Redkar RG*, Masarweh M**, Howard ER***, Karani J****, Miele-Vergani G*****, Davenport M******
Department of Paediatric Surgery, Institute of Liver Studies3, King's College Hospital, London. *, Department of Paediatric Surgery, Institute of Liver Studies3, King's College Hospital, London. **, Department of Paediatric Surgery, Institute of Liver Studies3, King's College Hospital, London. ***, Department of Radiology & Institute of Liver Studies3, King's College Hospital, London. ****, Institute of Liver Studies3, King's College Hospital, London. *****, Department of Paediatric Surgery,Institute of Liver Studies3, King's College Hospital, London. ******
Introduction: Obstructive jaundice caused by intraluminal bile plugs, sludge or gallstones is uncommon in infancy and has been termed the inspissated bile syndrome (IBS). The aim of this review was to analyze etiologic factors, and review recent experience with its treatment and long-term outcome.

Methods: A retrospective review of infants with IBS who presented to a single center during the period Jan 1994 to May 2005 was undertaken.

Clinical series: Fifty-two infants (37M:15F) presented at a median age of 12 (range 3 - 40 weeks with conjugated jaundice (n = 49) and acholic stools (n = 38). Possible predisposing factors were noted in 39 (75%). These included gastrointestinal pathology (n = 17), prematurity (n = 16), total parenteral nutrition (n = 14), sepsis (n = 13) and other miscellaneous causes like IUGR, birth asphyxia and hemolysis. Only thirteen (25%) infants had an uncomplicated neonatal history. Biliary ultrasound was the most useful primary investigation and diagnostic in 46 (89%). Inspissated bile was identified in 33, while the other 13 showed a dilated biliary tree. Percutaneous transhepatic cholangiography PTC) was performed in 26 (50%) infants and was therapeutic in 18 (flushing in 17, balloon sphincteroplasty in 1). ERCP (n = 4) and latterly MRCP )n = 5) were also useful in identifying the pathology. Underlying structural anomalies of the bile ducts were shown in 16 (30%) infants (choledochal malformation (n = 8), stricture (n = 3), abnormal ductal anatomy (n = 3) and a long common channel (n = 4). Laparotomy was performed in 13 (25%) infants. The procedures performed were cholecystectomy and hepatico-jejunostomy (n = 4), open flushing of the bile ducts (n = 7), cholecystectomy and reconstruction of the bile ducts (n = 1) and transduodenal sphincteroplasty (n = 1).

Results: Twenty-one (40%) had a spontaneous resolution of which 14 have an entirely normal biliary tract and liver biochemistry profile. Four patients in this group are monitored for gallstones while three have their dilated ducts observed. In the remainder, therapeutic PTC (n = 18, 34%) and open surgery (n = 13, 25%) were necessary to clear the biliary tract. Of the infants that had a PTC flushing, 9 have an entirely normal biliary tree with normal liver function tests while 6 have their dilated bile ducts being observed and 2 have silent gallstones. At a mean period of follow up of 3.7 years, thirty-three out of 49 infants (67%) are now jaundice-free with an apparently normal biliary tract. Six children (12%) have asymptomatic gall bladder stones. None (17%) children have non-obstructive, non-progressive extrahepatic duct dilatation biliary tree. One child with intermittent jaundice after PTC-flushing, proved to have familial intrahepatic cholestasis and underwent liver transplantation.

Conclusion: Inspissated bile syndrome is a cause of surgical jaundice in this age group and is of heterogenous aetiology. The majority will require intervention, either radiological or surgical, but the long-term outlook is excellent.
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