Biliary Diversion by Use of the Appendix (Cholecystoappendicostomy) in Progressive Familial Intrahepatic Cholestasis

Rebhandl, Winfried; Felberbauer, F. X.; Turnbull, Julia R.; Paya, Kurosh; Barcik, U.; Huber, Wolf Dietrich; Whitington, Peter F.; Horcher, Ernst

doi:10.1097/00005176-199902000-00026

Cited by 28 publications

(25 citation statements)

References 12 publications

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“…PIBD was performed through a cholecystojejunal and jejunal colic anastomosis, where a 15 to 20 cm segment of mid jejunum was constructed as a conduit between the gall bladder and colon. PEBD was performed through a novel surgical approach of cholecysto-appendicostomy [10]. Here, the appendix was used as the conduit between the gall bladder and abdominal skin.…”

Section: Resultsmentioning

confidence: 99%

Therapeutic Interventions in Progressive Familial Intrahepatic Cholestasis: Experience from a Tertiary Care Centre in North India

et al. 2011

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Medical therapy has limited value in managing symptoms of progressive familial intrahepatic cholestasis (PFIC). Liver transplantation (LT) is the only definite therapy for progressive liver failure and intractable pruritis. In recent years, biliary diversion (BD) has also shown therapeutic promise. This study was designed to review the experience of management and outcome of seven PFIC patients. Two children each had type II and III and three had type I/II PFIC, respectively. Medical treatment was successful in only one. Decompensated cirrhosis had already set in four children. They underwent a living related LT. There was one post transplant mortality. Remaining all children had a normal graft function at a mean follow-up of 50 months. One patient of PFIC type I/II received internal and another of PFIC type II received external BD. Both patients were asymptomatic at follow-up of 19 and 23 months respectively. Nontransplant surgical options should be offered to noncirrhotic children with PFIC.

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Section: Resultsmentioning

confidence: 99%

Therapeutic Interventions in Progressive Familial Intrahepatic Cholestasis: Experience from a Tertiary Care Centre in North India

et al. 2011

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“…12,13 It may be suitable in small infants with severe hepatomegaly and may thus result in the lower placement of gall bladder, in which the distance between the gall bladder and right lower quadrant is quite small.…”

Section: Discussionmentioning

confidence: 99%

“…Various types of external and internal biliary diversions such as a catheter cholecystostomy, 8,9 T-tube choledochostomy, 8 cholecystocutaneostomy, 9 cholecystocolonostomy, ileal bypass or ileal exclusion, 8,10,11 and a cholecystoappendicostomy 12,13 have been tried in order to control pruritus. All these operations have their own results, complications, advantages, and disadvantages.…”

Section: Introductionmentioning

confidence: 99%

“…9,14,15 Partial external biliary diversion has been used with promising results for pruritus, jaundice, and a hepatocellular histology in some patients. [9][10][11][12][13][15][16][17][18][19] In this study, our experience in using this technique and the early results of PEBD in patients with PFIC are reported with a review of the current literature on the topic.…”

Section: Introductionmentioning

confidence: 99%

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Partial external biliary diversion for the treatment of intractable pruritus in children with progressive familial intrahepatic cholestasis: Report of two cases

et al. 2008

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Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120-200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.

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“…The consequences of this procedure certainly include interruption of the enterohepatic circulation of bile acids [14][15][16][17]. An external stoma can also be formed by using the appendix creating a cholecystoappendicectomy as reported by Rebhandl et al [18]. Another alternative is ileal exclusion (IE), which has also been performed in cases with previous cholecystectomy or as rescue treatment [2,15,19,20].…”

Section: Fic1 Deficiencymentioning

confidence: 99%

Familial Intrahepatic Cholestasis

Grammatikopoulos

Thompson

2015

Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Biliary Diversion by Use of the Appendix (Cholecystoappendicostomy) in Progressive Familial Intrahepatic Cholestasis

Cited by 28 publications

References 12 publications

Therapeutic Interventions in Progressive Familial Intrahepatic Cholestasis: Experience from a Tertiary Care Centre in North India

Therapeutic Interventions in Progressive Familial Intrahepatic Cholestasis: Experience from a Tertiary Care Centre in North India

Partial external biliary diversion for the treatment of intractable pruritus in children with progressive familial intrahepatic cholestasis: Report of two cases

Familial Intrahepatic Cholestasis

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