Journal of Ultrasound in Medicine, Vol 16, Issue 4 235-240, Copyright © 1997 by American Institute of Ultrasound in Medicine
High-resolution ultrasonography in the differential diagnosis of cystic diseases of the kidney in infancy and childhood: preliminary experience
M. Jain, G. W. LeQuesne, A. J. Bourne and P. Henning
Department of Paediatric Ultrasound, Women's and Children's Hospital, North Adelaide, South Australia.
Autosomal recessive polycystic kidney disease, autosomal dominant
polycystic disease, and glomerulocystic disease may all appear in the
perinatal period as bilaterally enlarged echogenic kidneys. Current
ultrasonographic equipment can better demonstrate the underlying pathologic
state and assist in the differentiation of these conditions. The primary
abnormality in autosomal recessive polycystic kidney disease is at the
level of the collecting ducts, which are dilated and saccular. The nephrons
remain normal. These dilated ectatic tubules are seen in their usual
distribution as a radial array, with major ducts being perpendicular to the
renal capsule, in both the renal cortex and the medulla. The peripheral
renal cortex does not normally contain collecting ducts and remains
unaffected in patients with mild disease. Autosomal dominant polycystic
disease is characterized by cystic changes involving both the nephron and
the collecting ducts. The nephron may become cystic at any point. Multiple
discrete cysts of varying sizes are seen in both the renal cortex and the
medulla in the severely affected infant. Subcapsular cysts are seen
regularly. Glomerulocystic disease is an unusual sporadic condition
characterized by the cystic dilation of the space of Bowman and the
proximal convoluted tubule. On ultrasonographic examination tiny, isolated
cysts, usually smaller than those occurring in autosomal dominant
polycystic kidney disease, are seen in the echogenic renal cortex and may
extend to the periphery of the kidney. No cysts are seen in the renal
medulla. Correlation between pathologic findings and sonographic images is
of value in correctly diagnosing these conditions.
