|
1
|
Silva CLM, Puthanveetil PN, Oliveira SD. Liver bypass in the development of pathogen-associated pulmonary vascular disease: contribution of mesocaval and portosystemic shunts. Am J Physiol Gastrointest Liver Physiol 2025; 328:G791-G800. [PMID: 40323787 DOI: 10.1152/ajpgi.00409.2024] [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: 01/03/2025] [Revised: 02/18/2025] [Accepted: 04/24/2025] [Indexed: 05/07/2025]
Abstract
Portosystemic and mesocaval shunts are aberrant vascular connections that bypass hepatic detoxification process, directly linking the portal to the systemic circulation. These shunts, whether congenital or acquired, might play a pivotal role in the pathogenesis of systemic inflammatory diseases, such as schistosomiasis-associated pulmonary hypertension (Sch-PH) by facilitating the dissemination of pathogen-derived eggs and antigens from the gut and mesentery into the lungs. Beyond the translocation of Schistosoma mansoni eggs, emerging evidence implicates that gut-lung microbiome dysbiosis contributes to the development of pulmonary hypertension (PH) in the preclinical animal model of Sch-PH. Sch-PH emerges as a chronic complication of schistosomiasis and evolves silently, progressively increasing the mean pulmonary arterial pressure and vascular resistance, leading to right heart hypertrophy, failure, and significant morbidity and mortality. Chronic schistosomiasis is often linked to the development of portal hypertension, which significantly contributes to the formation of the porto/mesocaval shunt as a compensatory response that can have far-reaching implications on pulmonary vascular physiology. In addition, portal hypertension compromises the integrity of the intestinal barrier, exacerbating peritoneal and mesenteric inflammation, potentially facilitating microbial and metabolite entrance into the systemic circulation. This article briefly discusses the mechanisms by which porto/mesocaval shunts contribute to PH, especially Group I PH, focusing on the interplay between portosystemic shunting, microbial translocation, and systemic dissemination of proinflammatory metabolites.
Collapse
Affiliation(s)
- Claudia Lucia Martins Silva
- Biochemical and Molecular Pharmacology Laboratory, Institute of Biomedical Sciences, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil
| | - Prasanth N Puthanveetil
- Chicago College of Osteopathic Medicine, Midwestern University, Downers Grove, Illinois, United States
- Department of Pharmacology, College of Graduate Studies, Midwestern University, Downers Grove, Illinois, United States
| | - Suellen Darc Oliveira
- Vascular Immunobiology Laboratory, Department of Anesthesiology, College of Medicine, University of Illinois Chicago, Chicago, Illinois, United States
- Vascular Immunobiology Laboratory, Department of Physiology and Biophysics, University of Illinois Chicago, Chicago, Illinois, United States
| |
Collapse
|
|
2
|
Li W, Liu B, Feng H. Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review. Vasc Endovascular Surg 2025; 59:176-182. [PMID: 39262296 DOI: 10.1177/15385744241278870] [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: 09/13/2024]
Abstract
Background: Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. Purpose: We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein. A mini literature review of AF patients presented with gastrointestinal (GI) tract bleeding. Research design: Case report and literature review. Data Collection: An electronic search of PubMed was performed from inception to December 2023. Results: 34 AF patients presented with GI tract bleeding were identified published in the literature. The proportion of type II AF patients presenting with GI bleeding is greater (79%). Conclusions: We regard that both surgical ligation and endovascular closure of the shunt are effective and safe treatments for these patients, but coils embolization alone may not be sufficient to completely close the shunt when the shunt flow is high.
Collapse
Affiliation(s)
- Wenrui Li
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Bin Liu
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Hai Feng
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| |
Collapse
|
|
3
|
Jerbi B, Chourou H, Ben Aziza R, Jelassi O, Sdiri Y, Belhadj Ammar W, Kacem S, Aloui N, Achour R. Congenital portosystemic shunts: experience of a tertiary Tunisian referral center. AJOG GLOBAL REPORTS 2024; 4:100409. [PMID: 39512762 PMCID: PMC11541822 DOI: 10.1016/j.xagr.2024.100409] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2024] Open
Abstract
Congenital portosystemic shunt is a rare condition in which communications between the systemic venous circulation and the portal veins drain blood directly into the systemic circulation. Diagnosis may occur from the prenatal period to adulthood. Nevertheless, diagnosing and treating a congenital portosystemic shunt, particularly in the perinatal stage, remain challenging, as multiple complications can occur. This study aimed to describe the clinical characteristics of 3 cases of congenital portosystemic shunts diagnosed during pregnancy or the neonatal period, the diagnostic procedures, and their outcomes. This study reported 3 cases of full-term newborns with a congenital portosystemic shunt diagnosed at neonatal age. Case 1 was antenatally diagnosed with a congenital portosystemic shunt, which was confirmed postnatally via computed tomography and was associated with malformed ductus venosus and hypoplasia of the right portal vein. Cases 2 and 3 were siblings: a boy who had diffuse hemangiomatosis and a congenital portosystemic shunt complicated with severe persistent pulmonary hypertension and a girl who presented with a congenital portosystemic shunt and Kell alloimmunization. Congenital portosystemic shunts can be detected on prenatal ultrasounds during the etiologic workup of one of its complications or may be incidentally identified later in life. Children with congenital portosystemic shunts may develop various biological abnormalities, such as pulmonary hypertension, hypoxemia, encephalopathy, and liver tumors. A multidisciplinary approach and standardized protocols are required to optimize the management of congenital portosystemic shunts and minimize the short- and long-term consequences of congenital portosystemic shunts.
Collapse
Affiliation(s)
- Bilel Jerbi
- University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Jerbi and Jelassi)
| | - Hajer Chourou
- Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Chourou, Aziza, Sdiri, Ammar, and Kacem)
| | - Rim Ben Aziza
- Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Chourou, Aziza, Sdiri, Ammar, and Kacem)
| | - Omar Jelassi
- University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Jerbi and Jelassi)
| | - Yosra Sdiri
- Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Chourou, Aziza, Sdiri, Ammar, and Kacem)
| | - Wafa Belhadj Ammar
- Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Chourou, Aziza, Sdiri, Ammar, and Kacem)
| | - Samia Kacem
- Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Chourou, Aziza, Sdiri, Ammar, and Kacem)
| | - Nadia Aloui
- Department of Radiology, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Aloui)
| | - Radhouane Achour
- Department of Emergency, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunis, Tunisia (Achour)
| |
Collapse
|
|
4
|
Akel R, Aoun R. Incidental diagnosis of silent extrahepatic portosystemic shunt. Clin Case Rep 2024; 12:e8904. [PMID: 38711838 PMCID: PMC11070487 DOI: 10.1002/ccr3.8904] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2024] [Revised: 04/09/2024] [Accepted: 04/23/2024] [Indexed: 05/08/2024] Open
Abstract
Extrahepatic portosystemic shunts are a very rare disease. Early diagnosis and appropriate treatment are needed to prevent serious complications. In asymptomatic patients, conservative treatment is an option.
Collapse
Affiliation(s)
- Rhea Akel
- Department of Medical ImagingGuy Chatiliez Hospital CenterTourcoingFrance
| | - Rany Aoun
- Department of Digestive SurgeryGermon and Gauthier Hospital CenterBethuneFrance
| |
Collapse
|
|
5
|
Zhang LJ, Liu XY, Chen TF, Xu ZY, Yin HJ. Type II Abernethy malformation with cystic fibrosis in a 12-year-old girl: A case report. World J Clin Cases 2023; 11:7865-7871. [DOI: 10.12998/wjcc.v11.i32.7865] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2023] [Revised: 10/21/2023] [Accepted: 11/02/2023] [Indexed: 11/16/2023] Open
Abstract
BACKGROUND Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is an uncommon malformation resulting from aberrant development of the portal venous system. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene. It mainly affects the exocrine glands of the respiratory, digestive and reproductive systems. It is considered extremely rare in the Asian population. We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF.
CASE SUMMARY A 12-year-old girl presented with a medical history of recurring respiratory infections and hemoptysis, and chest computed tomography (CT) showed bronchiectasis. Whole exome sequencing was performed for the patient, yielding findings that revealed a compound heterozygous variant of the CFTR gene: c.233_c.234insT/p.Trp79fsTer3 (maternal origin); c.2909G>A/p.Gly970Asp (paternal origin). CF was diagnosed. The physician’s attention was drawn to the presence of splenomegaly during disease progression. Abdominal enhanced CT revealed splenomegaly, compression of the left kidney, and multiple tortuous dilated vascular shadows were seen at the splenic hilum, which flowed back into the left renal vein and portal vein, suggesting Abernethy malformation type II. Intraoperatively, the abnormal blood flow was seen to merge into the inferior vena cava through the left renal vein without hepatic processing, and the pathology of liver biopsy showed hypoplastic, dilated or absent portal vein branches, both of which supported the diagnosis of Abernethy malformation type II. This represents the initial documented instance of Abernethy malformation accompanied by a CFTR gene mutation in the existing body of literature.
CONCLUSION Coexisting Abernethy malformation and CF are rare. Detailed medical history information, abdominal enhanced CT, venography and genetic testing contribute to diagnosis as well as differential diagnosis.
Collapse
Affiliation(s)
- Li-Jie Zhang
- Xuzhou Medical University, Xuzhou 221000, Jiangsu Province, China
| | - Xing-Yu Liu
- Department of Pediatric Surgery, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Teng-Fei Chen
- Department of General Surgery, Nanjing Drum Tower Hospital Group Suqian Hospital, Suqian 223800, Jiangsu Province, China
| | - Zhong-Ya Xu
- Department of Pediatric Surgery, Children's Hospital of Nanjing Medical University, Nanjing 210000, China
| | - Han-Jun Yin
- Department of Pediatrics, Nanjing Drum Tower Hospital Group Suqian Hospital, Suqian 223800, Jiangsu Province, China
| |
Collapse
|
|
6
|
Zhang D, Wu X, Yang Z, Xu C, Wang X, Sun L, Cai J, Wang J. Successful Liver Transplantation From a Deceased Donor With Congenital Extrahepatic Portosystemic Shunt: A Case Report. Transplant Proc 2023; 55:1943-1945. [PMID: 37612151 DOI: 10.1016/j.transproceed.2023.07.014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2023] [Accepted: 07/31/2023] [Indexed: 08/25/2023]
Abstract
Congenital extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. We present a case of congenital extrahepatic portosystemic shunt occurring in a 42-year-old man who died of cerebral hemorrhage and donated his liver. His portal vein angiography revealed that the main portal vein communicated with the left renal vein, suggesting a portosystemic shunt. A liver biopsy showed that the liver tissue structure was normal. His liver was not involved, and the transplantation was carried out smoothly. The recipient recovered smoothly, and the function of the transplanted liver was good.
Collapse
Affiliation(s)
- Di Zhang
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Xiaodong Wu
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Zizhen Yang
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Chuanshen Xu
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Xin Wang
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Lingling Sun
- Pathology Department, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Jinzhen Cai
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China
| | - Jianhong Wang
- Organ Transplantation Center, the Affiliated Hospital of Qingdao University, Qingdao Shandong, China.
| |
Collapse
|
|
7
|
Yoshizawa K, Yonezawa N, Takei T, Sugiki K. Adult‐onset noncirrhotic hyperammonemic encephalopathy probably caused by splenorenal shunts. Clin Case Rep 2022; 10:e05774. [PMID: 35498349 PMCID: PMC9036195 DOI: 10.1002/ccr3.5774] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2021] [Revised: 03/17/2022] [Accepted: 04/04/2022] [Indexed: 11/08/2022] Open
Affiliation(s)
- Kazuhiro Yoshizawa
- Department of Emergency and Critical Care Medicine Yokohama City Minato Red Cross Hospital Yokohama Japan
| | - Naoki Yonezawa
- Department of Emergency and Critical Care Medicine Yokohama City Minato Red Cross Hospital Yokohama Japan
| | - Tetsuhiro Takei
- Department of Emergency and Critical Care Medicine Yokohama City Minato Red Cross Hospital Yokohama Japan
| | - Kei Sugiki
- Department of Emergency and Critical Care Medicine Yokohama City Minato Red Cross Hospital Yokohama Japan
| |
Collapse
|
|
8
|
De Benedictis A, Pietrobattista A, Talamanca LF, Monti L, Paolantonio G, Natali GL, Bua A, Savioli A, Alessandra Marasi E, Randi F, Carai A, Spada M, Marras CE. “De novo” brain arteriovenous malformation in a child with congenital porto-systemic shunt and multisystemic angiomas. Clin Neurol Neurosurg 2022; 217:107236. [DOI: 10.1016/j.clineuro.2022.107236] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2022] [Revised: 03/28/2022] [Accepted: 03/29/2022] [Indexed: 11/24/2022]
|
|
9
|
Grant CN, Rhee D, Tracy ET, Aldrink JH, Baertschiger RM, Lautz TB, Glick RD, Rodeberg DA, Ehrlich PF, Christison-Lagay E. Pediatric solid tumors and associated cancer predisposition syndromes: Workup, management, and surveillance. A summary from the APSA Cancer Committee. J Pediatr Surg 2022; 57:430-442. [PMID: 34503817 DOI: 10.1016/j.jpedsurg.2021.08.008] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2021] [Revised: 08/11/2021] [Accepted: 08/19/2021] [Indexed: 02/06/2023]
Abstract
BACKGROUND/PURPOSE Cancer predisposition syndromes (CPS) are a heterogeneous group of inherited disorders that greatly increase the risk of developing malignancies. CPS are particularly relevant to pediatric surgeons since nearly 10% of cancer diagnoses are due to inherited genetic traits, and CPS often contribute to cancer development during childhood. MATERIALS/METHODS The English language literature was searched for manuscripts, practice guidelines, and society statements on "cancer predisposition syndromes in children". Following review of these manuscripts and cross-referencing of their bibliographies, tables were created to summarize findings of the most common CPS associated with surgically treated pediatric solid malignancies. RESULTS Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors, such as WT. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms' tumor, hepatoblastoma, and medullary thyroid cancer. Frequently associated non-tumor manifestations of these CPS are also included as a guide to increase surgeon awareness. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available. CONCLUSION Pediatric surgeons play an important role as surgical oncologists and are often the first point of contact for children with solid tumors. In their role of delivering a diagnosis and developing a follow-up and treatment plan as part of a multidisciplinary team, familiarity with common CPS will ensure evidence-based practices are followed, including important principles such as organ preservation and intensified surveillance plans. This review defines and summarizes the CPS associated with common childhood solid tumors encountered by the pediatric surgeon, as well as common non-cancerous disease stigmata that may help guide diagnosis. TYPE OF STUDY Summary paper. LEVEL OF EVIDENCE 5.
Collapse
Affiliation(s)
- Christa N Grant
- Division of Pediatric Surgery, Penn State Children's Hospital, Milton S. Hershey Medical Center, Hershey, PA, United States.
| | - Daniel Rhee
- Division of Pediatric Surgery, Department of Surgery, Johns Hopkins School of Medicine, Baltimore, MD, United States
| | - Elisabeth T Tracy
- Division of Pediatric Surgery, Department of Surgery, Duke University Medical Center, Durham, NC, United States
| | - Jennifer H Aldrink
- Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Reto M Baertschiger
- Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
| | - Timothy B Lautz
- Division of Pediatric Surgery, Ann & Robert H Lurie Children's Hospital of Chicago, Northwestern University, Chicago, IL, United States
| | - Richard D Glick
- Division of Pediatric Surgery, Department of Surgery, Zucker School of Medicine at Hofstra/Northwell, Cohen Children's Medical Center, New Hyde Park, NY, United States
| | - David A Rodeberg
- Division of Pediatric Surgery, East Carolina Medical Center, Greenville, NC, United States
| | - Peter F Ehrlich
- Division of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan, United States
| | - Emily Christison-Lagay
- Division of Pediatric Surgery, Yale-New Haven Children's Hospital, Yale School of Medicine, New Haven, CT, United States
| |
Collapse
|
|
10
|
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]
|
|
11
|
Del Valle K, DuBrock HM. Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease. Compr Physiol 2021; 11:3281-3302. [PMID: 34636408 DOI: 10.1002/cphy.c210009] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Pulmonary vascular disease is a frequent complication of chronic liver disease and portal hypertension, affecting up to 30% of patients. There are two distinct pulmonary vascular complications of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS affects 25% of patients with chronic liver disease and is characterized by intrapulmonary vasodilatation and abnormal arterial oxygenation. HPS negatively impacts quality of life and is associated with a 2-fold increased risk of death compared to controls with liver disease without HPS. Angiogenesis, endothelin-1 mediated endothelial dysfunction, monocyte influx, and alveolar type 2 cell dysfunction seem to play important roles in disease pathogenesis but there are currently no effective medical therapies. Fortunately, HPS resolves following liver transplant (LT) with improvements in hypoxemia. POPH is a subtype of pulmonary arterial hypertension (PAH) characterized by an elevated mean pulmonary arterial pressure and pulmonary vascular resistance in the setting of normal left-sided filling pressures. POPH affects 5% to 6% of patients with chronic liver disease. Although the pathogenesis has not been fully elucidated, endothelial dysfunction, inflammation, and estrogen signaling have been identified as key pathways involved in disease pathogenesis. POPH is typically treated with PAH targeted therapy and may also improve with liver transplantation in selected patients. This article highlights what is currently known regarding the diagnosis, management, pathobiology, and outcomes of HPS and POPH. Ongoing research is needed to improve understanding of the pathophysiology and outcomes of these distinct and often misunderstood pulmonary vascular complications of liver disease. © 2021 American Physiological Society. Compr Physiol 11:1-22, 2021.
Collapse
|