Ileal Exclusion for Intractable Pruritus in Alagille Syndrome

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.

• 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