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Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report

INTRODUCTION AND IMPORTANCE: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic porta...

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Autores principales: Tamiru, Raja, Hailemariam, Tesfahunegn, Wakjira, Eyasu, Hailu, Samuel Sisay
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10391669/
https://www.ncbi.nlm.nih.gov/pubmed/37481969
http://dx.doi.org/10.1016/j.ijscr.2023.108553
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author Tamiru, Raja
Hailemariam, Tesfahunegn
Wakjira, Eyasu
Hailu, Samuel Sisay
author_facet Tamiru, Raja
Hailemariam, Tesfahunegn
Wakjira, Eyasu
Hailu, Samuel Sisay
author_sort Tamiru, Raja
collection PubMed
description INTRODUCTION AND IMPORTANCE: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities. CASE PRESENTATION: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences. CLINICAL DISCUSSION: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS. CONCLUSION: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.
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spelling pubmed-103916692023-08-02 Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report Tamiru, Raja Hailemariam, Tesfahunegn Wakjira, Eyasu Hailu, Samuel Sisay Int J Surg Case Rep Case Report INTRODUCTION AND IMPORTANCE: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities. CASE PRESENTATION: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences. CLINICAL DISCUSSION: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS. CONCLUSION: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis. Elsevier 2023-07-20 /pmc/articles/PMC10391669/ /pubmed/37481969 http://dx.doi.org/10.1016/j.ijscr.2023.108553 Text en © 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Tamiru, Raja
Hailemariam, Tesfahunegn
Wakjira, Eyasu
Hailu, Samuel Sisay
Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title_full Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title_fullStr Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title_full_unstemmed Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title_short Congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: A case report
title_sort congenital extrahepatic portosystemic shunt masquerading as chronic portal vein thrombosis: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10391669/
https://www.ncbi.nlm.nih.gov/pubmed/37481969
http://dx.doi.org/10.1016/j.ijscr.2023.108553
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