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Inspissated bile syndrome: A rare cause of neonatal cholestasis

  • Shan-Ming Chen
    Affiliations
    Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan

    Department of Pediatrics, School of Medicine, Chung Shan Medical University, Taichung, Taiwan
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  • Yuan-Ya Liao
    Affiliations
    School of Medicine, Chung Shan Medical University, Taichung, Taiwan

    Department of Surgery, Chung Shan Medical University Hospital, Taichung, Taiwan
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  • Ching-Pin Lin
    Correspondence
    Corresponding author. Division of Hepatology and Gastroenterology, Department of Internal Medicine, Chung Shan Medical University Hospital, No. 110 Jianguo North Road, Section 1, Taichung City, 40201, Taiwan. Tel.: +886 4 2473 9595 ext. 32332; fax: +886 4 2471 0934.
    Affiliations
    Division of Hepatology and Gastroenterology, Department of Internal Medicine, Chung Shan Medical University Hospital, Taichung, Taiwan

    Institute of Biochemistry, Microbiology and Immunology, Chung Shan Medical University, Taichung, Taiwan
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Open AccessPublished:April 30, 2020DOI:https://doi.org/10.1016/j.pedneo.2020.03.002
      A male neonate with a birthweight of 3220 g was transferred to our hospital on the eighth day of life because of progressive neonatal hyperbilirubinemia. His serum total bilirubin and direct bilirubin levels were 22.3 and 0.8 mg/dL, respectively. After 72 h of adequate hydration and phototherapy, these levels had increased to 26.0 and 5.8 mg/dL, respectively. Other liver function tests were normal except for gamma-glutamyl transpeptidase (234 IU/L) and alkaline phosphatase (230 IU/L). An obstruction of the common bile duct (CBD) due to biliary sludge was demonstrated on abdominal ultrasonography (Fig. 1A and B) at 12 days of age and in follow-up magnetic resonance cholangiopancreatography 3 days after ultrasound examination (Fig. 2). Intraoperative cholangiography via the punctured gall bladder was performed at 16 days of age because of no dissolution of the bile sludge and no improvements in bilirubin levels after oral ursodeoxycholic acid (UDCA) therapy. Inspissated black sludge was noted in the drainage catheter after saline lavage of the bile duct (Supplementary Fig. S1). Ultrasonography on the fourth day post operation revealed significant clearance and decreased the diameter of the CBD (Supplementary Figure S2). The patient's serum bilirubin levels returned to normal 40 days after surgery, and other biochemical test results normalized at 3 months of age.
      Fig. 1
      Fig. 1Abdominal ultrasonography showed mild intrahepatic biliary dilatation and a dilated common bile duct (CBD) (6 mm) filled with a slurry-like substance (A; indicated by arrow). A transverse ultrasound scan of the CBD showed that it was dilated and filled with slurry (B).
      Fig. 2
      Fig. 2Magnetic resonance cholangiopancreatography showed a small gallbladder, diffuse moderate dilatation of intra- and extrahepatic biliary ducts, and abrupt tapering of the CBD.
      Inspissated bile syndrome (IBS) is defined as extrahepatic obstruction of the bile duct by bile sludge without congenital biliary malformations, bile acid synthesis defects, or hepatocellular causes of jaundice. The estimated incidence of IBS is 1 in 175,000 live births, accounting for 8% of all types of surgical jaundice during infancy.
      • Bollu B.K.
      • Dawrant M.J.
      • Thacker K.
      • Thomas G.
      • Chenapragadda M.
      • Gaskin K.
      • et al.
      Inspissated bile syndrome; Safe and effective minimally invasive treatment with percutaneous cholecystostomy in neonates and infants.
      Several risk factors have been associated with the formation of biliary sludge in infancy, including prematurity, parenteral nutrition, dehydration, hemolytic diseases, cystic fibrosis, congenital heart diseases, cefotaxime, ceftriaxone, and sepsis.
      • Bollu B.K.
      • Dawrant M.J.
      • Thacker K.
      • Thomas G.
      • Chenapragadda M.
      • Gaskin K.
      • et al.
      Inspissated bile syndrome; Safe and effective minimally invasive treatment with percutaneous cholecystostomy in neonates and infants.
      In some cases, biliary sludge has been reported to resolve spontaneously with or without UDCA treatment.
      • Chang S.H.
      • Joo S.M.
      • Yoon C.S.
      • Lee K.H.
      • Lee S.M.
      Percutaneous transhepatic biliary drainage in a two-month-old infant with inspissated bile syndrome.
      Surgical intervention is indicated when the bile duct is dilated to >3 mm with persistent jaundice.
      • Chang S.H.
      • Joo S.M.
      • Yoon C.S.
      • Lee K.H.
      • Lee S.M.
      Percutaneous transhepatic biliary drainage in a two-month-old infant with inspissated bile syndrome.
      ,
      • Berger S.
      • Schibli S.
      • Stranzinger E.
      • Cholewa D.
      One-stage laparoscopic surgery for inspissated bile syndrome: case report and review of surgical techniques.
      Berger et al. described their clinical experience of successfully treating a 6-week-old infant with IBS via laparoscope-aided cholecystostomy with biliary duct lavage.
      • Berger S.
      • Schibli S.
      • Stranzinger E.
      • Cholewa D.
      One-stage laparoscopic surgery for inspissated bile syndrome: case report and review of surgical techniques.

      Declaration of Competing Interest

      The authors declare no conflicts of interest regarding this manuscript.

      Appendix A. Supplementary data

      Figs1
      Figs1Inspissated black sludge was noted in the drainage catheter after intraoperative cholangiography with saline lavage of the bile duct.
      Figs2
      Figs2Postoperative ultrasonography on the fourth day revealed significant clearance and a decreased CBD diameter (<2 mm).

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