Hyperparathyroidism in Otolaryngology and Facial Plastic Surgery Workup

  • Author: James LaBagnara, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Aug 23, 2011
 

Laboratory Studies

  • Diagnosis is made based on hypercalcemia and elevated parathyroid hormone (PTH) levels. Other abnormal laboratory findings may include elevated BUN and creatinine levels, hyperchloremic acidosis, reduced serum bicarbonate levels due to renal bicarbonate casting, hypophosphatemia, elevated alkaline phosphatase levels and hypercalciuria.
  • Other causes of hypercalcemia (eg, paraneoplastic syndromes, malignancies, Paget disease, drug-induced causes, dietary causes) are not associated with PTH level elevation. However, occasionally, primary hyperparathyroidism and malignancy-related hypercalcemia may coexist.
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Imaging Studies

  • The importance of preoperative localization studies can substantially reduce operative time, cost, and patient morbidity. This is important in the era of managed care and operating room cost containment. Without preoperative localization, a parathyroid adenoma is successfully identified and removed in more than 95% of patients, although this may require exploration of all 4 glands.
  • Accurate localization can limit exploration to the identified side, allowing rapid removal of the adenoma. If a second adenoma is present, both adenomas are frequently identified with preoperative ultrasonography and sestamibi scan, even if the lesion is in an ectopic location. Localization may not reduce the need for a later reexploration for a mediastinal adenoma. A list of noninvasive imaging modalities and their usefulness and ease of performance appears below, in order of increasing cost, as follows:
    • High-resolution ultrasonography: In the hands of an experienced ultrasonographer, this method is the most economic and may provide maximum information. It shows enlarged parathyroid glands and their relationship to relevant neck anatomy, thyroid nodules, and lymph nodes. High-resolution ultrasonography can reveal multiple adenomas, hyperplasia of all 4 glands, and glands in ectopic cervical locations such as within the carotid sheath or thyroid. However, high-resolution ultrasonography can not identify mediastinal adenomas.
    • Technetium-99m labeled sestamibi scan
      • This nuclear material has a specific affinity for abnormal parathyroid tissue. Although uptake also occurs in thyroid tissue, technetium-99m rapidly diminishes in the thyroid but is retained in the parathyroid mitochondria.
      • Sestamibi scan is useful in identifying single and multiple parathyroid adenomas and hyperplasia. Sestamibi also can reveal ectopic glands.
      • Although sestamibi is most often used preoperatively, it can also be used intraoperatively. Intrathoracic adenomas can also be identified despite the overlying sternum. A sestamibi scan that fails to reveal an adenoma in a patient with hypercalcemia and elevated PTH levels may suggest diffuse hyperplasia of all 4 glands or the presence of an adenoma that has a cell population that consists mainly of chief cells.
      • Although sestamibi is very sensitive with single adenomas, it fails to reveal 17% of second adenomas and 55% of hyperplastic glands. The outcome of the sestamibi scan is most influenced by the size of the adenoma; scans of lesions less than 2 cm in size are often difficult to interpret. Since sestamibi is concentrated in mitochondria, the sensitivity of sestamibi has histopathologic considerations that vary by the predominant cell type within the adenoma.
      • Adenomas that are rich in Oxyphil cells have a higher mitochondrial content, greater metabolic activity, and increased radiotracer uptake. Adenomas that are predominantly chief cells have minimal mitochondrial content and minimal radiotracer uptake.
    • CT scan: CT scanning provides excellent spatial resolution and greater detail than the images obtained in a single plane. CT scans can be reconstructed for additional views. The location of an enlarged gland can be precisely defined in relation to adjacent anatomy. CT scan is helpful in locating mediastinal adenomas as well.
    • MRI: MRI provides excellent contrast resolution; images can be formatted in multiple planes (ie, axial, coronal, sagittal). Increased vascularity of the adenomas is ideal for identification with this modality. MRI may be useful in locating mediastinal adenomas.
  • Combination of ultrasonography and sestamibi scan provides maximum information and is cost effective.
  • CT scan or MRI for mediastinum adenomas may be required when an adenoma is suspected in the thorax.
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Histologic Findings

In primary hyperparathyroidism, the adenomas represent true neoplasms. Diffuse hyperplasia occurs in the absence of an adenoma. Hyperplasia of all 4 glands is often dramatic in renal (secondary) hyperparathyroidism with significantly increased gland volumes and weights.

Frozen section differentiation of an adenoma from hyperplasia is difficult for the pathologist. In the operating room, the surgeon primarily wishes to know that the specimen contains parathyroid tissue. An adenoma can be identified on permanent section if the surrounding halo or rim of fat is visible along with certain cellular characteristics. An experienced surgeon can usually identify an adenoma in situ based on its size and color as compared with a normal parathyroid gland, lymph node, or globule of fat.

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Contributor Information and Disclosures
Author

James LaBagnara, MD  Chief, Division of Otolaryngology, Department of Surgery, St Joseph's Regional Medical Center; Associate Clinical Professor of Otolaryngology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School

James LaBagnara, MD is a member of the following medical societies: Academy of Medicine of New Jersey, Aerospace Medical Association, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, American Society for Head and Neck Surgery, and Medical Society of New Jersey

Disclosure: Nothing to disclose.

Specialty Editor Board

Mimi S Kokoska, MD  Associate Professor, Department of Otolaryngology-Head and Neck Surgery, University of Arkansas for Medical Sciences; Chief, Department of Otolaryngology-Head and Neck Surgery, Central Arkansas Veterans Healthcare System

Mimi S Kokoska, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Physician Executives, American College of Surgeons, American Head and Neck Society, and Arkansas Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Karen H Calhoun, MD, FACS, FAAOA  Professor, Department of Otolaryngology-Head and Neck Surgery, Ohio State University College of Medicine

Karen H Calhoun, MD, FACS, FAAOA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Medical Association, American Rhinologic Society, Association for Research in Otolaryngology, Society of University Otolaryngologists-Head and Neck Surgeons, Southern Medical Association, Texas Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA  Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo Consulting; Medvoy Ownership interest Management position; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

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Normal parathyroid glands as seen during a thyroidectomy. The large arrow points to the superior parathyroid. The thinner arrow points to the inferior parathyroid. The forceps points toward the recurrent laryngeal nerve. The patient's head is toward the right.
Table 1. Genetics, Findings, and Associated Conditions
DisorderInheritanceGeneChromosomePenetrance and FindingsAssociated



Conditions and



Cancers



MEN1Autosomal



dominant



MEN111q1390% penetrance,



multiple glands



Pituitary,



neuroendocrine,



pancreatic tumors;



foregut carcinoid



MEN2AAutosomal



dominant



RET10q21Low penetrance, (approximately 20%),



usually single adenoma,



may be multiglandular



Medullary carcinoma



thyroid (C-cell)



pheochromocytoma



HPT-JTAutosomal



dominant



HRPT21q21-q32Cystic parathyroid



tumors, 15% risk of CA



Jaw tumors, renal



lesions



FIHPTAutosomal



dominant



Autosomal



dominant



HRPT2



MEN1



1q21-q32



11q13



Adenoma, multiglandular



Adenoma, multiglandular



...
ADMHAutosomal



dominant



CASR3q13-q21Multiglandular adenoma...
FHH...CASR



heterozygous



3q13-q21Mildly hyperplasticMildest form of



hyperparathyroidism



NSHPT...CASR



homozygous



3q13-q21Markedly hyperplasticSeverest form of



hyperparathyroidism,



very high PTH level,



severe



hypercalcemia



Table 2. Surgical Therapy in Patients With Hereditary Parathyroid Disorders
DisorderDominant FeatureTreatmentNotable Facts
MEN1HyperparathyroidismTotal parathyroidectomy with search



for ectopic



supernumerary glands;



transcervical



thymectomy;



autotransplantation



Recurrence inevitable
MEN2AMedullary carcinomaRemoval of single



adenoma,



normal-appearing



glands left in situ



Milder



hyperparathyroidism;



often asymptomatic



HPT-JTSevere hypercalcemia;



cystic parathyroid tumors



Uniglandular but



total parathyroidectomy



(may reduce risk of cancer)



Only 30 families



reported; 15% risk of



carcinoma



FIHPT...Complex



management.



Single adenoma treated with resection;



multiglandular disease treated with subtotal parathyroidectomy



Linked to MEN1 gene,



HRPT gene and CASR



gene mutation



ADMH...Subtotal parathyroidectomyCASR mutation
FHHUsually asymptomatic;



hypercalcemia at birth but little long-term



morbidity



No benefit from



parathyroid surgery



of mildly enlarged



glands; total parathyroidectomy with autotransplantation for severe forms



CASR mutation
NSHPTVery high PTH level, severe



hypercalcemia



Total parathyroidectomy within first



months of life (condition often lethal)



CASR mutation
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