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Case Reports
. 2019 Oct-Dec;24(4):294-296.
doi: 10.4103/jiaps.JIAPS_132_18.

Previously Undescribed Anomalies of Hepatic Artery and Portal Venous Anatomy in a Case of Extrahepatic Biliary Atresia and its Implications

Prabudh Goel  1 , Minu Bajpai  1 , Kanika Sharma  1 , Priyanka Naranje  2
Affiliations
Case Reports

Previously Undescribed Anomalies of Hepatic Artery and Portal Venous Anatomy in a Case of Extrahepatic Biliary Atresia and its Implications

Prabudh Goel et al. J Indian Assoc Pediatr Surg. 2019 Oct-Dec.

Abstract

A search on PubMed and Web of Science revealed scarcity of the literature on anomalies of hepatic artery or portal vein and the presence of arterioportal fistula in biliary atresia; although, it has long-lasting implications for both the patient and the surgeon, including hepato-pancreato-biliary surgeons, pediatric surgeons (who perform Kasai's portoenterostomy), liver transplant surgeons, and interventional radiologists. We report a case of extrahepatic biliary atresia with multiple anomalies involving the hepatic arteries, portal vein, cystic artery, arterioportal fistula and shunting, intrahepatic portal vein radicals, kidney, and external genitalia. The merits of the case from various standpoints including its implications for etiopathogenesis, caution during surgical anesthesia or postoperative management, and enrichment of the literature have been discussed.

Keywords: Anomalies; arterioportal shunting; congenital; extrahepatic biliary atresia; hepatic artery; hypospadias; liver transplant; portal vein; renal agenesis.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Arterial phase computed tomography angiography of maximum-intensity-projection images in coronal plane showing (a) replaced hepatic artery arising from the left gastric artery (horizontal arrow pointing left) and entering the liver through the left lobe. Splenic artery (vertical arrow pointing upward) was normal, (b) absent of the left kidney and the left renal artery (vertical arrow pointing downwards); the empty renal fossa is occupied by bowel loops
Figure 2
Figure 2
(a) Coronal venous phase of maximum-intensity-projection images showing the formation of the main portal vein (horizontal arrow facing left) at porta by the confluence of splenic vein (vertical arrow pointing down) and superior mesenteric vein (vertical arrow pointing upwards). (b) Normal caliber right branch of the portal vein (horizontal arrow pointing right) and the attenuated left portal vein (horizontal arrow pointing left). (c) Venous phase axial computed tomography image showing disorganized intrahepatic portal vein branches (arrows). (d) Arterial phase axial computed tomography image showing the transient hepatic attenuation difference phenomenon, observed as an area of enhancement in the left lobe of the liver (encircled) with early opacification of the left portal vein suggesting arterioportal shunt
See this image and copyright information in PMC

References

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    1. Braun MA, Collins MB, Wright P. An aberrant right hepatic artery from the right renal artery: Anatomical vignette. Cardiovasc Intervent Radiol. 1991;14:349–51. - PubMed

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