Authors: Fred E Avni Gretel Guissard Michelle Hall Françoise Janssen Viviane DeMaertelaer Françoise Rypens
Publish Date: 2002/01/11
Volume: 32, Issue: 3, Pages: 169-174
Abstract
Objective To determine which US changes occur with time in children affected by autosomal recessive ARPKD and autosomal dominant polycystic kidney disease ADPKD and whether any of these changes correlate with the onset of renal failure Materials and methods We reviewed the US features of 29 patients 16 ARPKD 13 ADPK imaged by at least two US examinations We analysed the size and echogenicity of the kidneys corticomedullary differentiation CMD the presence location and size of cysts and any other anomaly that developed with time In order to determine whether a relationship could be found between any of the US changes and the onset of the renal failure based on a glomerular filtration rate 50 ml/min per 173 m2 a Pearson exact chisquare test was calculated Results For ARPKD renal size was above 4 standard deviations SD in 10 of 16 patients but it remained stable during evolution 10/16 The kidneys appeared hyperechoic 16/16 without CMD in the majority 11/16 of patients Changes in the appearance of CMD over time were observed in five patients Small cysts 1 cm were present at the time of diagnosis in seven patients larger cysts 1 cm in three A diffuse microcystic pattern was observed in three patients Diffuse hyperechoic foci developed in 14 patients – 13 of whom had developed renal failure at the time of the examination or rapidly thereafter statistical correlation P=00125 For ADPKD renal size was between 0–2 SD in 7 of 13 patients and above 2 SD in the other 6 Renal echogenicity was normal in five difficult to assess in five and the kidneys appeared hyperechoic without CMD in three patients Cysts larger than 1 cm were present in 8 of 12 patients 3 cm in 5 In four patients the cysts measured less than 1 cm In the last child the diagnosis had been made antenatally and the first cysts appeared at the age of 6 months The size of the kidneys 13/13 and of the cysts 11/13 remained stable No renal failure occurred Conclusions ARPKD may manifest with various US patterns and there may be evolution in the appearances over time Our study confirms a significant relationship between the development of diffuse hyperechoic foci and the onset of renal failure In older children ARPKD and ADPKD may closely resemble each other Large 3 cm cysts are the US hallmark for the diagnosis of ADPKD furthermore fewer US changes occur with time during childhood in ADPKD
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