Hum Pathol 1980 Sep;11(5 Suppl):549-60

Morphometric analysis of liver lesions in cystic diseases of childhood

Landing BH, Wells TR, Claireaux AE

Abstract
Morphometric analysis of the liver lesions in patients with various forms of
cystic disease of the liver in childhood demonstrates that the entities called
the congenital, perinatal, and infantile forms of polycystic disease produce the
same liver lesion. The data suggest that these entities are actually the same
disorder, for which the name infantile polycystic disease (of the liver and
kidneys) is recommended. In all patients with this autosomal recessive disorder
there is involvement of both the liver and the kidneys, and patients present
with hepatonephromegaly at birth or in early infancy. Similarly, the entity
called the juvenile form of polycystic disease produces the same liver lesion as
does the disease to which the term congenital hepatic fibrosis has been applied
to describe patients presenting in later childhood with portal hypertension. The
latter name is recommended to distinguish this condition from infantile
polycystic disease. Nephromegaly is not a feature of this also autosomal
recessive disease, but renal insufficiency, progressing slowly through later
childhood and adolescence, is. The hepatic lesions of infantile polycystic
disease and congenital hepatic fibrosis differ from those of other disorders
causing cystic lesions of the liver and kidneys, either regularly (Meckel's
syndrome, Jeune's syndrome) or variably (vaginal atresia syndrome, tuberous
sclerosis, medullary cystic disease)