Abstract
Purpose
Children with cholestatic disorders have undergone liver transplantation for intractable pruritus unresponsive to medical therapy even in the absence of liver failure. Biliary diversion procedures interrupt the entero-hepatic circulation of bile acids allowing them to be excreted in the feces thereby lowering the total bile acid pool. We evaluated the outcome of partial internal biliary diversion (PIBD) in children with intractable pruritus from inherited cholestatic disorders.
Methods
The records of children who underwent PIBD over a 4-year period were reviewed for etiology of liver disease, demographic data, preoperative and postoperative biochemical profile and improvement of pruritus. Standard statistical methods were used for analysis.
Results
Of the 12 children, 10 had progressive familial intrahepatic cholestasis (PFIC) and 2 had Alagille syndrome (AS). PIBD was done using an isolated jejunal loop as a conduit from gall bladder to mid ascending colon. Median period of follow up was 30 months. Pruritus resolved in nine children with significant reduction of serum bile acids (P < 0.02).
Conclusion
To our knowledge, this is the largest reported series of children with PIBD. PIBD is a safe, well-tolerated and effective alternative to liver transplant in children with PFIC and AS who have intractable pruritus in the absence of synthetic liver failure.
Similar content being viewed by others
References
Jacquemin E (2012) Progressive familial intrahepatic cholestasis. Clin Res Hepatol Gastroenterol 36:S26–S35
Bull LN, Carlton VE, Stricker NL, Bhaharloo S, De Young JA, Freimer NB et al (1997) Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity. Hepatology 26(1):155–164
Lee CN, Tiao MM, Chen HJ et al (2013) Characteristics and outcome of liver transplantation in children with alagille syndrome. A single-center experience. Pediatr Neonatol 55(2):135–138
Kronsten V, Fitzpatrick E, Baker A (2013) Management of cholestatic pruritus in pediatric patients with Alagille syndrome: the King’s College Hospital experience. J Pediatr Gastroenterol Nutr 57(2):149–154
Wanty C, Joomye R, Van Hoorebeek N et al (2004) Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy. Acta Gastroenterol Belg 67(4):313–319
Ismail H, Kalcinski P, Markiewicz M et al (1999) Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant 3(3):219–224
Bassas A, Chehab M, Hebby H et al (2003) Living related liver transplantation in 13 cases of progressive familial intrahepatic cholestasis. Transplant Proc. 35(8):3003–3005
Mattei P, von Allmen D, Piccoli D, Rand E (2006) Relief of intractable pruritus in Alagille syndrome by partial external biliary diversion. J Pediatr Surg 41(1):104–107
Whitington PF, Whitington GL (1988) Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 95(1):130–136
Bustorff-Silva J, SbraggiaNeto L, Olimpio H, de Alcantara RV, Matsushima E, De Tommaso A (2007) Partial internal biliary diversion through a cholecystojejunocolonic anastomosis-a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report. J Pediatr Surg 42(8):1337–1340
Ganesh R, Suresh N, Sathiyasekaran M, Ramachandran P (2011) Partial internal biliary diversion: a solution for pruritus in progressive familial intrahepatic cholestasis type 1. Saudi J Gastroenterol 17(3):212–214
Sheflin-Findling S, Arnon R, Lee S, Chu J, Henderling F, Kerkar N, Iyer K (2012) Partial internal biliary diversion for Alagille syndrome: case report and review of the literature. J Pediatr Surg 47(7):1453–1456
Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnwelt CA (1998) Ileal exclusion for Byler’s disease: an alternative surgical approach with promising early result for pruritus. J Pediatr Surg 33(2):220–224
Kalicinski PJ, Ismail H, Jankowska I et al (2003) Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 13(5):307–311
Metzelder ML, Petersen C, Melter M, Ure BM (2006) Modified laparoscopic external biliary diversion for benign recurrent intrahepatic cholestasis in obese adolescents. Pediatr Surg Int 22(6):551–553
Diao M, Li L, Zhang JS, Ye M, Cheng W (2013) Laparoscopic cholecystocolostomy: a novel surgical approach for the treatment of progressive familial intrahepatic cholestasis. Ann Surg 258(6):1028–1033
Kurbegov AV, Setchell KD, Haas JE et al (2003) Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile. Gastroenterology 125(4):1227–1234
Melter M, Rodeck B, Kardoff R et al (2000) Progressive familial intrahepatic cholestasis: partial internal biliary diversion normalizes serum lipids and improves growth in non-cirrhotic patients. Am J Gastroenterol 95(12):3522–3528
Jankowska I, Czubkowski P, Kalicinski P et al (2014) Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 58(1):92–95
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ramachandran, P., Shanmugam, N.P., Sinani, S.A. et al. Outcome of partial internal biliary diversion for intractable pruritus in children with cholestatic liver disease. Pediatr Surg Int 30, 1045–1049 (2014). https://doi.org/10.1007/s00383-014-3559-x
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-014-3559-x