Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Apr 27, 2020; 12(4): 160-169
Published online Apr 27, 2020. doi: 10.4254/wjh.v12.i4.160
Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature
Roberta Angelico, Guglielmo Paolantonio, Monica Paoletti, Chiara Grimaldi, Maria Cristina Saffioti, Lidia Monti, Manila Candusso, Massimo Rollo, Marco Spada
Roberta Angelico, Monica Paoletti, Chiara Grimaldi, Maria Cristina Saffioti, Marco Spada, Department of Abdominal Transplantation and Hepatobiliary and Pancreatic Surgery, Bambino Gesù Children’s Hospital IRCCS, Rome 00165, Italy
Roberta Angelico, Department of Surgery, Hepato-bilio-pancreatic Surgery and Transplant Unit, University of Rome Tor Vergata, Fondazione PVT, Rome 00133, Italy
Guglielmo Paolantonio, Massimo Rollo, Interventional Radiology Unit, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, Rome 00165, Italy
Lidia Monti, Department of Imaging, Bambino Gesù Children’s Hospital IRCCS, Rome 00165, Italy
Manila Candusso, Division of Hepatogastroenterology, Bambino Gesù Children’s Hospital IRCCS, Rome 00165, Italy
Author contributions: Angelico R, Paolantonio G, Spada M and Rollo M designed research; Candusso M, Paoletti M and Grimaldi G performed research; Saffioti MC, Candusso M and Monti L contributed analytic tools; Angelico R, Paolantoni G and Paoletti M analyzed data; Angelico R and Spada M wrote the paper.
Informed consent statement: Informed consent was obtained from the patient for publication of their information.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Marco Spada, MD, PhD, FEBS, Department of Abdominal Transplantation and Hepatobiliary and Pancreatic Surgery, Bambino Gesù Children’s Hospital IRCCS, Piazza Sant'Onofrio 4, Rome 00165, Italy. marco.spada@opbg.net
Received: October 21, 2019
Peer-review started: October 21, 2019
First decision: November 22, 2019
Revised: February 17, 2020
Accepted: February 23, 2020
Article in press: February 23, 2020
Published online: April 27, 2020
Abstract
BACKGROUND

Congenital intrahepatic arterioportal fistula (IAPF) is a rare vascular malformation in infants that causes severe portal hypertension (PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children. Furthermore, indications are not defined.

CASE SUMMARY

We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multi-step endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised. The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascular-surgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.

CONCLUSION

The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

Keywords: Liver, Intrahepatic arterioportal fistula, Congenital malformation, Portal hypertension, Radiological embolization, Hepatic surgery, Case report

Core tip: Complex congenital intrahepatic arterioportal fistula (IAPF) is a rare vascular malformation causing severe portal hypertension, gastrointestinal hemorrhage and ascites in infants, for whom the therapeutic approach is challenging due to the children’s small size and their poor clinical condition. Radiological embolization often isn’t effective for complex lesions due to impossibility to embolize small afferent arterial branches, while surgical treatments (liver resection or liver transplantation) are associated with severe morbidity and mortality. We aimed to present a novel endovascular-surgical hybrid approach in an infant with complex congenital IAFP, providing a literature review on the treatment options and outcomes for congenital IAFP.