Clinical caseAssociation of N-acetylcysteine and glucagon during percutaneous cholangiography in the treatment of inspissated bile syndromeEfficacité de l’association N-acétylcystéine-glucagon au cours de la cholangiographie percutanée dans le traitement du syndrome de bile épaisse
Introduction
Inspissated bile syndrome (IBS) remains an infrequent disease in the newborn. In IBS, extrahepatic bile ducts become obstructed by solid plugs of inspissated bile or dense bile sludge. Standard treatment of IBS is percutaneous transhepatic cholangiography with saline irrigation of the biliary tract. Despite the application of this recommended management, some biliary tract obstructions may persist. We report on the case of a 3-month-old infant with continuous biliary obstruction caused by IBS, which was successfully treated with the association of N-acetylcysteine (NAC) and glucagon.
Section snippets
Case report
This female infant first presented in the emergency room at 2.5 months of age. The pregnancy had been uneventful and the infant was born by cesarean section at 40 weeks of gestation. Her birthweight was 4300 g. There was no previous family history of cholelithiasis or hemolytic disorders. After an asymptomatic period, the parents observed icterus and clay colored stools at 15 days of age. The observations were confirmed by the physician in charge and the initial laboratory tests were consistent
Discussion
Conjugated hyperbilirubinemia of the neonate is a condition requiring careful investigation [1]. The diagnosis of biliary atresia must first be eliminated. IBS is a less common cause of infantile jaundice. It is defined as an obstruction of the common bile duct by bile sludge in full-term infants without anatomical abnormalities or congenital chemical bile defects. In a significant proportion of neonates, no recognized predisposing factors are present; however, in some situations, it is a
Conclusion
To our knowledge, this case is the first reported treatment of IBS using a combination of intravenous glucagon and in-situ N-acetylcysteine in an infant. We suggest that management of IBS in neonates with interventional radiology could be improved by the addition of glucagon and N-acetylcysteine. Their use, even as first-line agents, would allow more rapid clearance of the sludge and could prevent possible infectious complications of indwelling catheters as well as decrease the need for
Disclosure of interest
The authors declare that they have no conflicts of interest concerning this article.
Acknowledgements
The authors thank Richard Medeiros, Rouen University Hospital Medical Editor, and Geraldine Pettersen, MD, Sherbooke University Hospital, for their valuable advice in editing the manuscript.
References (7)
- et al.
Cholelithiasis in infancy: a study of 40 cases
J Pediatr
(1993) - et al.
Percutaneous management of bile-plug syndrome: a case report
J Pediatr Surg
(2011) - et al.
Addition of N-acetylcysteine to aqueous model bile systems accelerates dissolution of cholesterol gallstones
Gastroenterology
(1990)
Cited by (6)
Pediatric Cholestatic Syndromes
2017, Zakim and Boyer's Hepatology: A Textbook of Liver DiseaseSuccessful Treatment of Refractory Inspissated Bile Syndrome Using Gastrografin
2023, Journal of Clinical Interventional Radiology ISVIRIn vitro effect of 20% n-acetylcysteine on the viscosity of normal canine bile
2019, American Journal of Veterinary ResearchSAGES clinical spotlight review: intraoperative cholangiography
2017, Surgical EndoscopyPediatric cholangiopathies: diseases of the gallbladder and biliary tract
2017, Abdominal Radiology