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DOI: 10.1055/s-0032-1308695
Ileal Exclusion for Intractable Pruritus in Alagille Syndrome
Publication History
16 September 2011
29 September 2011
Publication Date:
09 May 2012 (online)
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Introduction
Intractable pruritus due to an accumulation of bile acids in the circulation is the most debilitating symptom of Alagille syndrome (AGS), leading to a markedly impaired quality of life.[1] [2] Some patients are refractory to conventional medical therapy.[1] [3] In these patients, effective treatment requires surgical intervention, either biliary diversion[1] [4] [5] or liver transplantation.[2] Liver transplantation is also considered to treat AGS-related symptoms even in the absence of liver failure or cirrhosis,[1] [2] [4] although only 10% of AGS patients progress to end-stage liver disease.
Compared with liver transplantation, partial external biliary diversion (PEBD) represents a less invasive procedure to effectively treat pruritus in patients with progressive cholestasis.[1] [4] [6] [7] This technique interrupts enterohepatic bile acid circulation in an attempt to decrease the bile acid pool by using a conduit between the dome of the gallbladder and the skin to allow external drainage of bile.[1] [3] Although several reports have studied the use of this technique in progressive familial intrahepatic cholestasis (PFIC), there is less data available on the treatment of symptoms in AGS.[1] [4] [5] [6]
Terminal ileal exclusion (IE) is an alternative surgical method for internal biliary diversion and avoids the need for an external biliary fistula.[8] In this technique, 15% of the terminal portion of the small intestine is bypassed through direct anastomosis of the proximal ileum to the ascending colon.[8] This procedure has been used in PFIC,[8] but there is only one study demonstrating the effective use of IE in the treatment of AGS.[3]
This case report was approved by our institutional review board (approval number: 1073-2011), and written informed consent for the procedure was obtained from the patient.
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References
- 1 Emerick KM, Whitington PF. Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome. Hepatology 2002; 35 (6) 1501-1506
- 2 Kamath BM, Schwarz KB, Hadzić N. Alagille syndrome and liver transplantation. J Pediatr Gastroenterol Nutr 2010; 50 (1) 11-15
- 3 Modi BP, Suh MY, Jonas MM, Lillehei C, Kim HB. Ileal exclusion for refractory symptomatic cholestasis in Alagille syndrome. J Pediatr Surg 2007; 42 (5) 800-805
- 4 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 (1) 104-107 , discussion 104–107
- 5 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 (2) 216-221
- 6 Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988; 95 (1) 130-136
- 7 Metzelder ML, Bottländer M, Melter M, Petersen C, Ure BM. Laparoscopic partial external biliary diversion procedure in progressive familial intrahepatic cholestasis: a new approach. Surg Endosc 2005; 19 (12) 1641-1643
- 8 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 (2) 220-224