Partial internal biliary diversion for patients with progressive familial intrahepatic cholestasis type 1.

Division of Pediatric Surgery, Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Pediatric Surgery International (Impact Factor: 1.22). 01/2012; 28(1):51-4. DOI: 10.1007/s00383-011-3018-x
Source: PubMed

ABSTRACT We herein report a case of progressive familial intrahepatic cholestasis with partial internal biliary diversion (PIBD). Although by using PIBD an external stoma can be avoided, exposure of the ileocecal junction to bile reflux as well as the effects of the direct bile flow on the colonic mucosa require further investigation.

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    ABSTRACT: Background We speculated that Roux-en-Y cholecysto-colonic diversion was as effective for treating children with progressive familial intrahepatic cholestasis (PFIC) as partial biliary diversion. The feasibility of the novel approach in bypassing bile was investigated in rabbits. Methods 24 rabbits were randomly divided into three groups: sham operated group (Group1), 30 cm limb group (Group 2), and 10 cm limb group (Group 3). Group 2 or 3 underwent a Roux-en-Y cholecystocolonic anastomose with a 30-or 10-cm-long Roux limb. 99mTcEHIDA dynamic biligraphy was used to detect alterations of bile flow among the three groups at 1 year postoperatively. TBA levels and histological changes were also evaluated. Results All animals survived and developed normally without clinical symptoms during 1 year follow-up. Bile was diverted into colon directly after cholecystocolonic anastomosis. In group 3, E20 and E35 values were (77.27 ± 6.15) % and (90.39 ± 1.49) % respectively. Gallbladder emptying was accelerated in 10 cm short limb group than in 30 cm long limb group. The ratio of bile shunt was (0.547 ± 0.182), which was also more than that in group 2 (p < 0.05). The activity-time curve for the gallbladder area in group 2 looks like a wave. A significant reduction in TBA level was observed in group 2 and 3 (p < 0.05). Conclusions Roux-en-Y cholecystocolonic bypass was safe, feasible. Its effectiveness is related to the length of Roux loop. Cholecystocolonic bypass led to a significant loss of bile acids in healthy rabbits and might be considered for bile diversion in pediatric patients with selected cholestatic diseases.
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