Cystic Fibrosis Liver Disease: Endovascular, endoscopic and surgical
considerations
Abstract
Advanced cystic fibrosis liver disease (aCFLD) with portal hypertension
develops in a minority of people (5-7.5%) with Cystic Fibrosis (CF),
necessitating surgical interventions, including portosystemic shunting,
gastrostomy tube placement, and in some cases, liver transplantation.
This manuscript also aims to explore the different endoscopic,
endovascular and surgical procedures needed to diagnose and manage
aCFLD, including liver biopsy, endoscopic retrograde
cholangiopancreatography, intraoperative cholangiograms, hepatic venous
pressure gradient measurement and portosystemic shunt procedures in
patients with aCFLD. By utilizing advanced diagnostic and surgical
techniques, healthcare professionals can effectively manage aCFLD and
improve patient outcomes.