Thyroid

Embryology:
The thyroid gland arises as an outpouching of the primitive
foregut around the third week of gestation. It originates at the
base of the tongue at the foramen cecum.

During its descent, the anlage remains connected to the

foramen cecum via an epithelial-lined tube known as the
thyroglossal duct.

Developmental Abnormalities
Thyroglossal Duct Cyst and Sinus:
Thyroglossal duct cysts are the most commonly encountered
congenital cervical anomalies. During the fifth week of
gestation, the thyroglossal duct lumen starts to obliterate, and
the duct disappears by the eighth week of gestation.

Rarely, the thyroglossal duct may persist in whole, or in

part.

Thyroglossal duct cysts may occur anywhere along the

migratory path of the thyroid although 80% are found
injuxtaposition to the hyoid bone.

They are usually asymptomatic but occasionally become

infected by oral bacteria, prompting the patient to seek
medical advice. Thyroglossal duct sinuses result from infection
of the cyst secondary to spontaneous or surgical drainage of
the cyst and are accompanied by minor inflammation of the
surrounding skin. Histologically, thyroglossal duct cysts are
lined by pseudostratified ciliated columnar epithelium and
squamous epithelium, with heterotopic thyroid tissue present
in 20% of cases.

The diagnosis usually is established by observing a 1- to 2-cm,

smooth, well-defined midline neck mass that moves upward
with protrusion of the tongue. Routine thyroid imaging is not
necessary although thyroid scintigraphy and ultrasound have
been performed to document the presence of normal thyroid
tissue in the neck. Treatment involves the "Sistrunk operation,"
which consists of en bloc cystectomy and excision of the central
hyoid bone to minimize recurrence. Approximately 1% of
thyroglossal duct cysts are found to contain cancer, which is
usually papillary (85%). The role of total thyroidectomy in this
setting is controversial, but is advised in older patients with
large tumors, particularly if there are additional thyroid nodules
and evidence of cyst wall invasion or lymph node metastases.1
Squamous, Hürthle cell, and anaplastic cancers also have been
reported but are rare. Medullary thyroid cancers (MTCs) are,
however, not found in thyroglossal duct cysts.

Lingual Thyroid
A lingual thyroid represents a failure of the median thyroid
anlage to descend normally and may be the only thyroid tissue
present. Intervention becomes necessary for obstructive
symptoms such as choking, dysphagia, airway obstruction, or
hemorrhage. Many of these patients develop hypothyroidism.
Medical treatment options include administration of exogenous
thyroid hormone to suppress thyroid-stimulating hormone
(TSH) and radioactive iodine (RAI) ablation followed by
hormone replacement. Surgical excision is rarely needed but, if
required, should be preceded by an evaluation of normal thyroid
tissue in the neck to avoid inadvertently rendering the patient
hypothyroid.

Ectopic Thyroid
Normal thyroid tissue may be found anywhere in the central
neck compartment, including the esophagus, trachea, and
anterior mediastinum. Thyroid tissue has been observed adjacent
to the aortic arch, in the aortopulmonary window, within the
upper pericardium, or in the interventricular septum. Often,
"tongues" of thyroid tissue are seen to extend off the inferior
poles of the gland and are particularly apparent in large goiters.
Thyroid tissue situated lateral to the carotid sheath and jugular
vein, previously termed lateral aberrant thyroid, almost always
represents metastatic thyroid cancer in lymph nodes, and not
remnants of the lateral anlage that had failed to fuse with the
main thyroid, as previously suggested by Crile. Even if not
readily apparent on physical examination or ultrasound imaging,
the ipsilateral thyroid lobe contains a focus of papillary thyroid
cancer (PTC), which may be microscopic.

Pyramidal Lobe
Normally the thyroglossal duct atrophies, although it may
remain as a fibrous band. In about 50% of individuals, the distal
end that connects to the thyroid persists as a pyramidal lobe
projecting up from the isthmus, lying just to the left or right of
the midline. In the normal individual, the pyramidal lobe is not
palpable, but in disorders resulting in thyroid hypertrophy (e.g.,
Graves' disease, diffuse nodular goiter, or lymphocytic
thyroiditis), the pyramidal lobe usually is enlarged and palpable.

Thyroid Anatomy
The adult thyroid gland is brown in color and firm in
consistency, and is located posterior to the strap muscles.
The normal thyroid gland weighs approximately 20 g, but gland
weight varies with body weight and iodine intake.

Blood Supply
The superior thyroid arteries arise from the ipsilateral external
carotid arteries and divide into anterior and posterior branches at
the apices of the thyroid lobes. The inferior thyroid arteries arise
from the thyrocervical trunk shortly after their origin from the
subclavian arteries. The inferior thyroid arteries travel upward in
the neck posterior to the carotid sheath to enter the thyroid lobes
at their midpoint. A thyroidea ima artery arises directly from the
aorta or innominate in 1 to 4% of individuals to enter the
isthmus or replace a missing inferior thyroid artery. The inferior
thyroid artery crosses the recurrent laryngeal nerve (RLN),
necessitating identification of the RLN before the arterial
branches can be ligated. The venous drainage of the thyroid
gland occurs via multiple small surface veins, which coalesce to
form three sets of veins—the superior, middle, and inferior
thyroid veins. The superior thyroid veins run with the superior
thyroid arteries bilaterally. The middle vein or veins are the least
consistent. The superior and middle veins drain directly into the
internal jugular veins. The inferior veins often form a plexus,
which drains into the brachiocephalic veins.

Nerves
The left RLN arises from the vagus nerve where it crosses the
aortic arch, loops around the ligamentum arteriosum, and
ascends medially in the neck within the tracheoesophageal
groove. The right RLN arises from the vagus at its crossing with
the right subclavian artery. The nerve usually passes posterior to
the artery before ascending in the neck, its course being more
oblique than the left RLN. Along their course in the neck, the
RLNs may branch, and pass anterior, posterior, or interdigitate
with branches of the inferior thyroid artery . The right RLN may
be nonrecurrent in 0.5 to 1% of individuals and often is
associated with a vascular anomaly. Nonrecurrent left RLNs are
rare but have been reported in patients with situs inversus and a
right-sided aortic arch. The RLN may branch in its course in the
neck and identification of a small nerve should alert the surgeon
to this possibility. Identification of the nerves or their branches
often necessitates mobilization of the most lateral and posterior
extent of the thyroid gland, the tubercle of Zuckerkandl, at the
level of the cricoid cartilage. The last segments of the nerves
often course below the tubercle and are closely approximated to
the ligament of Berry. Branches of the nerve may traverse the
ligament in 25% of individuals, and are particularly vulnerable
to injury at this junction. The RLNs terminate by entering the
larynx posterior to the cricothyroid muscle. The RLNs innervate
all the intrinsic muscles of the larynx, except the cricothyroid
muscles, which are innervated by the external laryngeal nerves.
Injury to one RLN leads to paralysis of the ipsilateral vocal
cord, which comes to lie in the paramedian or the abducted
position. The paramedian position results in a normal, but weak
voice, whereas the abducted position leads to a hoarse voice and
an ineffective cough. Bilateral RLN injury may lead to airway
obstruction, necessitating emergency tracheostomy, or loss of
voice. If both cords come to lie in an abducted position, air
movement can occur, but the patient has an ineffective cough
and is at increased risk of repeated respiratory tract infections
from aspiration.

Benign Thyroid Disorders

Hyperthyroidism
The clinical manifestations of hyperthyroidism result from an
excess of circulating thyroid hormone. Hyperthyroidism may
arise from a number of conditions. It is important to distinguish
disorders such as Graves' disease and toxic nodular goiters that
result from increased production of thyroid hormone from those
disorders that lead to a release of stored hormone from injury to
the thyroid gland (thyroiditis) or from other nonthyroid gland–
related conditions. The former disorders lead to an increase in
radioactive iodine uptake (RAIU), whereas the latter group is
characterized by low RAIU. Of these disorders, Graves' disease,
toxic multinodular goiter, and solitary toxic nodule are most
relevant to the surgeon.
Differential Diagnosis of Hyperthyroidism

Increased Hormone Synthesis Release of Preformed Hormone (Decreased

(Increased RAIU) RAIU)
Graves' disease (diffuse toxic Thyroiditis—acute phase of Hashimoto's
goiter) thyroiditis, subacute thyroiditis
Toxic multinodular goiter
Plummer's disease (toxic adenoma)
Drug induced—amiodarone, iodine Factitious (iatrogenic) thyrotoxicosis
"Hamburger thyrotoxicosis"
Thyroid cancer
Struma ovarii
Hydatidiform mole
TSH-secreting pituitary adenoma

RAIU = radioactive iodine uptake; TSH = thyroid-stimulating hormone.

Diffuse Toxic Goiter (Graves' Disease)

Graves' disease is by far the most common cause of
hyperthyroidism in North America, accounting for 60 to 80% of
cases. It is an autoimmune disease with a strong familial
predisposition, female preponderance (5:1), and peak incidence
between the ages of 40 to 60 years. Graves' disease is
characterized by thyrotoxicosis, diffuse goiter, and
extrathyroidal conditions including ophthalmopathy,
dermopathy (pretibial myxedema), thyroid acropachy,
gynecomastia, and other manifestations.
Clinical Features
The clinical manifestations of Graves' disease can be divided
into those related to hyperthyroidism and those specific to
Graves' disease.

Hyperthyroid symptoms include heat intolerance, increased

sweating and thirst, and weight loss despite adequate caloric
intake. Symptoms of increased adrenergic stimulation include
palpitations, nervousness, fatigue, emotional lability,
hyperkinesis, and tremors. The most common GI symptoms
include increased frequency of bowel movements and diarrhea.
Female patients often develop amenorrhea, decreased fertility,
and an increased incidence of miscarriages. Children experience
rapid growth with early bone maturation, whereas older patients
may present with cardiovascular complications such as atrial
fibrillation and congestive heart failure.

Approximately 50% of patients with Graves' disease also

develop clinically evident ophthalmopathy, and dermopathy
occurs in 1 to 2% of patients. It is characterized by deposition of
glycosaminoglycans leading to thickened skin in the pretibial
region and dorsum of the foot.

Diagnostic Tests

The diagnosis of hyperthyroidism is made by a suppressed TSH

with or without an elevated free T4 or T3 level.

Treatment
Graves' disease may be treated by any of three treatment
modalities—antithyroid drugs, thyroid ablation with radioactive
131
I, and thyroidectomy.
Hypothyroidism
Deficiency in circulating levels of thyroid hormone leads to
hypothyroidism and, in neonates, to cretinism, which is
characterized by neurologic impairment and mental retardation.
Hypothyroidism also may occur in Pendred's syndrome
(associated with deafness) and Turner's syndrome.

Causes of Hypothyroidism

Primary (Increased TSH Secondary (Decreased Tertiary

Levels) TSH Levels)
Hashimoto's thyroiditis Pituitary tumor Hypothalamic
RAI therapy for Graves' disease Pituitary resection or insufficiency
ablation Resistance to thyroid
Postthyroidectomy hormone

Excessive iodine intake

Subacute thyroiditis
Medications: antithyroid drugs,
lithium
Rare: iodine deficiency,
dyshormogenesis

RAI = radioactive iodine; TSH = thyroid-stimulating hormone.

Clinical Features
Failure of thyroid gland development or function in utero leads
to cretinism and characteristic facies similar to those of children
with Down syndrome and dwarfism. Failure to thrive and severe
mental retardation often are present. Immediate testing and
treatment with thyroid hormone at birth can lessen the
neurologic and intellectual deficits. Hypothyroidism developing
in childhood or adolescence results in delayed development and
may also lead to abdominal distention, umbilical hernia, and
rectal prolapse. In adults, symptoms in general are nonspecific,
including tiredness, weight gain, cold intolerance, constipation,
and menorrhagia. Patients with severe hypothyroidism or
myxedema develop characteristic facial features due to the
deposition of glycosaminoglycans in the subcutaneous tissues,
leading to facial and periorbital puffiness. The skin becomes
rough and dry and often develops a yellowish hue from reduced
conversion of carotene to vitamin A. Hair becomes dry and
brittle, and severe hair loss may occur. There is also a
characteristic loss of the outer two thirds of the eyebrows. An
enlarged tongue may impair speech, which is already slowed, in
keeping with the impairment of mental processes. Patients may
also have nonspecific abdominal pain accompanied by
distention and constipation. Libido and fertility are impaired in
both sexes. Cardiovascular changes in hypothyroidism include
bradycardia, cardiomegaly, pericardial effusion, reduced cardiac
output, and pulmonary effusions. When hypothyroidism occurs
as a result of pituitary failure, other features of hypopituitarism,
such as pale, waxy skin; loss of body hair; and atrophic
genitalia, may be present.

Laboratory Findings
Hypothyroidism is characterized by low circulating levels of T4
and T3. Raised TSH levels are found in primary thyroid failure,
whereas secondary hypothyroidism is characterized by low TSH
levels that do not increase following TRH stimulation.

Treatment
T4 is the treatment of choice and is administered in dosages
varying from 50 to 200u g per day, depending upon the patient's
size and condition.