Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease.
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.


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.
Yang
Huiqi
H
Department of Paediatric Surgery, University Medical Centre Groningen/Beatrix Children's Hospital, Groningen, The Netherlands.
Porte
Robert J
RJ
Verkade
Henkjan J
HJ
De Langen
Zacharias J
ZJ
Hulscher
Jan B F
JB
D016738:Alagille Syndrome / Q000150:complications / Q000601:surgery*; D001647:Bile Acids and Salts / Q000097:blood*; D001653:Bile Ducts, Intrahepatic / Q000601:surgery; D001662:Biliary Tract Surgical Procedures; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000235:genetics / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D030342:Genetic Diseases, Inborn / Q000601:surgery; D006128:Growth; D006801:Humans; D015994:Incidence; D007223:Infant; D008103:Liver Cirrhosis / Q000453:epidemiology / Q000209:etiology; D016031:Liver Transplantation / Q000706:statistics & numerical data; D008297:Male; D011537:Pruritus / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome
D001647:Bile Acids and Salts