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Key Points

  1. Total thyroidectomy should be performed for all primary thyroid malignancies with the exception of anaplastic thyroid cancer.

  2. Protection of the recurrent laryngeal nerves is critical in thyroid and parathyroid procedures. Understanding of the anatomy and its potential variations can help prevent iatrogenic injuries.

  3. Ectopic and supernumerary parathyroid glands can pose a significant challenge to the surgeon. The surgeon should be prepared with sufficient preoperative imaging and knowledge of where to search for missing parathyroid glands.

  4. Minimally invasive adrenalectomy is less painful and associated with a quicker recovery in appropriately selected patients.

  5. Pheochromocytoma needs to be ruled out prior to resection of an adrenal mass. Patients with a pheochromocytoma require specific preoperative management with alpha-blockade.

Introduction

Functional endocrine neoplasms in the pediatric patient are rare; however, these lesions are the most frequent indication for surgical resection of endocrine glands. Oftentimes, the surgeon may need to perform a resection not only to alleviate symptoms due to hormone hypersecretion but to distinguish between benign and malignant neoplastic processes as well, particularly in this age where advanced radiographic imaging is bringing more incidental lesions to the clinician's attention. Other disorders of the endocrine glands may come to the surgeon's attention due to pathologic hyperfunction, enlargement, or for risk reduction. The surgeon should feel comfortable in the technical aspects of the surgical management of endocrine neoplasms and other endocrinopathies. In this chapter we focus on operative techniques for the management of thyroid, parathyroid, and adrenal neoplasms and endocrinopathies.

Thyroid Gland

Essentials of Diagnosis

  1. While thyroid nodules are less common in childhood compared to adults, the incidence of malignancy is higher.

  2. Medullary thyroid carcinoma may be indicative of multiple endocrine neoplasia syndrome IIA or IIB and pheochromocytoma should be ruled out in these patients.

  3. Ultrasound-guided fine needle aspiration is useful to evaluate a thyroid nodule in the adolescent but sampling error makes it a less useful diagnostic test in the pre-adolescent.

  4. The MACIS score is a prognostic tool for papillary and follicular variant thyroid cancers that is well established in adults. Its utility in the pediatric patient is not well studied.

Embryology

During the third fetal week, the thyroid gland originates as an outpouching from the foramen cecum at the base of the tongue. This outpouching solidifies into a bilobed structure as it descends below the cricoid cartilage, anterior to the trachea. Fibrous connective tissue, known as the ligament of Berry, anchors the thyroid gland to the trachea. The fourth branchial pouch gives rise to the para-follicular C-cells which will produce calcitonin. These cells are found laterally at the junction of the upper two-thirds of the thyroid gland, and for this reason, medullary thyroid carcinomas (which arise from the para-follicular C-cells) tend to be localized to this region.

Anatomy

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