Treatments for Multicystic Dysplastic Kidney in Children

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What treatments are available for multicystic dysplastic kidney?

Background

In the past, the precise diagnosis was difficult to determine, so all multicystic dysplastic kidneys were removed by nephrectomy (removal of the diseased kidney). With advances in medical imaging, the correct diagnosis can now be established with near certainty. This has led some clinicians to begin recommending surveillance instead of nephrectomy.

Monitoring

There is no uniform consensus on the best form of treatment for this condition. The condition is being monitored by a national registry (National Multicystic Kidney Registry), and some of their findings include the following:

  • The risk of developing a malignancy within a multicystic dysplastic kidney is low. Since 1957 there have only been nine reported cases.
  • The development of hypertension (high blood pressure) is also low, with only four patients of 508 within the Registry developing hypertension.

Currently, most pediatric urologists who recommend monitoring the kidney perform serial ultrasound examinations until the kidney totally regresses or becomes undetectable by ultrasound.

Surgery

Surgical removal is an option, and many parents have elected this form of treatment. With surgery, there is no further need for routine/serial surveillance because there is no risk of hypertension or a tumor arising from the kidney.

If you choose this option, your child's kidney may be removed through a small skin incision or by a procedure called a laparoscopic nephrectomy (kidney removal). Children's is one of the few hospitals in the world to use robotic surgery technology for nephrectomy procedures. In 2001, we were the first pediatric hospital to acquire a surgical robot, and today, Children's surgeons perform more pediatric robotic surgeries than any other hospital in the world.

Laparoscopic nephrectomy may be:

  • transperitoneal (performed from the front) - This approach is sometimes better if your child's ureter (the tube connecting the kidney with the bladder) also needs to be removed.
  • retroperitoneal (performed from the back or side) - Most kidneys can be removed using this approach, which seems to permit even more rapid recovery.

During your child's laparoscopic nephrectomy:

  1. She will be put under general anesthesia.
  2. Only three or four punctures are used. Her kidney is removed through one of the puncture sites, sometimes using a small bag inserted into the surgical area to allow the kidney to be pulled out.
  3. She is kept overnight in the hospital and then allowed to go home.

Surgery may also be appropriate when the multicystic dysplastic kidney is very large and causes difficulty with feeding or breathing due to its size.

This is a congenitally developed lesion and not related to the adult condition known as adult polycystic kidney disease.

What is the long-term outlook for a child with a complete or partial nephrectomy?

The long-term outlook for a child with a complete or partial nephrectomy is very good, since most people have a second, normally functioning kidney that can adequately meet the body's needs.

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- Sandra L. Fenwick, President and CEO

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