The Decision to Have a Bilateral Adrenalectomy
Alicia M. Held, MSW
I assume that by the time you have reached this point you are a lay expert on Cushing’s syndrome, and I expect you could probably give a few endocrinologists a run for their money too. So I will not be going into much detail about the ins and outs of Cushing’s syndrome itself in this piece. If you have somehow found this piece and you know nothing about Cushing’s syndrome please see the wealth of information elsewhere on this site. What I would like to go into in some depth in this piece is what processes go into the decision to have a bilateral adrenalectomy, both from a medical perspective and with some anecdotes from my own personal experience. It is my hope that this piece provides not only reliable and important information to help with your decision, but also helps to reinforce that you are not alone in going through this!
What Is a Bilateral Andrenalectomy?
Bilateral adrenalectomy is a surgical procedure in which both of the adrenal glands are removed in totality. As a reminder, the adrenal glands are endocrine glands, an organ that produces hormones and that sit atop each of your kidneys. Normally, they are approximately the size of your thumb and produce the hormones, cortisol, aldosterone, the adrenaline hormones-epinephrine and norepinephrine, and a small amount of sex hormones-DHEA and androgens (SAGES, 2014). Previously, a large 6-10 inch incision had to be made in the abdomen, similar to that used for a cesarean section, and the glands were removed in that fashion. However, almost all bilateral adrenalectomies now performed are done laparoscopically, with small incisions being made in either the abdomen, side or back, depending on surgeon technique; and instruments are inserted into these incisions to dissect and remove the adrenal glands (SAGES). After the adrenal glands are removed patients will need to supplement mineralcorticoid and glucocorticoid hormones for the rest of their lives (Liu et al., 2007).
Why Would Someone Need a BLA?
The most common cause for a bilateral adrenalectomy is patients who have Cushing’s syndrome and have undergone previous surgery or treatments without a resolution of their Cushing’s symptoms or a reduction the their excess cortisol production (Liu et al.). Bilateral adrenalectomies may also be performed on someone who has not had any previous treatment for Cushing’s syndrome but needs an immediate reversal of their hypercortisolemia (Liu et al.); however, it is generally a last resort in the treatment of Cushing’s syndrome after all other treatments have been tried and failed.
How Do I Decide If A Bilateral Adrenalectomy Is The Right Decision For Me?
This really gets to the meat of the issue for me. This decision is so personal, and the path to the decision to have a bilateral adrenalectomy is different for each person. Some people have had multiple pituitary surgeries and are still suffering from symptoms of Cushing’s syndrome. Others have had one surgery but have tried multiple medications to mitigate some of the effects of Cushing’s but they either did not work or they could not tolerate some of the extreme side effects. No matter how you come to this decision, chances are you have had at least one surgery and tried multiple modalities to find that elusive Cushing’s cure before considering what is really your last option, a bilateral adrenalectomy. To assist you in making this very important medical decision, from here on I will be weighing the risks and benefits of a bilateral adrenalectomy using the most reliable and up to date information available, and I will share my story on how I came to make this decision for myself.
What are your options? It is very important to understand that while a bilateral adrenalectomy is a viable option for the reversal of hypercortisolemia in Cushing’s syndrome, there are also many inherent risks with the procedure, and it is essential that you have reviewed all of your options as you approach this decision. For many people a second, third, or even fourth pituitary surgery might be the preferred treatment modality before pursuing a bilateral adrenalectomy. Studies have shown that patients have as much as a 70% chance of remission from Cushing’s syndrome from a second pituitary surgery (Liu et al.). Some factors to consider in whether another pituitary surgery might be the better option for you are:
- Was your surgeon an expert in treating pituitary tumors?
- If the surgeon who performed your first pituitary surgery had not performed hundreds of the same surgery prior to yours, you may not have had the best care or surgical technique in removing your pituitary tumor. Pituitary tumors are small, difficult to locate on MRI, and the endoscopic and microscopic approaches require years of practice (McCutcheon, 2008). If your surgeon was not skilled in this type of surgery it may be prudent to receive a second opinion from a top neurosurgeon who is well known for their treatment of pituitary microadenomas. While there are risks associated with every surgery, pituitary surgery generally has less inherent risk than a bilateral adrenalectomy and does not require life long hormone supplementation in most cases. If you can achieve a cure through an additional pituitary surgery this might be a viable option to investigate. The downside is, however, there is also a chance that an additional surgery may not be successful, it could cause additional damage to your pituitary, more time will have passed, and you will be back evaluating this decision again in a year or more.
- What were your post surgery findings?
- Studies show that having a cortisol level less than 2 μg/dl 72 hours after surgery provides a good predictor for long-term remission (Liu et al.). If you had a cortisol level higher than 2 μg/dl you have a much higher chance of recurrence and might even be a candidate for an immediate reoperation for tumor resection or a possible partial hypophysectomy, where part of the pituitary gland is removed along with the tumor (Liu et al.). Studies have shown a remission rate of approximately 67% in those situations, however, there is a high chance of reduced pituitary function which could result in the need for hormone replacement (Liu et al.)
- Another good predictor for a successful secondary pituitary surgery is if you had positive pathology confirming the presence of a tumor in your initial tumor removal (Liu et al.). The risk of an unsuccessful second pituitary surgery increase when there was no clear adenoma in the initial pituitary surgery, and the risk of hypopituitarism, i.e. loss of pituitary function, from additional pituitary surgeries increases to over 50% when no clear tumor was found during the initial surgery (Liu et al.). This means that patients with chemical evidence of hypercortisolism but no tumor visualized on MRI or identified in a primary pituitary surgery are not likely candidates to have successful remission from additional pituitary surgeries.
- Is there a visible tumor on your most recent MRI?
- If you have a tumor, or an area highly suspicious for tumor, visible on your MRI it is definitely in your best interest to seek the opinion of a renowned pituitary specialist to evaluate your case on whether an additional pituitary surgery is the treatment option you should pursue. Any person considering a BLA should have an MRI to check for residual tumor, not only because a pituitary surgery presents less risks and less long term adverse health effects, but because of the risk of Nelson’s syndrome. Nelson’s syndrome is a rare side effect of bilateral adrenalectomy, caused by residual tumor in the pituitary rapidly expanding and secreting excess ACTH due to the loss of hormonal feedback after the removal of the adrenal glands (Wilson, 2013). Nelson’s syndrome is rare, but like many other things related to Cushing’s, experts disagree on the prevalence of Nelson’s syndrome after a BLA. Studies place the occurrence of Nelson’s syndrome somewhere between 8-44% of patients who undergo a BLA, but they do agree that the incidence of Nelson’s seems to be falling due to enhanced MRI techniques that can spot microadenomas at an earlier stage (Wilson). Symptoms of Nelson’s syndrome include headache, increased skin pigmentation, and in rare cases the pituitary tumor may become malignant (Wilson). Therefore, it is in your very best interest while you are contemplating the decision to have a bilateral adrenalectomy that you have an MRI done at high resolution and receive an evaluation by a highly qualified neurosurgeon to be sure there is absolutely no target for a repeat pituitary surgery.
Besides the option of an additional pituitary surgery, there is the option of medical treatment, radiation therapy or a combination of both. I will not go into the specifics of each of those treatments as they are delineated in detail elsewhere on this site. However, it is important, while considering a bilateral adrenalectomy that you review these options with an edocrinologist or neurosurgeon that you trust. There are new medical treatments available, and gamma knife surgery has made huge gains in the treatment of Cushing’s. Either of these might be a valid option for you that does not require surgery or permanent and life-long replacement of hormones.
What are the risks and benefits? As stated before, all surgery has inherent risk, and a bilateral adrenalectomy is no exception. In fact, this surgery carries with it unique risks and challenges that will last a lifetime. Risks that are associated with almost all abdominal surgeries include chances of bleeding, possible bowel tears leading to infection, significant pain, and rarely there is the chance of death. Prior to the widespread use of the laparoscopic approach in bilateral adrenalectomies the mortality rate was between 3-6%, with a post surgery morbidity (long-term difficulties) rate near 40% (Fernandez-Cruz, et al., 1996). However, with the overwhelming use of the laparoscopic technique today, mortality has dropped to near zero and morbidity rates are approximately 10% (Thompson et al., 2007).
In patients undergoing a bilateral adrenalectomy the risks do not end after the procedure though. Because cortisol is a vital hormone needed for proper body functioning, there are chances that you will have difficulty maintaining a correct physiological dose of hormone, with 20% of patients having one adrenal crisis after surgery, and 30% having multiple adrenal crises requiring emergency intervention throughout their lives (Thompson et al.). Although patient self-reports place the number of people seeking emergency care for an adrenal crisis at closer to 65% (Hawn, Cook, Deveney, Sheppard, 2002). An adrenal crisis is a life-threatening condition in which the body does not get enough cortisol. Symptoms of an adrenal crisis include, nausea, vomiting, severe fatigue, confusion and disorientation, low blood pressure, and loss of consciousness (Rennert, 2014). Failure to get appropriate emergency medical treatment can result in death.
In addition, there also remains the risk that, although rare, removal of the adrenal glands will not alleviate the symptoms of Cushing’s syndrome. Some patients have tissue outside of their adrenal glands that may not be removed at the time of surgery which can continue to produce cortisol and Cushing’s symptoms (Thompson et al.). Therefore, having undergone a potentially dangerous procedure there is the small chance that you will still be left with symptoms of Cushing’s.
As you can see, the decision to undergo a BLA should not be taken lightly, however, there are obvious benefits associated with the procedure. Approximately 90% of patients reported that they were satisfied or very satisfied with the outcome of their BLA and would make the same decision again (Thompson et al.). Patients reported resolution of symptoms including excess hair growth, emotional lability, muscle weakness, and weight loss (Liu et al.). And although when compared to their healthy counterparts, many patients still score lower on physical and mental quality of life scales; most patients self-report a significant reduction in their Cushing’s symptoms and a dramatic increase in their quality of life after bliateral adrenalectomy (Thompson et al.).
What Would You Do If It Was You?
I feel fairly confident in answering this question, as I have navigated this maze in my own life. I was diagnosed with Cushing’s disease in 2009 after being sick and misdiagnosed for approximately 10 years. I underwent an endoscopic transspheoidal pituitary tumor removal in April of 2010. The surgeon felt good that he had resected all abnormal looking tissue, and pathology confirmed a tumor and normal gland boundaries on what he had removed. My 72 hour cortisol level was 2.2 μg/dl, right on the border of what would be considered successful resolution of hypercortisolemia after surgery. I went home on steroid replacement and was able to taper fairly easily over the next few months. I had approximately 6-9 months where I felt great, lost weight, reduced my blood pressure medication, and even went back to work part-time. However, within a year post-op I started to notice symptoms of Cushing’s returning and I began the tedious work of being rediagnosed with Cushing’s. I was officially rediagnosed in 2011 and was placed on Ketoconazole to try to control the symptoms of my Cushing’s. At a lower dose of Keto there was no impact on my symptoms. I had to raise my dose to 800mg a day before I found some relief. However, at this high dose I suffered from constant and severe daily nausea and after about 10 months I was no longer able to tolerate the medication. I spent another year doing repeat testing, and finally I was cleared by my doctor for a bilateral adrenalectomy in 2014.
I followed many of the steps I outlined for you above. I had my MRI reviewed by a renowned neurosurgeon to make sure there was no surgical target in my pituitary gland. I had some trepidation going forward making this decision, however, I felt that I carefully weighed my options, reviewed the risks and benefits, discussed them with medical professionals and family, and I was secure in the decision that I had made. The path to a BLA for me was frustrating, filled with many hurdles and delays, like it is for so many others, but do not get discouraged. Like many people I spoke to throughout the process, who previously had undergone a BLA; I felt like if I did not do something, Cushing’s would eventually take my life, sooner rather than later. I understand there are great risks associated with a bilateral adrenalectomy, however, for me, the benefits physically, emotionally, and in the ability to live my life out to its fullest, far outweighed the risks of the procedure and future health issues.
My BLA surgery was textbook, and I had very little pain or side effects immediately after surgery. I began to let myself hope again that I could find a normal future on the other side of Cushing’s. However, within a few months of surgery I was having difficulties, and my road post-BLA has been fraught with a baby step forward and giant leaps back. Luckily, for most people considering a BLA I am in the very rare minority of those who do not see immediate relief from a bilateral adrenalectomy, I have seen some successes though, minor weight loss, improved sleep, reduction of blood pressure and other small victories. And even with what I know now, if I had to make the decision to have a BLA a hundred times again, one hundred times I would choose to have the surgery. I know in my heart that Cushing’s was going to kill me, and in the end the bilateral adrenalectomy was the best option, personally, to save my life.
I am so sorry that your journey with Cushing’s syndrome has brought you to this place. However, I sincerely hope that the information you find here has helped in some small part to assuage your fears and help you to make an informed decision in regards to your medical care. And more than that, I hope my story, and the stories of others here, have helped to assure you that you are not alone in this process, and that there is hope for a life after Cushing’s!
- Fernandez-Cruz, L., Saenz, A., Bennaroch, G., Astudillo, E., Taura, P., & Sabater, L. (1996). Laparosocpic unilateral and bilateral adrenalectomy for Cushing’s syndrome: Transperitoneal and retroperitoneal approaches. Annals of Surgery, 224 (6), 727-736. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1235468/pdf/annsurg00034-0051.pdf
- Hawn, M., Cook, D., Deveney, C. & Sheppard, B. (2002). Quality of life after bilateral adrenalectomy for Cushing’s disease. Surgery 132, 1064-1069. Retrieved from http://home.comcast.net/~staticnrg/Cushings/QualityOfLifeBLA.pdf
- Liu, J.K., Fleseriu, M., Delashaw Jr., J., Ciric, I., Couldwell, W. (2007). Treatment options for Cushing disease after unsuccessful transsphenoidal surgery. Neurosurgery Focus, 23 (3), E8. Retreived from http://www.medscape.com/viewarticle/566309_1
- Rennert, N.J., (2014, Feb 26). Acute adrenal crisis. U.S. National Library of Medicine. Retreived from http://www.nlm.nih.gov/medlineplus/ency/article/000357.htm
- Society of American Gastrointestinal Endoscopic Surgeons (SAGES). (2014). Patient information for laparoscopic adrenal gland removal (adrenalectomy) from SAGES. Retrieved from http://www.sages.org/publications/patient-information/patient-information-for-laparoscopic-adrenal-gland-removal-adrenalectomy-from-sages/
- Thompson, S.K., Hatman, A.V., Ludlam, W.H., Deveney, C.W., Loriaux, L. & Sheppard, B.C. (2007). Improved quality of life after bilateral laparoscopic adrenalectomy for Cushing’s diease. Annals of Surgery, 245 (5), 790-794. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1877068/
- Wilson, T.A., Ed. Kemp, S. (2013, Feb 11). Nelson syndrome. Medscape. Retrieved from http://emedicine.medscape.com/article/923425-overview#a0101