Laparoscopic aided cholecystostomy as a treatment of inspissated bile syndrome Anna Gunnarsdóttir a, ⁎ , Peter Holmqvist b , Einar Arnbjörnsson a , Carl-Magnus Kullendorff a a Department of Pediatric Surgery, Lund University Hospital, 221 85 Lund, Sweden b Department of Pediatrics, Lund University Hospital, 221 85 Lund, Sweden Received 1 November 2007; accepted 18 December 2007 Key words: Inspissated bile syndrome; Bile sludge; Bile plug syndrome; Cholecystostomy; Children; Ursodeoxycholic acid Abstract We report a case of a newborn girl with inspissated bile syndrome (IBS) that did not respond to treatment with oral ursodeoxycholic acid (Ursofalk). A solution was found using laparoscopic aided cholecystostomy with an indwelling catheter for local Ursofalk flushing in the gallbladder and the choledochus. This is the first report of a laparoscopic aided management of IBS without cholecystectomy or exploration of the bile ducts. This minimal invasive approach showed a clear advantage for the patient. There were no complications. The method is recommended in the treatment of IBS. © 2008 Elsevier Inc. All rights reserved. Neonatal jaundice is common, seen in 25% to 50% of all normal newborn infants, and requires rapid evaluation [1]. It is usually physiologic, unconjugated, and self-limiting. Less common cause of infantile jaundice is a rise in conjugated bilirubin owing to obstruction of the extrahepatic bile ducts, often needing surgical solutions. A metabolic derangement leading to biliary sludge (BS) or inspissated bile is also found in children with cystic fibrosis. The first report of inspissated bile syndrome (IBS) causing obstruction of the biliary tract was by Ladd [2] in 1935. The diagnosis is based on medical history and typical ultrasound features with dilated biliary tract and partly ductal obstruction after the exclusion of other known causes. Jaundice and weight stagnation accompanying more or less alcoholic stools and hepatomegaly are most often the only signs of this disease. The onset of jaundice in IBS occurs later than in biliary atresia. In the 1950s to 1960s, this condition was well recognized, giving obstructive jaundice, because of the prevalence of untreated maternal Rh and ABO blood group incompa- tibility. Today, IBS is less common, and the incidence in England is reported to be 1 in 175,000 live births and counts for about 8% of all surgical jaundice during infancy [3]. The treatment of IBS is generally percutaneous trans- hepatic cholangiography with contemporary therapeutic saline lavage of the biliary tree. Percutaneous transhepatic cholangiography in an infant is a difficult procedure because of the small size of the intrahepatic gall ducts. Moreover, it is sometimes difficult to obtain a sufficient flushing pressure. Failure of this treatment will entail a laparotomy for drainage of the inspissated bile [4]. Here, we, for the first time, report a successful resolution of IBS in an otherwise healthy infant with laparoscopic aided cholecystostomy, leaving a catheter in place to be used for local irrigation of the biliary tracts. ⁎ Corresponding author. Tel.: +46 46 17 83 05; fax: +46 46 17 22 99. E-mail address: anna.gunnarsdottir@med.lu.se (A. Gunnarsdóttir). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.12.068 Journal of Pediatric Surgery (2008) 43, E33–E35