{
  "abstract": "Progressive familial intrahepatic cholestasis type 1 (PFIC1), or Byler disease, is a rare genetic disorder that disrupts bile flow in children. 1 Although liver transplantation (LT) offers a curative option, post-LT patients with PFIC1 often experience complications like chronic refractory diarrhoea and graft steatosis due to bile acid malabsorption.2 3 Surgical biliary diversion is commonly used before or after LT to alleviate symptoms such as severe diarrhoea and pruritus by reducing bile acid load.4 Recently, ileal bile acid transporter (IBAT) inhibitors have shown promise as a non-surgical alternative for managing symptoms in PFIC1 patients without prior LT.5 However, not all PFIC1 patients require biliary diversion post-LT, due to variability in symptom presentation and timing of complications in addition to the unclear full pathophysiology of the disease. To address these challenges, we developed a novel technique for marking the Roux loop during transplantation.This marking allows for minimally invasive access to the Roux loop for surgical biliary diversion if it becomes necessary post-transplant, providing a flexible and less invasive management option for these patients if needed.This retrospective observational case series involved three paediatric patients with PFIC1 who underwent liver transplantation at ages 2.5 years, 3 years and 7 months, and 4.5 years respectively .All patients underwent transplantation due to end-stage liver disease. Two of these patients had biliary diversion pre transplant(one required it post transplant as well), and 1 case did not have biliary diversion before or after transplant .All three cases had a novel technique of marking their Roux loop by radiopaque marker during the LT procedure (figure 1). This marking aimed to facilitate less invasive future interventions if biliary diversion (BD) was required. Of the three patients, one developed severe refractory diarrhoea with severe hepatic steatosis post-LT and underwent partial external biliary diversion via interventional radiology, utilizing the pre-marked Roux loop without the need for major surgery. The remaining two patients did not need biliary diversion at the time of the study, as they exhibited only mild hepatic steatosis and their diarrhoea was not severe. In the case requiring biliary diversion (performed 7 months after second liver transplantation due to severe watery diarrhoea), the patient exhibited significant clinical and histopathological improvements following the procedure.The frequency of bowel movements decreased as well as the loperamide dose. Liver histology showed a reduction in hepatic steatosis from severe to moderate, with no progression of fibrosis. Due to stomal complications and patient preference, the external biliary diversion was later converted to an internal/external biliary diversion with a jejuno-colonic anastomosis.After this modification, the patient demonstrated good weight gain and reduced stool frequency to 2–3 times daily.Marking the Roux loop enabled efficient and minimally invasive access to the biliary system for the intervention, avoiding more invasive surgical approaches.In conclusion, marking the Roux loop during liver transplantation for PFIC1 is an innovative technique that allows for simpler, less invasive management of post-transplant complications. Further research is needed to confirm these benefits in larger patient groups.Abstract OC54 Figure 1Radiopaque marker in Roux loop for post-transplant accessReferences Nakanishi Y, Saxena R. Pathophysiology and diseases of the proximal pathways of the biliary system. Arch Pathol Lab Med. 2015;139:858–66.Nguyen KD, Sundaram V, Ayoub WS. Atypical causes of cholestasis. World J Gastroenterol. 2014;20:9418–26.Egawa H, Yorifuji T, Sumazaki R, et al. Intractable diarrhea after liver transplantation for Byler’s disease: successful treatment with bile adsorptive resin. Liver Transpl. 2002;8:714–6.Shanmugam N, Reddy MS, Anand L, et al. Total internal biliary diversion for post-liver transplant PFIC-1-related allograft injury. J Clin Exp Hepatol. 2022;12(1):212.Loomes KM, Squires RH, Kelly D, et al. Maralixibat for the treatment of PFIC: long-term, IBAT inhibition in an open-label, phase 2 study. Hepatol Commun. 2022;6(9):2379–2390.",
  "authors": [
    {
      "affiliations": [
        "Birmingham Children’s Hospital",
        "Ain Shams University"
      ],
      "name": "AA Amin"
    },
    {
      "affiliations": [
        "Birmingham Children’s Hospital"
      ],
      "name": "GL Gupte"
    },
    {
      "affiliations": [
        "Birmingham Children’s Hospital"
      ],
      "name": "AK Unny"
    },
    {
      "affiliations": [
        "Birmingham Children’s Hospital"
      ],
      "name": "K Sharif"
    }
  ],
  "title": "OC54 Novel technique for management of post-transplant refractory diarrhoea in children with PFIC type 1",
  "uid": "26b1d785-c51d-5c08-8d7a-9baaccec83a3"
}