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Imaging of infantile polycystic kidney disease with some rare association
1Radiology Division, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Insights
Infantile polycystic kidney disease (IPKD) in children is best diagnosed using ultrasound. This noninvasive imaging technique allows for repeat examinations, making it ideal for antenatal and postnatal assessment of enlarged kidneys and associated complications.
Area of Science:
- Pediatric Nephrology
- Medical Imaging
- Genitourinary Radiology
Background:
- Infantile polycystic kidney disease (IPKD) is a severe genetic disorder affecting the kidneys.
- Early and accurate diagnosis is crucial for managing IPKD and its complications.
- Various imaging modalities are used, but their diagnostic efficacy in IPKD varies.
Purpose of the Study:
- To evaluate the diagnostic utility of ultrasound and other imaging techniques in pediatric IPKD.
- To characterize the sonographic findings in infantile polycystic kidney disease.
- To compare the effectiveness of sonography with other imaging methods for IPKD.
Main Methods:
- Retrospective analysis of 17 pediatric patients diagnosed with IPKD (age 1 day to 6 years).
- Evaluation using ultrasound (sonography) and other imaging techniques.
- Detailed assessment of renal morphology, echogenicity, and associated anomalies.
Main Results:
- Most patients presented with bilaterally enlarged kidneys, hyperechoic parenchyma, and poor differentiation of renal structures.
- Renal cysts of varying sizes were observed; however, some cases showed well-defined outlines or dilated collecting systems.
- Hepatomegaly was present in 12 cases, with rare findings including liver cysts, Meckel syndrome, renal stones, reflux, and calcification.
Conclusions:
- Sonography, alongside intravenous pyelography, confirms diagnostic value in IPKD.
- Ultrasound's noninvasiveness and suitability for repeat antenatal/postnatal examinations establish it as the preferred imaging modality for infantile polycystic kidney disease.
Abstract:
Seventeen patients, age 1 day to 6 years with infantile polycystic kidney disease were evaluated with ultrasound and other imaging techniques. Most patients showed bilaterally enlarged kidneys with hyperechoic renal parenchyma, which had poor differentiation in outlines as well as between renal sinus, cortex and medulla. Cysts of various sizes were also identified in the kidneys. However, a third of these cases showed well-defined renal outlines, normal echogenic cortical rim, whilst dilated renal collecting systems were seen in another third of the cases. Twelve cases showed hepatomegaly. Few rare findings such as liver cysts, associated Meckel syndrome, renal stone, bilateral vesicoureteric reflux and renal calcification were also noted. This study confirms the diagnostic value of sonography and intravenous pyelography examination. However, the noninvasiveness and the opportunity for repeat examination in antenatal and postnatal life makes sonography the ideal investigative modality.