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Biliary Disorders, Anomalies, and Malignancies in Children
Curtis L Simmons1, Laura K Harper1, Mittun C Patel1
1From the Department of Radiology, Phoenix Children's Hospital, 1919 E Thomas Rd, Main Tower, Phoenix, AZ 85016 (C.L.S., M.C.P., R.N.S., L.G., D.R.B.); Department of Radiology, Mayo Clinic, Phoenix, Ariz (L.K.H.); Department of Radiology, UT Health San Antonio, San Antonio, Tex (V.S.K.); and Baylor College of Medicine, Houston, Tex (E.T.).
Insights
Pediatric biliary disorders are diverse, ranging from congenital anomalies to inflammatory conditions. Accurate diagnosis relies on a multimodal imaging approach, with ultrasound as the initial tool, complemented by MRI and MRCP for detailed assessment.
Area of Science:
- Pediatric Radiology
- Gastroenterology
- Medical Imaging
Background:
- Biliary abnormalities in children present a broader spectrum than in adults, are rarely neoplastic, and often stem from congenital or embryologic origins.
- Common pediatric biliary disorders include anatomical gallbladder anomalies, anomalous pancreaticobiliary tracts, cholestatic processes, congenital cysts, and genetic conditions, alongside benign entities like motility and inflammatory disorders.
Purpose of the Study:
- To outline the diverse range of pediatric biliary disorders and emphasize the challenges in their diagnosis using a single imaging modality.
- To highlight the essential role of a multimodal imaging strategy in the accurate diagnosis and management of pediatric biliary abnormalities.
Main Methods:
- Ultrasound (US) is the primary imaging modality for initial evaluation due to its accessibility, safety, and cost-effectiveness.
- Magnetic Resonance Imaging (MRI), Computed Tomography (CT), and nuclear medicine provide complementary anatomic and functional information.
- Hepatobiliary-specific contrast-enhanced MRI and Magnetic Resonance Cholangiopancreatography (MRCP) offer superior visualization of biliary anatomy compared to traditional MRI and Endoscopic Retrograde Cholangiopancreatography (ERCP).
Main Results:
- Distinguishing between various pediatric biliary entities is challenging with a single imaging technique.
- A combination of US and MRCP is crucial for diagnosing conditions like choledochal cysts.
- Suspected biliary atresia necessitates a multimodal diagnostic approach for timely intervention.
Conclusions:
- A comprehensive understanding of imaging appearances is vital for appropriate workup, differential diagnosis, and management of pediatric biliary abnormalities.
- Multimodal imaging, integrating US, MRI, and MRCP, is essential for accurate diagnosis and guiding treatment strategies in pediatric biliary disorders.
- Biopsy may be required for definitive diagnosis of both benign and malignant biliary masses in children.
Abstract:
Biliary abnormalities in children are uncommon, and the spectrum of biliary disorders is broader than in adult patients. Unlike in adults, biliary disorders in children are rarely neoplastic and are more commonly rhabdomyosarcoma rather than cholangiocarcinoma. Pediatric biliary disorders may be embryologic or congenital, such as anatomic gallbladder anomalies, anomalous pancreaticobiliary tracts, various cholestatic processes, congenital cystic lesions, or genetic conditions. They may also be benign, such as biliary filling anomalies, biliary motility disorders, and biliary inflammatory and infectious disorders. Distinguishing these entities with a single imaging modality is challenging. US is the primary imaging modality for initial evaluation of biliary abnormalities in children, due to its wide availability, lack of ionizing radiation, and low cost and because it requires no sedation. Other examinations such as MRI, CT, and nuclear medicine examinations may provide anatomic and functional information to narrow the diagnosis further. Hepatobiliary-specific contrast material with MRI can provide better assessment of biliary anatomy on delayed images than can traditional MRI contrast material. MR cholangiopancreatography (MRCP) allows visualization of the intra- and extrahepatic biliary ducts, which may not be possible with endoscopic retrograde cholangiopancreatography (ERCP). Suspected biliary atresia requires multiple modalities for diagnosis and timely treatment. Determining the type of choledochal cyst calls for a combination of initial US and MRCP. Many benign and malignant biliary masses require biopsy for definitive diagnosis. Knowledge of the imaging appearances of different pediatric biliary abnormalities is necessary for appropriate imaging workup, providing a diagnosis or differential diagnosis, and guiding appropriate management. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.
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