Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease
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Published on: March 25, 2022
Huiqi Yang1, Robert J Porte, Henkjan J Verkade
1Department of Paediatric Surgery, University Medical Centre Groningen/Beatrix Children's Hospital, Groningen, The Netherlands.
Insights
Partial external biliary diversion (PEBD) relieved pruritus in 75% of children with cholestatic liver diseases. This treatment improved bile salt levels and growth in most patients, but longer follow-up is needed.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Interventions for Liver Disease
Background:
- Progressive familial intrahepatic cholestasis (PFIC) and Alagille disease are severe pediatric liver conditions.
- Partial external biliary diversion (PEBD) is a surgical option for these conditions, but long-term outcomes are not well-established.
Observation:
- A retrospective review analyzed 14 children (11 PFIC, 3 Alagille) who underwent PEBD between 2000-2005.
- Preoperative characteristics included significant pruritus, growth failure (50% <-2 SDS), elevated bile salts (median 318 µmol/L), and varying degrees of liver fibrosis.
Findings:
- At a median follow-up of 3.1 years, 75% of patients experienced relief from pruritus.
- Postoperative improvements were observed in bile salt concentrations (decreased in mild/moderate fibrosis) and growth (27% below -2 SDS at 2 years).
- One patient (7%) required liver transplantation; PEBD did not reduce bile salts in those with severe preoperative fibrosis.
Implications:
- PEBD offers significant symptom relief and biochemical/growth improvements in pediatric cholestatic liver diseases.
- The procedure's effectiveness in severe fibrosis and its potential to delay or prevent liver transplantation require further long-term investigation.
Background:
Partial external biliary diversion (PEBD) is a promising treatment for children with progressive familial intrahepatic cholestasis (PFIC) and Alagille disease. Little is known about long-term outcomes.
Patients And Methods:
A retrospective chart review of all patients undergoing PEBD in the University Medical Centre of Groningen (UMCG).
Results:
Between 2000 and 2005, PEBD was performed on 14 children with severe pruritus (PFIC 11, mean age 5.3 +/- 4.4 years; Alagille 3, mean age 7.4 +/- 4.2 years). Stature was <-2 standard deviation score (SDS) in 50%. Median preoperative serum bile salt concentration was 318 micromol/L (range 23-527 micromol/L). Twenty-nine percent had severe liver fibrosis and 71% had mild or moderate fibrosis. Median follow-up was 3.1 years (range 2.0-5.7 years). One patient (7%) underwent a liver transplantation at 3.2 years post-PEBD. Two years postoperatively, 50% were without pruritus and 21% had mild pruritus. In 29%, pruritus had not diminished; 3 of them had severe fibrosis preoperatively. In patients with mild or moderate fibrosis, PEBD decreased serum bile salts (105 micromol/L [range 8-269 micromol/L] 2 years postoperatively). Bile salts did not decrease in the patients with severe fibrosis. Two years after PEBD, 27% had a stature below -2 SDS.
Conclusions:
At median follow-up of 3.1 years after PEBD, pruritus has been relieved in 75%. Bile salts level and growth are improved in most patients. Longer follow-up is needed to determine whether PEBD can postpone or avoid the demand for liver transplantation.