1
|
Liu Z, Shen T, Xia K, He J, Rui T, Chen W. Classification of variant portal vein anatomy based on three-dimensional CT: surgical implications. Surg Radiol Anat 2024; 46:1177-1184. [PMID: 38963433 PMCID: PMC11246292 DOI: 10.1007/s00276-024-03427-5] [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] [Received: 04/17/2024] [Accepted: 06/19/2024] [Indexed: 07/05/2024]
Abstract
PURPOSES The purpose of this study was to develop a new and more comprehensive classification system for portal vein (PV) variations using three-dimensional visualization and evaluation (3DVE) and to discuss the prevalence rates and clinical implications of the variants. METHODS The anatomies of PVs were tracked and analyzed by using three-dimensional visualization of CT images acquired between 2013 and 2022. Scans from 200 adults were evaluated and a total of 178 patients (N = 178) were included in the study. The new classification system, named BLB classification, was developed based on the level of the absent PV branch in each variant anatomy. RESULTS Using the BLB classification system, PVs were divided into thirteen subtypes. Only 82.6-84.8% of the portal veins of the 178 patients were depicted in Atri's, Cheng's or Covey's classification, compared with 100% identified by the BLB classification. The BLB classification was validated against external data sets from previous studies, with 97.0-98.9% of patients classified by the BLB system. CONCLUSION Variant PV anatomies are more commonly seen based on 3DVE than in previous reports. The BLB classification covers almost all portal vein variants and may be used for planning liver surgery.
Collapse
Affiliation(s)
- Zheyu Liu
- Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, P.R. China
| | - Tianni Shen
- Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, P.R. China
| | - Kexin Xia
- Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, P.R. China
| | - Junye He
- Shanghai Jiao Tong University, Shanghai, 200240, P.R. China
| | - Tianhao Rui
- Shanghai Jiao Tong University, Shanghai, 200240, P.R. China
| | - Wei Chen
- Department of Biliary-Pancreatic Surgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, 160 Pujian Road, Shanghai, 200127, P.R. China.
| |
Collapse
|
2
|
Layton BM, Lapsia SK. The Portal Vein: A Comprehensive Review. Radiographics 2023; 43:e230058. [PMID: 37856316 DOI: 10.1148/rg.230058] [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: 10/21/2023]
Abstract
Radiologists are familiar with the appearances of a normal portal vein; variations in its anatomy are commonplace and require careful consideration due to the implications for surgery. These alterations in portal vein anatomy have characteristic appearances that are clearly depicted on CT, MR, and US images. Similarly, there are numerous congenital and acquired disorders of the portal vein that are deleterious to its function and can be diagnosed by using imaging alone. Some of these conditions have subtle imaging features, and some are conspicuous at imaging but poorly understood or underrecognized. The authors examine imaging appearances of the portal vein, first by outlining the classic and variant anatomy and then by describing each of the disorders that impact portal vein function. The imaging appearances of portal vein abnormalities discussed in this review include (a) occlusion from and differentiation between bland thrombus and tumor in vein and the changes associated with resultant hepatic artery buffer response changes, cavernous transformation of the portal vein, and portal biliopathy; (b) ascending thrombophlebitis of the portal vein (pylephlebitis); (c) portal hypertension and its causes and sequelae; (d) the newly described disease entity portosinusoidal vascular disorder; and (e) intra- and extrahepatic shunts of the portal system, both congenital and acquired (including Abernethy malformations), and the associated risks. Current understanding of the pathophysiologic processes of each of these disorders is considered to aid the approach to reporting. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.
Collapse
Affiliation(s)
- Benjamin M Layton
- From the Department of Radiology, East Lancashire Hospitals Trust, Royal Blackburn Hospital, Haslingden Rd, Blackburn, BB2 3HH, England (B.M.L., S.K.L.)
| | - Snehal K Lapsia
- From the Department of Radiology, East Lancashire Hospitals Trust, Royal Blackburn Hospital, Haslingden Rd, Blackburn, BB2 3HH, England (B.M.L., S.K.L.)
| |
Collapse
|
3
|
Nohomovich B, Nguyen MH, Fakhoury J, Cameron RC, Gomes T. Down Syndrome Patients with Congenital Portosystemic Shunts: A Case Report and Review. Case Rep Gastroenterol 2023; 17:367-375. [PMID: 38111805 PMCID: PMC10727516 DOI: 10.1159/000535477] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2023] [Accepted: 11/22/2023] [Indexed: 12/20/2023] Open
Abstract
Introduction Down syndrome is due to trisomy 21 and is characterized by intellectual disability, dysmorphic facial features, congenital malformations, and gastrointestinal abnormalities. There is an increased appreciation of congenital portosystemic shunts in Down syndrome patients. Congenital portosystemic shunts have been associated with many defects in body systems, including cardiac, metabolic, and neurological. Case Presentation Herein, we describe a portosystemic shunt in a Down syndrome patient that resulted in hyperammonemia with altered mental status and choreiform movements. Computed tomography angiography of the abdomen and pelvis identified a connection between the right portal vein and inferior vena cava. An 18 mm Amplatzer PFO closure device was placed within the congenital shunt, significantly improving symptoms. The patient has no sequelae from the related shunt or the device at the 2-year follow-up. We extensively reviewed the literature and identified cases of portosystemic shunts in Down syndrome patients. Shunts can either be extrahepatic or intrahepatic and are classified by vasculature connections. Conclusion From our literature review and case presentation, we identify other conditions in patients, including cardiac and gastrointestinal defects. We then review the available treatment options, whether observation or surgical, depending on the patient's clinical picture.
Collapse
Affiliation(s)
- Brian Nohomovich
- Department of Internal Medicine, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA
| | - Minh H.N. Nguyen
- Department of Pediatrics, University of South Florida, St. Petersburg, FL, USA
| | - Joseph Fakhoury
- Pediatric Hospital Medicine, Bronson Children’s Hospital, Kalamazoo, MI, USA
| | - Russell C. Cameron
- Pediatric Gastroenterology, Bronson Children’s Hospital, Kalamazoo, MI, USA
| | - Theotonius Gomes
- Department of Internal Medicine, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA
- Department of Pediatrics and Adolescent Medicine, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA
| |
Collapse
|
4
|
kayedi M, Kian B, Teimouri A. A rare Abernethy Ib malformation was initially misdiagnosed as chronic portal vein thrombosis in a 27-year-old female. Radiol Case Rep 2022; 17:3551-3555. [PMID: 35923331 PMCID: PMC9340080 DOI: 10.1016/j.radcr.2022.06.093] [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: 06/22/2022] [Accepted: 06/26/2022] [Indexed: 10/26/2022] Open
|
5
|
Kruszyna T, Rogala J, Jędrychowski T, Ignacak E, Bętkowska-Prokop A, Jaśkowski P, Richter P. Kidney Transplantation in Abernethy Malformation: A Case Report. Transplant Proc 2022; 54:1155-1157. [DOI: 10.1016/j.transproceed.2022.02.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2022] [Accepted: 02/18/2022] [Indexed: 02/07/2023]
|
6
|
Dubovan P, Tomáš M, Pavlendová J, Dolník J, Aziri R, Pinďák D. Superior mesenteric vein absence with intestinal malrotation: a case report. BMC Surg 2022; 22:39. [PMID: 35114982 PMCID: PMC8812031 DOI: 10.1186/s12893-022-01490-6] [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: 01/13/2021] [Accepted: 01/20/2022] [Indexed: 11/20/2022] Open
Abstract
Background Congenital abnormalities are not very common and are even rarer when two or more are combined. Congenital malformation of the superior mesenteric vein may not affect normal development, or it may lead to moderate or even severe symptoms. In combination with intestinal malrotation, however, it may lead to the need for surgical intervention in the early years of life. Case presentation We present the case of a 22-year-old patient who had been diagnosed with iron deficiency anaemia at the age of two months. As a result of the absence of the proximal section of the superior mesenteric vein, the patient has always needed iron supplements and an occasional erythrocyte transfusion. This has resulted from the formation of collaterals throughout the small bowel, causing chronic blood loss with its clinical manifestation. Although, there are some congenital abnormalities of the superior mesenteric vein, the absence of the superior mesenteric vein is rare, and in this case the clinical course was quite severe. Therefore, we planned bypass surgery for this patient to reduce the duodenal collaterals and resolve the persistent anaemia caused by chronic blood loss from the duodenum. We successfully performed the surgery consisting of the formation of anastomosis between the large collateral vein from the distal end of the superior mesenteric vein and the anterior inferior pancreaticoduodenal vein. Conclusion The purpose of this case report is to describe the rare anatomical malformation of the superior mesenteric vein accompanied by intestinal malrotation, with its potential clinical implications regarding symptoms, clinical presentation, and the impact on potential surgery planning.
Collapse
Affiliation(s)
- Peter Dubovan
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic.,Department of Surgical Oncology, Faculty of Medicine, Slovak Medical University, Klenova 1, 833 10, Bratislava, Slovak Republic
| | - Miroslav Tomáš
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic.
| | - Jana Pavlendová
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic
| | - Jozef Dolník
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic
| | - Ramadan Aziri
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic.,Department of Surgical Oncology, Faculty of Medicine, Slovak Medical University, Klenova 1, 833 10, Bratislava, Slovak Republic
| | - Daniel Pinďák
- Department of Surgical Oncology, National Cancer Institute Bratislava, Klenova 1, 833 10, Bratislava, Slovak Republic.,Department of Surgical Oncology, Faculty of Medicine, Slovak Medical University, Klenova 1, 833 10, Bratislava, Slovak Republic
| |
Collapse
|
7
|
Katsourakis A, Chytas D, Filo E, Chatzis I, Chouridis P, Komsis G, Noussios G. Incidence of Extrahepatic Portal Vein Anatomic Variations and Their Clinical Implications in Daily Practice. J Clin Med Res 2021; 13:460-465. [PMID: 34691319 PMCID: PMC8510651 DOI: 10.14740/jocmr4581] [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: 08/20/2021] [Accepted: 09/02/2021] [Indexed: 12/04/2022] Open
Abstract
Anatomical variations of the portal vein are relatively common and can affect the outcomes of hepatic resections, transplantations and interventional radiological procedures. The aim of this study was to review the literature regarding extrahepatic portal vein anomalies. Two main databases were searched for suitable articles, and results concerning more than 3,700 patients were included in the analysis. The most common anatomical variations of the portal vein were trifurcation and having a right posterior portal vein as the first branch of the main portal vein; these anomalies were found in 11.7% and 10.8% of cases, respectively.
Collapse
Affiliation(s)
| | - Dimitrios Chytas
- Department of Physiotherapy, University of Peloponnese, Sparta, Greece
| | - Eva Filo
- Department of General Surgery, Agios Dimitrios General Hospital, Thessaloniki, Greece
| | - Iosif Chatzis
- Department of General Surgery, Agios Dimitrios General Hospital, Thessaloniki, Greece
| | | | - Georgios Komsis
- Department of Physical Education and Sports Sciences at Serres, Aristotle University of Thessaloniki, Thessaloniki, Greece
| | - George Noussios
- Department of Physical Education and Sports Sciences at Serres, Aristotle University of Thessaloniki, Thessaloniki, Greece
| |
Collapse
|
8
|
Bhattacharya D, Sasikumar D, Gopalakrishnan A, Anoop A. Ventricular septal defect with pulmonary arterial hypertension in an infant: Is there something more than what meets the eye? Ann Pediatr Cardiol 2021; 14:554-556. [PMID: 35527760 PMCID: PMC9075548 DOI: 10.4103/apc.apc_226_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2020] [Revised: 02/26/2021] [Accepted: 05/10/2021] [Indexed: 12/02/2022] Open
Abstract
An 11-month-old girl was detected to have muscular ventricular septal defect with hyperkinetic pulmonary hypertension (PH) was urgently operated upon. On follow-up, her PH worsened, resulting in right ventricular dysfunction and was later detected to have absent portal vein.
Collapse
Affiliation(s)
- Deepanjan Bhattacharya
- Department of Cardiology, Sree Chitra Tirunal Insitute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
| | - Deepa Sasikumar
- Department of Cardiology, Sree Chitra Tirunal Insitute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
| | - Arun Gopalakrishnan
- Department of Cardiology, Sree Chitra Tirunal Insitute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
| | - A Anoop
- Department of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Insitute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
| |
Collapse
|
9
|
Ghuman SS, Gupta S, Buxi TBS, Rawat KS, Yadav A, Mehta N, Sud S. The Abernethy malformation-myriad imaging manifestations of a single entity. Indian J Radiol Imaging 2021; 26:364-372. [PMID: 27857464 PMCID: PMC5036336 DOI: 10.4103/0971-3026.190420] [Citation(s) in RCA: 30] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.
Collapse
Affiliation(s)
- Samarjit S Ghuman
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| | - Saumya Gupta
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| | - T B S Buxi
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| | - Kishan S Rawat
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| | - Anurag Yadav
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| | - Naimish Mehta
- Department of Surgical Gastroenterology, Sir Gangaram Hospital, New Delhi, India
| | - Seema Sud
- Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
| |
Collapse
|
10
|
Marra P, Dulcetta L, Pellegrinelli C, D'Antiga L, Sironi S. Percutaneous transhepatic treatment of a unique portal vein malformation with portal hypertension in a pediatric patient. CVIR Endovasc 2021; 4:47. [PMID: 34097180 PMCID: PMC8184909 DOI: 10.1186/s42155-021-00239-1] [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: 03/16/2021] [Accepted: 05/28/2021] [Indexed: 11/11/2022] Open
Abstract
Background Anomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies. Case presentation This case report describes the diagnosis and interventional management of a singular abnormality in the portal venous system of an 8-year-old female that led to severe portal hypertension and acute variceal bleeding. Peculiar imaging findings were not pathognomonic for any of the known congenital and acquired portal vein anomalies: absence of a normal extrahepatic portal vein; splenic and mesenteric veins merging into a dilated left gastric vein; presence of an aberrant mesenteric venous collateral with a stenotic connection with the intrahepatic right portal branch; and absence of porto-systemic shunt. The case was successfully managed with percutaneous transhepatic portography and angioplasty. Conclusions Prompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding.
Collapse
Affiliation(s)
- Paolo Marra
- Department of Radiology - Papa Giovanni XXIII Hospital, University of Milano Bicocca, Piazza OMS 1, 24127, Bergamo, Italy.
| | - Ludovico Dulcetta
- Department of Radiology - Papa Giovanni XXIII Hospital, University of Milano Bicocca, Piazza OMS 1, 24127, Bergamo, Italy
| | - Claudia Pellegrinelli
- Paediatric Hepatology, Gastroenterology, and Transplantation - Papa Giovanni XXIII Hospital, Bergamo, Italy
| | - Lorenzo D'Antiga
- Paediatric Hepatology, Gastroenterology, and Transplantation - Papa Giovanni XXIII Hospital, Bergamo, Italy
| | - Sandro Sironi
- Department of Radiology - Papa Giovanni XXIII Hospital, University of Milano Bicocca, Piazza OMS 1, 24127, Bergamo, Italy
| |
Collapse
|
11
|
Nandan R, Jana M, Yadav DK, Goel P. Cystic biliary atresia with congenital absence of portal vein. BMJ Case Rep 2021; 14:14/1/e239205. [PMID: 33462044 PMCID: PMC7813356 DOI: 10.1136/bcr-2020-239205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023] Open
Abstract
A 126-day female child presented with jaundice since day 7 of life. She was icteric and had hepatosplenomegaly. Radiological investigations revealed three extrahepatic and multiple intrahepatic biliary cysts, absent gall bladder and portal vein. These findings were confirmed on laparotomy. To the best of our knowledge, this is the first report of cystic biliary atresia associated with congenital absence of portal vein.
Collapse
Affiliation(s)
- Ruchira Nandan
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, Delhi, India
| | - Manisha Jana
- Radiodiagnosis, All India Institute of Medical Sciences (AIIMS), New Delhi, Delhi, India
| | - Devendra Kumar Yadav
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, Delhi, India
| | - Prabudh Goel
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, Delhi, India
| |
Collapse
|
12
|
Evaluation of portal vein variations in multidetector CT. JOURNAL OF SURGERY AND MEDICINE 2020. [DOI: 10.28982/josam.717046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
|
13
|
Peček J, Fister P, Homan M. Abernethy syndrome in Slovenian children: Five case reports and review of literature. World J Gastroenterol 2020; 26:5731-5744. [PMID: 33088165 PMCID: PMC7545390 DOI: 10.3748/wjg.v26.i37.5731] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/30/2020] [Revised: 08/11/2020] [Accepted: 09/17/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Abernethy syndrome is a congenital vascular anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt. Although the number of recognized and reported cases is gradually increasing, Abernethy syndrome is still a rare disease entity, with an estimated prevalence between 1 per 30000 to 1 per 50000 cases. With this case series, we aimed to contribute to the growing knowledge of potential clinical presentations, course and complications of congenital portosystemic shunts (CPSS) in children.
CASE SUMMARY Five children are presented in this case series: One female and four males, two with an intrahepatic CPSS and three with an extrahepatic CPSS. The first patient, who was diagnosed with an intrahepatic CPSS, presented with gastrointestinal bleeding, abdominal pain and hyperammonaemia at six years of age. He underwent a percutaneous embolization of his shunt and has remained asymptomatic ever since. The second patient presented with direct hyperbilirubinemia in the neonatal period and his intrahepatic CPSS later spontaneously regressed. The third patient had pulmonary hypertension and hyperammonaemia due to complete portal vein agenesis and underwent liver transplantation at five years of age. The fourth patient was diagnosed immediately after birth, when evaluated due to another congenital vascular anomaly, and the last patient presented as a teenager with recurrent bone fractures associated with severe osteoporosis. In addition, the last two patients are characterised by benign liver nodules; however, they are clinically stable on symptomatic therapy.
CONCLUSION Abernethy syndrome is a rare anomaly with diverse clinical features, affecting almost all organ systems and presenting at any age.
Collapse
Affiliation(s)
- Jerneja Peček
- Division of Paediatrics, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
| | - Petja Fister
- Department of Neonatology, Division of Paediatrics, University Medical Centre Ljubljana; Faculty of medicine, University of Ljubljana, Ljubljana 1000, Slovenia
| | - Matjaž Homan
- Department of Gastroenterology, Hepatology and Nutrition, Division of Paediatrics, University Medical Centre Ljubljana; Faculty of Medicine, University of Ljubljana, Ljubljana 1000, Slovenia
| |
Collapse
|
14
|
Musa J, Madani K, Saliaj K, Cai J, Guy A, Saraci B, Kola E, Ceka E. Asymptomatic presentation of a congenital malformation of the portal vein with portosystemic shunt. Radiol Case Rep 2020; 15:2009-2014. [PMID: 32874401 PMCID: PMC7452029 DOI: 10.1016/j.radcr.2020.07.076] [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: 07/28/2020] [Accepted: 07/28/2020] [Indexed: 11/16/2022] Open
Abstract
Malformations of the portal venous system consist of congenital and acquired anomalies. Congenital portosystemic shunts represent rare vascular developmental anomalies that allow partial or complete diversion of blood flow from the portal venous system to the systemic venous system, bypassing the liver. Congenital portosystemic shunts may be associated with malformations or congenital absence of the portal vein, and it was first described by John Abernethy in 1793. Most cases are diagnosed in early childhood, but some congenital shunts may remain asymptomatic and are encountered incidentally because of the widespread use of computed tomography and magnetic resonance imaging. In this report, we discuss the case of a 40-year-old female who presented to the Emergency Department with right upper quadrant pain, nausea, and vomiting. Clinical presentation and abdominal computed tomography angiography were consistent with the diagnosis of calculous cholecystitis and congenital absence of portal vein with intrahepatic portosystemic shunts. We discuss the importance of radiology in diagnosing such incidental malformations, coupled with a review of the current literature on this topic.
Collapse
Affiliation(s)
- Juna Musa
- Department of Surgery, Mayo Clinic, Rochester, MN 55905, USA
| | - Kulsum Madani
- Deccan College of Medical Sciences, Telengana, India
| | | | - Jason Cai
- Department of Radiology, Mayo Clinic, Rochester, MN, USA
| | - Ali Guy
- Department of Physical Medicine and Rehabilitation, New York University School of Medicine Medical Center, USA
| | - Blerina Saraci
- Department of Radiology, Regional Hospital, Durres, Albania
| | - Erisa Kola
- Department of Pathology and Forensic Medicine, Tirana, Albania
| | - Elton Ceka
- Department of Radiology, Regional Hospital, Durres, Albania
| |
Collapse
|
15
|
Danko M, Malinowska A, Moszczyńska E, Pawłowska J, Szarras-Czapnik M, Walewska-Wolf M, Wątrobińska U, Szalecki M. Porto-systemic shunt - a rare cause of hyperandrogenism in children. Two case reports and review of literature. J Pediatr Endocrinol Metab 2020; 33:/j/jpem.ahead-of-print/jpem-2020-0123/jpem-2020-0123.xml. [PMID: 32651988 DOI: 10.1515/jpem-2020-0123] [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: 03/17/2020] [Accepted: 04/27/2020] [Indexed: 11/15/2022]
Abstract
Objectives The main cause of hyperandrogenism in children is congenital adrenal hyperplasia, adrenal and gonadal tumors, polycystic ovary syndrome (PCOs) and Cushing's disease. In the last 20 years several descriptions of girls with hyperandrogenism and venous porto-systemic shunts appeared in literature. Case presentation First case is an eleven and a half-year-old girl, was admitted to Department of Endocrinology because of symptoms of hyperandrogenism. Laboratory tests revealed high serum testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEAS). The ammonia concentration was also increased. In the abdominal angio-CT scans persistent umbilical vein which connected portal and femoral vein was found. The second case was a seven-year-old boy with symptoms of precocious puberty. Blood tests also revealed high concentration of testosterone, androstenedione, DHEAS and ammonia. Imaging studies showed persistent ductus venosus. Conclusion Although pathophysiological relation is not clear, porto-systemic shunts should be considered as a cause of hyperandrogenism of unknown origin in children.
Collapse
Affiliation(s)
- Mikołaj Danko
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
- Department of Paediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland
| | - Anna Malinowska
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
| | - Elżbieta Moszczyńska
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
| | - Joanna Pawłowska
- Department of Gastrology, Hepatology and Feeding Disorders, The Children's Memorial Health Institute, Warsaw, Poland
| | - Maria Szarras-Czapnik
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
| | - Małgorzata Walewska-Wolf
- Outpatient Paediatric Gynecology Clinic, The Children's Memorial Health Institute, Warsaw, Poland
| | - Urszula Wątrobińska
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
| | - Mieczysław Szalecki
- Department of Paediatric Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland
- Collegium Medicum, Jan Kochanowski University, Kielce, Poland
| |
Collapse
|
16
|
Li P, Hidaka M, Hamada T, Ikeda S, Ono S, Maruya Y, Kugiyama T, Hara T, Yoshimoto T, Adachi T, Tanaka T, Miyoshi T, Murakami S, Huang Y, Kanetaka K, Eguchi S. Living donor liver transplantation for congenital absence of portal vein in portal venous reconstruction with a great saphenous vein graft. Surg Case Rep 2020; 6:153. [PMID: 32601822 PMCID: PMC7324451 DOI: 10.1186/s40792-020-00916-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2020] [Accepted: 06/19/2020] [Indexed: 11/21/2022] Open
Abstract
Background Congenital absence of portal vein (CAPV) is a rare structural anomaly in which the portal vein (PV) blood that normally flow into the liver directly drains into the systemic venous system through other collateral circulation. Congenital portal vein shunts (CPSs) is classified into types I and II according to the absence or presence of the intrahepatic portal vein, respectively. The CPS type I is also known as CAPV. The liver transplantation may be the only treatment option for CAPV. The key point of liver transplantation for CAPV is the reconstruction of the PV. Case presentation A 29-year-old man was diagnosed with CAPV with splenomegaly and gastroesophageal varix when being treated for pancytopenia and liver dysfunction. A living donor liver transplantation was performed for him using the right lobe which had been donated by his mother. The PV was reconstructed using his own great saphenous vein (GSV) as a graft vein. The end of the GSV graft was anastomosed to the inferior mesenteric vein while the other end was anastomosed to the vein graft of the right hepatic vein from the explanted liver. Conclusion Using the patient’s own GSV for PV reconstruction during living donor transplantation in the patient with CAPV seems to be an effective method.
Collapse
Affiliation(s)
- Peilin Li
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Masaaki Hidaka
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Takashi Hamada
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Satoshi Ikeda
- Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Shinichiro Ono
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Yasuhiro Maruya
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Tota Kugiyama
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Takanobu Hara
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Tomoko Yoshimoto
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Tomohiko Adachi
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Takayuki Tanaka
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Takayuki Miyoshi
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Shunsuke Murakami
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Yu Huang
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Kengo Kanetaka
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan
| | - Susumu Eguchi
- Department of Surgery, Nagasaki University Graduate School of Biomedical Science, 1-7-1 Sakamoto, Nagasaki, 852-8102, Japan.
| |
Collapse
|
17
|
Tang H, Song P, Wang Z, Han B, Meng X, Pan Y, Meng X, Duan W. A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment. Intractable Rare Dis Res 2020; 9:64-70. [PMID: 32494552 PMCID: PMC7263991 DOI: 10.5582/irdr.2020.03005] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
Extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. The clinical importance and manifestations of this vascular abnormality range from asymptomatic cases to liver or metabolic dysfunctions of various degrees. Congenital extrahepatic portosystemic shunt, also termed as Abernethy malformation, is a very rare congenital vascular malformation in which splenomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial extrahepatic shunt. So far, limited cases of congenital extrahepatic portosystemic shunt have been reported. In this review, incidence, mechanisms, complications, diagnoses and treatments of congenital extrahepatic portosystemic shunt are described.
Collapse
Affiliation(s)
- Haowen Tang
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Peipei Song
- The Institute for Global Health Policy Research, Bureau of International Health Cooperation, National Center for Global Health and Medicine, Tokyo, Japan
| | - Zhiqiang Wang
- Department of Hepatobiliary Surgery, Qinghai Red Cross Hospital, Xining, China
| | - Bing Han
- Department of General Surgery, Shanxian Central Hospital, Heze, China
| | - Xiangfei Meng
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Yingwei Pan
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Xuan Meng
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
- Address correspondence to:Weidong Duan and Xuan Meng, Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Haidian, Beijing 100853, China. E-mail: (Duan WD), (Meng X)
| | - Weidong Duan
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
- Address correspondence to:Weidong Duan and Xuan Meng, Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Haidian, Beijing 100853, China. E-mail: (Duan WD), (Meng X)
| |
Collapse
|
18
|
Bessho H, Tanaka S, Ishihara A, Kato S, Toshiyama R, Hama N, Mori K, Mano M, Miyamoto A, Ishida H, Hirao M, Mita E. Hepatocellular carcinoma in an adult patient with congenital absence of the portal vein type II: A case report. JGH OPEN 2020; 4:766-768. [PMID: 32782969 PMCID: PMC7411543 DOI: 10.1002/jgh3.12312] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/03/2020] [Revised: 01/23/2020] [Accepted: 01/29/2020] [Indexed: 11/23/2022]
Abstract
Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins. CAPV is classified into two types based on the absence (type I) or presence (type II) of portal venous flow into the hepatic parenchyma and is associated with multiple other anomalies such as usually benign hepatic tumors. There have been only two case reports describing hepatocellular carcinoma (HCC) in patients with CAPV type II to date. We report the third such patient. A 50‐year‐old woman was referred to our hospital for management of a giant hepatic tumor. Contrast‐enhanced computed tomography (CECT) indicated a huge mass occupying the right lobe of the liver; the radiological diagnosis was HCC. CECT also demonstrated that the superior mesenteric vein (SMV) and the splenic vein (SpV) joined to form a shunt draining into the left renal vein and that a hypoplastic portal vein branched from the confluence of the SMV and SpV and drained into the liver, indicating that the CAPV was type II. Liver resection was successfully performed to treat the HCC, and the pathological diagnosis was well‐differentiated HCC. Seven months after the operation, a recurrent small HCC was detected and treated with radiofrequency ablation without complications. The patient has been carefully followed for 6 months to date without any evidence of further recurrence. Patients with CAPV are predisposed to developing HCC and require close surveillance.
Collapse
Affiliation(s)
- Hiroki Bessho
- Department of Gastroenterology and Hepatology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Satoshi Tanaka
- Department of Gastroenterology and Hepatology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Akio Ishihara
- Department of Gastroenterology and Hepatology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Shinya Kato
- Department of Surgery National Hospital Organization Osaka National Hospital Osaka Japan
| | - Reishi Toshiyama
- Department of Surgery National Hospital Organization Osaka National Hospital Osaka Japan
| | - Naoki Hama
- Department of Surgery National Hospital Organization Osaka National Hospital Osaka Japan
| | - Kiyoshi Mori
- Department of Pathology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Masayuki Mano
- Department of Pathology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Atsushi Miyamoto
- Department of Surgery National Hospital Organization Osaka National Hospital Osaka Japan
| | - Hisashi Ishida
- Department of Gastroenterology and Hepatology National Hospital Organization Osaka National Hospital Osaka Japan
| | - Motohiro Hirao
- Department of Surgery National Hospital Organization Osaka National Hospital Osaka Japan
| | - Eiji Mita
- Department of Gastroenterology and Hepatology National Hospital Organization Osaka National Hospital Osaka Japan
| |
Collapse
|
19
|
Guo C, Zhong YM, Wang Q, Hu LW, Gu XH, Shao H, Wu W, Cao JF, Qiu HS. Diagnostic accuracy of multi-slice computed tomography in children with Abernethy malformation. BMC Med Imaging 2019; 19:97. [PMID: 31847826 PMCID: PMC6918646 DOI: 10.1186/s12880-019-0396-3] [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: 07/03/2019] [Accepted: 11/28/2019] [Indexed: 12/23/2022] Open
Abstract
Background Abernethy malformation is a rare congenital abnormality. Imaging examination is an important method for the diagnosis. The purpose of this study was to demonstrate manifestations of multi-slice computed tomography (MSCT) in Abernethy malformation and its diagnostic accuracy. Methods Fourteen children with Abernethy malformation were admitted to our center in China between July 2011 and January 2018. All 14 patients (eight males and six females) received MSCT and digital subtraction angiography (DSA) while eight patients also received ultrasound. The patients’ age ranged from 1 to 14 (median age 8 years old). The clinical records of the patients were retrospectively reviewed. MSCT raw data were transferred to an Advantage Windows 4.2 or 4.6 workstation (General Electric Medical Systems, Waukesha, WI). We compared the findings of MSCT with DSA and surgical results in order to ascertain diagnostic accuracy. Results Three cases had type Ib Abernethy malformation and eleven cases had type II. Two cases of type II Abernethy malformation were misdiagnosed as type Ib using MSCT. Comparing the findings of MSCT with DSA and surgical results, the accuracy of MSCT was 85.7% (12/14), in which 100.0% (3/3) were type Ib and 81.8% (9/11) were type II. Clinical information included congenital heart disease, pulmonary hypertension, diffuse pulmonary arteriovenous fistula, abnormal liver function, hepatic nodules, elevated blood ammonia, and hepatic encephalopathy. Eleven cases were treated after diagnosis. One patient with Abernethy malformation type Ib (1/3) underwent liver transplantation. Seven patients with Abernethy malformation type II (7/11) were treated by shunt occlusion, received laparoscopy, or were treated with open surgical ligation. Another three patients (3/11) with Abernethy malformation type II were treated by interventional portocaval shunt occlusion under DSA. Conclusion MSCT attains excellent capability in diagnosing type II Abernethy malformation and further shows the location of the portocaval shunt. DSA can help when it is hard to determine diagnosis between Abernethy type Ib and II using MSCT.
Collapse
Affiliation(s)
- Chen Guo
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Yu-Min Zhong
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China.
| | - Qian Wang
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Li-Wei Hu
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Xiao-Hong Gu
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Hong Shao
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Wei Wu
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Jian-Feng Cao
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| | - Hai-Sheng Qiu
- Diagnostic Imaging Center, Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University School of Medicine, 1678 Dong Fang Road, Shanghai, 200127, People's Republic of China
| |
Collapse
|
20
|
Zhou M, Zhang J, Luo L, Wang B, Zheng R, Li L, Jing H, Zhang S. Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation. Ann Vasc Surg 2019; 65:285.e1-285.e5. [PMID: 31705994 DOI: 10.1016/j.avsg.2019.10.094] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2019] [Revised: 10/01/2019] [Accepted: 10/30/2019] [Indexed: 12/20/2022]
Abstract
Abernethy malformation is a very rare congenital vascular malformation consisting of diversion of portal blood away from liver, and it is commonly associated with multiple congenital anomalies. Here, we present a case of a male from China with nonspecific abdominal pain associated with an unusual pattern of type II Abernethy malformation, whose was diagnosed with a portosystemic shunt via a giant portal-inferior vena cava fistula (17.22 mm in diameter). The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of a type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.
Collapse
Affiliation(s)
- Mantian Zhou
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Juqiang Zhang
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Lei Luo
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Botian Wang
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Renjun Zheng
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Linpeng Li
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Hailing Jing
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China
| | - Shengzu Zhang
- Department of Interventional Vascular Surgery, The Affiliated Zhuzhou Hospital, Xiangya Medical College CSU, Zhuzhou City, Hunan Province, China.
| |
Collapse
|
21
|
Kemp SR. Sonography Aids in Diagnosis of Congenital Absence of Portal Vein in Patient With Marfan Syndrome: A Case Study. JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY 2019. [DOI: 10.1177/8756479319848747] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Skylar R. Kemp
- Department of Radiology Ultrasound, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
| |
Collapse
|
22
|
Yangın-Ergon E, Ermis N, Colak R, Polat B, Alkan-Ozdemir S, Yıldız M, Kulalı F, Omur-Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol 2019; 8:163-166. [PMID: 30828559 PMCID: PMC6395480 DOI: 10.5005/jp-journals-10018-1283] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2018] [Accepted: 11/20/2018] [Indexed: 12/30/2022] Open
Abstract
Introduction Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. Case report The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). Conclusion Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases.How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166.
Collapse
Affiliation(s)
- Ezgi Yangın-Ergon
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Nebahat Ermis
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Ruya Colak
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Burak Polat
- Department of Pediatric Radiology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Senem Alkan-Ozdemir
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Meral Yıldız
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Ferit Kulalı
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Cigden Omur-Ecevit
- Department of Pediatric Gastroenterology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| | - Orkan Ergun
- Department of Pediatric Surgery and Transplantation, Ege University Hospital, Izmir, Turkey
| | - Sebnem Calkavur
- Department of Neonatology, Dr. BehCet Uz Pediatric Diseases and Surgery Education Research Hospital, Izmir, Turkey
| |
Collapse
|
23
|
Madhusudhan KS, Vyas S, Sharma S, Srivastava DN, Gupta AK. Portal vein abnormalities: an imaging review. Clin Imaging 2018; 52:70-78. [DOI: 10.1016/j.clinimag.2018.07.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2018] [Revised: 06/01/2018] [Accepted: 07/04/2018] [Indexed: 12/20/2022]
|
24
|
Sanada Y, Mizuta K. Congenital absence of the portal vein: translated version. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2018; 25:359-369. [PMID: 30160060 DOI: 10.1002/jhbp.572] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward.
Collapse
Affiliation(s)
- Yukihiro Sanada
- Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan
| | - Koichi Mizuta
- Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan
| |
Collapse
|
25
|
Jha RC, Khera SS, Kalaria AD. Portal Vein Thrombosis: Imaging the Spectrum of Disease With an Emphasis on MRI Features. AJR Am J Roentgenol 2018; 211:14-24. [PMID: 29792748 DOI: 10.2214/ajr.18.19548] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/30/2023]
Affiliation(s)
- Reena C. Jha
- Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd, NW, Lombardi, G-184, Washington, DC 20007
| | | | | |
Collapse
|
26
|
Kroencke T, Murnauer M, Jordan FA, Blodow V, Ruhnke H, Schaller T, Märkl B. Radioembolization for Hepatocellular Carcinoma Arising in the Setting of a Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation). Cardiovasc Intervent Radiol 2018; 41:1285-1290. [PMID: 29675774 DOI: 10.1007/s00270-018-1965-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2017] [Accepted: 04/12/2018] [Indexed: 11/24/2022]
Abstract
In a 50-year-old male with an incidentally discovered hepatocellular carcinoma, a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy syndrome) was diagnosed by imaging. This study aims to discuss the variant anatomy relevant to this splanchnic vascular malformation, review its association with the development of liver neoplasms, and report the safety and efficacy of TARE followed by resection for a HCC arising in this rare condition.
Collapse
Affiliation(s)
- Thomas Kroencke
- Department of Diagnostic and Interventional Radiology and Neuroradiology, Klinikum Augsburg, Stenglinstraße 2, 86156, Augsburg, Germany.
| | - Marcus Murnauer
- Department of General, Visceral and Transplant Surgery, Klinikum Augsburg, Augsburg, Germany
| | | | - Vera Blodow
- Department of Nuclear Medicine, Klinikum Augsburg, Augsburg, Germany
| | - Hannes Ruhnke
- Department of Diagnostic and Interventional Radiology and Neuroradiology, Klinikum Augsburg, Stenglinstraße 2, 86156, Augsburg, Germany
| | - Tina Schaller
- Institute of Pathology, Klinikum Augsburg, Augsburg, Germany
| | - Bruno Märkl
- Institute of Pathology, Klinikum Augsburg, Augsburg, Germany
| |
Collapse
|
27
|
Christou N, Dib N, Chuffart E, Taibi A, Durand-Fontanier S, Valleix D, Mathonnet M. Stepwise management of hepatocellular carcinoma associated with Abernethy syndrome. Clin Case Rep 2018; 6:930-934. [PMID: 29744090 PMCID: PMC5930207 DOI: 10.1002/ccr3.1384] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2017] [Revised: 09/05/2017] [Accepted: 10/18/2017] [Indexed: 12/22/2022] Open
Abstract
Patients with congenital agenesis of the portal vein may develop hepatocellular tumors due to enhanced arterial blood flow. These tumors may be benign (FNH, adenomas) or malignant (hepatoblastoma, HCC). Liver resection can be proposed, and preoperative arterial embolization may decrease blood loss during surgery. Liver transplantation with PV reconstruction is also an option.
Collapse
Affiliation(s)
- Niki Christou
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Nabil Dib
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Etienne Chuffart
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Abdelkader Taibi
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Sylvaine Durand-Fontanier
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Denis Valleix
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Muriel Mathonnet
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| |
Collapse
|
28
|
Holloway A, Groot L, van der Schaaf K. Congenital absence of the portal vein in a cat. JFMS Open Rep 2018; 4:2055116917749079. [PMID: 29372068 PMCID: PMC5774732 DOI: 10.1177/2055116917749079] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
CASE SUMMARY A 9-month-old female neutered domestic shorthair cat presented with a history of episodic ptyalism, lethargy and abnormal behaviour. The clinical signs together with elevated pre- and post-prandial bile acid concentrations were consistent with hepatic encephalopathy (HE). In the absence of a portosystemic shunt (PSS) on abdominal ultrasound, medical management of HE was established with a protein-restricted diet and lactulose and the neurological signs resolved. Following an episode of acute vomiting and haemorrhagic diarrhoea at 19 months of age abdominal ultrasonography was repeated. The portal vein could not be demonstrated ultrasonographically; instead, portal vein tributaries were tortuous and communicated with the caudal vena cava (CdVC) at the level of the left kidney. CT angiography (CTA) confirmed the absence of the portal vein. CTA demonstrated the tortuous terminations of the portal tributaries, and several systemic veins, draining into the CdVC via a large-diameter paracaval vessel at the level of the left kidney. Gastrointestinal signs were stabilised and medical management for HE of a protein-restricted diet and lactulose was re-established. RELEVANCE AND NOVEL INFORMATION Congenital absence of the portal vein has not been described previously in the cat and should be considered in cats presenting with signs suggestive of a PSS and HE. The portal vein in the cat can be demonstrated using ultrasound, but complex congenital vascular malformations of the portal or systemic abdominal veins should be characterised using CTA and further distinguished from other vascular anomalies that may present with similar ultrasonographic features.
Collapse
Affiliation(s)
| | - Louise Groot
- Goddard Veterinary Hospital, Wanstead, London, UK
| | | |
Collapse
|
29
|
Martín-Llahí M, Albillos A, Bañares R, Berzigotti A, García-Criado MÁ, Genescà J, Hernández-Gea V, Llop-Herrera E, Masnou-Ridaura H, Mateo J, Navascués CA, Puente Á, Romero-Gutiérrez M, Simón-Talero M, Téllez L, Turon F, Villanueva C, Zarrabeitia R, García-Pagán JC. Enfermedades vasculares del hígado. Guías Clínicas de la Sociedad Catalana de Digestología y de la Asociación Española para el Estudio del Hígado. GASTROENTEROLOGIA Y HEPATOLOGIA 2017; 40:538-580. [PMID: 28610817 DOI: 10.1016/j.gastrohep.2017.03.011] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/13/2017] [Accepted: 03/29/2017] [Indexed: 12/11/2022]
|
30
|
Benedict M, Rodriguez-Davalos M, Emre S, Walther Z, Morotti R. Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) With Associated Hepatocellular Carcinoma: Case Report and Literature Review. Pediatr Dev Pathol 2017; 20:354-362. [PMID: 28727971 DOI: 10.1177/1093526616686458] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein, we report a case of Abernethy malformation, type Ib, in a 12-month-old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years with Abernethy malformation.
Collapse
Affiliation(s)
- Mark Benedict
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
| | | | - Sukru Emre
- 2 Department of Surgery, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Zenta Walther
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Raffaella Morotti
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
| |
Collapse
|
31
|
Gunasekaran SS, Gaba RC. Anatomic Variations of the Right Portal Vein: Prevalence, Imaging Features, and Implications for Successful Transjugular Intrahepatic Portosystemic Shunt Creation. J Clin Imaging Sci 2017; 7:14. [PMID: 28515965 PMCID: PMC5385700 DOI: 10.4103/jcis.jcis_10_17] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2017] [Accepted: 02/08/2017] [Indexed: 12/20/2022] Open
Abstract
Given the widespread use of transjugular intrahepatic portosystemic shunt (TIPS) creation for the treatment of portal hypertensive complications, a working knowledge of portal venous anatomy is critical for interventional radiologists. The right portal vein - which is most commonly accessed during TIPS - is subject to various anatomic variants that may potentially impact procedure success. This pictorial essay characterizes the anatomic patterns of the right portal vein branching in terms of type and frequency based on case series review. The work also explains the potential procedural implications of the right portal vein anatomic variations as they pertain to TIPS technical success.
Collapse
Affiliation(s)
- Senthil S Gunasekaran
- Department of Radiology, Division of Interventional Radiology, University of Illinois Hospital and Health Sciences System, Chicago, IL 60612, USA
| | - Ron C Gaba
- Department of Radiology, Division of Interventional Radiology, University of Illinois Hospital and Health Sciences System, Chicago, IL 60612, USA
| |
Collapse
|
32
|
Ghandour A, Partovi S, Karuppasamy K, Rajiah P. Congenital anomalies of the IVC-embryological perspective and clinical relevance. Cardiovasc Diagn Ther 2016; 6:482-492. [PMID: 28123970 DOI: 10.21037/cdt.2016.11.18] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
With the increased use of cross-sectional imaging, systemic venous anomalies are more frequently being recognized in asymptomatic patients. Accurate characterization of systemic venous anomalies plays a major role in the appropriate selection of a surgical approach or interventional procedure. In this article, we review common and uncommon inferior vena cava (IVC) anomalies. We describe the embryological basis and clinical implications of these anomalies, particularly from an interventional radiology perspective. We also discuss the complications and treatments of these anomalies.
Collapse
Affiliation(s)
- Abed Ghandour
- Imaging Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Sasan Partovi
- Department of Radiology, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA
| | | | - Prabhakar Rajiah
- Cardiothoracic Imaging, Radiology Department, UT Southwestern Medical Center, Dallas, Texas, USA
| |
Collapse
|
33
|
Shah A, Aziz A, Awwad A, Ramjas G, Higashi Y. Incidental radiological diagnosis of asymptomatic Abernethy malformations-two case reports. BJR Case Rep 2016; 3:20150496. [PMID: 30363319 PMCID: PMC6159285 DOI: 10.1259/bjrcr.20150496] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2015] [Accepted: 06/28/2016] [Indexed: 01/05/2023] Open
Abstract
The diagnosis of the rare congenital extrahepatic portosystemic shunts is of clinical significance because of the risk of hepatic encephalopathy; liver dysfunction; and associated cardiac, gastrointestinal, vascular, skeletal and genitourinary anomalies. This article describes two varying cases showing the same type of the extrahepatic congenital shunts (Type II). Both the patients were clinically asymptomatic. The first patient initially presented with unprovoked deep venous thrombosis and a staging CT scan was performed to identify any potential underlying malignancy. The second was a polytrauma patient in whom a congenital extrahepatic portosystemic shunt was identified on the CT scan performed to investigate the trauma-related injuries. The first case underwent hepatological investigations, including a fibroscan to rule out liver fibrosis, and was diagnosed as having a Type II congenital malformation, while the second case is under observation post recovery from his traumatic injuries and will be subsequently referred to the hepatology team in the future. Although uncommon, extrahepatic portosystemic shunts can cause significant morbidity and mortality, and all new cases diagnosed radiologically should be further investigated by referring them to a hepatologist.
Collapse
Affiliation(s)
- Ali Shah
- Trauma and Orthopaedics, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK
| | - Abdul Aziz
- Trauma and Orthopaedics, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK
| | | | - Greg Ramjas
- Interventional Radiology, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK
| | - Yutaro Higashi
- Radiology Department, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK
| |
Collapse
|
34
|
Congenital Extrahepatic Portosystemic Shunts: Spectrum of Findings on Ultrasound, Computed Tomography, and Magnetic Resonance Imaging. Radiol Res Pract 2016; 2015:181958. [PMID: 26858845 PMCID: PMC4691495 DOI: 10.1155/2015/181958] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2015] [Accepted: 11/15/2015] [Indexed: 11/23/2022] Open
Abstract
Congenital extrahepatic portosystemic shunt (CEPS) is a rare disorder characterised by partial or complete diversion of portomesenteric blood into systemic veins via congenital shunts. Type I is characterised by complete lack of intrahepatic portal venous blood flow due to an end to side fistula between main portal vein and the inferior vena cava. Type II on the other hand is characterised by partial preservation of portal blood supply to liver and side to side fistula between main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. The presentation of these patients is variable. Focal liver lesions, most commonly nodular regenerative hyperplasia, are an important clue to the underlying condition. This pictorial essay covers imaging characteristics in abdominopelvic region.
Collapse
|
35
|
Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 2015; 46:2418-21. [PMID: 25242797 DOI: 10.1016/j.transproceed.2014.04.012] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2014] [Accepted: 04/01/2014] [Indexed: 02/07/2023]
Abstract
Congenital absence of the portal vein (CAPV) is a rare congenital anomaly in which the superior mesenteric veins (SMV) and splenic veins converge and bypass the liver, effectively draining directly into the systemic venous circulation via the inferior vena cava (IVC), or alternatively the renal or iliac vein, creating a native portosystemic shunt. Portosystemic shunting results in clinical manifestations of hepatic encephalopathy as well as a predisposition to focal nodular hyperplasia and tumors, including adenomas, hepatoblastoma, and hepatocellular carcinoma (HCC), by the disruption of enterohepatic blood flow. Historically, CAPV has been thought to be a rare condition found mainly at autopsy, however, in recent years due to advances in radiological techniques, CAPV detection has increased. Herein we describe a patient with known CAPV who initially underwent hepatic resection for HCC. During surveillance, additional masses were discovered and were identified as recurrent HCC. Unfortunately, this patient was not a candidate for further resection or locoregional therapy. We demonstrate that transplantation is a challenging but technically viable option for treatment of HCC complicating adenomatosis-associated CAPV.
Collapse
|
36
|
Brasoveanu V, Ionescu MI, Grigorie R, Mihaila M, Bacalbasa N, Dumitru R, Herlea V, Iorgescu A, Tomescu D, Popescu I. Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review. AMERICAN JOURNAL OF CASE REPORTS 2015; 16:637-44. [PMID: 26386552 PMCID: PMC4581685 DOI: 10.12659/ajcr.895235] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
BACKGROUND Abernethy malformation (AM), or congenital absence of portal vein (CAPV), is a very rare disease which tends to be associated with the development of benign or malignant tumors, usually in children or young adults. CASE REPORT We report the case of a 21-year-old woman diagnosed with type Ib AM (portal vein draining directly into the inferior vena cava) and unresectable liver adenomatosis. The patient presented mild liver dysfunction and was largely asymptomatic. Living donor liver transplantation was performed using a left hemiliver graft from her mother. Postoperatively, the patient attained optimal liver function and at 9-month follow-up has returned to normal life. CONCLUSIONS We consider that living donor liver transplantation is the best therapeutic solution for AM associated with unresectable liver adenomatosis, especially because compared to receiving a whole liver graft, the waiting time on the liver transplantation list is much shorter.
Collapse
Affiliation(s)
- Vladislav Brasoveanu
- "Dan Setlacec" Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania
| | - Mihnea Ioan Ionescu
- "Dan Setlacec" Center for General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania
| | - Razvan Grigorie
- "Dan Setlacec" Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania
| | - Mariana Mihaila
- Department of Internal Medicine, Fundeni Clinical Institute, Bucharest, Romania
| | - Nicolae Bacalbasa
- "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania
| | - Radu Dumitru
- Department of Radiology and Imagistics, Fundeni Clinical Institute, Bucharest, Romania
| | - Vlad Herlea
- Department of Morphopathology, Fundeni Clinical Institute, Bucharest, Romania
| | - Andreea Iorgescu
- Department of Morphopathology, Fundeni Clinical Institute, Bucharest, Romania
| | - Dana Tomescu
- Department of Anesthesiology, Fundeni Clinical Institute, Bucharest, Romania
| | - Irinel Popescu
- "Dan Setlacec" Center for General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania
| |
Collapse
|
37
|
Sureka B, Patidar Y, Bansal K, Rajesh S, Agrawal N, Arora A. Portal vein variations in 1000 patients: surgical and radiological importance. Br J Radiol 2015; 88:20150326. [PMID: 26283261 PMCID: PMC4743455 DOI: 10.1259/bjr.20150326] [Citation(s) in RCA: 51] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2015] [Revised: 08/02/2015] [Accepted: 08/17/2015] [Indexed: 12/29/2022] Open
Abstract
OBJECTIVE The purpose of the study was to evaluate the spectrum and incidence of intrahepatic portal vein (PV) variations on triphasic abdomen multidetector CT (MDCT) and to discuss the surgical and radiological implications. METHODS A retrospective review of 1000 triphasic MDCT abdomen scans was performed in patients sent for various liver and other abdominal pathologies between January 2014 and August 2014. A total of 967 patients (N = 967) were included in the study. The variations in branching pattern of PV were classified according to classification used by Covey et al (Covey AM, Brody LA, Getrajdman GI, Sofocleous CT, Brown KT. Incidence, patterns, and clinical relevance of variant portal vein anatomy. AJR Am J Roentgenol 2004; 183: 1055-64) and Koç et al (Koç Z, Oğuzkurt L, Ulusan S. Portal vein variations: clinical implications and frequencies in routine abdominal CT. Diagn Interv Radiol 2007; 13: 75-80). RESULTS Normal anatomy (Type I) was seen in 773 patients (79.94%) out of 967 patients in our study. Trifurcation (Type II) anomaly was seen in 66 (6.83%) of cases. Right posterior vein as first branch of main PV (Type III) anomaly was seen in 48 (4.96%) of cases. Type IV anomaly and Type V anomaly was seen in 26 (2.69%) and 13 (1.34%) cases, respectively. 19 cases showed other types of variations. CONCLUSION Variations in the hepatic PV branching patterns are commonly seen that are similar to variations in hepatic artery and biliary anatomy. Knowledge of these variations is extremely important for transplant surgeons and intervention radiologists. ADVANCES IN KNOWLEDGE Awareness of the variations in PV branching patterns is essential for intervention radiologists and vascular surgeons and avoids major catastrophic events.
Collapse
Affiliation(s)
- Binit Sureka
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| | - Yashwant Patidar
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| | - Kalpana Bansal
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| | - S Rajesh
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| | - Nitesh Agrawal
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| | - Ankur Arora
- Department of Radiology/Interventional Radiology, Institute of Liver and Biliary Sciences, Vasant Kunj, New Delhi
| |
Collapse
|
38
|
Watson CJE, Harper SJF. Anatomical variation and its management in transplantation. Am J Transplant 2015; 15:1459-71. [PMID: 25981150 DOI: 10.1111/ajt.13310] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2015] [Accepted: 03/05/2015] [Indexed: 01/25/2023]
Abstract
Variant anatomy may be challenging at retrieval, with failure to identify variance being associated with organ damage, particularly vascular damage. On implantation, some variants demand nonstandard techniques of reconstruction or implantation. This review covers the common and less common anatomical variants of the liver, kidney and pancreas, and gives guidance as to how they may be managed during organ retrieval and implantation.
Collapse
Affiliation(s)
- C J E Watson
- Department of Surgery, University of Cambridge, Cambridge, UK.,Cambridge NIHR Biomedical Research Centre, Addenbrooke's Hospital, Cambridge, UK
| | - S J F Harper
- Department of Surgery, University of Cambridge, Cambridge, UK.,Cambridge NIHR Biomedical Research Centre, Addenbrooke's Hospital, Cambridge, UK
| |
Collapse
|
39
|
Kanazawa H, Nosaka S, Miyazaki O, Sakamoto S, Fukuda A, Shigeta T, Nakazawa A, Kasahara M. The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 2015; 50:688-95. [PMID: 25840084 DOI: 10.1016/j.jpedsurg.2015.01.009] [Citation(s) in RCA: 62] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/14/2014] [Revised: 12/25/2014] [Accepted: 01/13/2015] [Indexed: 10/24/2022]
Abstract
BACKGROUND/PURPOSE Liver transplantation was previously indicated as a curative operation for congenital absence of portal vein. Recent advances in radiological interventional techniques can precisely visualize the architecture of the intrahepatic portal system (IHPS). Therefore, the therapeutic approach for congenital portosystemic shunt (CPS) needs to be reevaluated from a viewpoint of radiological appearances. The aim of this study was to propose the IHPS classification which could explain the pathophysiological characteristics and play a complementary role of a therapeutic approach and management for CPS. METHODS Nineteen patients with CPS were retrospectively reviewed. The median age at diagnosis was 6.8 years old. Eighteen of these patients underwent angiography with a shunt occlusion test and were classified based of the severity of the hypoplasia of IHPS. RESULTS The eighteen cases who could undergo the shunt occlusion test were classified into mild (n=7), moderate (n=6) and severe types (n=5) according to the IHPS classification. The IHPS classification correlated with the portal venous pressure under shunt occlusion, the histopathological findings, postoperative portal venous flow and liver regeneration. Shunt closure resulted in dramatic improvement in the laboratory data and subclinical encephalopathy. Two patients with the severe type suffered from sepsis associated with portal hypertension after treatment, and from the portal flow steal phenomenon because of the development of unexpected collateral vessels. The patients with the severe type had a high risk of postoperative complications after shunt closure in one step, even if the PVP was relatively low during the shunt occlusion test. CONCLUSION The IHPS could be visualized by the shunt occlusion test. The IHPS classification reflected the clinicopathological features of CPS, and was useful to determine the therapeutic approach and management for CPS.
Collapse
Affiliation(s)
- Hiroyuki Kanazawa
- Transplantation Center, National Center for Child Health & Development, Tokyo, Japan.
| | - Shunsuke Nosaka
- Department of Radiology, National Center for Child Health & Development, Tokyo, Japan
| | - Osamu Miyazaki
- Department of Radiology, National Center for Child Health & Development, Tokyo, Japan
| | - Seisuke Sakamoto
- Transplantation Center, National Center for Child Health & Development, Tokyo, Japan
| | - Akinari Fukuda
- Transplantation Center, National Center for Child Health & Development, Tokyo, Japan
| | - Takanobu Shigeta
- Transplantation Center, National Center for Child Health & Development, Tokyo, Japan
| | - Atsuko Nakazawa
- Department of Clinical Pathology, National Center for Child Health & Development, Tokyo, Japan
| | - Mureo Kasahara
- Transplantation Center, National Center for Child Health & Development, Tokyo, Japan
| |
Collapse
|
40
|
Congenital portosystemic shunt: our experience. Case Rep Pediatr 2015; 2015:691618. [PMID: 25709849 PMCID: PMC4332460 DOI: 10.1155/2015/691618] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2014] [Revised: 01/09/2015] [Accepted: 01/12/2015] [Indexed: 01/15/2023] Open
Abstract
Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.
Collapse
|
41
|
Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein--Case report and a review of literature. Clin Anat 2015; 23:750-8. [PMID: 20533511 DOI: 10.1002/ca.21007] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2010] [Revised: 04/13/2010] [Accepted: 04/20/2010] [Indexed: 01/30/2023]
Abstract
Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and the splenic venous drainage bypass the liver and drain into systemic veins through various venous shunts. To our knowledge, we have reviewed all 83 cases of CAPV, since first described in 1793. This equates to a rate of almost 2.5 cases per year over the last 30 years. Morgan and Superina (1994, J. Pediatr. Surg. 29:1239-1241) proposed the following classification of portosystemic anomalies; either the liver is not perfused with portal blood because of a complete shunt (Type I) or the liver is perfused with portal blood due to the presence of a partial shunt (Type II). In our case, abdominal venous blood drained into the suprarenal inferior vena cava via the left renal vein and dilated left gastric veins. After analyzing all reported cases, we recognize that more than 65% of patients are females and more than 30% of all published cases had been diagnosed by the age of 5 years. Additional anomalies are common in CAPV. In the reported cases, more then 22% of patients had congenital heart disease. Other commonly found anomalies include abnormalities of the spleen, urinary and male genital tract, brain as well as skeletal anomalies. Hepatic changes such as focal nodular hyperplasia, hepatocellular carcinoma, and hepatoblastoma are diagnosed in more then 40% of patients. This article also illustrates the radiological findings of CAPV. Radiological evaluation by ultrasound, CT, and MRI is helpful to detect coexisting abnormalities.
Collapse
Affiliation(s)
- Jana Mistinova
- Department of Radiology, Faculty of Medicine and University Hospital, Comenius University Bratislava, Slovak Republic
| | | | | |
Collapse
|
42
|
Kwapisz L, Wells MM, AlJudaibi B. Abernethy malformation: congenital absence of the portal vein. Can J Gastroenterol Hepatol 2014; 28:587-8. [PMID: 25575105 PMCID: PMC4277169 DOI: 10.1155/2014/675812] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/29/2014] [Accepted: 09/17/2014] [Indexed: 11/17/2022] Open
Affiliation(s)
- Lukasz Kwapisz
- Department of Internal Medicine, Western University, London, Ontario
| | - Malcolm M Wells
- Department of Gastroenterology and Hepatology, Western University, London, Ontario
| | - Bandar AlJudaibi
- Department of Gastroenterology and Hepatology, Western University, London, Ontario
- Department of Gastroenterology, King Khalid University Hospital, King Saud University, Saudi Arabia
| |
Collapse
|
43
|
Zhang K, Wang Q, Wang H, Ye H, Guo A, Duan W. Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature. Eur J Med Res 2014; 19:63. [PMID: 25424550 PMCID: PMC4260376 DOI: 10.1186/s40001-014-0063-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/04/2014] [Accepted: 11/04/2014] [Indexed: 02/05/2023] Open
Abstract
Background Patients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations. Case presentation Congenital absence of the portal vein (CAPV) is a rare malformation. We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD). The CT and MRI features of this case are described, and previously reported cases are reviewed. Conclusions CAPV is a rare congenital anomaly and in such patients, clarifying the site of portosystemic shunts, liver disease, and other anomalies is critical for appropriate treatment selection and accurate prognosis determination. Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.
Collapse
Affiliation(s)
- Kun Zhang
- Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| | - Qingjun Wang
- Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| | - Haiyi Wang
- Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| | - Huiyi Ye
- Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| | - Aitao Guo
- Department of Pathology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| | - Weidong Duan
- Department of Hepatobiliary Surgery, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China.
| |
Collapse
|
44
|
Kim JH, Hwang SE, Rodríguez-Vázquez JF, Murakami G, Cho BH. Liver agenesis with omphalocele: a report of two human embryos using serial histological sections. Pediatr Dev Pathol 2014; 17:431-40. [PMID: 25133969 DOI: 10.2350/14-05-1484-oa.1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
We identified 2 human embryos, with crown-rump lengths (CRLs) of 22 mm and 23 mm and a gestational age of approximately 7 weeks (O'Rahilly's stage 21-22), with liver agenesis and omphalocele. Serial histological sections were prepared of the entire body of one specimen, whereas sections of the neck, including the upper part of the heart, were missed for the other specimen as a result of tissue damage during the abortion. In addition, isolated omphalocele was assessed in another embryo (CRL = 25 mm) for comparison with atypical omphalocele in the embryos with liver agenesis. The 2 embryos with liver agenesis were characterized by (1) the absence of the anterior part of the diaphragm; (2) abnormality in the venous pole of the heart; (3) a normal stomach in the left upper abdominal cavity; and (4) normal pancreas development with normal midgut rotation. The most likely cause of liver agenesis, when combined with isolated omphalocele, was a defect in the anterior extension or migration of the septum transversum rather than a mechanical separation of the hepatic diverticulum from the septum transversum.
Collapse
Affiliation(s)
- Ji Hyun Kim
- 1 Department of Anatomy, Chonbuk National University Medical School, Jeonju, Republic of Korea
| | | | | | | | | |
Collapse
|
45
|
Raghuram KA, Bijulal S, Krishnamoorthy KM, Tharakan JA. Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol 2014; 34:1882-5. [PMID: 22843201 DOI: 10.1007/s00246-012-0428-z] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2012] [Accepted: 06/27/2012] [Indexed: 10/28/2022]
Abstract
A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.
Collapse
Affiliation(s)
- Krishnan A Raghuram
- Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Medical College P O, Thiruvananthapuram, 695011, India,
| | | | | | | |
Collapse
|
46
|
Tan GMY, Tay ELW, Poh KK. An unusual case of anomalous origin of the right coronary artery and hepatic focal nodular hyperplasia. QJM 2014; 107:145-6. [PMID: 23983268 DOI: 10.1093/qjmed/hct177] [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: 11/13/2022] Open
Abstract
Anomalous origin of the right coronary artery (ARCA) and focal nodular hyperplasia (FNH) are frequently reported in association with congenital heart abnormalities but not with each other. We propose that both conditions may share common origins in a maladative hyperplastic response to differential vascular flow due to developmental arterial malformations or aberrant Notch signalling during simultaneous gut and cardiac vasculorigenesis.
Collapse
Affiliation(s)
- G M Y Tan
- Cardiac Department, National University Heart Centre, 1E, Kent Ridge Road, NUHS Tower Block, Level 9, Singapore 119228, Singapore.
| | | | | |
Collapse
|
47
|
Histological and immunohistochemical revision of hepatocellular adenomas: a learning experience. Int J Hepatol 2013; 2013:398308. [PMID: 23533787 PMCID: PMC3603421 DOI: 10.1155/2013/398308] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2012] [Accepted: 01/19/2013] [Indexed: 01/20/2023] Open
Abstract
Light has been shed on the genotype/phenotype correlation in hepatocellular adenoma (HCA) recognizing HNF1 α -inactivated HCA (H-HCA), inflammatory HCA (IHCA), and β -catenin-activated HCA (b-HCA). We reviewed retrospectively our surgical HCA series to learn how to recognize the different subtypes histopathologically and how to interpret adequately their immunohistochemical staining. From January 1992 to January 2012, 37 patients underwent surgical resection for HCA in our institution. Nine had H-HCA (25%) characterized by steatosis and loss of L-FABP expression; 20 had IHCA (55.5%) showing CRP and/or SAA expression, sinusoidal dilatation, and variable inflammation; and 1 patient had both H-HCA and IHCA. In 5 patients (14%), b-HCA with GS and β -catenin nuclear positivity was diagnosed, two already with hepatocellular carcinoma. Two cases (5.5%) remained unclassified. One of the b-HCA showed also the H-HCA histological and immunohistochemical characteristics suggesting a subgroup of β -catenin-activated/HNF1 α -inactivated HCA, another b-HCA exhibited the IHCA histological and immunohistochemical characteristics suggesting a subgroup of β -catenin-activated/inflammatory HCA. Interestingly, three patients had underlying vascular abnormalities. Using the recently published criteria enabled us to classify histopathologically our retrospective HCA surgical series with accurate recognition of b-HCA for which we confirm the higher risk of malignant transformation. We also underlined the association between HCA and vascular abnormalities.
Collapse
|
48
|
Caputo S, Manganiello CAT. Severe portopulmonary hypertension associated with an unusual pattern of abernethy malformation: three-dimensional computed tomography view. Echocardiography 2012; 29:E275-7. [PMID: 22957945 DOI: 10.1111/j.1540-8175.2012.01816.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Affiliation(s)
- Salvatore Caputo
- Department of Cardiology, Ospedali Riuniti Marche Nord Hospital, Fano, Italy
| | | |
Collapse
|
49
|
Guérin F, Blanc T, Gauthier F, Abella SF, Branchereau S. Congenital portosystemic vascular malformations. Semin Pediatr Surg 2012; 21:233-44. [PMID: 22800976 DOI: 10.1053/j.sempedsurg.2012.05.006] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.
Collapse
Affiliation(s)
- Florent Guérin
- Department of Pediatric Surgery, Bicêtre Hospital, Paris, France.
| | | | | | | | | |
Collapse
|
50
|
Loomba RS, Telega GW, Gudausky TM. Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 2012; 47:E25-31. [PMID: 22595606 DOI: 10.1016/j.jpedsurg.2011.12.031] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2011] [Revised: 12/12/2011] [Accepted: 12/29/2011] [Indexed: 11/18/2022]
Abstract
We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.
Collapse
Affiliation(s)
- Rohit S Loomba
- Children's Hospital of Wisconsin/Herma Heart Center, Milwaukee, WI 53226, USA.
| | | | | |
Collapse
|