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Urinary and Genital Disorders (Children)

Segmental Multicystic Kidney



Our 19 week male fetus was diagnosed with a slightly enlarged kidney. Subsequent ultrasounds indicate that there appears to be cyst(s) in the upper portion. The pregnancy has progressed well (presently 35 weeks) with no change in kidney size or appearance. The cysts appear to remain in only one area of the kidney. Amniotic fluid is normal and the fetus is healthy and active. I have several questions:

My father (deceased) has a non functioning kidney removed when he was a toddler. Is this condition hereditary?

Is it a sign of other congenitive issues?

It is my understanding that cysts form early in the fetal development, completely takeover and prevent any/all kidney development? Discussions with various doctors indicates that there is a kidney, with cysts forming in only one area.

Perhaps this is a father`s wishful thinking, but is it possible for this to be confused with something else?



This condition does not sound serious. It sounds like there is a duplex kidney with maldevelopment/dysplasia in the upper half of the kidney. Assuming the rest of the kidney is normal and the opposite kidney is normal, then it is unlikely to need treatment, either surgical or medical.

It sounds like there is what is termed a segmental multicystic dysplastic kidney. It is confirmed by an ultrasound study after birth, and generally a few follow-up ultrasounds are performed over the first few years of life. In many of these cases, a small ureterocele or bubble in the base of the bladder is observed also. That does not need treatment. In some cases pediatric urologists recommend a kidney scan and a voiding cystourethrogram as part of the initial radiologic evaluation.

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Response by:

Jack S Elder, MD, FACS, FAAP Jack S Elder, MD, FACS, FAAP
Clinical Professor of Urology
School of Medicine
Case Western Reserve University