Internal biliary diversion may become a therapeutic option to treat severe symptoms of Alagille syndrome (ALGS), according to a study recently published in the Journal of Surgical Case Reports.

“It should be considered prior to transplantation when evaluating surgical options for patients with [ALGS] and symptoms refractory to medical management,” the authors wrote.

This case series described 3 patients previously diagnosed with ALGS, 2 females and 1 male aged 12, 2, and 1 year, respectively. They all had persistent cholestasis that did not respond to medical treatment with ursodeoxycholic acid, rifampin, and at least 1 of the following drugs: antihistamines or bile acid-binding resins.


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Given the latter scenario, the patients underwent an internal biliary diversion through a Roux-en-Y cholecystocolostomy, 2 via a laparoscopic-assisted approach, and 1 through open surgery, with a single incision in the right upper quadrant.

The patients’ preoperative pruritus scores were grade 4 in 2 of them and grade 1 in the other. Only 1 had xanthomas classified as grade 4 that led to disability and disfigurement. All postoperative pruritus scores were grade 1, and the xanthoma further decreased to grade 3.

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The postsurgical period transcurred without complications in 2 cases, while the other patient evacuated a hematoma during the immediate postoperative period, undergoing a second surgery, and observed no active bleeding. However, the same patient presented with cholangitis treated with antibiotics, and about 1 year after the initial surgery, he received a liver transplant due to progressive liver failure.

This surgical approach has been previously described for patients with other cholestatic diseases, such as progressive familial intrahepatic cholestasis. Regardless, this may be the first report on individuals with ALGS.

Although 1 patient did present with complications that ended up requiring a liver transplant, the results of this study call for further research on this technique to consider it as the first-line option. Despite the fact that complications after an internal biliary diversion may exist, the significant morbidity transplant carriers have for the remaining of their life makes it important to consider exhausting all other alternatives before selecting this approach.

“Cholecystocolostomy improves patient symptoms without physiologic derangement and can be performed using minimally invasive techniques,” the authors concluded.

Reference

Durgin J, Crum R, Kim H, Cuenca A. Outcomes of internal biliary diversion using cholecystocolostomy for patients with severe Alagille syndrome. J Surg Case Rep. Published online July 4, 2022. doi:10.1093/jscr/rjac307