We present a case of a 14-month-old male who was referred to our facility with complete failure to thrive since birth and had developed melena stools intermittently for 1 month. The haemoglobin at presentation was 7.0 g/dl. The patient was seen at a peripheral facility but the referral to a specialist was delayed. An ultrasound and contrast-enhanced CT of the abdomen was performed which showed a 2.4cm x 2.2cm tubular structure in the right hepatic lobe with early arterial filling from the left hepatic artery and draining into left portal vein (Figure 1 and 2). Early high flow retrograde arterial contrast filling of the left portal vein, the portal vein and the superior mesenteric vein, splenic vein and their tributaries was also seen. The portal vein, left portal vein, common hepatic and left hepatic artery were prominent. No other liver lesion was seen. There were also features of portal hypertension with periportal collateral vessels.
A multidisciplinary meeting was held to discuss the treatment options between the paediatrician, paediatric surgeon and interventional radiologist. The child was a poor surgical candidate as he was severely malnourished with a fairly central lesion in the right lobe of the liver. Therefore, a decision was made to attempt to treat at least the arterial inflow via an endovascular approach.
The procedure was performed under general anaesthesia. Under ultrasound guidance, a 4Fr 7cm hydrophilic sheath (Angiogate TransRadial Introducer Kit by Kimal, United Kingdom) was placed retrogradely into the right common femoral artery. Intraarterial hourly heparin bolus was administered to prevent thrombosis at the access site and lower limb. Access to the celiac axis and then common hepatic artery was obtained using hydrophilic standard angled guide wire (Merit Laureate by MeritMedical, Ireland) and 4 Fr Cobra 2 catheter (Performa Angiographic catheter by MeritMedical, Mexico). The left intrahepatic arterioportal malformation was demonstrated receiving blood supply from the left hepatic artery and draining into left portal venous vein (Figure 3).
A decision was made to perform coil embolization of the main feeder as distally possible using a 2.4Fr microcatheter (Progreat by Terumo) to deploy a 4mm detachable micro-coils (Concerto by Medtronic, The Netherlands). There was some flow still noted and hence a second 5mm detachable micro-coil (Concerto by Medtronic, The Netherlands) was put in place which stopped the flow (Figure 4).
There was good outcome with one tiny feeder opening immediately post embolisation. digital subtraction angiography of the superior mesenteric artery showed no additional supply to the fistula.
An ultrasound scan performed 1 week after the procedure confirmed thrombosis of the fistula with antegrade flow in the portal vein (Figure 4). This was maintained on further follow up scans.
A further hospital admission 2 months after the procedure was necessary to manage refeeding syndrome. However, the patient did well clinically for a number of months once discharge, gaining significant weight and no further melaena. Unfortunately, he succumbed 8 months after the procedure secondary to an unrelated, acute respiratory illness.