Partial Biliary Diversion May Promote Long-Term Relief of Pruritus and Native Liver Survival in Children with Cholestatic Liver Diseases

Abstract

Introduction Rare cholestatic liver diseases may cause debilitating pruritus in children. Partial biliary diversion (PBD) may relieve pruritus and postpone liver transplantation which is the only other alternative when conservative treatment fails. The aim was to report long-term outcome after PBD in a population of 26 million people during a 25-year period.

Materials and Methods This is an international, multicenter retrospective study reviewing medical journals. Complications were graded according to the Clavien–Dindo classification system.

Results Thirty-three patients, 14 males, underwent PBD at a median of 1.5 (0.3–13) years at four Nordic pediatric surgical centers. Progressive familial intrahepatic cholestasis was the most common underlying condition. Initially, all patients got external diversion, either cholecystojejunostomy (25 patients) or button placed in the gallbladder or a jejunal conduit. Early complications occurred in 14 (42%) patients, of which 3 were Clavien–Dindo grade 3. Long-term stoma-related complications were common (55%). Twenty secondary surgeries were performed due to stoma problems such as prolapse, stricture, and bleeding, or conversion to another form of PBD. Thirteen children have undergone liver transplantation, and two are listed for transplantation due to inefficient effect of PBD on pruritus. Serum levels of bile acids in the first week after PBD construction were significantly lower in patients with good relief of pruritus than in those with poor effect (13 [2–192] vs. 148 [5–383] μmol/L; p = 0.02).

Conclusion PBD may ensure long-term satisfactory effect on intolerable pruritus and native liver survival in children with cholestatic liver disease. However, stoma-related problems and reoperations are common.

Keywords

partial biliary diversion - cholestatic liver disease - progressive familial intrahepatic cholestasis

Publikationsverlauf

Eingereicht: 26. März 2020

Angenommen: 18. Juni 2020

Artikel online veröffentlicht:
24. Juli 2020

• References

• 1 Vitale G, Gitto S, Vukotic R, Raimondi F, Andreone P. Familial intrahepatic cholestasis: new and wide perspectives. Dig Liver Dis 2019; 51 (07) 922-933
• 2 Kamath BM, Baker A, Houwen R, Todorova L, Kerkar N. Systematic review: the epidemiology, natural history, and burden of Alagille syndrome. J Pediatr Gastroenterol Nutr 2018; 67 (02) 148-156
• 3 Drivdal M, Holven KB, Retterstøl K, Aagenaes Ø, Kase BF. A nine year follow-up study of patients with lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome). Scand J Clin Lab Invest 2018; 78 (7-8): 566-574
• 4 Yang H, Porte RJ, Verkade HJ, De Langen ZJ, Hulscher JB. Partial external biliary diversion in children with progressive familial intrahepatic cholestasis and Alagille disease. J Pediatr Gastroenterol Nutr 2009; 49 (02) 216-221
• 5 Ramachandran P, Shanmugam NP, Sinani SA. et al. Outcome of partial internal biliary diversion for intractable pruritus in children with cholestatic liver disease. Pediatr Surg Int 2014; 30 (10) 1045-1049
• 6 Diao M, Li L, Zhang JS, Ye M, Cheng W. Laparoscopic cholecystocolostomy: a novel surgical approach for the treatment of progressive familial intrahepatic cholestasis. Ann Surg 2013; 258 (06) 1028-1033
• 7 Arnell H, Papadogiannakis N, Zemack H, Knisely AS, Németh A, Fischler B. Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion. J Pediatr Gastroenterol Nutr 2010; 51 (04) 494-499
• 8 Bull LN, Pawlikowska L, Strautnieks S. et al. Outcomes of surgical management of familial intrahepatic cholestasis 1 and bile salt export protein deficiencies. Hepatol Commun 2018; 2 (05) 515-528
• 9 Wang KS, Tiao G, Bass LM. et al; Childhood Liver Disease Research Network (ChiLDReN). Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis. Hepatology 2017; 65 (05) 1645-1654
• 10 Clavien PA, Barkun J, de Oliveira ML. et al. The Clavien-Dindo classification of surgical complications: five-year experience. Ann Surg 2009; 250 (02) 187-196
• 11 Halaweish I, Chwals WJ. Long-term outcome after partial external biliary diversion for progressive familial intrahepatic cholestasis. J Pediatr Surg 2010; 45 (05) 934-937
• 12 Ismail H, Kaliciński P, Markiewicz M. et al. Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant 1999; 3 (03) 219-224
• 13 Erginel B, Soysal FG, Durmaz O, Celik A, Salman T. Long-term outcomes of six patients after partial internal biliary diversion for progressive familial intrahepatic cholestasis. J Pediatr Surg 2018; 53 (03) 468-471
• 14 Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988; 95 (01) 130-136
• 15 Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnweit CA. Ileal exclusion for Byler's disease: an alternative surgical approach with promising early results for pruritus. J Pediatr Surg 1998; 33 (02) 220-224
• 16 Kaliciński PJ, Ismail H, Jankowska I. et al. Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 2003; 13 (05) 307-311
• 17 Clifton MS, Romero R, Ricketts RR. Button cholecystostomy for management of progressive familial intrahepatic cholestasis syndromes. J Pediatr Surg 2011; 46 (02) 304-307
• 18 Schukfeh N, Gerner P, Paul A, Kathemann S, Metzelder M. Laparoscopic button cholecystostomy for progressive familial intrahepatic cholestasis in two children. Eur J Pediatr Surg 2014; 24 (05) 433-436
• 19 Arnell H, Bergdahl S, Papadogiannakis N, Nemeth A, Fischler B. Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis. J Pediatr Surg 2008; 43 (07) 1312-1320
• 20 Mattei P, von Allmen D, Piccoli D, Rand E. Relief of intractable pruritus in Alagille syndrome by partial external biliary diversion. J Pediatr Surg 2006; 41 (01) 104-107
• 21 Wassman S, Pfister ED, Kuebler JF. et al. Quality of life in patients with progressive familial intrahepatic cholestasis: no difference between post-liver transplantation and post-partial external biliary diversion. J Pediatr Gastroenterol Nutr 2018; 67 (05) 643-648