu A, Costache DO. Portal vein thrombosis: A concise review (Review). Exp Ther Med 2021; 22:759. [PMID: 34035856 DOI: 10.3892/etm.2021.10191] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Accepted: 02/19/2021] [Indexed: 12/14/2022] Open
Abstract
Portal vein thrombosis (PVT) is a frequent complication in cirrhotic patients, but it may also exist as a basic vascular condition even without any liver damage. Local and systemic factors play a significant role in the pathogenesis of PVT; yet, in practice, more than one factor may be identified. PVT can be considered a result of liver fibrosis and hepatic insufficiency. The JAK2 mutation has been accepted as a factor producing PVT. In general, the anticoagulants are recommended but this therapy should be used carefully in treating patients that associate coagulopathy or thrombocytopenia and esophageal varices. Acute PVT without bowel infarction has a good prognosis. In liver cirrhosis, the mortality due to hemorrhage is higher than in chronic PVT. Therefore, for the patients with PVT, the survival rate is decreased by 55% in two years, due to hepatic insufficiency. Regarding the treatment, LMWH (low molecular weight heparine) is the most utilized in patients with cirrhosis, non-malignancies, infections, or those who are awaiting a liver transplant. DOACs (direct-acting oral anticoagulants) may be used in the rest of the medical conditions, being safe and equal to LMWH.
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Affiliation(s)
- Raluca S Costache
- Department of Gastroenterology, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania.,5th Clinical Department, Gastroenterology and Internal Medicine Discipline, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Andreea S Dragomirică
- Department of Gastroenterology, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania
| | - Elena A Dumitraș
- Department of Gastroenterology, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania
| | - Jinga Mariana
- Department of Gastroenterology, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania.,5th Clinical Department, Gastroenterology and Internal Medicine Discipline, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Ana Căruntu
- Department of Maxillofacial Surgery, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania
| | - Andrada Popescu
- Department of Gastroenterology, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania.,5th Clinical Department, Gastroenterology and Internal Medicine Discipline, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania
| | - Daniel O Costache
- Department of Research, 'Carol Davila' University Central Emergency Military Hospital, 010825 Bucharest, Romania
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Surgical shunts for extrahepatic portal vein obstruction in pediatric patients: a systematic review. HPB (Oxford) 2021; 23:656-665. [PMID: 33388243 DOI: 10.1016/j.hpb.2020.11.1149] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2020] [Accepted: 11/29/2020] [Indexed: 12/12/2022]
Abstract
BACKGROUND Extrahepatic portal vein obstruction (EHPVO) causes portal hypertension in noncirrhotic children. Among surgical treatments, it is unclear whether the meso-Rex shunt (MRS) or portosystemic shunt (PSS) offers lower post-operative morbidity and superior patency over time. Our objective was to evaluate long-term outcomes comparing MRS and PSS for pediatric patients with EHPVO. METHODS A systematic review was conducted of articles reporting children undergoing surgical shunts for EHPVO from 1/2000-2/2020. Of 87 articles screened, 22 were eligible for inclusion. The primary outcome was shunt thrombosis and secondary outcomes included non-operative complications, stenosis, and re-operation. RESULTS Eighteen of 22 studies were of good quality and four had fair quality. Of 461 patients included, 340 underwent MRS and 121 underwent PSS. MRS were associated with a higher rate of post-operative thrombosis when compared to PSS (14.1% vs 5.8%, p = 0.021). There were 40/340 MRS patients (11.8%) that required at least one re-operation for either shunt thrombosis or stenosis, versus 5/121 PSS patients (4.1%), p = 0.019. CONCLUSION Both MRS and PSS result in acceptable long-term patency rates, but the more technically demanding MRS is associated with higher post-shunt thrombosis, often requiring further operative intervention. This study suggests that PSS may offer advantages for pediatric patients with EHPVO.
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Wang JB, Gao Y, Liu JW, Dai MG, Yang SW, Ye B. Gastroesophageal varices in a patient presenting with essential thrombocythemia: A case report. World J Clin Cases 2021; 9:1871-1876. [PMID: 33748236 PMCID: PMC7953392 DOI: 10.12998/wjcc.v9.i8.1871] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/16/2020] [Revised: 01/03/2021] [Accepted: 01/28/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Gastroesophageal varices are a rare complication of essential thrombocythemia (ET). ET is a chronic myeloproliferative neoplasm (MPN) characterized by an increased number of blood platelets.
CASE SUMMARY A 46-year-old woman, who denied a history of liver disease, was admitted to our hospital on presentation of hematemesis. Laboratory examination revealed a hemoglobin level of 83 g/L, and a platelet count of 397 × 109/L. The appearance of gastric and esophageal varices with red colored signs as displayed by an urgent endoscopy was followed by endoscopic variceal ligation and endoscopic tissue adhesive. Abdominal computed tomography revealed cirrhosis, marked splenomegaly, portal vein thrombosis and portal hypertension. In addition, bone marrow biopsy and evidence of mutated Janus kinase 2, substantiated the onset of ET. The patient was asymptomatic with regular routine blood testing during the 6-mo follow-up period. Therefore, in this case, gastroesophageal varices were induced by ET.
CONCLUSION MPN should be given considerable attention when performing differential diagnoses in patients with gastroesophageal varices. An integrated approach such as laboratory tests, radiological examination, and pathological biopsy, should be included to allow optimal decisions and management.
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Affiliation(s)
- Jian-Bo Wang
- Department of Gastroenterology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
| | - Yang Gao
- Department of Radiology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
| | - Jun-Wei Liu
- Department of Gastroenterology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
| | - Mu-Gen Dai
- Department of Gastroenterology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
| | - Shang-Wen Yang
- Department of Gastroenterology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
| | - Bin Ye
- Department of Gastroenterology, Lishui Hospital of Zhejiang University, The Fifth Affiliated Hospital of Wenzhou Medical University, Lishui Central Hospital, Lishui 323000, Zhejiang Province, China
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Elhence A, Gamanagatti SR, Shalimar. Transjugular Intrahepatic Portosystemic Shunt for Non-malignant Portal Vein Thrombosis. PORTAL VEIN THROMBOSIS 2021:117-129. [DOI: 10.1007/978-981-33-6538-4_9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Kawachi S, Chiba N, Nakagawa M, Kobayashi T, Hikita K, Sano T, Tomita K, Hirano H, Abe Y, Obara H, Shimazu M. Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literature. BMC Surg 2020; 20:257. [PMID: 33121468 PMCID: PMC7597044 DOI: 10.1186/s12893-020-00921-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2020] [Accepted: 10/22/2020] [Indexed: 01/08/2023] Open
Abstract
Background Idiopathic portal hypertension (IPH) generally has a good prognosis and rarely results in liver transplantation. Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis. Case presentation We report the case of a 51-year-old female patient diagnosed with IPH more than 20 years ago. She suffered severe jaundice, massive ascites, and encephalopathy at the time of her visit to our hospital. The patient’s extrahepatic portal vein showed a scar-like stenosis, and the portal flow was completely hepatofugal. Collateral circulation such as the splenorenal shunt was well developed, and multiple splenic artery aneurysms up to 2 cm were observed in the splenic hilum. Her Model for End-Stage Liver Disease score increased to over 40 because of renal dysfunction, requiring temporary dialysis. We performed LDLT using her husband’s right lobe graft and splenectomy. The extrahepatic stenotic portal vein was completely resected, and the superficial femoral vein (SFV) graft collected from the recipient’s right leg was used for portal reconstruction as an interposition graft. Although the clinical course after LDLT had many complications, the patient was discharged on postoperative day 113 and has been fine for 2 years after LDLT. Histopathologically, the explanted liver had obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal cirrhosis. Conclusion This case showed that severe IPH is occasionally associated with extrahepatic portal vein stenosis and can be treated with LDLT with portal vein reconstruction using an interposition graft. It was also suggested that the SFV is a useful choice for the interposition graft.
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Affiliation(s)
- Shigeyuki Kawachi
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan.
| | - Naokazu Chiba
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Masashi Nakagawa
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Toshimichi Kobayashi
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Kosuke Hikita
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Toru Sano
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Koichi Tomita
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Hiroshi Hirano
- Departmet of Diagnostic Pathology, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
| | - Yuta Abe
- Department of Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 1608582, Japan
| | - Hideaki Obara
- Department of Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 1608582, Japan
| | - Motohide Shimazu
- Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 1930998, Japan
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Pediatric living donor liver transplantation with non-anatomical portal vein reconstruction in idiopathic extrahepatic portal vein thrombosis. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2020.101510] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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Nicoară-Farcău O, Rusu I, Stefănescu H, Tanțău M, Badea RI, Procopeț B. Diagnostic challenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease. World J Gastroenterol 2020; 26:3000-3011. [PMID: 32587444 PMCID: PMC7304099 DOI: 10.3748/wjg.v26.i22.3000] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 03/31/2020] [Accepted: 05/27/2020] [Indexed: 02/06/2023] Open
Abstract
Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension, which differ from cirrhosis through histological alterations, hemodynamic characterization and, clinical outcome. Because of the similarities in clinical presentation and imaging signs, frequently these patients, and particularly those with porto-sinusoidal vascular disease (PSVD), are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis. The most challenging differentiation to be considered is between PSVD and cirrhosis and, although not pathognomonic, liver biopsy is still the standard of diagnosis. Although they still require extended validation before being broadly used, new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease, like transient elastography, contrast-enhanced ultrasound or metabolomic profiling, have shown promising results. Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction, especially now when it is known that 40% of patients suffering from PSVD develop portal vein thrombosis. In this particular case, once the portal vein thrombosis occurred, the diagnosis of PSVD is impossible according to the current guidelines. Moreover, so far, the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances. In this review we highlighted the diagnostic challenges regarding the PSVD, as well as the current techniques used in the evaluation of these patients.
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Affiliation(s)
- Oana Nicoară-Farcău
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Ioana Rusu
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Pathology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Horia Stefănescu
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Marcel Tanțău
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Radu Ion Badea
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Imagistic Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
| | - Bogdan Procopeț
- University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca 400000, Romania
- Gastroenterology Department, Regional Institute of Gastroenterology and Hepatology “O. Fodor”, Cluj-Napoca 400000, Romania
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CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know. Insights Imaging 2020; 11:48. [PMID: 32185572 PMCID: PMC7078419 DOI: 10.1186/s13244-020-00852-z] [Citation(s) in RCA: 29] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2019] [Accepted: 02/21/2020] [Indexed: 12/11/2022] Open
Abstract
Abdominopelvic vascular compression syndromes include a variety of uncommon conditions characterized by either extrinsic compression of blood vessels by adjacent anatomical structures (i.e., median arcuate ligament syndrome, nutcracker syndrome, May-Thurner syndrome) or compression of hollow viscera by adjacent vessels (i.e., superior mesenteric artery syndrome, ureteropelvic junction obstruction, ureteral vascular compression syndromes, portal biliopathy). These syndromes can be unexpectedly diagnosed even in asymptomatic patients and the predisposing anatomic conditions can be incidentally discovered on imaging examinations performed for other indications, or they can manifest with atypical abdominal symptoms and acute complications, which may lead to significant morbidity if unrecognized. Although computed tomography (CT) is an accurate noninvasive technique for their detection, the diagnosis remains challenging due to the uncommon clinical presentation and often overlooked imaging features. Dynamic imaging may be performed in order to evaluate patients with inconstant symptoms manifesting in a specific position. The purposes of this paper are to review the CT imaging findings of abdominopelvic vascular compression syndromes, correlating with anatomical variants and to provide key features for the noninvasive imaging diagnosis.
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Di Giorgio A, D'Antiga L. Reply to comment: Etiology of non-cirrhotic portal vein thrombosis in children: Few or many causes? Dig Liver Dis 2020; 52:120-121. [PMID: 31648919 DOI: 10.1016/j.dld.2019.09.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2019] [Accepted: 09/26/2019] [Indexed: 12/11/2022]
Affiliation(s)
- Angelo Di Giorgio
- Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Lorenzo D'Antiga
- Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy.
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Roncati L, Manenti A, Gallo G, Bernardelli G, Farinetti A. Prognostic Elements of Portal Vein Thrombosis. Ann Vasc Surg 2019; 65:e307-e308. [PMID: 31639482 DOI: 10.1016/j.avsg.2019.10.035] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2019] [Revised: 10/11/2019] [Accepted: 10/13/2019] [Indexed: 02/07/2023]
Affiliation(s)
- Luca Roncati
- Department of Pathology, University of Modena, Polyclinic Hospital, Modena, Italy
| | - Antonio Manenti
- Department of Surgery, University of Modena, Polyclinic Hospital, Modena, Italy.
| | - Graziana Gallo
- Department of Pathology, University of Modena, Polyclinic Hospital, Modena, Italy
| | - Giuditta Bernardelli
- Department of Pathology, University of Modena, Polyclinic Hospital, Modena, Italy
| | - Alberto Farinetti
- Department of Surgery, University of Modena, Polyclinic Hospital, Modena, Italy
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Mohapatra N, Sandhyav R, Agrawal N, Arora A, Chattopadhyay TK. Effect of Proximal Splenorenal Shunt on Intraoperative Portal Venous Pressure and Its Correlation with Clinical Outcomes. Indian J Surg 2019. [DOI: 10.1007/s12262-018-1803-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
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Di Giorgio A, De Angelis P, Cheli M, Vajro P, Iorio R, Cananzi M, Riva S, Maggiore G, Indolfi G, Calvo PL, Nicastro E, D'Antiga L. Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study. Dig Liver Dis 2019; 51:1179-1184. [PMID: 30928422 DOI: 10.1016/j.dld.2019.02.014] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/26/2018] [Revised: 12/20/2018] [Accepted: 02/24/2019] [Indexed: 02/06/2023]
Abstract
OBJECTIVES Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome. METHODS We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery. RESULTS 187 patients, mean age at diagnosis 4 ± 3.7 years, had a history of prematurity (61%); UVC (65%); neonatal illnesses (79%). The diagnosis followed the detection of splenomegaly (40%), gastrointestinal bleeding (36%), hypersplenism (6%), or was incidental (18%). Of 71 patients who had endoscopy at presentation 62 (87%) had oesophageal varices. After 11.3 years' follow up 63 (34%) required surgery or TIPS. Ten-year survival rate was 98%, with 90% shunt patency. Spleen size, variceal bleeding and hypersplenism at presentation were predictors of surgery or TIPS (p < 0.05). CONCLUSION PVT is associated with congenital and acquired co-morbidities. History of prematurity, neonatal illnesses and UVC should lead to rule out PVT. Large spleen, variceal bleeding and hypersplenism at presentation predict the need for eventual surgery in a third of cases.
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Affiliation(s)
- Angelo Di Giorgio
- Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy
| | - Paola De Angelis
- Paediatric Surgery and Endoscopy, Ospedale Pediatrico Bambino Gesù, Roma, Italy
| | - Maurizio Cheli
- Paediatric Surgery, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy
| | - Pietro Vajro
- Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana" Section of Pediatrics, University of Salerno, Baronissi (Salerno), Italy
| | - Raffaele Iorio
- Paediatric Liver Unit, Department of Translational Medical Science, University of Naples Federico II, Naples, Italy
| | - Mara Cananzi
- Unit of Pediatric Gastroenterology and Hepatology, Dpt. of Women's and Children's Health, University Hospital of Padova, Italy
| | - Silvia Riva
- Paediatric department and transplantation, Ismett, Palermo, Italy
| | - Giuseppe Maggiore
- Paediatric Section of the Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | | | - Pier Luigi Calvo
- Paediatric Gastroenterology, Department of Pediatrics, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza di Torino, University of Torino, Torino, Italy
| | - Emanuele Nicastro
- Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy
| | - Lorenzo D'Antiga
- Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy.
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Hernández-Gea V, De Gottardi A, Leebeek FWG, Rautou PE, Salem R, Garcia-Pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol 2019; 71:175-199. [PMID: 30822449 DOI: 10.1016/j.jhep.2019.02.015] [Citation(s) in RCA: 72] [Impact Index Per Article: 12.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2018] [Revised: 02/15/2019] [Accepted: 02/19/2019] [Indexed: 12/11/2022]
Abstract
Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.
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Affiliation(s)
- Virginia Hernández-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, European Reference Network for Rare Vascular Liver Diseases, Universitat de Barcelona, Spain
| | - Andrea De Gottardi
- Hepatology, University Clinic of Visceral Medicine and Surgery, Inselspital, and Department of Biomedical Research, University of Bern, Bern, Switzerland
| | - Frank W G Leebeek
- Department of Haematology, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - Pierre-Emmanuel Rautou
- Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Hôpital Beaujon, AP-HP, Clichy, France; Inserm, UMR-970, Paris Cardiovascular Research Center, PARCC, Paris, France
| | - Riad Salem
- Department of Radiology, Section of Interventional Radiology, Northwestern University, Chicago, IL, USA
| | - Juan Carlos Garcia-Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic de Barcelona, IDIBAPS, CIBERehd, European Reference Network for Rare Vascular Liver Diseases, Universitat de Barcelona, Spain.
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Gupta P, Kalra N, Gulati A, Chandel K, Priyaranjan P, Dahal P, Sinha SK, Duseja A, Dhiman RK, Sandhu MS. Changes in liver morphology in patients with extrahepatic portal venous obstruction: a retrospective magnetic resonance imaging study. Br J Radiol 2019; 92:20180890. [PMID: 30924678 PMCID: PMC6592076 DOI: 10.1259/bjr.20180890] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2018] [Revised: 03/21/2019] [Accepted: 03/25/2019] [Indexed: 12/14/2022] Open
Abstract
OBJECTIVE To investigate the liver parenchymal changes on MRI in patients with extrahepatic portal venous obstruction (EHPVO). METHODS This was a retrospective evaluation of the MRI studies in patients with EHPVO between January 2016 and April 2018. The diagnosis of EHPVO in each case had been established on the basis of clinical and Doppler parameters. Following findings were recorded on MRI: liver size; outline; overall volume redistribution; volume redistribution of caudate, right and left lobes; signal heterogeneity, intrahepatic biliary radicle dilatation, focal liver lesions, gallstones and ascites. RESULTS A total of 164 MRI studies were evaluated. Median age was 27 years (range, 6-70). There were 90 (54.9%) males and 74 (45.1%) females. The median liver size was 14 cm (range 5-17). The median spleen size was 15.9 cm (range, 6-28). A spleen size ≥12 cm was seen in 136 (82.9%) patients. The liver outline was nodular in 32 (19.5%) patients. Volume redistribution was seen in 63 (38.4%) patients. Caudate lobe hypertrophy was seen in 49 (29.9%) patients. Right lobe atrophy and hypertrophy was seen in 4 (2.4%) and 1 (0.6%) patients respectively. Left lobe atrophy and hypertrophy was seen in 15 (9.1%) and 10 (6.1%) patients respectively. 30 MRI (18.3%) showed heterogeneous signal intensity. Focal lesions were seen in 22 (13.4%) patients. Intrahepatic biliary radicle dilatation, gallstones and ascites were seen in 120 (73.2%), 54 (32.9%) and 26 (15.9%) patients respectively. CONCLUSION Liver morphological changes are common in EHPVO. ADVANCES IN KNOWLEDGE The morphological changes in liver in patients with EHPVO have not been described previously. This information will prevent misdiagnosis of this condition as cirrhosis.
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Affiliation(s)
- Pankaj Gupta
- Department of Gastroenterology, PGIMER, Chandigarh, India
| | - Naveen Kalra
- Radiodiagnosis and Imaging, PGIMER, Chandigarh, India
| | - Ajay Gulati
- Radiodiagnosis and Imaging, PGIMER, Chandigarh, India
| | | | | | - Prajwal Dahal
- Radiodiagnosis and Imaging, PGIMER, Chandigarh, India
| | - Saroj K Sinha
- Department of Gastroenterology, PGIMER, Chandigarh, India
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Alexopoulou A, Mani I, Tiniakos DG, Kontopidou F, Tsironi I, Noutsou M, Pantelidaki H, Dourakis SP. Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report. World J Hepatol 2019; 11:483-488. [PMID: 31183008 PMCID: PMC6547289 DOI: 10.4254/wjh.v11.i5.483] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2019] [Revised: 05/15/2019] [Accepted: 05/21/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Idiopathic non-cirrhotic portal hypertension (INCPH) is mainly associated with thrombophilia in Western countries. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disease that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias. Portal and hepatic venous thrombosis were reported in PNH. A rare case of INCPH complicating PNH is described.
CASE SUMMARY A 63-year old woman with a 2-year past medical history of PNH without treatment was admitted because of jaundice and refractory ascites requiring large volume paracentesis. Liver histology revealed portal venopathy with portal fibrosis and sclerosis, nodular regenerative hyperplasia, parenchymal ischemic changes, and focal sinusoidal and perivenular fibrosis without bridging fibrosis or cirrhosis, all indicative of INCPH. The flow cytometry confirmed PNH diagnosis and eculizumab treatment was initiated. Her condition was improved gradually, bilirubin was normalized 6 months following initiation of eculizumab, and 1 year later diuretics were stopped.
CONCLUSION Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria.
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Affiliation(s)
- Alexandra Alexopoulou
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Iliana Mani
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Dina G Tiniakos
- Institute of Cellular Medicine, Faculty of Medical Sciences, Newcastle University, Framlington Place, Newcastle NE2 4HH, United Kingdom
- Department of Pathology, Aretaieion Hospital, National and Kapodistrian University of Athens, Athens 11528, Greece
| | - Flora Kontopidou
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Ioanna Tsironi
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Marina Noutsou
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Helen Pantelidaki
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
| | - Spyros P Dourakis
- 2nd Department of Medicine, Medical School, National and Kapodistrian University of Athens, Hippokration General Hospital, Athens 11527, Greece
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