Findings in percutaneous trans-hepatic cholecysto-cholangiography in neonates and infants presenting with conjugated hyperbilirubinemia: emphasis on differential diagnosis and cholangiographic patterns

Dimitri A Parra; Shannon E Peters; Ruhail Kohli; Racha Chamlati; Bairbre L Connolly; Justyna M Wolinska; Vicky L Ng; Michael J Temple; Philip R John; Binita M Kamath; Simon C Ling; Annie Fecteau; Afsaneh Amirabadi; Joao G Amaral

doi:10.1186/s12887-022-03816-y

Findings in percutaneous trans-hepatic cholecysto-cholangiography in neonates and infants presenting with conjugated hyperbilirubinemia: emphasis on differential diagnosis and cholangiographic patterns

BMC Pediatr. 2023 Jan 14;23(1):22. doi: 10.1186/s12887-022-03816-y.

Authors

Affiliations

¹ Division of Image Guided therapy, Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. dimitri.parra@sickkids.ca.
² Department of Family & Community Medicine, University of Toronto, Toronto, ON, Canada.
³ Division of Gastroenterology and Hepatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
⁴ Department of Psychiatry, St. Elizabeth's Medical Center, Brighton, MA, USA.
⁵ Division of Image Guided therapy, Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
⁶ Division of Pediatric Surgery, Children's Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada.
⁷ Division of Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
⁸ Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Abstract

Background: Biliary atresia (BA) is one of the causes of conjugated hyperbilirubinemia in infants which if untreated leads to end-stage liver disease and death. Percutaneous Trans-hepatic Cholecysto-Cholangiography (PTCC) is a minimally invasive study which can be utilized in the diagnostic work-up of these patients. This study's purpose is to describe the experience with PTCC in neonates, the imaging findings encountered, and the abnormal patterns which warrant further investigation.

Methods: A 16-year single-center retrospective study of patients with persistent neonatal cholestasis (suspected BA) undergoing PTCC. Patient demographics, laboratory values, PTCC images, pathology and surgical reports were reviewed.

Results: 73 patients underwent PTCC (68% male, mean age 8.7 weeks, mean weight 4.0 Kg). The majority of studies were normal (55%). Abnormal patterns were identified in 33 cases, 79% were diagnosed with BA and 12% with Alagille syndrome. Non-opacification of the common hepatic duct with a narrowed common bile duct (42%) and isolated small gallbladder (38%) were the most common patterns in BA.

Conclusion: PTCC is a minimally invasive study in the diagnostic work-up of infants presenting with conjugated hyperbilirubinemia (suspected BA). Further invasive investigations or surgery can be avoided when results are normal.

Keywords: Biliary atresia; Conjugated hyperbilirubinemia, Alagille’s syndrome; PTCC; Pediatric cholangiography.

MeSH terms

Biliary Atresia* / diagnosis
Biliary Atresia* / diagnostic imaging
Cholangiography / methods
Cholestasis* / diagnostic imaging
Cholestasis* / etiology
Diagnosis, Differential
Female
Gallbladder / diagnostic imaging
Humans
Hyperbilirubinemia / etiology
Infant
Infant, Newborn
Male
Retrospective Studies