University of Maryland Medical Center surgeon Andrew Kramer, M.D., answers questions about this innovative treatment to treat polycystic kidney disease. Recently, PKD patient Jamie Cadiz underwent a successful bilateral nephrectomy transplant at the Medical Center. To make an appointment, please send an e-mail here with your contact information.
What is a simultaneous bilateral nephrectomy transplant?
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A bilateral nephrectomy is when both kidneys are removed. A simultaneous bilateral transplant is when the kidneys are removed and then a kidney transplant is done in the same operation.
What condition is this used to treat?
Polycystic kidney disease (PKD), and potentially it could also be used for renovascular hypertension and kidney cancer.
What is PKD?
Polycystic kidney disease is the most common genetic, life-threatening disease that affects more than 600,000 Americans and an estimated 12.5 million people worldwide.
Polycystic means multiple cysts. In effect, PKD denotes multiple cysts on each kidney. These cysts grow and multiply over time, also causing the mass of the kidney to increase. It can also cause hypertension of the kidneys, as well as infection and pain. Ultimately, the diseased kidney shuts down, causing end-stage renal disease, for which dialysis and transplantation are the only forms of treatment.
PKD comes in two forms. Autosomal dominant polycystic kidney disease (ADPKD) is the most common, affecting 1-in-400 to 1-in-500 adults. Autosomal recessive polycystic kidney disease (ARPKD) is far less common, affecting 1-in-10,000 at a far younger age, including newborns, infants and children.
How many simultaneous bilateral nephrectomy transplants have been done at the University of Maryland Medical Center?
At the University of Maryland, we have done 50 total simultaneous bilateral nephrectomies then transplant for the treatment of PKD.
How common a treatment is this for PKD — to do the combined nephrectomy transplant?
This is not a common treatment for PKD. The University of Maryland Medical Center is one of the few centers nationwide that offers this treatment.
Normally with a kidney transplant the old kidneys are left in, but with PKD, since they have cysts in them, would the surgeon normally remove the old kidneys before they do the transplant?
There’s no protocol or consensus on it. This has not been an accepted case of transplant nephrectomy because of the risk of infection and how big an operation it is. That’s why there is not a consensus. Some people take them out way before, some people take them out after. I don’t think many surgeons do it all at once.
Sometimes you take out the native kidneys before transplantation, sometimes you don’t. I’d say for most of the transplants done, they don’t take out the diseased kidneys if it’s due to the standard conditions such as hypertensive nephropathy or nephrosis (congential). But patients who have renovascular hypertension would have their kidneys removed.
What are the benefits of combining the two operations?
One operation relieves patients of all the pain and suffering from the kidneys. They’re getting a transplant all at once so they can go home; the whole problem is solved.
I think there are two points: One, it’s unique and a good treatment option that makes sense and seems to work. And when it works it’s better than the other alternatives -- removing the kidneys before the transplant, doing it after, or leaving the kidneys in.
What are the risks?
The possible disadvantages would be if it weren’t working, if all our grafts were failing but we haven’t had a case of that yet.
But we wouldn’t do something that would increase the chance of infections. We’ve had good success and the cases I’ve been involved in haven’t had any problems. That’s not always the scientific method, but in this case there’s no model so we started doing it with good results.
Once the old kidneys are removed, does that cure the patient of PKD?
No. PKD is an autosomoal dominant disease. The liver will continue to grow cysts. Patients won’t get hypertension or renal pain, but they do get cysts in other places such as the liver and pancreas. But those cysts won’t cause the same type of problems that the kidney cysts do.
Is the transplant the only treatment option?
It’s the only cure for the renal manifestations of the disease, but there are other treatments. Some people get frequent cyst decortications (removal of part or all of the outer surface of the kidney). Sometimes if one kidney is having infections or pain, laparoscopically you can just go in and open some of those cysts. For some people, that is shown to be palliative. Draining the cysts is another palliative treatment. If the kidneys function some people get by with cyst decortications.
What should patients with PKD consider the University of Maryland Medical Center?
I think we can prove that based on patient testimonials -- because we’ve done thousands of transplants and we’ve done several that involve this -- that these patients are thriving and are doing well.
I don’t think a complex operation such as this would work just anywhere; this is a job that really should be done in a center such as Maryland.
We have an experienced team of urologists, transplant, plastic surgery and general surgeons and that experience is important. And it’s an advantage to go to a place where the urologists have done a number of these and we do it quickly. We’re doing this in the same location as a transplant program that’s done 2,000 transplants, so I think working with them is important because if it is a hard case they have the experience to come in and do a transplant in that situation.
That team approach is what makes us unique. The operation is successful if done in the right hands. It requires a center with the depth of expertise that we have.