A pseudotumor, giant regenerative nodule, or macroregenerative nodule is an unusual benign hepatic lesion in biliary atresia (BA) patients. This tumor may mimic malignant transformation and may preclude liver transplantation (LT). The clinical and imaging surveillance of patients after the Kasai procedure is therefore an important aspect of management of BA patients. Our objective is to report our experience and describe the incidence, imaging, and pathologic features of pseudotumors in BA patients awaiting LT. From August 1990 to December 2006, 133 LTs for BA were performed. Five (3.8%; 4 female, 1 male) patients were diagnosed with pseudotumor. The patients' records were reviewed. The diagnostic imaging modalities used were abdominal ultrasound (US), computed tomography (CT) scan, and magnetic resonance imaging (MRI). Histologic confirmation of the lesions was obtained in all cases. All underwent the Kasai operation in early infancy. Six of 7 lesions in 4 of 5 patients were demonstrated by pretransplant imaging. Two of 7 tumors were detected by US. Five of 7 lesions were detected by CT, and 5 of 7 lesions were demonstrated by MRI. In 1 patient, the lesion was not seen in the US, CT, or MRI but was found during surgery and confirmed by histology. An additional tumor was found incidentally during histologic examination in a patient previously diagnosed to have 2 tumors by CT and MRI. In another patient diagnosed to have 2 tumors on imaging, pathology revealed only a single tumor. In conclusion, although unusual, pseudotumor should be included in the differential diagnosis of liver masses in BA children.
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http://dx.doi.org/10.1002/lt.21320 | DOI Listing |
Children (Basel)
January 2025
Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London SE5 9RS, UK.
Biliary atresia (BA) is an obliterative disease of the bile ducts affecting between 1 in 10,000-20,000 infants with a predominance in Asian countries. It is clinically heterogeneous with a number of distinct variants (e.g.
View Article and Find Full Text PDFBiomedicines
January 2025
Faculty of Health Sciences, Health Science Investigation Center of University of Beira Interior (CICS-UBI), 6200-506 Covilhã, Portugal.
Biliary atresia (BA) is a progressive hepatobiliary disease in infants, leading to liver failure and the need for transplantation. While its etiopathogenesis remains unclear, recent studies suggest primary cilia (PC) disruption plays a role. This study investigates correlations between PC and cytoplasmic tubulin (TUBA4A) alterations with hypoxia in patients with the isolated form of BA, focusing on native liver survival.
View Article and Find Full Text PDFFront Bioeng Biotechnol
January 2025
Department of Hepatobiliary Surgery, Haikou Affiliated Hospital of Central South University Xiangya School of Medicine, Haikou, Hainan, China.
Biliary duct injury, biliary atresia (BA), biliary tract tumors, primary sclerosing cholangitis (PSC), and other diseases are commonly encountered in clinical practice within the digestive system. To gain a better understanding of the pathogenesis and development of these diseases and explore more effective treatment methods, organoid technology has recently garnered significant attention. Organoids are three-dimensional structures derived from stem/progenitor cells that can faithfully mimic the intricate structure and physiological function of tissues or organs .
View Article and Find Full Text PDFClin Exp Hepatol
March 2024
Pediatric Surgery, Nagoya City University Midori Municipal Hospital, Japan.
Aim Of The Study: This study aimed to establish an objective, simple, and minimally invasive screening method to detect patients with biliary atresia during neonatal checkups by using indocyanine green (ICG) fluorescence in the stool.
Material And Methods: We produced a rat model of extrahepatic biliary obstruction (group O, = 9) and compared the stools from these rats with those of control group rats (group C, = 6) by a fluorescence technique. ICG was administered (0.
J Pediatr Gastroenterol Nutr
January 2025
Department of Pediatrics, Children's Hospital of Colorado, University of Colorado, Aurora, Colorado, USA.
Pediatric cholestatic liver diseases are rare conditions that can result from multiple specific underlying etiologies. Among the most common etiologies of pediatric cholestatic liver diseases are biliary atresia, Alagille syndrome (ALGS), and inherited disorders of bile acid transport. These diseases are characterized by episodic or chronic unremitting cholestasis.
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