Outcome of Bilateral Adrenalectomy in Cushing's Syndrome: A Systematic Review

[~MaryO~]

1. Katrin Ritzel,
2. Felix Beuschlein,
3. Anne Mickisch,
4. Andrea Osswald,
5. Harald J. Schneider,
6. Jochen Schopohl and
7. Martin Reincke

-Author Affiliations

1. Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, D-80336 München, Germany

1. Address all correspondence and requests for reprints to: Martin Reincke, M.D., Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Ziemssenstrasse 1, D-80336 München, Germany. E-mail:Martin.Reincke@med.uni-muenchen.de.

Abstract

Background: The outcome of bilateral adrenalectomy (BADx) in patients with Cushing’s syndrome (CS) is not well characterized.

Methods: A literature search was performed with the search terms “bilateral adrenalectomy” or “total adrenalectomy” and “Cushing’s” or “Cushing.” Immediate and long-term outcomes after BADx in CS were analyzed using descriptive statistics (median [range]).

Results: From 549 screened publications, 37 studies met inclusion criteria (1320 patients, 82% having Cushing’s disease [CD], 13% having ectopic CS, and 5% having primary adrenal hyperplasia).

Surgical morbidity and mortality of BADx (23 studies, 739 patients) were 18% (6–31) and 3% (0–15), respectively. In patients with CD, surgical mortality was below 1%. Although residual cortisol secretion due to accessory adrenal tissue or adrenal remnants was found in 3–34% (5 studies, 236 patients), less than 2% had a relapse of CS.

Symptoms of hypercortisolism (eg, hypertension, obesity, or depression) improved in the majority of the patients after BADx (7 studies, 195 patients). The number of adrenal crises per 100 patient-years was 9.3 (6 studies, 203 patients).

Nelson’s syndrome occurred in 21% (0–47) of the patients (24 studies, 768 patients). Mortality (23 studies, 690 patients) was 17% (0–88) at a follow-up of 41 months (14–294). Remarkably, 46% of the patients died in the first year after surgery. The median ages at death were 62 years (CD) and 53 years (ectopic CS).

Conclusion: BADx is relatively safe and provides adequate success. Excess mortality within the first year after surgery suggests that intensive clinical care for patients after BADx is warranted.

Footnotes

• For editorial see page 3974

• Abbreviations:

  BADx bilateral adrenalectomy BAH bilateral adrenal hypercortisolism CD Cushing’s disease CS Cushing’s syndrome ECS ectopic CS NS Nelson’s syndrome QOL quality of life TSS transsphenoidal surgery.

   

• Copyright © 2013 by The Endocrine Society

From http://jcem.endojournals.org/content/98/10/3939.abstract

[Katski]

Thanks for posting this. I have had a lot of questions regarding the outcome of A BLA. I'm waiting in the results of my last 3 salivaries, and I am pretty sure I know the outcome (rats).

Is it me, or does it seem like there are alot of people having BLA's?

Kathy

[tallyo]

Thank you for posting this article MaryO.  This is another article I am going to give to my husband to read.

 

Katski sorry you are boarding the BLA train.  It’s no fun.  

[philb]

BLA has been fun for me so far! I was literally dying with Cushing's. I no longer have Cushing's and I'm going to live. 3 weeks  out and it's been a better 3 weeks than the last 3 years

[Door]

Am I the only one completely freaked out by the fact that 46% of the participants DIED within the first year??? Did I read it wrong or something?

[JessP]

You read that right. I suspect that their age also had something to do with it. (The median was 62 y/o for those with Cushing's disease and 52 y/o for those with ectopic Cushing's syndrome, which means some patients had to be older than those. Of course some were younger, too.) Perhaps they didn't tolerate surgery well or were inactive post-op and got pneumonia or some other problem. It doesn't help that they were likely overweight going into surgery given that they had Cushing's.

[Door]

I'm such a worrier by nature that if I ever had to have a BLA I'd worry myself to death (literally) over the fear of dying from the BLA. Either that or I'd have medically induced cushing's from all of the steroids I'd be pumping myself full of in fear of dying! lol. I know. I'm crazy.

I'm curious as to how you feel about this outcome.

[JessP]

I'm convinced that Cushing's will kill me. My immune system is so bad that when I get a cold I'll have it a minimum of 10 days. The flu or anything more serious I'll have for 3 weeks. I'm sure that one of these days I'll get sick and my body just won't be able to fight it. I'd hate to die of pneumonia in my 20s or 30s. ...or I'd get injured, get an infection, and turn septic. It takes me weeks to heal from minor injuries like scratches.

 

So, given that, I'm willing to take my chances with a BLA. Most sources say that it's fairly safe. That, and my qualify of life sucks. I'm managing because I have to. I'm the only source of income for my family so I have to work. I'm taking 10 credits this semester and still working full-time because I just don't have a choice, but it's an awful experience. There's a lot I'm willing to risk to get anything better than this.

[Door]

One thing that might help you with the infections/slow healing is zinc and vitamin c. I know that ever since all of this stuff started with me my zinc is chronically low. I have to take zinc because if I don't my level drops. I've gotten to where I can tell by just symptoms now. But I used to heal extremely slow. I had a mild toe infection for over a year if that tells you anything, and it wasn't until I started zinc that it cleared up. What antibiotics couldn't cure zinc did. I usually have colds for about a month if I don't take my zinc.

 

I read a study on the intrawebz that said cushing's patients should take zinc. This bastard affects more than just vitamin d apparently! I also have to take magnesium. Dr. F. Even suggests magnesium. If i don't take it my fatigue and weakness is almost unbearable.

Just some tips to help you in the future. I completely understand your position. I myself am terrified of the possibility that I may need a bla in the future but if it came down to it I know I'd go through with it for the same reasons you will.

[betseebee]

So 46 percent died within the first year after the BLA? That scares the daylights out of me. Aack. Chickening out. 

[Door]

I wonder what the exact cause of death is for most of them. Adrenal crisis???

[smcsk8]

I was miserable with Cushing's. I am not sorry I had a bla and do think Cushing's would've eventually killed me.

 

I think most people on this board who end up with a bla do really well. Good doctors matter.

 

And I suspect the cause of death for those subjects in that study is something else. Adrenal crisis can kill you, but you have notice!

[qosmio]

I think this abstract is confusingly worded.  I don't think it is saying that 46% of all patients died.  That sentence is referring to the sentence previous, which states a 17% mortality across all studies at the 41 month mark.  Then it goes into more detail by saying that nearly half (the 46%) of that 17% didn't make it past the first 12 months.  There is no way the conclusion of this abstract would state that BLA is "relatively safe" if half the subjects died.

 

Mortality (23 studies, 690 patients) was 17% (0–88) at a follow-up of 41 months (14–294). Remarkably, 46% of the patients died in the first year after surgery.

[philb]

Exactamundo.  BLA is as safe as you make it to be. Be aware of your body and it's needs. There are many, many people who have this done, not just Cushing's patients and with a good doc and knowledge you can live a normal life.

 

Cushing's disease on the other hand is unmanageable and deadly. I was on borrowed time when I had my BLA 3 weeks ago.
 

[Door]

Qosmio, thanks for catching that! I'm not even diagnosed yet and was quaking in my boots! That makes me feel way better.

 

I was also quite confused as to why they would claim it to be relatively safe. I just thought "well, I guess it's because slightly more patients lived than died." Lol! Whew.

[smcsk8]

Yeah they missed a modifier. It should say something like "of those 17%" or "of those patients" so it was clear which patients the 46% referred to.

[betseebee]

Thank you all for clarifying what the Cushie brain can overlook. Breathing easier now.

[JessP]

Ah, you guys are right. Sorry!