eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Head & Neck Surgery
Hyperparathyroidism: Treatment
Updated: Aug 31, 2009
Treatment
Medical Therapy
Controversy exists regarding the need and timing of surgery in asymptomatic patients who have slow progressive parathyroid disease. If patients are to be medically observed, the potential renal and bone disease should be periodically assessed.
In patients who are not candidates for surgery, ultrasound-guided alcohol ablation and angiographic embolization are considerations.
Medical management of hyperparathyroidism is generally reserved for patients with poor medical conditions, advanced age with mild hypercalcemia, no evidence of dementia, and no significant bone demineralization. Serum calcium levels should be only mildly elevated, and renal status, bone density, and bone mass should be normal. Severe hypercalcemia (> 14mg/dL) may be a medical emergency, and the initial treatment requires intravenous saline infusions, diuresis, calcium binders and bisphosphonates.) Medical follow-up care may also be indicated when neck exploration has not been successful.
Medical follow-up care should include biannual measurements of serum calcium levels, parathyroid hormone (PTH) levels, and bone mass, as well as assessment of renal status. Ask patients specifically about symptoms of weakness, fatigue, and depression.
Surgical Therapy
The surgical procedure selected to remove the pathologic gland or glands depends on the philosophy and experience of the surgeon and the nature of the disease in each patient. A focused parathyroidectomy is effective in most cases; however, some controversy exists regarding the following procedures:
- Unilateral versus bilateral explorations in patients with primary hyperparathyroidism
- Total parathyroidectomy versus seven-eighth parathyroidectomy in patients with chronic renal failure or diffuse, nonrenal hyperplasia.
Intraoperative Details
Adenoma and hyperplasia
Parathyroid adenomas vary in size and shape and are often bilobed. A halo of fat, at least at one pole, usually surrounds parathyroid adenomas and aids in identification. The gland often turns red-brown when manipulated, probably because of vasospasm of its feeding artery.
Classical surgical approach
This technique was most often performed between 1930 and 1960. The standard, time-honored (but dated) approach uses a typical thyroidectomy collar incision with wide exposure. The upper and lower subplatysmal flaps are raised, and the anterior cervical veins are not ligated and are left on the anterior surface of the strap muscles. The strap muscles are separated in the midline, exposing the thyroid isthmus. On the side to be dissected, the area lateral to the inferior thyroid veins is explored first, and an inferior adenoma is often visible with minimal dissection. If an inferior adenoma is not visible, the lateral aspect of the thyroid lobe is rotated anteromedially and the superior gland is usually seen on the posterior superior surface of the lobe. When present, the middle thyroid vein may require ligation.
The inferior thyroid artery (ITA) is identified just medial to the carotid artery, and a vessel loop is placed on the ITA. The artery is followed medially, and the recurrent laryngeal nerve (RLN) is always identified passing either over or under the artery. With careful dissection, the distal branches of the artery can be seen feeding each normal or pathologic parathyroid gland. Before the gland is removed, the RLN is followed to its point of entry into the cricothyroid membrane. Bleeding is usually minimal with this technique; minimal bleeding is controlled with bipolar cautery. The distal arterial branches are tied, when necessary.
Once the adenoma has been removed and sent for frozen section diagnosis of parathyroid tissue, the ipsilateral gland is identified. If the gland appears normal, it is not disturbed. If the gland is enlarged, biopsy is performed, and the specimen sent to confirm the presence of hyperplasia. If both glands are found to be hyperplastic, the opposite side of the neck is explored. If the 2 contralateral glands are confirmed as hyperplastic, most surgeons perform either a seven-eighth parathyroidectomy or a total parathyroidectomy with autotransplantation of a small fragment into forearm musculature for easy access in the future.
General endotracheal anesthesia was standard. Operative time was longer since a bilateral exploration was done. A postoperative drain was routinely used and an overnight stay was typical.
Minimally invasive approach
This technique became popular in the 1990s and is most often used today. A small transverse incision is placed in the lower neck, off the midline, on the side of the adenoma. Both ipsilateral parathyroid glands can be reached through the small incision with aggressive retraction. The dissection is minimal and exposure is limited. The amount of dissection may be further reduced if intraoperative PTH monitoring or intraoperative radioguidance is used. Instead of separating the strap muscles in the midline, the lateral aspect of the sternothyroid and sternohyoid muscles are separated from the medial border of the sternocleidomastoid muscle. The carotid sheath is exposed and the adenoma removed.
This approach can easily be performed with laryngeal mask anesthesia or under local anesthesia with intravenous sedation in carefully selected patients. This approach has been proven to be safe in adult patients of all ages, including the elderly.6 Morbidity is reduced. A drain is not required. Operative time is further reduced, and often the patient can be discharged from the same day unit within an hour or 2 after surgery, even in morbidly obese patients. Preoperative loading with oral calcium citrate makes postoperative hypocalcemia extremely unlikely.
There is no learning curve for this technique in the hands of an experienced parathyroid surgeon.
Renal (secondary) hyperparathyroidism
Generally, but not always, diffuse hyperplasia of all 4 glands is present. The glands may be voluminous and weigh in excess of 100 grams. The classic surgical approach is used. Each RLN is identified. All abnormal parathyroid tissue is removed. When fewer than 4 glands are hyperplastic, the normal sized glands may be left intact. When 4 large hyperplastic glands are removed, a small fragment of one gland may be reimplanted into a forearm muscle or strap muscle. The treating nephrologist often prefers reimplantation in order to preserve some parathyroid function. Total postoperative hypoparathyroidism is more debilitating and more difficult to medically manage than an autotransplanted patient with a small fragment of revitalized parathyroid tissue.
Tertiary hyperparathyroidism
In patients with tertiary hyperparathyroidism, the residual tissue or adenoma is usually identified preoperatively with ultrasonography and sestamibi scan, allowing rapid access to the involved area. Scarring from prior surgery can be problematic and may place the RLN at risk of iatrogenic injury. The surgical approach in this situation is to proceed to the localized site immediately and to remove the lesion with as little dissection as possible. The RLN may not be routinely exposed, especially if severe scarring is present from prior surgery. This surgery usually removes all remaining parathyroid tissue and results in permanent hypoparathyroidism.
Parathyroid carcinoma
When the rare parathyroid carcinoma is encountered, the goal is wide local excision with selective neck dissection, which allows removal of metastatic nodal disease and all involved soft tissues. The mass is usually large, gray-white, and locally invasive. Aggressively removing the mass, along with the ipsilateral thyroid gland and adjacent soft tissue, is required. Postoperative radiotherapy may be necessary in cases with residual tumor. Patients with recurrent parathyroid carcinoma can be identified based on rising PTH levels.
Familial parathyroid disorders
As mentioned above, the hereditary parathyroid disorders require an advanced knowledge of the genetics of transmission, associated conditions, and special treatment considerations (see Table 2).
Table 2. Surgical Therapy in Patients With Hereditary Parathyroid Disorders
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Table
| Disorder | Dominant Feature | Treatment | Notable Facts |
| MEN1 | Hyperparathyroidism | Total parathyroidectomy with search | Recurrence inevitable |
| MEN2A | Medullary carcinoma | Removal of single | Milder |
| HPT-JT | Severe hypercalcemia; | Uniglandular but | Only 30 families |
| FIHPT | ... | Complex | Linked to MEN1 gene, |
| ADMH | ... | Subtotal parathyroidectomy | CASR mutation |
| FHH | Usually asymptomatic; | No benefit from | CASR mutation |
| NSHPT | Very high PTH level, severe | Total parathyroidectomy within first | CASR mutation |
| Disorder | Dominant Feature | Treatment | Notable Facts |
| MEN1 | Hyperparathyroidism | Total parathyroidectomy with search | Recurrence inevitable |
| MEN2A | Medullary carcinoma | Removal of single | Milder |
| HPT-JT | Severe hypercalcemia; | Uniglandular but | Only 30 families |
| FIHPT | ... | Complex | Linked to MEN1 gene, |
| ADMH | ... | Subtotal parathyroidectomy | CASR mutation |
| FHH | Usually asymptomatic; | No benefit from | CASR mutation |
| NSHPT | Very high PTH level, severe | Total parathyroidectomy within first | CASR mutation |
Ancillary intraoperative tools
- Intraoperative rapid PTH monitoring (i-PTH): Although intraoperative rapid PTH monitoring is not necessary for routine cases in which the location of the adenoma is preoperatively known, it has special value in reexplorations and when a second adenoma is suspected. The chemoluminescent immunoreactive technique has demonstrated PTH to have a half life of 3-5 minutes. The circulating PTH level has been shown to be reduced by 50% within 10 minutes following successful removal of an adenoma. Additional equipment, time, and expense are necessary. A venous baseline sample is taken prior to the skin incision and then at 5 and 10 minutes after specimen removal. The turnaround time for results, in the best of hands, is 10-15 minutes.
- Intraoperative total serum calcium monitoring: In all cases, the serum calcium level has been shown to drop within 5 minutes of successful removal of a parathyroid adenoma. Calcium monitoring is less expensive than i-PTH monitoring and is readily available in all hospitals.
- Intraoperative sestamibi radiomonitoring (gamma probe):7 Minimally invasive radioguided parathyroidectomy requires that surgery be performed within 1.5-3 hours after the injection of sestamibi so that the adenoma still has a high gamma emission. The hand-held probe guides the surgeon to the area of increased activity. The excised specimen still emits radioactivity greater than 20% of the background check.
- Endoscopic parathyroidectomy
- The pure endoscopic approach requires preoperative localization of a single adenoma. The technique is contraindicated in patients with a history of prior neck surgery, prior neck radiation, abnormal anatomy, and multigland disease. Reported complications include dramatic subcutaneous emphysema from chin to scrotum. Hypercapnia and tachycardia have also been reported. Exposure is limited and the working space is small.
- A video-assisted technique uses carbon dioxide insufflation for only 3 minutes at the start of the procedure and begins the dissection under the strap muscles. A small skin incision (1.5 cm) in the midline is made and used to perform the parathyroidectomy on the video monitor.
- Transaxillary and submammary endoscopic approaches: Emerging reports describe transaxillary and submammary endoscopic approaches to parathyroid disease that use laparoscopic techniques.
Postoperative Details
Monitoring of serum calcium levels and management of hypocalcemia are the main tasks of postoperative care. This is minimized by preoperative calcium and vitamin D loading. Prevention of a wound seroma with an appropriate closed suction drain is necessary if a wide dissection as been performed. The skin is approximated with a skin adhesive (Dermabond). Sutures or staples, if used, are removed in 3 days; removal is delayed in patients with renal disease.
Transient hypocalcemia in patients with primary hyperparathyroidism is generally mild, and the serum calcium level slowly drops and gradually returns to normal without tetany. Oral calcium supplements may be given for a period of weeks with calcitriol (Rocaltrol) 0.25 mg 2 or 3 times per day to enhance GI absorption.
Dramatic hypocalcemia due to severe bone hunger is observed in patients with secondary and tertiary parathyroidectomy due to marked calcium depletion from bone, which is often rapidly and acutely reversed in the first few hours postoperatively in patients with chronic renal disease. Hungry bone uptake of ionized serum calcium is so swift that a continuous calcium infusion is often needed to avoid tetany. Continuous calcium infusion is required until oral calcium and vitamin D supplements begin to maintain the serum calcium levels near the reference range. Calcitriol (Calcijex) injection during dialysis is often required.
Calcitriol (the active form of vitamin D-3) injection in patients with renal disease, a population in whom treatment proves difficult, stimulates intestinal calcium absorption and aids in the treatment of chronic hypocalcemia.
Follow-up
A primary care physician or surgeon observes patients with primary hyperparathyroidism annually to monitor serum calcium levels; patients with genetic parathyroid disorders may develop a second adenoma or recurrent hyperparathyroidism.
Patients with chronic renal disease are monitored indefinitely because of the nature of their disease and its intimate relationship to kidney disease and phosphorus retention.
Complications
Transient hypocalcemia is anticipated but may be mild and without clinical signs. This is especially true if the patient is preloaded with calcium citrate and vitamin D for 1-2 weeks prior to surgery. Difficult-to-manage severe hypocalcemia can lead to tetany if not treated. Permanent hypoparathyroidism and recurrent laryngeal nerve injury are also potential complications. If the adenoma is not found or if a second adenoma is not identified, hypercalcemia will persist.
More on Hyperparathyroidism |
| Overview: Hyperparathyroidism |
| Workup: Hyperparathyroidism |
 Treatment: Hyperparathyroidism |
| Follow-up: Hyperparathyroidism |
| Multimedia: Hyperparathyroidism |
| References |
| Further Reading |
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Further Reading
Clinical guidelines
National Institute for Health and Clinical Excellence (NICE). Cinacalcet for the treatment of secondary hyperparathyroidism in patients with end-stage renal disease on maintenance dialysis therapy. London (UK): National Institute for Health and Clinical Excellence (NICE); 2007 Jan. 28 p.
AACE/AAES Task Force on Primary Hyperparathyroidism. The American Association of Clinical Endocrinologists and the American Association of Endocrine Surgeons position statement on the diagnosis and management of primary hyperparathyroidism. Endocr Pract 2005 Jan-Feb;11(1):49-54.
Keywords
hyperparathyroidism, osteitis fibrosa cystica, parathyroid enlargement, parathyroid glands, parathyroid adenoma, renal stones, nephrocalcinosis, parathyroid hormone, PTH, diffuse hyperplasia, parathyroid carcinoma, multiple endocrine neoplasia type 1, MEN1, Wermer syndrome, multiple endocrine neoplasia 2A, MEN2A, Sipple syndrome, familial hypocalciuric hypercalcemia, FHH, familial benign hypercalcemia, cystic parathyroid adenomas, primary hyperparathyroidism, secondary hyperparathyroidism, tertiary hyperparathyroidism, autonomous hypercalcemic hyperparathyroidism, fibrous osseous dysplasia, cystic bone lesion, giant cell lesion, parathyroidectomy, seven-eighth parathyroidectomy, total parathyroidectomy, thyroid problems, thyroid abnormalities, parathyroid problems, parathyroid abnormalities, thyroid glands, osteitis, HPT-JT, jaw tumor syndrome, familial isolated hyperparathyroidism, neonatal severe hyperparathyroidism
