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Congenital hepatic fibrosis with an unusual pulmonary lesion
  1. Roger Williams,
  2. Peter J. Scheuer,
  3. Brian E. Heard
  1. Department of Medicine, Royal Free Hospital, London
  2. Department of Pathology, Royal Free Hospital, London
  3. Department of Pathology, Postgraduate Medical School, London


    The necropsy findings in a 5-year-old girl with congenital hepatic fibrosis are described. She had been followed since the age of 18 months and finally developed hepatocellular failure. Extensive haemorrhagic necrosis was found at necropsy and was probably related to the terminal illness. Other unusual findings were areas of typical post-necrotic cirrhosis and marked centrilobular and diffuse emphysema of the lungs. It is suggested that the latter was also congenital in origin.

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