Multicystic Dysplastic Kidney

As Jillian and I begin what we hope is our final month in CHOP (during this stay, anyway) I wanted to do a series of posts to help me keep my head straight about all the issues Aurelia faces/faced/might face. It will help us maintain a running record of Aurelia's treatment and I think it might help some folks get a better understanding of the complexity of some of the things we mention here.

So, I'm starting with Multicystic Dysplastic Kidney, or MCDK. Aurelia has a right MCDK and a normal left kidney. Her left kidney does present a bit higher than normal in her body but functions like a champ. This was discovered during our Fetal Health visits last summer after the HLHS revelation. In essence, MCDK is a fetal malformation of the kidney where multiple cysts of varying sizes fill a kidney. This affliction impairs kidney function, obviously, but in Aurelia's case she has maintained very slight functioning up to the present.

We monitored her kidney every two weeks with an ultrasound, and now that she's born we'll be keeping an eye on it with regular imaging diagnostics. Typically infants with MCDK will experience regression with the damaged kidney, resulting in either kidney death or actual degradation of the organ until it shrivels up and *poof* disappears. Given that Aurelia still has very minimal function in this kidney, it's not likely that we'll see either of the previous outcomes happen for her.

To help you get a feel for what this looks like, here's an image of an infant with the exact same issue affecting Aurelia - a MCD right kidney and a normally functioning left kidney:

The right kidney actually appears on the left of the screen, but you can see the cysts of varying sizes. quite clearly. This is a highly detailed MRI, but even on an ultrasound you can clearly see the cysts present in the kidney. Aurelia has had a renal ultrasound (RUS) and a voiding cystourethrogram (VCUG) to determine the severity of her defect and the level of kidney function present.

Of particular concern is any back-flow of urine from the bladder into the damaged kidney which can cause catastrophic infection (not your normal UTI) and other risks, such as rupturing cysts, etc. Aurelia presents no such complications at this point. The slight function will likely end at some point and we may have to make a decision about how to proceed with the kidney; let it die and see if it will shrink and disappear or remove the kidney via surgery to prevent any future complications.

Treatment includes a daily regiment of Amoxicillin to prevent the infection and it is possible that she will take that for her lifetime. One problematic side-effect to the early and frequent use of antibiotics in CHD babies is an increase in the occurrence of NEC, which did appear in Aurelia after one month of treatment for her MCDK.

A great site that summarizes much of this information is http://www.childrenshospital.org/az/Site1318/mainpageS1318P0.html


The urology team here at CHOP