PostKasai Portoenterostomy Dissection of a Branch of the Right Hepatic Artery – A Rare Complication

ABSTRACT

Hepatic artery branch dissection is a lesser-known complication after biliary atresia surgery. The presentation is massive upper gastrointestinal bleeding and rapid drop in hemoglobin levels. We present a 6-week-old infant with biliary atresia who had dissection of a branch of the right hepatic artery which was managed by angioembolization.

INTRODUCTION

The Kasai portoenterostomy (KPE) remains the primary surgical intervention that aims to restore bile flow in infants with biliary atresia. Dissection of a hepatic artery branch is rare after KPE and has not yet been reported in English literature. It presents a significant challenge due to its potential for massive upper gastrointestinal (GI) bleeding, posing a life-threatening risk to the patient. Here, we present a case of hepatic artery branch dissection following KPE.

CASE REPORT

A 6-week-old healthy and active male infant underwent intraoperative cholangiogram and KPE for extrahepatic biliary atresia. Preoperatively, his liver function test suggested a total bilirubin of 10 mg% with >50% direct component; liver enzymes were borderline; complete blood count, renal function test, and the coagulation profile were normal. The fasting and postprandial ultrasonography and hepatobiliary iminodiacetic acid scan suggested obstructed drainage. Cytomegalovirus immunoglobulins (Ig) G and IgM titers were raised in both the mother and baby.

Postoperatively, he passed greenish-yellow stools by 3^rd postoperative day and icterus started decreasing. Feeds were started on the 5^th postoperative day, which was well-tolerated by the patient. On the 8^th postoperative day, he developed multiple episodes of massive upper GI bleed in the form of blood mixed stools and blood in vomitus. The baby was transfused with packed red blood cells (RBC), and simultaneously, the cause of GI bleed was investigated. Nuclear RBC scan suggested bleeding from the portoenterostomy site [Figure 1]. Digital subtraction angiography (DSA) confirmed dissection in the right hepatic artery branch from its origin [Figure 2a] which was embolized by 66% glue (N-butyl cyanoacrylate). Postembolization, the affected area could be seen being supplied by collaterals [Figure 2b].

Figure 1.

Image of nuclear red blood cells scan suggesting bleeding from the portoenterostomy site

Figure 2.

Digital subtraction angiography showing dissection in the right hepatic artery branch from its origin (a) and postembolization image (b) showing the area supplied by the embolized artery being supplied by collaterals

The baby recovered well. He is now asymptomatic and on follow-up.

DISCUSSION

To the best of our knowledge, no such case has been reported after Kasai procedure. Similar complications reported in literature are hepatic artery pseudoaneurysms; their causes have been variously reported in literature to be – hypertension, vascular diseases, pancreatitis, diabetes mellitus, autoimmune disease, infections, malignancies, trauma, regional inflammation or infection, or biliary intervention.[1,2,3,4]

In KPE, the obliterated biliary remnant is excised, and the portal plate is drained with a Roux-en-Y portoenterostomy at the level of porta hepatis just above the bifurcation of the portal vein. The hepatic artery and its branches lie posterior to the portal vein bifurcation. Being in the vicinity of the vascular hilum renders it vulnerable to injury while dissection of porta hepatis and the anastomosis. Such subtle injuries while taking sutures of the anastomosis or while achieving hemostasis might go unnoticed and later lead to dissection of the artery and its consequences. This probably explains the etiopathogenesis of the arterial dissection in our case.

The presenting symptoms are jaundice, biliary colic, and GI hemorrhage (Quincke’s triad).[2] Our patient had undergone KPE wherein a greater flow of (infected) bile might have washed up and corroded the already injured (by cautery/dissection) intimal layer of the right hepatic artery branch leading to the dissection and GI hemorrhage. Computed tomography angiography, RBC scan, and DSA help in diagnosis.[2] DSA is therapeutic as well in the form of endovascular treatment.[2]

CONCLUSION

Identifying and isolating the vascular structures intraoperatively, especially at anastomosis, can help to prevent inadvertent injury to the vasculature. Prompt identification and diagnosis of postoperative GI bleed in such patients is mandatory for management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.