Percutaneous Transhepatic Biliary Drainage in a Two-Month-Old Infant with Inspissated Bile Syndrome

Inspissated bile syndrome (IBS) is a relatively rare condition. Many treatment options are available, including medication, surgery, and surgical interventions, such as insertion of cholecystostomy drain, endoscopic retrograde cholangiopancreatography, internal biliary drainage, and percutaneous transhepatic biliary drainage (PTBD). We herein report the first case of IBS that was successfully treated with PTBD in a two-month-old infant in Korea. PTBD was initiated on postnatal day 72. On postnatal day 105, we confirmed complete improvement and successfully removed the catheters. This report suggests that PTBD is a viable and safe treatment option for obstructive jaundice in very young infants.

Use of Omega-3 Polyunsaturated Fatty Acids to Treat Inspissated Bile Syndrome: A Case Report / 대한소아소화기영양학회지

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

Obstructive jaundice in neonates.

The causes of jaundice in the first few weeks of life may be categorised into hematologic, enzymatic/metabolic, infectious and obstructive. Obstructive jaundice results from an interruption in the drainage of bile in the biliary system. Surgical causes of jaundice in neonates are biliary atresia, inspissated bile syndrome, intrahepatic hypoplasia, choledochal cyst, Caroli’s disease and spontaneous perforation of the bile duct. Pediatricians should be aware of the pernicious consequences of unresolved biliary obstruction and should thus refer neonates or infants with inexplicable jaundice for surgical exploration at an earlier age.