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Li W, Liu B, Feng H. Abernethy Malformation and Gastrointestinal Bleeding: A Case Report and Literature Review. Vasc Endovascular Surg 2025; 59:176-182. [PMID: 39262296 DOI: 10.1177/15385744241278870] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/13/2024]
Abstract
Background: Congenital extrahepatic portosystemic shunt (CEPS), also termed Abernethy malformation (AF) is a rare anomaly of the splanchnic venous system. Several approaches, including shunt closures through surgical or radiological interventions and liver transplantations, have been proposed, but clear comparisons among different treatment strategies are still unavailable. Purpose: We report a case in which an unusual portosystemic shunt was present between the dilated inferior mesenteric vein (IMV) to the right ovarian vein. A mini literature review of AF patients presented with gastrointestinal (GI) tract bleeding. Research design: Case report and literature review. Data Collection: An electronic search of PubMed was performed from inception to December 2023. Results: 34 AF patients presented with GI tract bleeding were identified published in the literature. The proportion of type II AF patients presenting with GI bleeding is greater (79%). Conclusions: We regard that both surgical ligation and endovascular closure of the shunt are effective and safe treatments for these patients, but coils embolization alone may not be sufficient to completely close the shunt when the shunt flow is high.
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Affiliation(s)
- Wenrui Li
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Bin Liu
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Hai Feng
- Department of Vascular Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China
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2
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Kono M, Akahane M, Yoshioka N, Hirano Y, Ohizumi Y, Fukuda I, Fujiwara S, Ishiguro T, Kiryu S. A rare, asymptomatic case of an extrahepatic portocaval shunt between the posterior aspect of the portal vein and the inferior vena cava. Surg Radiol Anat 2025; 47:63. [PMID: 39873868 DOI: 10.1007/s00276-025-03576-1] [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: 12/13/2024] [Accepted: 01/17/2025] [Indexed: 01/30/2025]
Abstract
PURPOSE We present the case of a rare extrahepatic portocaval shunt that resulted in communication of the portal vein and the inferior vena cava (IVC) at the level between two right renal veins that was incidentally diagnosed with contrast-enhanced computed tomography (CECT) in an asymptomatic patient. METHODS A woman in her sixties with abdominal pain and diarrhea of unclear origin underwent exploratory abdominal CECT. RESULTS The CECT incidentally revealed an extrahepatic portocaval shunt, whereby a vessel arising from the portal vein superior to the confluence of the superior mesenteric and splenic veins drained into the posterior aspect of the IVC between two right renal veins. CONCLUSION The identified vessel was likely a remnant communication between the supra-subcardinal anastomosis and the primitive portal vein. This type of portocaval shunt is rare; although there are no reports of its asymptomatic presence in an adult, recognition thereof may help to identify the cause of symptoms related to hepatic encephalopathy or liver dysfunction. Such a shunt may also provide a minimally invasive approach to the portal vein in procedures that typically rely on access via the transhepatic route.
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Affiliation(s)
- Mei Kono
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan.
| | - Masaaki Akahane
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Naoki Yoshioka
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Yuichiro Hirano
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Yuji Ohizumi
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Issei Fukuda
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Shin Fujiwara
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Taiga Ishiguro
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
| | - Shigeru Kiryu
- Department of Radiology, International University of Health and Welfare Narita Hospital, 852 Hatakeda, Narita, 286-8520, Chiba, Japan
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3
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Bakhru S, Koneti NR. The physiological implications of absent ductus venosus during fetal and post-natal life. Ann Pediatr Cardiol 2024; 17:257-263. [PMID: 39698422 PMCID: PMC11651408 DOI: 10.4103/apc.apc_93_24] [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: 05/12/2024] [Revised: 07/21/2024] [Accepted: 09/07/2024] [Indexed: 12/20/2024] Open
Abstract
Background The ductus venosus (DV) is a pivotal component of fetal circulation. Absent DV (ADV) is associated with structural defects, portal vein (PV) anomalies, and chromosomal anomalies. This observational study aims to investigate the impact of ADV on fetal circulation and postnatal outcomes. Materials and Methods This observational study was conducted from August 2016 to January 2020 at a fetal and pediatric cardiac center. The DV was evaluated as part of routine fetal echocardiography. Cases of ADV were identified. Blood flow and exit points of the umbilical vein were studied. Cardiothoracic ratio, hydrops, and PV were assessed during the initial and follow-up scans. The postnatal evaluation included an ultrasound abdomen and computed tomography with triple-phase imaging to assess portosystemic shunts (PSSs). Serum ammonia levels were monitored. Results Twelve patients with ADV were identified. The median maternal age and median gestational age were 27.5 years and 22 weeks, respectively. Four patients had intrahepatic drainage, while eight had extrahepatic drainage. All patients (100%) exhibited cardiomegaly, but none developed hydrops. Four patients had persistent PSS postnatally. All four patients with PSS had asymptomatic hyperammonemia. Two patients underwent transcatheter closure of PSS. The intrahepatic variant showed good PV anatomy with no evidence of PSS. Conclusions DV evaluation should be performed during fetal echocardiography. ADV can lead to cardiomegaly and dilation of the right atrium and ventricle. PSS can be a potential sequela of the extrahepatic variant of ADV.
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Affiliation(s)
- Shweta Bakhru
- Department of Pediatric Cardiology, Rainbow Children’s Heart Institute, Hyderabad, Telangana, India
| | - Nageswara Rao Koneti
- Department of Pediatric Cardiology, Rainbow Children’s Heart Institute, Hyderabad, Telangana, India
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Yan J, Yin J, Zhang D, Lv C, Pang W, Chen Y. An unusual interventional approach to treat Type 2 Abernethy malformation in children: two case reports. Gastroenterol Rep (Oxf) 2024; 12:goad075. [PMID: 38187097 PMCID: PMC10769814 DOI: 10.1093/gastro/goad075] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/12/2023] [Revised: 11/09/2023] [Accepted: 12/05/2023] [Indexed: 01/09/2024] Open
Affiliation(s)
- Jiayu Yan
- Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
| | - Jie Yin
- Department of Radiology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
| | - Dan Zhang
- Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
| | - Chuankai Lv
- Department of Ultrasound, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
| | - Wenbo Pang
- Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
| | - Yajun Chen
- Department of General Surgery, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, P. R. China
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Pawal S, Arkar R. A Rare Cause of Recurrent Encephalopathy in a Septuagenarian: A Case Report. Cureus 2023; 15:e41015. [PMID: 37519484 PMCID: PMC10372537 DOI: 10.7759/cureus.41015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/26/2023] [Indexed: 08/01/2023] Open
Abstract
The Abernethy malformation is an extremely rare congenital, extrahepatic, portosystemic shunt. There are many problems associated with this abnormal portovenous shunting and subsequent reduced hepatic portal venous flow. With the advances in non-invasive imaging technologies, these cases are diagnosed in more numbers; however, the presentation of patients is varied and the natural history is not completely known. The presenting symptom of the portosystemic shunt is mainly hyperammonemia, leading to encephalopathy. Management varies depending on the type of shunt and its clinical course; hence, the classification of the congenital portosystemic shunt is important in these patients.
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Affiliation(s)
- Sambhaji Pawal
- Department of Interventional Radiology, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Pune, IND
| | - Rahul Arkar
- Department of Interventional Radiology, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Pune, IND
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6
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Nagao Y, Toshida K, Morinaga A, Tomiyama T, Kosai Y, Shimagaki T, Tomino T, Wang H, Kurihara T, Toshima T, Morita K, Itoh S, Harada N, Yoshizumi T. Caution for living donor liver transplantation with congenital portosystemic shunt: a case report. Surg Case Rep 2022; 8:190. [PMID: 36201092 PMCID: PMC9537393 DOI: 10.1186/s40792-022-01533-3] [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: 04/24/2022] [Accepted: 09/13/2022] [Indexed: 11/05/2022] Open
Abstract
Background Congenital portosystemic shunt is an infrequent abnormal connection between the portal vascular system and the systemic circulation. Portosystemic shunts are common findings in patients with cirrhosis, causing gastroesophageal varices, hepatic encephalopathy, and others. However, there is no consensus or literature describing how to manage asymptomatic patients with portosystemic shunts and normal liver. Case presentation The patient was a 39-year-old female who underwent donor right hepatectomy for living donor liver transplantation. The patient was healthy by nature, however, developed hepatic encephalopathy after the surgery due to a development of portosystemic shunt. Portosystemic shunt stole portal blood flow, and imaging modalities revealed narrowing of the portal trunk, representing prolonged depletion of portal blood flow. Balloon-occluded retrograde transvenous obliteration (B-RTO) was performed for occlusion of the portosystemic shunt. B-RTO increased portal blood flow, and hepatic encephalopathy with hyperammonemia was successfully resolved without the outbreak of any other symptom of portal hypertension. Conclusions A congenital portosystemic shunt itself is not a contraindication for donor hepatectomy, but perioperative endovascular shunts occlusion or intraoperative ligature of these shunts should be considered.
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Affiliation(s)
- Yoshihiro Nagao
- grid.411248.a0000 0004 0404 8415Department of Advanced Medical Initiatives, Kyushu University Hospital, Fukuoka, Japan ,grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Katsuya Toshida
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Akinari Morinaga
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Takahiro Tomiyama
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Yukiko Kosai
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Tomonari Shimagaki
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Takahiro Tomino
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Huanlin Wang
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Takeshi Kurihara
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Takeo Toshima
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Kazutoyo Morita
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Shinji Itoh
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Noboru Harada
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
| | - Tomoharu Yoshizumi
- grid.177174.30000 0001 2242 4849Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 Japan
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7
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Zeng P, Qu Z, Sun H, Zhao J. Status of congenital extrahepatic portosystemic shunt. Chin Med J (Engl) 2022; 135:00029330-990000000-00115. [PMID: 35941726 PMCID: PMC9532025 DOI: 10.1097/cm9.0000000000001925] [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: 03/18/2021] [Indexed: 12/05/2022] Open
Affiliation(s)
- Pan Zeng
- Pediatric Surgery, Graduate Training Base of Jinzhou Medical University, Affiliated Hospital of Hubei Medical College, Taihe Hospital, Shiyan, Hubei 442000, China
| | - Zhenfan Qu
- Pediatric Surgery, Graduate Training Base of Jinzhou Medical University, Affiliated Hospital of Hubei Medical College, Taihe Hospital, Shiyan, Hubei 442000, China
| | - Heng Sun
- Pediatric Surgery, Graduate Training Base of Jinzhou Medical University, Affiliated Hospital of Hubei Medical College, Taihe Hospital, Shiyan, Hubei 442000, China
| | - Junbo Zhao
- Pediatric Surgery, Graduate Training Base of Jinzhou Medical University, Affiliated Hospital of Hubei Medical College, Taihe Hospital, Shiyan, Hubei 442000, China
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8
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Ghasemi-Rad M, Smuclovisky E, Cleveland H, Hernandez JA. Endovascular treatment of a portosystemic shunt presenting with hypoglycemia; case presentation and review of literature. Clin Imaging 2022; 83:131-137. [PMID: 35033850 DOI: 10.1016/j.clinimag.2021.12.020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2021] [Revised: 12/13/2021] [Accepted: 12/23/2021] [Indexed: 11/03/2022]
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9
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Nakajima H, Kodo K. A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2021.102052] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022] Open
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10
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Andrade G, Facas J, Marques P, Mónica AN, Donato P. Congenital extrahepatic portosystemic shunt type II occluded with cardiac closure device. Radiol Case Rep 2021; 16:3802-3806. [PMID: 34691344 PMCID: PMC8511717 DOI: 10.1016/j.radcr.2021.09.020] [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: 09/08/2021] [Accepted: 09/10/2021] [Indexed: 11/21/2022] Open
Abstract
Congenital extrahepatic portosystemic shunts are a rare cause of lower gastrointestinal bleeding in children. They result from the connection of a systemic vessel with the portomesenteric vasculature before the division of the main portal vein. Herein, we report a case of a congenital extrahepatic portosystemic shunts type II in a 4-year-old male diagnosed by Doppler ultrasonography during the investigation of abdominal pain and recurrent hematochezia, later confirmed by computed tomography. Conventional angiography with a balloon occlusion test revealed patent intrahepatic portal branches not depicted by previous imaging techniques. Successful shunt closure was achieved by endovascular approach with an Amplatzer Septal Occluder without complications.
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Affiliation(s)
- Gisela Andrade
- Department of Radiology, Hospital Prof. Doutor Fernando Fonseca EPE, Amadora, Portugal
| | - João Facas
- Medical Imaging Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
| | - Pedro Marques
- Department of Radiology, Hospital Prof. Doutor Fernando Fonseca EPE, Amadora, Portugal
| | - Ana Nassauer Mónica
- Department of Radiology, Hospital Prof. Doutor Fernando Fonseca EPE, Amadora, Portugal
| | - Paulo Donato
- Medical Imaging Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
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11
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AKDULUM İ, AKYÜZ M, GÜRÜN E, ÖZTÜRK M, SIĞIRCI A, BOYUNAĞA Ö. CT-angiography and doppler ultrasound evaluation of congenital portosystemic shunts. ACTA MEDICA ALANYA 2021. [DOI: 10.30565/medalanya.828133] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
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12
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Tanaka H, Saijo Y, Tomonari T, Tanaka T, Taniguchi T, Yagi S, Okamoto K, Miyamoto H, Sogabe M, Sato Y, Muguruma N, Tsuneyama K, Sata M, Takayama T. An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration. Intern Med 2021; 60:1839-1845. [PMID: 33456037 PMCID: PMC8263181 DOI: 10.2169/internalmedicine.5914-20] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.
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Affiliation(s)
- Hironori Tanaka
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Yoshihito Saijo
- Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Tetsu Tomonari
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Takahiro Tanaka
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Tatsuya Taniguchi
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Shusuke Yagi
- Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Koichi Okamoto
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Hiroshi Miyamoto
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Masahiro Sogabe
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Yasushi Sato
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Naoki Muguruma
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Koichi Tsuneyama
- Department of Pathology and Laboratory Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Masataka Sata
- Department of Cardiovascular Medicine, Tokushima University Graduate School of Biomedical Sciences, Japan
| | - Tetsuji Takayama
- Department of Gastroenterology and Oncology, Tokushima University Graduate School of Biomedical Sciences, Japan
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13
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Uchida H, Sakamoto S, Kasahara M, Kudo H, Okajima H, Nio M, Umeshita K, Ohdan H, Egawa H, Uemoto S. Longterm Outcome of Liver Transplantation for Congenital Extrahepatic Portosystemic Shunt. Liver Transpl 2021; 27:236-247. [PMID: 37160013 DOI: 10.1002/lt.25805] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2019] [Revised: 04/01/2020] [Accepted: 05/01/2020] [Indexed: 02/07/2023]
Abstract
Liver transplantation (LT) is often viewed as the last resort for the treatment of congenital extrahepatic portosystemic shunt (CEPS) due to advancement of imaging and interventional radiology techniques. However, some patients still require LT, and criteria for LT are yet to be determined. We conducted a national survey of patients undergoing LT for CEPS between June 1998 and August 2018 and evaluated the clinical data and outcomes with a review of previously reported patients from the English-language medical literature. A total of 26 patients underwent LT in Japan at a median age of 5.2 years old. The most common indications for LT were persistent hyperammonemia (54%) and liver mass (50%), followed by pulmonary complications (38%). Pulmonary complications in all patients, including intrapulmonary shunt and pulmonary hypertension (PH), were improved after LT. Regarding the 29 previously reported patients in the English-language literature, a liver nodule (49%), including hepatoblastoma and hepatocellular carcinoma, was the most common indication for LT, followed by pulmonary complications (34%). A total of 25 (96%) patients in our survey and 26 (90%) patients in the literature review were alive with a median follow-up period of 9.5 and 1.6 years, respectively. Although LT has a limited role in management of CEPS, our study indicated that LT was safe as an alternative treatment for select patients with malignant tumor or pulmonary complications and those with complications related to new portosystemic collateral vessels after shunt closure, such as PH or hepatopulmonary syndrome.
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Affiliation(s)
- Hajime Uchida
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Seisuke Sakamoto
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Mureo Kasahara
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Hironori Kudo
- Department of Pediatric Surgery, Tohoku University, Miyagi, Japan
| | - Hideaki Okajima
- Department of Pediatric Surgery, Kanazawa Medical University, Ishikawa, Japan
| | - Masaki Nio
- Department of Pediatric Surgery, Tohoku University, Miyagi, Japan
| | - Koji Umeshita
- Department of Surgery, Osaka University, Osaka, Japan
| | - Hideki Ohdan
- Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Hiroto Egawa
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Shinji Uemoto
- Department of Surgery, Kyoto University, Kyoto, Japan
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14
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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.
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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
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15
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Rohringer TJ, Ng VL, Amaral JG, Parra DA. Embolization of congenital portosystemic shunt presenting after pediatric liver transplantation: Case report and literature review. Pediatr Transplant 2020; 24:e13713. [PMID: 32406984 DOI: 10.1111/petr.13713] [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: 07/05/2019] [Revised: 09/28/2019] [Accepted: 03/23/2020] [Indexed: 11/30/2022]
Abstract
This case report describes a 13-year 10-month-old girl who underwent a deceased-donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans-hepatic porto-venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro-coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post-LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS.
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Affiliation(s)
- Taryn J Rohringer
- Faculty of Medicine, University of Toronto, Toronto, ON, Canada.,Division of Image Guided Therapy, Diagnostic Imaging Department, The Hospital for Sick Children, Toronto, ON, Canada
| | - Vicky L Ng
- Faculty of Medicine, University of Toronto, Toronto, ON, Canada.,Division of Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada
| | - Joao G Amaral
- Faculty of Medicine, University of Toronto, Toronto, ON, Canada.,Division of Image Guided Therapy, Diagnostic Imaging Department, The Hospital for Sick Children, Toronto, ON, Canada
| | - Dimitri A Parra
- Faculty of Medicine, University of Toronto, Toronto, ON, Canada.,Division of Image Guided Therapy, Diagnostic Imaging Department, The Hospital for Sick Children, Toronto, ON, Canada
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16
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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.
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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
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17
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Nam HD. Living-donor liver transplantation for Abernethy malformation - case report and review of literature. Ann Hepatobiliary Pancreat Surg 2020; 24:203-208. [PMID: 32457268 PMCID: PMC7271108 DOI: 10.14701/ahbps.2020.24.2.203] [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] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2019] [Revised: 01/23/2020] [Accepted: 01/29/2020] [Indexed: 11/17/2022] Open
Abstract
Abernethy malformation was named for the rare congenital absence of the portal vein (CAPV), also known as congenital extrahepatic portal-systemic shunts (CEPS). This could be classified as complete (type 1) or incomplete shunt (type 2) according to Morgan-Superina classification. Its presentation may show under variable signs and symptoms such as hepatopulmonary syndrome, hyper-ammonia, hepatic masses and liver failure…. This usually combined with other congenital anomalies (cardiac anomaly, trisomy…). This report presented a 10 year-old boy with growth retardation and mild mental recognition, intermittent hyperammonia, elevated liver enzymes, huge inoperable mass in the right liver. MS CT and MRI findings: hyperplasia of liver parenchyma with superior mesenteric vein confluenced with splenic vein before draining directly into the inferior vena cava (Abernethy anomaly type 1b). Living donor liver transplantation underwent using right lobe from his mother. Anatomopathology findings of the native liver showed chronic hepatitis with cirrhosis 4/6 Knodel-Ishak. Postoperatively, the patient still attained optimal liver function and has returned to normal life at 12-month follow-up. Liver transplantation was a reasonable indication for CAPV type 1. Living donor liver transplantation was effective and practical in the scarcity of donor organ.
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Affiliation(s)
- Hoang Duc Nam
- HBP-Liver Transplant Center, Vinmec Times City International Hospital, Hanoi, Vietnam
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18
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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.
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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)
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19
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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.
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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
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20
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Glonnegger H, Schulze M, Kathemann S, Berg S, Füllgraf H, Tannapfel A, Gerner P, Grohmann J, Niemeyer C, Hettmer S. Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt. Front Pediatr 2020; 8:501. [PMID: 32984213 PMCID: PMC7477041 DOI: 10.3389/fped.2020.00501] [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] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2020] [Accepted: 07/15/2020] [Indexed: 12/19/2022] Open
Abstract
Congenital extrahepatic portosystemic shunts (CEPS), previously also described as Abernethy malformations, are rare malformations in which the extrahepatic portal system directly communicates with the vena cava inferior, thereby bypassing the liver. A hypoplastic portal vein (PV) exists in most cases. CEPS have been associated with the development of liver nodules, ranging from mostly focal nodular hyperplasia (FNH) to hepatic adenoma (HA) and even hepatocellular carcinoma (HCC). Tumor development in CEPS may be due to changes in perfusion pressures, oxygen supply or endocrine imbalances. It is important to rule out CEPS in children with liver tumors, because resection could impede future shunt occlusion procedures, and benign masses may regress after shunt occlusion. Here, we review the case of a 9-years-old male with CEPS and hepatic nuclear Factor 1-alpha (HNF-1-alpha) inactivated HA to raise awareness of this condition and review histopathological changes in the liver of CEPS.
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Affiliation(s)
- Hannah Glonnegger
- Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
| | - Maren Schulze
- Department of Transplant and General Surgery, University Hospital Essen, Essen, Germany
| | - Simone Kathemann
- Department of Transplant and General Surgery, University Hospital Essen, Essen, Germany
| | - Sebastian Berg
- Division of Pediatric Radiology, Department of Radiology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
| | - Hannah Füllgraf
- Department of Pathology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
| | - Andrea Tannapfel
- Faculty of Medicine, Medical Center, Institute for Pathology, Ruhr-University Bochum, Bochum, Germany
| | - Patrick Gerner
- Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
| | - Jochen Grohmann
- Department of Congenital Heart Defects and Pediatric Cardiology, Faculty of Medicine, University Heart Center Freiburg-Bad Krozingen, University of Freiburg, Freiburg im Breisgau, Germany
| | - Charlotte Niemeyer
- Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
| | - Simone Hettmer
- Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany
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21
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Azad S, Arya A, Sitaraman R, Garg A. Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature. Ann Pediatr Cardiol 2019; 12:240-247. [PMID: 31516281 PMCID: PMC6716315 DOI: 10.4103/apc.apc_185_18] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/28/2023] Open
Abstract
Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.
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Affiliation(s)
- Sushil Azad
- Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, New Delhi, India
| | - Adhi Arya
- Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, New Delhi, India
| | - Radhakrishnan Sitaraman
- Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, New Delhi, India
| | - Amit Garg
- Department of Radiodiagnosis, Fortis Escorts Heart Institute, New Delhi, India
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22
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Ponziani FR, Faccia M, Zocco MA, Giannelli V, Pellicelli A, Ettorre GM, De Matthaeis N, Pizzolante F, De Gaetano AM, Riccardi L, Pompili M, Rapaccini GL. Congenital extrahepatic portosystemic shunt: description of four cases and review of the literature. J Ultrasound 2018; 22:349-358. [PMID: 30357760 DOI: 10.1007/s40477-018-0329-y] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 09/24/2018] [Indexed: 12/12/2022] Open
Abstract
Congenital extrahepatic portosystemic shunt (CEPS), also known as Abernethy malformation, is a rare condition in which the splenomesenteric blood drains directly into a systemic vein, bypassing the liver through a complete or partial shunt. The diagnosis is frequently made during childhood in the setting of neonatal cholestasis, hypergalactosemia, failure to thrive, mental retardation or other congenital defects. In adulthood, CEPS is usually found incidentally during diagnostic work-up for abdominal pain, liver test abnormalities, liver nodules, portopulmonary hypertension, portopulmonary syndrome or portosystemic encephalopathy. The diagnosis depends on imaging and portal venography, but sometimes only liver biopsy can be resolutive, demonstrating the absence of venules within the portal areas. Here we report four recent cases of Abernethy malformation diagnosed in young adults, in which ultrasound (US) was the initial imaging technique and allowed to suspect the diagnosis. Furthermore, we reviewed clinical presentations, associated anomalies and treatment of the 310 cases of CEPS previously reported in the literature.
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Affiliation(s)
- Francesca Romana Ponziani
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | - Mariella Faccia
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy.
| | - Maria Assunta Zocco
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | | | | | - Giuseppe Maria Ettorre
- Division of General Surgery and Liver Transplantation, San Camillo Hospital, Rome, Italy
| | - Nicoletta De Matthaeis
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | - Fabrizio Pizzolante
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | | | - Laura Riccardi
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | - Maurizio Pompili
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
| | - Gian Ludovico Rapaccini
- Internal Medicine, Gastroenterology and Hepatology, Fondazione IRCCS Agostino Gemelli Hospital, Largo Agostino Gemelli 8, 00168, Rome, Italy
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23
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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.
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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
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24
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Sharma S, Bobhate PR, Sable S, Kumar S, Yadav K, Maheshwari S, Amin S, Chauhan A, Varma V, Kapoor S, Kumaran V. Abernethy malformation: Single-center experience from India with review of literature. Indian J Gastroenterol 2018; 37:359-364. [PMID: 30187299 DOI: 10.1007/s12664-018-0884-3] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2018] [Accepted: 08/05/2018] [Indexed: 02/04/2023]
Abstract
Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. This is a retrospective analysis of data from nine patients with Abernethy malformation at a single center. This is a referral center for Pediatric Cardiology and for Hepatobiliary and Pancreatic Surgery. The patients presented to the Pulmonary Hypertension Clinic/the Hepatobiliary Surgery Clinic. Out of nine patients, four were male. Type II Abernethy malformation was present in five patients whereas three patients had type I malformation. One of the patients had communication between inferior mesenteric vein and internal iliac vein. Five out of nine patients were erroneously diagnosed as idiopathic primary pulmonary hypertension and were treated with vasodilators. One patient required living donor liver transplant. One patient was managed with surgical shunt closure whereas two patients required transcatheter shunt closure. The rest of the patients were managed conservatively. Abernethy malformation is more common than previously thought and the diagnosis is often missed. There are various management options for Abernethy malformation, which includes surgical or transcatheter shunt closure and liver transplant. Management of Abernethy malformation depends upon type, presentation, and size of shunt.
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Affiliation(s)
- Swapnil Sharma
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India.
| | - Prashant R Bobhate
- Department of Pediatric Cardiology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Shailesh Sable
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Suneed Kumar
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Kapildev Yadav
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Sharad Maheshwari
- Department of Radiology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Saista Amin
- Department of Pediatric Hepatology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Ashutosh Chauhan
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Vibha Varma
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Sorabh Kapoor
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Vinay Kumaran
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
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25
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Zhang B, Wu KT, Li L, Lai HY. Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature. Cardiovasc Intervent Radiol 2018; 41:1121-1127. [PMID: 29691614 DOI: 10.1007/s00270-018-1972-6] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2017] [Accepted: 04/20/2018] [Indexed: 11/30/2022]
Abstract
Hematochezia is a rare clinical presentation of congenital extrahepatic portosystemic shunt (CEPS). We describe a series of three patients with type II CEPS presenting as hematochezia that were treated by catheter embolization, followed by a brief review of published articles. Hematochezia of the patients was due to the giant inferior mesenteric vein, superior rectal vein and colonic varices. The catheter embolization was successfully accomplished in all of the patients. After a mean follow-up of 27 months, no serious adverse effects were observed. For unexplained massive hematochezia, CEPS needs to be considered as a differential diagnosis. Based on our present results and the review of the literature, transcatheter permanent embolization of the giant inferior mesenteric vein might be an effective and safe treatment for type II CEPS.Level of Evidence Level 4, case series.
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Affiliation(s)
- Bo Zhang
- Department of Radiology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510655, China.
| | - Ke-Tong Wu
- Department of Radiology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510655, China
| | - Lin Li
- Department of Radiology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510655, China
| | - Hai-Yang Lai
- Department of Radiology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510655, China
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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.
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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
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Mohtashami A, Kiat A, Cross J, Simon R, Curtin A. Catastrophic intraoperative bleeding due to congenital extrahepatic porto-systemic shunt anomaly: A surgical case report of two rare anomalies. Int J Surg Case Rep 2018; 44:161-165. [PMID: 29518666 PMCID: PMC5928286 DOI: 10.1016/j.ijscr.2018.02.034] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2018] [Accepted: 02/22/2018] [Indexed: 11/18/2022] Open
Abstract
INTRODUCTION Abernethy malformations are extremely rare congenital anomalous portosystemic shunts. We report the case of a patient with a rare variant Abernethy malformation between the superior mesenteric vein and left renal vein, associated with a massive jejunal diverticulum. PRESENTATION OF CASE A 37-year-old Caucasian female presented to our emergency department with severe abdominal pain and proceeded to laparotomy for a presumed small bowel obstruction. At laparotomy she was found to have a massive diverticulum at the duodeno-jejunal junction, which was intimately associated with a venous malformation and the anomalous portosystemic shunt. Whilst mobilising the diverticulum, the patient developed catastrophic haemorrhage from the malformation. The patient underwent a complicated post-operative course however was eventually stabilised. DISCUSSION We discuss the anatomy and pathophysiology of anomalous portosystemic shunts and propose an embryological origin for our patients' anomalies. CONCLUSION Abernethy malformations are rare however may be associated with other intra-abdominal pathology and extreme caution is required when operating on these patients.
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Affiliation(s)
- Ali Mohtashami
- Department of General Surgery, Lismore Base Hospital, Lismore, NSW 2480, Australia.
| | - Andrew Kiat
- Department of General Surgery, Lismore Base Hospital, Lismore, NSW 2480, Australia
| | - Jane Cross
- Department of General Surgery, Lismore Base Hospital, Lismore, NSW 2480, Australia
| | - Robert Simon
- Department of General Surgery, Lismore Base Hospital, Lismore, NSW 2480, Australia
| | - Austin Curtin
- Department of General Surgery, Lismore Base Hospital, Lismore, NSW 2480, Australia
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Papamichail M, Pizanias M, Heaton N. Congenital portosystemic venous shunt. Eur J Pediatr 2018; 177:285-294. [PMID: 29243189 PMCID: PMC5816775 DOI: 10.1007/s00431-017-3058-x] [Citation(s) in RCA: 98] [Impact Index Per Article: 14.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2017] [Revised: 11/24/2017] [Accepted: 11/28/2017] [Indexed: 12/12/2022]
Abstract
UNLABELLED Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension. CONCLUSION This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.
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Affiliation(s)
- M. Papamichail
- Department of Transplantation and Hepato-Pancreato-Biliary Surgery, Lahey Hospital and Medical Center, Burlington, Boston, MA 01805 USA
| | - M. Pizanias
- Department of Liver Transplantation, Hepatobiliary Pancreatic Surgery, King’s Healthcare Partners, King’s College Hospital NHS FT, Institute of Liver Studies, Denmark Hill, London, SE5 9RS UK
| | - N. Heaton
- Department of Liver Transplantation, Hepatobiliary Pancreatic Surgery, King’s Healthcare Partners, King’s College Hospital NHS FT, Institute of Liver Studies, Denmark Hill, London, SE5 9RS UK
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Mesquita RD, Sousa M, Vilaverde F, Cardoso R. Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature. BJR Case Rep 2017; 4:20170054. [PMID: 30363163 PMCID: PMC6159114 DOI: 10.1259/bjrcr.20170054] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2017] [Revised: 09/13/2017] [Accepted: 10/25/2017] [Indexed: 01/04/2023] Open
Abstract
The Abernethy malformation consists of a congenital extrahepatic portosystemic shunt and is believed to be extremely rare in humans. The potential implications of abnormal portovenous shunting and decreased hepatic portal flow are numerous and potentially serious. Although congenital extrahepatic portosystemic shunts are increasingly suspected and diagnosed in specialized centres, much of their clinical presentation and natural history is not fully understood. Symptoms of portosystemic shunt are mainly caused by increased levels of ammonia, which lead to signs of encephalopathy. Therapeutic options depend on the type of shunt and its clinical course, so the classification of the congenital portosystemic shunt is a key finding in these patients.
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Affiliation(s)
- Romeu Duarte Mesquita
- Department of Radiology, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal
| | - Marta Sousa
- Department of Radiology, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal
| | | | - Rosa Cardoso
- Department of Radiology, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal
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Mi XX, Li XG, Wang ZR, Lin L, Xu CH, Shi JP. Abernethy malformation associated with Caroli's syndrome in a patient with a PKHD1 mutation: a case report. Diagn Pathol 2017; 12:61. [PMID: 28814334 PMCID: PMC5559867 DOI: 10.1186/s13000-017-0647-y] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2017] [Accepted: 07/20/2017] [Indexed: 12/19/2022] Open
Abstract
Background Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli’s disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli’s disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli’s syndrome. The combination of Abernethy malformation and Caroli’s syndrome has not been reported previously. Case presentation We present the case of a 23-year-old female who was found to have both type II Abernethy malformation and Caroli’s syndrome. Radiological imaging was performed, including computed tomography with three-dimensional reconstruction and magnetic resonance imaging with (magnetic resonance cholangiopancreatography (MRCP), which revealed a side-to-side portocaval shunt, intrahepatic bile duct dilation, congenital hepatic fibrosis, and renal cysts. In addition, PKHD1 (polycystic kidney and hepatic disease 1) gene mutational analysis revealed a paternally inherited heterozygous missense mutation (c.1877A > G, p.Lys626Arg). A liver biopsy confirmed the pathological features of Caroli’s syndrome. Conclusions To our knowledge, this is the first reported case of a patient with both type II Abernethy malformation and Caroli’s syndrome diagnosed using a comprehensive approach that included imaging, mutational analysis, and liver biopsy. Additionally, this is the second reported case to date of an Asian patient presenting with liver and renal disorders with the same paternally inherited PKHD1 missense mutation.
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Affiliation(s)
- Xiao-Xiao Mi
- Institute of Translational Medicine, the Affiliated Hospital of Hangzhou Normal University, Hangzhou, Zhejiang, China
| | - Xiao-Guang Li
- Department of Infectious Diseases, The Second Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Zi-Rong Wang
- Department of Radiology, The Affiliated Hospital of Hangzhou Normal University, Hangzhou, Zhejiang, China
| | - Ling Lin
- Shanghai Biotecan Medical Diagnostics Corporation, Shanghai, China
| | - Chun-Hai Xu
- Department of Infectious Diseases, The Second Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China.
| | - Jun-Ping Shi
- Institute of Translational Medicine, the Affiliated Hospital of Hangzhou Normal University, Hangzhou, Zhejiang, China.
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Understanding the Pathophysiology of Portosystemic Shunt by Simulation Using an Electric Circuit. BIOMED RESEARCH INTERNATIONAL 2016; 2016:2097363. [PMID: 27868061 PMCID: PMC5102704 DOI: 10.1155/2016/2097363] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/03/2016] [Revised: 10/04/2016] [Accepted: 10/13/2016] [Indexed: 12/12/2022]
Abstract
Portosystemic shunt (PSS) without a definable cause is a rare condition, and most of the studies on this topic are small series or based on case reports. Moreover, no firm agreement has been reached on the definition and classification of various forms of PSS, which makes it difficult to compare and analyze the management. The blood flow can be seen very similar to an electric current, governed by Ohm's law. The simulation of PSS using an electric circuit, combined with the interpretation of reported management results, can provide intuitive insights into the underlying mechanism of PSS development. In this article, we have built a model of PSS using electric circuit symbols and explained clinical manifestations as well as the possible mechanisms underlying a PSS formation.
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Papamichail M, Ali A, Quaglia A, Karani J, Heaton N. Liver resection for the treatment of a congenital intrahepatic portosystemic venous shunt. Hepatobiliary Pancreat Dis Int 2016; 15:329-33. [PMID: 27298112 DOI: 10.1016/s1499-3872(16)60067-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Intrahepatic portosystemic shunts (IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis. It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications. When recognized, early intervention to occlude the shunt will reverse the associated complications. Literature review reports of surgical and radiological occlusion of the shunt, but due to its rarity, a standard therapeutic protocol has not been established. A case of a 38-year-old woman with abdominal pain and low grade encephalopathy, diagnosed with an IPSS and treated by right hepatectomy was reported.
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Affiliation(s)
- Michail Papamichail
- Department of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital: King's Healthcare Partners, King's College Hospital FT NHS Trust Institute of Liver Studies Denmark Hill, London SE5 9RS, UK.
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Achiron R, Kivilevitch Z. Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2016; 47:739-747. [PMID: 25988346 DOI: 10.1002/uog.14906] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/22/2015] [Revised: 05/10/2015] [Accepted: 05/12/2015] [Indexed: 06/04/2023]
Abstract
OBJECTIVES To review our experience with fetal umbilical-portal-systemic venous shunts (UPSVS), to devise an in-utero classification system, and to analyze their clinical and prognostic characteristics. METHODS This was a retrospective review of cases of UPSVS examined at an academic tertiary referral center over the 14-year period from 2001 to 2014 inclusive. The anatomical origin and drainage of the fetal umbilical, portal, ductus venosus and hepatic venous systems, and the integrity of the intrahepatic portal venous system (IHPVS), were assessed using two- and three-dimensional color Doppler sonography. Fetomaternal clinical characteristics and long-term outcome were investigated by means of medical files, imaging documentation and telephone interviews with the mothers. RESULTS Forty-four cases with UPSVS were reviewed. Prenatal classification was based on the anatomical origin of the shunt (umbilical, portal or ductal), with cases classified into four types: Type I, umbilical-systemic shunt (n = 9 (20.4%)); Type II, ductus venosus-systemic shunt (n = 19 (43.2%)); and Type III, portal-systemic shunt, divided into two subtypes: Type IIIa, intrahepatic portal-systemic shunt (n = 12 (27.2%)) and Type IIIb, extrahepatic portal-systemic shunt (n = 4 (9.1%)). Each type presented particular clinical and prognostic characteristics. The most important favorable prognostic factors were absence of associated major malformation and presence of the IHPVS. Thus, Type IIIa (intrahepatic portal-systemic shunt) had the best outcome. CONCLUSIONS Fetal UPSVS are anomalies with a broad spectrum of manifestations and prognoses. Accurate mapping of the IHPVS is paramount for optimizing prenatal counseling and postnatal care. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.
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Affiliation(s)
- R Achiron
- Department of Obstetrics and Gynecology, Chaim Sheba Medical Center, Tel-Hashomer, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Z Kivilevitch
- Maccabi Health Services, Ultrasound Unit, Negev Medical Center, Be'er Sheva, Israel
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Gong Y, Zhu H, Chen J, Chen Q, Ji M, Pa M, Zheng S, Qiao Z. Congenital portosystemic shunts with and without gastrointestinal bleeding - case series. Pediatr Radiol 2015. [PMID: 26209117 DOI: 10.1007/s00247-015-3417-6] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
BACKGROUND The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. OBJECTIVE To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). MATERIALS AND METHODS We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. RESULTS A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. CONCLUSION Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.
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Affiliation(s)
- Ying Gong
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Hui Zhu
- Department of Radiology, Fudan University Shanghai Cancer Center, Shanghai, 200032, the People's Republic of China
| | - Jun Chen
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Qi Chen
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Min Ji
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Mier Pa
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Shan Zheng
- Department of Surgery, Children's Hospital of Fudan University, Shanghai, 200032, the People's Republic of China
| | - Zhongwei Qiao
- Department of Radiology, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China.
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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.
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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
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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.
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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
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38
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Takeuchi H, Takeda Y, Takahashi M, Hayashi S, Fukuzawa Y, Nakano T. A rare congenital extrahepatic portosystemic shunt affecting the inferior mesenteric vein, inferior vena cava, and left ovarian vein. Surg Radiol Anat 2013; 36:729-32. [PMID: 24297630 DOI: 10.1007/s00276-013-1230-1] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/25/2013] [Accepted: 10/21/2013] [Indexed: 11/28/2022]
Abstract
PURPOSE To observe a case of congenital extrahepatic portosystemic shunt and discuss it from the embryological and clinical viewpoints. METHODS An 85-year-old female cadaver was employed for a dissection course at Aichi Medical University in 2009. RESULTS There was no evidence of liver cirrhosis macroscopically or microscopically. A portosystemic shunt was observed that involved communication between the inferior mesenteric vein, inferior vena cava (IVC), and left ovarian vein by a single Y-shaped shunt vessel. CONCLUSIONS To the best of our knowledge, this is the first reported case of the above-mentioned three veins being connected by a single Y-shaped shunt vessel. Considering the other venous diameters, the shunt appeared to flow into the splenic vein and IVC. It cannot be denied that this shunt may have led to hepatic encephalopathy, although the shunt effect may have been minimal. Embryological development of IVC appears to occur close to the plexus of anastomosing vitelline veins, forming the portal vein.
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Affiliation(s)
- Hajime Takeuchi
- School of Medicine, Aichi Medical University, Nagakute, Aichi, 480-1195, Japan
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Abstract
BACKGROUND Congenital extrahepatic portosystemic shunt (CEPS) is reported more frequently because of advances in imaging techniques. Liver transplantation (LT) is a therapeutic option, although the indications for LT are still controversial. METHODS This study reviewed 34 cases of LT for CEPS, including 30 cases reported in the English medical literature and the patients treated in our department, to collect the clinical data associated with LT. RESULTS The median age at diagnosis and LT was 3.7 and 6.8 years, respectively. Hepatic encephalopathy, including persistent hyperammonemia, was the most common indication of LT. Pulmonary complications, including hepatopulmonary syndrome and pulmonary hypertension, were the second most common indications of LT, and those patients underwent LT soon after the diagnosis. Although a shunt directly draining into the inferior vena cava was the most common type and managed by a simple direct anastomosis of the portal vein at LT, some cases required the modification of the portal vein reconstruction, such as interposition. Thirty patients were alive with a median follow-up period of 18 months. CONCLUSIONS LT for CEPS showed an excellent outcome. The development of pulmonary complications is an early indication for LT. Precise planning of portal vein reconstruction is required before LT.
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Schaeffer DF, Laiq S, Jang HJ, John R, Adeyi OA. Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt. Hum Pathol 2013; 44:432-7. [DOI: 10.1016/j.humpath.2012.08.018] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2012] [Revised: 08/21/2012] [Accepted: 08/22/2012] [Indexed: 10/27/2022]
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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.
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Affiliation(s)
- Florent Guérin
- Department of Pediatric Surgery, Bicêtre Hospital, Paris, France.
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van Steenbeek FG, van den Bossche L, Leegwater PAJ, Rothuizen J. Inherited liver shunts in dogs elucidate pathways regulating embryonic development and clinical disorders of the portal vein. Mamm Genome 2011; 23:76-84. [PMID: 22052005 PMCID: PMC3275728 DOI: 10.1007/s00335-011-9364-0] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/30/2011] [Accepted: 10/13/2011] [Indexed: 12/14/2022]
Abstract
Congenital disorders of the hepatic portal vasculature are rare in man but occur frequently in certain dog breeds. In dogs, there are two main subtypes: intrahepatic portosystemic shunts, which are considered to stem from defective closure of the embryonic ductus venosus, and extrahepatic shunts, which connect the splanchnic vascular system with the vena cava or vena azygos. Both subtypes result in nearly complete bypass of the liver by the portal blood flow. In both subtypes the development of the smaller branches of the portal vein tree in the liver is impaired and terminal branches delivering portal blood to the liver lobules are often lacking. The clinical signs are due to poor liver growth, development, and function. Patency of the ductus venosus seems to be a digenic trait in Irish wolfhounds, whereas Cairn terriers with extrahepatic portosystemic shunts display a more complex inheritance. The genes involved in these disorders cannot be identified with the sporadic human cases, but in dogs, the genome-wide study of the extrahepatic form is at an advanced stage. The canine disease may lead to the identification of novel genes and pathways cooperating in growth and development of the hepatic portal vein tree. The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis. Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.
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Affiliation(s)
- Frank G van Steenbeek
- Faculty of Veterinary Medicine, Utrecht University, Yalelaan 104, PO Box 80154, 3508 TD Utrecht, The Netherlands.
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Sanada Y, Urahashi T, Ihara Y, Wakiya T, Okada N, Yamada N, Egami S, Hishikawa S, Kawano Y, Ushijima K, Otomo S, Sakamoto K, Nakata M, Yasuda Y, Mizuta K. The role of operative intervention in management of congenital extrahepatic portosystemic shunt. Surgery 2011; 151:404-11. [PMID: 21962400 DOI: 10.1016/j.surg.2011.07.035] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2010] [Accepted: 07/08/2011] [Indexed: 01/07/2023]
Abstract
BACKGROUND AND AIMS Congenital extrahepatic portosystemic shunt (CEPS) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. It is still a matter of debate whether conservative or operative strategies should be used to treat symptomatic CEPS. The aim of this study was to evaluate the role of operative intervention in the management of CEPS. METHODS Between June 2004 and August 2010, 6 consecutive patients with symptomatic CEPS were treated in our department. There were 3 male and 3 female patients, with a median age of 3.5 years (range, 1-8). Their demographic, clinical, and laboratory data were analyzed. All patients were scheduled to undergo shunt ligation or liver transplantation (LT). RESULTS Living donor LT was carried out in 4 patients, and shunt ligation in 2. After a median follow-up of 25 months, all the patients are alive currently with marked relief of symptoms. CONCLUSION Shunt ligation or LT for symptomatic CEPS is potentially curative.
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Affiliation(s)
- Yukihiro Sanada
- Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan.
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Bekdache GN, Hamdan MA, Begam MA, Chedid F, Tamim MM, Mirghani H. Prenatal diagnosis of extrahepatic umbilicoportosystemic shunt: impact on postnatal management. J OBSTET GYNAECOL 2011; 31:542-3. [DOI: 10.3109/01443615.2011.580395] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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