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Alagille Syndrome

Internal biliary diversion with cholecystocolostomy can be considered for ALGS

Posted on July 8, 2022

In this case report, use of internal biliary diversion with Roux-en-Y cholecystocolostomy in 3 patients with severe pruritus due to Alagille syndrome (ALGS) is described.

This procedure has not been previously described for ALGS, although it has been for other biliary conditions.

Preoperative and post-operative lab values and symptom scores, with 0 being none and 4 being severe, were compared in a retrospective analysis. Mean preoperative pruritus and xanthoma scores were 3.33 (range, 2-4) and 1.33 (range, 0-4) and mean post-operative score was 1 and 1 at 2-month follow-up, respectively.

“Roux-en-Y cholecystocolostomy can be considered for ALGS, which is refractory to medical management,” the authors concluded. “This procedure accomplishes internal biliary diversion without significant physiologic derangements.”

Reference
Durgin JM, Crum R, Kim HB, et al. Outcomes of internal biliary diversion using cholecystocolostomy for patients with severe Alagille syndrome. J Surg Case Rep. 2022;2022(7):rjac307. doi: 10.1093/jscr/rjac307. PMID: 35794995; PMCID: PMC9252331.

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