Experiencing Parathyroid Disorders in Utah?

What are Parathyroid Glands?

These are 4 tiny glands that regulate the calcium in our body. They produce the parathyroid hormone (PTH), which affects many other things, to increase the calcium. It works directly on the bone cells, and makes them take away calcium from the bones and move it to the blood stream. It also works on the kidney cells, making the kidneys reabsorb calcium that would have otherwise gone from the blood to the urine, and place it back in the bloodstream. Finally, they also work on the kidney to activate the vitamin D. The activated vitamin D then works on the gut and makes it absorb more calcium, and also works on the bones and moves calcium from the bones to the bloodstream. There are multiple feedback mechanisms, to ensure that there is a very fine balance between the PTH, activated vitamin D, and calcium, so that the calcium level is always maintained at an optimal level.

Where are the Parathyroid Glands Located?

To understand where they are located, we need to know where they came from. While we are in our mother’s womb, the parathyroid glands emerge from the upper neck, and migrate down. Usually, they stop right behind the thyroid, with the 2 superior parathyroid glands ending up behind the upper part of each thyroid lobe, and the 2 inferior parathyroid glands ending up behind the lower part of each thyroid lobe. However, things don’t always go as planned, and the parathyroids are found in these usual locations in about 2 out of 3 people. For the rest, there is some variability, and some of the parathyroids can be found in the upper neck, lateral neck, lower neck, upper chest, or even in the middle of the chest close to the heart. Finally, in 1-2% of the patients, a parathyroid gland can be found inside the thyroid!

Usual locations of the parathyroid glands.Usual locations of the parathyroid glands.

Less usual locations of the parathyroid glands (black circles).

What is Hyperparathyroidism?

You have hyperparathyroidism when the parathyroid hormone level is above the normal range. There are 3 types of hyperparathyroidism: primary, secondary, and tertiary.

Primary hyperparathyroidism happens when one or more of the 4 parathyroid glands stops getting regulated by the feedback mechanisms discussed above, and starts overfunctioning independently. This is not normal, as it leads to an increase in the size of the gland(s), and too much parathyroid hormone circulating in the blood, which then makes the calcium increase, usually to levels above the normal range. Less commonly, the PTH may be high with a calcium in the normal reference range, because the labs were done early in the course of the disease. This is called “normocalcemic primary hyperparathyroidism”, and it is a diagnosis of exclusion, after other causes of hyperparathyroidism have been ruled out. If primary hyperparathyroidism is diagnosed, 85% of the patients will only have 1 hyperfunctioning parathyroid gland (parathyroid adenoma). Around 14% of the patients will have 2 or more parathyroid adenomas. Finally, <1% of the patients will have parathyroid cancer. Primary hyperparathyroidism is managed with surgical removal of the parathyroid adenoma(s), if the patient meets the criteria for surgery, which are discussed below. If the criteria are not met, then it is reasonable to observe the PTH and calcium level once a year without surgical intervention.

Secondary hyperparathyroidism happens when the PTH is elevated due to reasons other than autonomous hyperfunction of the parathyroid gland(s). The PTH is produced evenly by all 4 glands, and they can get slightly bigger but still remain tiny. In these cases, the calcium is either normal, or low. If your calcium is high, you do NOT have secondary hyperparathyroidism. On the other hand, secondary hyperparathyroidism is very common, and it has to be diagnosed properly, because surgery is NOT indicated for this. The most common causes of secondary hyperparathyroidism is low vitamin D, low calcium intake (through the diet or pills), and chronic kidney disease. The treatment for secondary hyperparathyroidism is calcium and vitamin D.

Tertiary hyperparathyroidism happens almost exclusively in patients with severe chronic kidney disease, who have either started, or are close to start dialysis, or have had a kidney transplant. It starts as secondary hyperparathyroidism due to the chronic kidney disease. However, due to the many years of stimulation of the parathyroid glands, at some point they stop responding to the feedback mechanisms and start working autonomously. When this happens, more than 1 glands are usually affected, and most commonly all 4 glands are affected. In order to diagnose tertiary hyperparathyroidism, you have to have severe chronic kidney disease, extremely elevated PTH, accompanied by many calcium levels above the normal range. Surgery is indicated only if the patients meet very specific criteria that indicate severe disease; otherwise this is managed with medication.

What are the Symptoms of High Calcium/Primary Hyperparathyroidism

High calcium can affect many organs in our body and cause a wide range of symptoms. These include fatigue, mental fogginess, mood changes, depression, anxiety, irritability, cognitive problems, disordered sleep, muscle cramps and pains, decreased muscle strength, decreased functional capacity, nausea, heartburn/indigestion, increased urination. Severely high calcium (hypercalcemic crisis) can lead to cardiovascular side effects, severe confusion/altered mental status, severe dehydration, acute renal failure, vomiting, abdominal pain, pancreatitis, acute kidney stones, and arrhythmia.

When is Surgery Indicated for Primary Hyperparathyroidism?

Over the past 15 years, the endocrine society guidelines and endocrine surgeon guidelines for surgical treatment of primary hyperparathyroidism have remained relatively stable, with small modifications. Overall, there are 5 indications for surgery for primary hyperparathyroidism. If at least 1 of these indications is met, then surgery is the preferred treatment:

  1. When there are symptoms that are clearly attributable to the hyperparathyroidism.
  2. When the blood calcium level is greater than 1 mg/dl above the upper limit of the reference range.
  3. When the kidneys have been affected. This is defined as high 24h urine calcium with increased kidney stone risk, history of kidney stones (regardless if they have pain), chronic kidney disease (based on creatinine levels), or calcium deposits in the kidneys as evidenced by images such as ultrasound (nephrocalcinosis).
  4. If you have osteoporosis, fractures attributed to osteoporosis, or vertebral fractures in the spine.
  5. If you are age 50 or younger.

Another consideration for surgery is if there is suspicion for parathyroid carcinoma, although this is exceedingly rare.

If Surgery is Indicated, How Do We Know Which Parathyroid Gland Should be Removed, and Where it is?

In order to minimize the risks and time of surgery, we do studies to localize the parathyroid adenoma(s). There are 2 studies that we perform in order to accomplish this.

The first study is ultrasound of your neck. This is a very good imaging study with a low cost, but the success of identifying the parathyroid adenoma greatly rests upon the experience of the sonographer: in centers with little experience in parathyroid localization, the adenoma can be identified in as little as 35% of the patients, while in centers with experienced sonographers, this rate increases to 82%! If the ultrasound is combined with a parathyroid sestamibi nuclear scan, this rate can increase to 92%! In addition, when a parathyroid adenoma is localized on ultrasound, this allows for a biopsy to provide proof that it is really there.

The second study is a parathyroid sestamibi scan. This is preferably done with SPECT, which provides better images and allows for better localization. This is more costly than ultrasound, and does not find all parathyroid adenomas. However, it is a good study when combined with ultrasound, because it can identify some parathyroid adenomas if they are in areas that the ultrasound cannot see, such as deep in the chest, or behind the trachea (windpipe).

A few years ago, a new tool called 4 dimensional CT scan was introduced to us. However, this has not been proven to be better than the combination of ultrasound and parathyroid sestamibi scan. In addition, it is much more expensive, and exposes the patient to a significant amount of radiation. Thus, we do not currently recommend the routine use of 4D CT.

Finally, even if all localizing studies fail to identify the parathyroid adenoma(s), this does not mean that surgery should not be performed! In fact, the most sensitive and reliable method of finding the adenoma(s) is surgery, as an experienced surgeon can identify these adenomas by visually inspecting the neck, even if the other studies could not find them.

Does Your Thyroid Need to be Evaluated Prior to Parathyroid Surgery?

Absolutely! Thyroid disease is very common in patients with primary hyperparathyroidism. Screening for thyroid dysfunction with thyroid function tests, and screening for thyroid nodules and cancer with a neck ultrasound, should be included in the evaluation of every patient. The thyroid ultrasound is accomplished at the same time as the parathyroid ultrasound, so no additional tests are needed. If a nodule is identified and meets criteria for biopsy, then this should be done prior to the parathyroid surgery, as, if it proves to be cancer, the thyroid can be removed at the same time as the parathyroid, and you can avoid a second surgery.

What Else Do You Need to Do to Prepare for Parathyroid Surgery?

The recommended calcium and vitamin D intake is the same for patients with primary hyperparathyroidism, as the general population. This means that you should aim to take at least 3 servings of dairy products daily to meet the calcium requirements. The vitamin D should be replaced with the guidance of your endocrinologist, as fast correction of low vitamin D can lead to significant elevations of calcium in patients with primary hyperparathyroidism.

How is Parathyroid Surgery Done?

There are 2 different approaches for parathyroid surgery:

Minimally Invasive Parathyroidectomy. This is a newer method, which takes advantage of the fact that 85% of patients with primary hyperparathyroidism have a single adenoma, and most of these will be identified with the above imaging techniques. This surgery is done by making a very small incision and going after the known parathyroid adenoma, which has already been localized. As soon as it is removed, a blood test for PTH is immediately done. If the PTH is at the expected level to prove cure, then the surgery is completed. If the PTH does not significantly decrease, then the parathyroid surgeon will perform further exploration of the neck to identify the hidden adenoma(s). When patients are appropriately selected for the Minimally Invasive Parathyroidectomy, cure can be achieved in 97-99% of the patients, all with a more limited surgery, shorter recovery, less postoperative discomfort, and smaller incision, as compared to the traditional bilateral exploration!

Bilateral Exploration. With this traditional approach, despite anything that the preoperative imaging shows, the surgeon opens and explores both sides of the neck in order to identify all 4 parathyroid glands. Then, all abnormal appearing parathyroid glands are removed. This is a time-tested approach, with very good results, and it is definitely the preferred approach when the preoperative localization studies have failed to identify the adenoma(s) or when there is a high suspicion that multiple glands are involved. However, it is no better than the Minimally Invasive Parathyroidectomy for the above appropriately selected patients and it is a more extensive surgery, with longer recovery period, more postoperative discomfort, and larger incision.

What are the Potential Complications of Parathyroid Surgery?

Parathyroid surgery can have complications due to damage of structures in the area of the operation. All of these complications happen more frequently if the surgery is done by an inexperienced surgeon. The complication rate dramatically drops at the hands of an experienced surgeon.

Recurrent laryngeal nerve paralysis. Since some of the parathyroid glands sit right next to the recurrent laryngeal nerves, which control the vocal cords and help have a clear voice, this can be damaged, and lead to hoarseness. If this happens, most of the time it is temporary and the nerve recovers, but sometimes it is permanent. If both nerves are damaged, then a permanent tracheostomy is needed because the vocal cords close and they cannot reopen, closing the trachea and not allowing breathing. Thankfully, this is extremely rare, and it can be avoided with a minimally invasive parathyroidectomy, as only one side of the neck is explored, and the recurrent laryngeal nerve on the other side is not exposed.

Hypoparathyroidism. This happens when many glands are removed, or their blood supply is damaged, and there is insufficient parathyroid tissue to produce PTH adequate to keep the calcium from dropping too low. If you develop hypoparathyroidism, you will require calcium and activated vitamin D products for the rest of your life. The risk of this complication is lower if there a single parathyroid adenoma, which has been successfully localized prior to surgery, as only the 2 parathyroid glands on one side of the neck are exposed during the operation.

Other complications. These are rare and also depend on the experience of the surgeon and anesthesiologist, and can include infection, bleeding, damage to other neck structures, problems with anesthesia, etc.

Special Situations:

Familial Hypocalciuric Hypercalcemia (FHH). This is an extremely rare genetic condition, in which the labs look like primary hyperparathyroidism, but you actually don’t have a parathyroid problem. It is caused by a genetic mutation in the kidney, making you reabsorb more calcium than normal. It does not have any symptoms, and nothing should be done about it. To differentiate from primary hyperparathyroidism, we do a 24h urine calcium at the same time as blood tests. However, if your labs look like primary hyperparathyroidism, you most likely do have it. FHH is 1 in a million, and the vast majority of the time it is found at a young age and other family members have it. If you don’t meet these criteria, you don’t have FHH.

Parathyroid cancer. This is also very rare, and is found in 1% of the patients with primary hyperparathyroidism. It typically presents with extremely high PTH and calcium levels, even hypercalcemic crisis. The chances of having parathyroid cancer are extremely low. However, it is important to have an experienced endocrinologist evaluate you, as suspicion of parathyroid cancer changes the evaluation and the surgical management.

Familial hyperparathyroidism. There are some genetic mutations that put patients at risk for primary hyperparathyroidism at a young age, and typically more than 1 glands are involved. These mutations may also put the patients at risk for other tumors. It is important to know if a genetic condition exists, because this information may change the type of surgery. It is generally recommended to undergo genetic testing if you are younger than 40 years with primary hyperparathyroidism with more than 1 gland involved, and also to consider genetic testing if you have a strong family history of primary hyperparathyroidism or other disorders that would make us suspect a genetic syndrome.

Why Choose the Thyroid Institute of Utah?

There are many reasons that you should choose the Thyroid institute of Utah, and they are all very important!

  • We have a team of doctors including endocrinologists and parathyroid surgeons, who are all experienced and specialize in parathyroid and thyroid disease.
  • Our evaluation starts from you! We will talk to you and listen to you. We will discuss your concerns and give you guidance. Then, our endocrinologists will proceed with a thorough evaluation with lab tests and imaging studies. This will help us find out if you truly have hyperparathyroidism, and which type it is. Based on the type, and the other indications discussed above, we will tell you if you need surgery, and if not, what treatments you need and what to expect in the future.
  • If you do need surgery, then our endocrinologists, who are expert sonographers and have performed countless ultrasounds for parathyroid localizations and many parathyroid biopsies, will perform a dedicated ultrasound of your neck, to try to localize the parathyroid adenoma(s), and also evaluate your thyroid. We will also order a parathyroid sestamibi scan with SPECT, to maximize the chances that the adenoma(s) will be localized. After the full evaluation has been completed, we will discuss the results with you, and refer you to our parathyroid surgeons.
  • Our parathyroid surgeons will then see you, and after reviewing all the studies that we performed, they will discuss with you the type of surgery that you need. The majority of the time, when the parathyroid adenoma has been localized, our surgeons will perform minimally invasive parathyroid surgery. We have excellent experience and outcomes with this surgery. We perform intraoperative PTH measurement to ensure success of surgery prior to closing. When the parathyroid adenoma has not been previously localized, or when the intraoperative PTH does not increase, then we will perform a traditional 4 gland exploration. That way, you can benefit from the less stressful minimally invasive parathyroidectomy, without having lower likelihood of cure!
  • Our parathyroid surgeons perform more than 50 parathyroid surgeries per year! Thus, they are considered high volume parathyroid surgeons, and you can enjoy a higher surgical success rate, with lower risk of complications. This is even more important in our area, as our data so far suggest that there is a higher rate of multi-gland disease. Only 72% of our patients have only 1 parathyroid adenoma, compared to the expected rate of 85%. In addition, 15% of our patients have 2 parathyroid adenomas, while 13% have 3 or 4 parathyroid glands involved. Together, these numbers make it even more important to have an experienced parathyroid surgeon, comfortable in both the minimally invasive parathyroidectomy, and the traditional 4 gland exploration. So far, our success rates have been excellent with persistence of hyperparathyroidism seen in only 5% of the patients!
  • The experience of our surgeons is also very important when looking at the complication rates. The recurrent laryngeal nerve is only injured temporarily 2% of the time, and these patients have all recovered their voice, so that the rate of permanent recurrent laryngeal nerve injury in our center is 0%! In terms of hypoparathyroidism/hypocalcemia after the surgery, temporary hypocalcemia is seen in 9% of the patients, with permanent hypocalcemia due to hypoparathyroidism seen in only 2% of patients, which is a very low risk, especially given the fact that we see multigland disease more frequently than the US average!
  • Finally, after the surgery, our endocrinologists will see you to make sure that your bone health, calcium and vitamin D intake are well taken care of, the hyperparathyroidism has been cured, and if hypocalcemia happens, to make sure it is treated.

So, call us today if your calcium or PTH is high, or for a second opinion before parathyroid surgery. We will do a thorough evaluation and take care of your treatment from start to finish!

Konstantinos Segkos, MD

Scott Shipley, MD