Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome.

Abstract

BACKGROUND:

Progressive familial intrahepatic cholestasis (PFIC) with low gamma-glutamyl transpeptidase (GGT) and Alagille syndrome are associated with persistent cholestasis and severe pruritus. Various types of biliary diversion have been used to reduce this pruritus and prevent liver dysfunction. We report our experience concerning the efficacy and safety of total biliary diversion (TBD) as an additional treatment option.

METHODS:

TBD was performed in four PFIC patients and one patient with Alagille syndrome, and was accomplished by anastomosing a jejunal segment to the choledochal duct terminating as an end stoma, or by disconnecting the choledochal duct after previous cholecystojejunocutaneostomy.

RESULTS:

TBD resulted in a marked improvement of symptoms and biochemical parameters in all PFIC patients. Despite relief of pruritus, cholestasis persisted in the Alagille patient. During 5-15years of follow-up, no clinical signs of fat malabsorption such as diarrhea or weight loss were encountered. However, to maintain adequate levels of fat-soluble vitamins, especially of vitamin K, substantial supplementation was necessary.

CONCLUSIONS:

Total biliary diversion can be a useful surgical treatment option for patients with low-GGT PFIC and possibly also Alagille syndrome, when partial biliary diversion is insufficient. It can be performed without inducing clinical signs of fat malabsorption although individualized supplementation of fat-soluble vitamins with careful monitoring is warranted.

• Authors: Wendy L van der Woerd, Freddy T Kokke, David C van der Zee, Roderick H Houwen
• Journal: Journal of pediatric surgery (J Pediatr Surg) Vol. 50 Issue 11 Pg. 1846-9 (Nov 2015) ISSN: 1531-5037 [Electronic] United States
• PMID: 26319776 (Publication Type: Journal Article)
• Copyright: Copyright © 2015 Elsevier Inc. All rights reserved.

Chemical References

• gamma-Glutamyltransferase

Topics

• Adolescent
• Alagille Syndrome (surgery)
• Anastomosis, Surgical (methods)
• Child
• Child, Preschool
• Cholestasis (complications, surgery)
• Cholestasis, Intrahepatic (enzymology, surgery)
• Female
• Humans
• Infant
• Jejunum (surgery)
• Liver (surgery)
• Male
• Pruritus (etiology, surgery)
• Surgical Stomas
• gamma-Glutamyltransferase (deficiency)