Sclerosing cholangitis with neonatal onset |
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| Authors: | O Amedee-Manesme O Bernard F Brunelle M Hadchouel C Polonovski J J Baudon P Beguet D Alagille |
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| Affiliation: | 1. Department of Paediatric Surgery, Kings College Hospital, London, UK (now, Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan);2. Institute of Liver Studies, Kings College Hospital, London, UK (now, Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan);1. Department of Paediatric Surgery, Institute of Medical, Sciences, Banaras Hindu University, Varanasi, U.P;2. Department of Radiology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, U.P;1. Department of Plastic, Reconstructive, and Aesthetic Surgery, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba, Chiba, 260-8670, Japan;2. Department of Plastic, Reconstructive, and Aesthetic Surgery, Japan Red Cross Maebashi Hospital, 389-1 Asakura-machi, Maebashi, Gunma, 371-0811, Japan;3. Department of Plastic and Reconstructive Surgery, Chiba Cancer Center Hospital, 666-2 Nitona-cho, Chuo-ku, Chiba, Chiba, 260-8717, Japan;4. Department of Plastic and Reconstructive Surgery, Shinyurigaoka General Hospital, 255 Furusawatuko, Asou-ku, Kawasaki, Kanagawa, 215-0026, Japan |
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| Abstract: | Sclerosing cholangitis is characterized by irregular narrowing of extrahepatic or intrahepatic bile ducts, and in adults is defined mainly by radiologic findings. We describe eight children with cholestasis from the first week of life, followed by early cirrhosis and portal hypertension. Histologic examination of the liver showed absence of interlobular bile ducts in the early cholestatic phase in two patients and biliary cirrhosis later in all patients. Radiologic examination by percutaneous cholecystography under ultrasound guidance, carried out at age 8 months to 9 years, disclosed abnormal intrahepatic bile ducts with rarefaction of segmental branches, stenosis, and focal dilation. The extrahepatic ducts were involved in six patients. No intestinal disease has been found in these patients. |
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