Journal of clinical and experimental hepatology | 2021
Portal Cavernoma Cholangiopathy in Children: An Evaluation Using Magnetic Resonance Cholangiography and Endoscopic Ultrasound
Abstract
Abstract Background Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extra hepatic portal venous obstruction (EHPVO) using Endoscopic Ultrasound (EUS) and Magnetic Resonance Cholangiography/Portovenography (MRC/MRPV). Methods 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity. Results All 53 children (100%) had PCC changes on MRC/EUS but none were symptomatic. Extra as well as intrahepatic ducts were involved in majority (85%) and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intra hepatic ducts whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the extra hepatic ducts. Increased CBD angulation predisposed to CBD strictures (p=0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade. Conclusions PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC.