Surgery for Secondary Hyperparathyroidism
Brendan C. Stack Jr
INTRODUCTION
Secondary hyperparathyroidism (sHPT) is not often a surgical disease. sHPT is in fact more prevalent than primary hyperthyroidism and is often not diagnosed or even considered by the surgeon evaluating the patient with hyperparathormonemia. Because of the high prevalence of sHPT, many patients presenting with primary hyperparathyroidism from either single- or multiple-gland disease in fact will have coexisting sHPT.
sHPT is most commonly a result of vitamin D deficiency. Vitamin D deficiency is now appreciated to be extremely prevalent but is not a surgical disease. If surgery is not urgent/emergent, it is advisable to hold off on surgery until the vitamin D level has been repleted to at least 30 ng/mL. This will remove confounding circumstances that might interfere with the proper treatment of the primary hyperparathyroidism, when postoperative parathyroid hormone (PTH) is elevated after a normal measurement(s) in the operating room.
sHPT has been recognized clinically for many decades. Classic sHPT is the second most common cause of sHPT and typically is seen in patients on chronic renal dialysis as a result of chronic renal insufficiency. Twenty-six million Americans have chronic kidney disease, and virtually all dialysis-dependent patients develop sHPT.
HISTORY
The classic history for sHPT is that of chronic renal failure requiring hemodialysis. These patients suffer from bone demineralization and chronic hypocalcemia, which serves to stimulate all parathyroid glands. This chronic parathyroid gland stimulation results in multigland hyperplasia. Usually years of dialysis are required before patients are referred for evaluation of sHPT, often with PTH values >500 ng/dL. These patients are evaluated for parathyroidectomy for a variety of reasons at this point but principally for an anticipated receipt of a donor kidney or to assist in the management of severe biochemical derangements that accompany chronic dialysis.
PHYSICAL EXAMINATION
Patients who have the suspicion of sHPT should undergo a comprehensive otolaryngologic and head and neck examination. Specific attention should be paid to the central and the inferior portions of the neck. There is a significant coexistence of thyroid lesions, and any thyroid lesions should be evaluated and managed based on their merits independent of the underlying diagnosis of sHPT. If surgery is contemplated, examination of the larynx should be undertaken to insure normal vocal cord function preoperatively. If a parathyroid is present as a palpable mass in the neck, concern is raised for parathyroid carcinoma.
INDICATIONS
The indication for surgery for sHPT is to correct hyperparathormonemia. This is desirable in patients being considered for renal transplant and patients in whom it is anticipated will be dialyzed for the rest of their life. Patients in the latter category may proceed to surgery for reasons of intractable bone disease, calciphylaxis, or other biochemical disorders refractory to medical treatment.
CONTRAINDICATIONS
The main contraindication to surgical treatment for sHPT is the poor medical condition of many of these patients on presentation for surgical consultation. These patients require inpatient observation, and their course can be complicated with profound postoperative hypocalcemia requiring intensive replacement therapy due to their significantly demineralized bones.
PREOPERATIVE PLANNING
Imaging Studies
Ultrasound (US) has become the most popular form of imaging of thyroid and parathyroid disease. Most US examinations can be performed in the outpatient setting, preferably by the thyroid/parathyroid surgeon. Dedicated thyroid ultrasonographers have a higher level of expertise in imaging in the central and inferior regions of the neck than the average radiologist who would customarily supervise a US technician in the radiology department and review only static images. The US imaging will not be able to see parathyroids that are not pathophysiologically enlarged. While US has more than 90% sensitivity in detecting single-gland parathyroid disease, in multigland sHPT the sensitivity is only 40% to 60%. The superior glands can be more challenging to image by US due to their posterior location and the overlying thyroid tissue. Inferior glands have a more variable location and higher likelihood of being ectopic. sHPT from vitamin D deficiency is a milder form, in which it would be uncommon to image any enlarged parathyroid glands by US in this patient population.
Sestamibi imaging is also frequently used for parathyroid localization, particularly if radio-guided surgical excision is planned. In patients in whom surgery is not indicated, this modality would not typically be required for the diagnosis or medical treatment of sHPT. Radio-guided surgery is a helpful adjunct in cases of renal-induced sHPT where the PTH is elevated above 1,000 pg/mL. Hyperplastic glands take up the radiotracer, assisting in the identification and confirmation of the identity of a hyperplastic gland.
Laboratory
A clear biochemical diagnosis of hyperparathyroidism should be the goal of the preoperative evaluation. For sHPT serum calcium and intact parathormone (PTH) should be measured. Typically the calcium is low normal or low, and the PTH is very elevated. Overall, low calcium reflects chronic depletion of the body’s calcium stores. Vitamin D testing is also very important in the sHPT patient population. If a patient is operated on for parathyroid disease with preexisting vitamin D deficiency, this could confound the results of any intraoperative parathyroid testing that might be used as part of the operative procedure, returning inappropriately elevated. Additionally, postoperative parathormonemia could result from vitamin D deficiency and not simply be viewed as a surgical failure.
SURGICAL TECHNIQUE
In the event of severe parathormonemia (>1,000 pg/mL) in patients with chronic renal insufficiency in which renal transplant is being contemplated, subtotal parathyroidectomy is indicated for sHPT. It is anticipated that with these patients, renal transplant will be successful, the kidney function will return to normal, and the chronic stimulation causing the parathyroid tissue to be hyperplastic will resolve. Therefore, a normal functioning parathyroid gland would be left in the neck with a small chance of needing additional revision surgery. If renal transplant is not contemplated or previously failed, total parathyroidectomy should be considered. This can be done in conjunction with primary or delayed autotransplantation in the forearm. I prefer primary autotransplantation. Cryopreservation is a critical resource to have when managing sHPT patients.
The Minimally Invasive Radio-Guided Parathyroidectomy
Once the diagnosis has been made and US imaging has been obtained, preoperative injection of sestamibi may facilitate the radio-guided approach to the parathyroidectomy. A 2-cm incision is made halfway between the
cricoid cartilage and the sternal notch as has been described by Norman. Once the incision is open, subcutaneous adipose tissue is removed down to the level of the strap muscles in the midline. This provides exposure and working space through this minimal access incision. Blunt dissection is then used in this pocket, and the incision is then kept open with a retractor. The midline is opened with a Bovie cautery. Both the superficial and deep levels of strap muscles are separated in the midline vertically. Both the unilateral and bilateral exploration can then be performed through this approach (Figs. 21.1, 21.2 and 21.3).
cricoid cartilage and the sternal notch as has been described by Norman. Once the incision is open, subcutaneous adipose tissue is removed down to the level of the strap muscles in the midline. This provides exposure and working space through this minimal access incision. Blunt dissection is then used in this pocket, and the incision is then kept open with a retractor. The midline is opened with a Bovie cautery. Both the superficial and deep levels of strap muscles are separated in the midline vertically. Both the unilateral and bilateral exploration can then be performed through this approach (Figs. 21.1, 21.2 and 21.3).