Laparoscopic button cholecystostomy for progressive familial intrahepatic cholestasis in two children

Area of Science:

• Hepatology
• Pediatric Surgery
• Minimally Invasive Surgery

Background:

• Progressive familial intrahepatic cholestasis (PFIC) can lead to severe liver disease, including cirrhosis and failure.
• Partial external biliary diversion (PEBD) is a potential treatment to prevent liver transplantation in non-cirrhotic PFIC patients.
• Traditional PEBD techniques can be complex, necessitating the development of simpler alternatives.

Purpose of the Study:

• To introduce and evaluate a novel laparoscopic technique for PEBD using a button cholecystostomy.
• To assess the safety and efficacy of this button technique in pediatric PFIC patients.

Main Methods:

• Two boys with PFIC underwent a laparoscopic button cholecystostomy using a 3-trocar approach.
• A 14 French MIC KEY button was inserted into the gallbladder fundus and secured.
• Outcomes measured included pruritus, serum bile acids, total bilirubin, and liver enzymes over a 6-month follow-up.

Main Results:

• The laparoscopic button cholecystostomy procedure was performed without complications in both patients.
• Significant relief of pruritus was observed in both children due to adequate bile drainage.
• Serum bile acids markedly decreased in both patients, with liver enzymes and bilirubin remaining within normal ranges.
• Excellent patient and parent acceptance of the button device was reported.

Conclusions:

• Laparoscopic button cholecystostomy is a simple, safe, and effective technique for PEBD in pediatric PFIC patients.
• This method achieves sufficient bile flow, leading to pruritus relief and avoiding the need for enteric anastomosis.
• The button technique offers a viable alternative for managing PFIC, potentially preventing the need for liver transplantation.