A case report of a challenging diagnosis of biliary atresia in a patient receiving total parenteral nutrition

• Published in: BMC pediatrics Vol. 19; no. 1; p. 72
• Main Authors: Agawu, Atu, Wehrman, Andrew, Pogoriler, Jennifer, Terry, Natalie A, Lin, Henry C
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 08-03-2019; BioMed Central; BMC
• Subjects: Age; Bile; Biliary atresia; Biliary Atresia - complications; Biliary Atresia - diagnosis; Bilirubin - blood; Case Report; Case reports; Causes of; Cholestasis; Cholestasis - etiology; Complications and side effects; Diagnosis; Diagnosis, Differential; Enteral nutrition; Etiology; Gallbladder; Gallbladder diseases; Hospitals; Humans; Hyperbilirubinemia - etiology; Infant; Infants; Laboratories; Liver; Liver - pathology; Liver biopsy; Liver diseases; Male; Medical diagnosis; Medical prognosis; Medical tests; Natural history; Nutrition; Organ transplantation; Ostomy; Parenteral nutrition; Parenteral Nutrition, Total - adverse effects; Pediatrics; Surgery; Systematic review; Total parenteral nutrition; Transplants & implants; Ultrasonic imaging; United States; United States > US; Liver biopsy; Biliary atresia; Case report; Cholestasis; Total parenteral nutrition
• ISSN: 1471-2431; 1471-2431
• DOI: 10.1186/s12887-019-1446-2

Total parenteral nutrition (TPN) and biliary atresia (BA) are common causes of cholestasis in infancy. The diagnosis of BA is time sensitive due to an inverse correlation between age at intervention (hepatic portoenterostomy - HPE) and survival without liver transplantation. Clinical, laboratory, and histologic features of BA and parenteral nutrition associated cholestasis (PNAC) are similar, creating a diagnostic dilemma for cholestatic infants on parenteral nutrition. There is limited published information about the natural history of PNAC including time to resolution, or diagnostic tests that distinguish BA from other etiologies of cholestasis.
We present a case of a child diagnosed with BA whose cholestasis began while receiving TPN. His clinical course was notable for transient resolution of his cholestasis after stopping parenteral nutrition and ultimate intraoperative diagnosis.
Clinicians who care for patients who frequently receive TPN should be aware that clinical, laboratory, imaging, and biopsy findings can be similar between BA and PNAC.