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9 Midline Cervical Swellings
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Fig. 9.6 (a) Thyroglossal cyst with the tongue in place. (b) The cyst with the tongue protruded. Note the upward move-
ment of the cyst
Fig. 9.7 (a) Thyroglossal cyst with the tongue in place. (b) The cyst with the tongue protruded. Note the upward move-
ment of the cyst. The cyst is large enough to mimic a goiter
that is dumb-bell-shaped lesion. It uctuates easily; however, few are not, being so tense. Many
are opaque due to desquamated epithelial cells or
debris of previous infection, few are illuminant
and many are too small to illuminate [2, 95–97].
A thyroglossal cyst is not attached to the skin and
local LNs should not be enlarged, unless the cyst
is secondarily infected.
present in the cyst might lead to goiter manifestations. However, the most serious complication is
the development of carcinoma.
Thyroglossal carcinoma has no sex predominance, with a peak incidence in the fourth decade
in females and in the sixth decade in males. Such
tumors are very rare and are most commonly
papillary thyroid carcinoma (PTC), followed
(equally) by mixed papillary–follicular and squamous cell carcinoma. Adenocarcinoma was also
9.9.6 Complications
reported. Pure follicular and anaplastic carcinomas are extremely rare. Medullary thyroid cancer
Complications include infection, which is inevitable, as the lymphatic tissues in its wall communicate with the neck LNs. Ectopic thyroid tissue
t.me/Dr_Mouayyad_AlbtousH
has never been reported. Their pathogenesis is
debatable, some presume they are metastasis
from occult PTC; however, most authors suggest
198
M. Sakr
there are de-novo neoplasms. Thyroglossal cyst
carcinoma should be suspected whenever the cyst
grows rapidly, when the US demonstrates a complex anechoic pattern, or in the presence of calcications. Still, they are treated by Sis trunk
operation with low recurrence rates. This may be
followed by thyroxin suppression therapy and
regular measurements of thyroglobulin, which
becomes undetectable if successful removal of
the carcinoma was achieved and as long as TSH
is adequately suppressed by thyroxin [1, 95].
9.9.7 Dierential Diagnosis
A thyroglossal cyst should be differentiated from
swellings in the midline of the neck, namely
dermoid cyst, sebaceous cyst, lipoma, enlarged
LN, laryngocele, ranula, ectopic thyroid tissue,
hypertrophied thyroid pyramidal lobe, and choristoma (mass of normal tissues found in abnormal locations). Imaging studies could be
equivocal. Close attention to the age at presentation, location of the lesion, relation to surrounding structures, and internal architecture can direct
the clinician to the correct diagnosis.
9.9.8 Evaluation
If serum thyroid function test results are
abnormal, thyroid scanning should be performed
to determine the amount of thyroid tissue in the
neck. Some investigators advocate routine US or
nuclear scanning to avoid permanent hypothyroidism [100].
9.9.8.1 Ultrasonography (US)
Ultrasound of the neck will show a thyroglossal
cyst as a well-circumscribed anechoic to
hypoechoic structure with posterior through
transmission [99]; there may be some internal
debris (Fig.9.8).
9.9.8.2 Computed Tomography
(CT)Scan
A thyroglossal cyst appears on CT scan as a
smooth, thin-walled hypo-attenuating mass in
close relation to the hyoid bone (Fig. 9.9)
[101–103].
9.9.8.3 Magnetic Resonance Imaging
(MRI)
On MRI, a thyroglossal cyst will be high signal
on T2-weighted images, and low to intermediate
signal on T1-weighted images, depending on the
degree of proteinaceous or hemorrhagic contents
[102].
Although benign midline neck swellings such as
thyroglossal duct cysts are often diagnosed clinically, the clinical presentation of infected cysts,
thyroglossal duct carcinoma, or other pathological lesions may be indistinguishable, necessitating diagnostic imaging. In patients with low
clinical suspicion of neoplasm, imaging evaluation may begin with ultrasonography (US). In the
presence of high clinical suspicion of tumor or
atypical US features such as solid component or
abnormal vascularity, CT scan or MRI is recommended to document an orthotopic thyroid gland
and to assess the anatomical extent and complications of the cyst, as well as pretreatment planning
[98]. In selected cases, diffusion-weighted or
dynamic contrast-enhanced MRI can be performed for evaluation of vascular malformations,
abscess, or suspicious cervical lymph nodes [99].
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Fig. 9.8 Ultrasound image (longitudinal) demonstrating
a well-circumscribed hypoechoic cyst containing tiny
hyper-echoic foci (debris)
9 Midline Cervical Swellings
Fig. 9.9 Computed tomography (CT) scan showing a
midline cystic mass (arrow) immediately below the hyoid
bone
9.9.9 Treatment
9.9.9.1 Sistrunk Operation
Patients with thyroglossal cyst may have various
problems such as cosmetic concerns, difculty in
breathing, pain, swelling, neck discomfort, and
dysphasia. Therefore, surgery has been considered the treatment of choice in this condition
[104–106] and the procedure of choice is the
“Sistrunk operation,” which was introduced in
1920 by Sistrunk [107]. It is performed through a
transverse incision over the cyst and involves
complete excision of the cyst, its tract, and the
central portion of the hyoid bone. If necessary,
excision extends to the base of the tongue [93, 94,
96, 97, 100].
Prior to excision, an imaging study is performed to identify functioning thyroid gland in
the lower neck. This ensures that the cyst does
not contain the only functioning thyroid tissue
in the patient, if any. The recurrence rate could
be decreased signicantly after Sistrunk procedure (1–5%) [104, 106, 108] as compared to
simple cyst excision (40–65%) [104–106,
109–112].
Gioacchini etal. (2015) reported that in their
meta-analysis study that included 24 articles,
comprising a total of 1371 subjects, the mean
local wound infection rate was 4% (range, 3–6%),
this being the most frequent complication following treatment [113].
199
Incomplete excision of the cyst leads to recurrence, and a recurrent lesion is more liable to
recur again. Factors predictive of recurrence
included more than two infections prior to surgery, age below 2 years, and inadequate initial
operation. Failure to remove the central part of
the hyoid bone results in recurrence in three out
of four patients [93, 94, 96, 97]. In the metaanalysis by Gioacchini et al., the mean overall
recurrence rate was 11% (range 9–14%) [113].
9.9.9.2 Percutaneous Ethanol Ablation
(PEA)
Although surgery is the treatment of choice of
treatment of a thyro-glossal cyst, it still has some
drawbacks such as the use of general anesthesia,
scars, and surgical morbidity [97, 104, 106, 108,
111, 112]. Thus, minimally invasive treatment
modalities have been introduced to avoid surgical
risk or morbidity. Ethanol ablation (EA) has been
reported to be effective, easy, and safe for the
treatment of cystic thyroid lesions [114–118].
Fewer studies on thyro-glossal cysts reported that
successful treatment was achieved with PEA in
60% of cases [119, 120].
9.10 Aortic Arch Aneurysm (AAA)
9.10.1 Denition
A true aneurysm is dened as a “pathological
dilation of a segment of a blood vessel involving
all three layers of the vessel wall and having at
least a 50% increase in diameter compared with
the expected normal diameter of the artery”
[121]. Aortic arch aneurysms (AAAs) include
any thoracic aneurysm that involves the brachiocephalic vessels [122, 123].
9.10.2 Epidemiology
Thoracic aortic aneurysms (TAAs) have an estimated incidence of approximately 10
cases/100,000 persons/year. AAAs account for
about 10% of the total cases of TAAs [123, 124].
Males are 2–4 times more commonly affected
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200
M. Sakr
than females, with the majority of patients in the
sixth and seventh decades of life [125].
9.10.3 Etiology
Atherosclerosis is the predominant etiology for
an isolated AAA. The causes include Marfan
syndrome, Loeys–Dietz syndrome, Ehlers–
Danlos syndrome, Turner syndrome, familial
TAA syndrome, and Behcet disease [126].
Deceleration injuries have been seen to cause
dilation of the segment just after the aortic arch
[121, 127].
Both infectious and noninfectious inammatory conditions of the aorta (aortitis) can also
result in TAA.These include syphilis, giant cell
arteritis, and Takayasu arteritis [127]. Along with
the risk factors of atherosclerosis such as smoking, hypertension, and hyper-cholesterolemia,
factors that increase aortic wall stress, including
pheochromocytoma, cocaine use, and weightlifting also increase the likelihood of development of TAA [125].
dentally on imaging ordered for other indications.
Vague chest discomfort, neck, and jaw pain may
occur with aneurysms involving the arch. A
large-sized aneurysm can impinge upon the adjacent anatomical structures and exert a local mass
effect including hoarseness, from left RLN
stretching; stridor, from tracheal or bronchial
compression; cough, dyspnea, and recurrent
pneumonitis, from lung compression; dysphagia,
from esophageal compression; and plethora and
edema, from compression of the SVC [122].
Aortic arch aneurysms can lead to lifethreatening cardiovascular and neurological complications, including aortic rupture presenting as
severe chest pain and hypotension or shock, aortic
dissection, and athero-embolism causing ischemic strokes. Some rare complications such as
aorto-esophageal or aorto-bronchial stula have
also been documented [129]. Embolization during the placement of the graft leading to stroke or
spinal ischemia from obstruction of spinal arteries, endo-leaks, and hematoma formation are also
some of the concerning perioperative complications associated with the aortic repair [130].
9.10.4 Histopathology
Histopathology of aortic aneurysm described as
cystic medial degeneration is characterized by
disintegration and loss of elastic bers with an
increase in the deposition of proteoglycans. Loss
of smooth muscle cells in tunica media is typically seen [121, 123]. Increased penetration of
vasa vasorum into the medial layer was reported.
As atherosclerosis has been found commonly in
relation to the AAA, histological data also depicts
the presence of atheroma or bro-fatty plaque in
the lesion. However, these changes have been
seen super-imposed on the degenerative medical
disease [128].
9.10.5 Clinical Presentation
Most patients with an AAA are asymptomatic or
directly present with life-threatening complications. Aneurysms are typically discovered inci-
9.10.6 Investigations
Computed tomography angiography (CTA) or
magnetic resonance angiography (MRA) is the
imaging modality of choice for accurate detection
and measurement of TAA.It is preferred to delineate aortic anatomy, size, branch artery involvement, and to rule out other differentials [122].
Echocardiography can also be used to visualize the aorta and its major branches; the suprasternal view is best for viewing the aortic arch
[131]. Coronary angiography and echocardiography are also performed as part of usual preoperative investigations done in order to determine the
need for a concomitant cardiac procedure. In
addition, as maintaining brain perfusion is critical while performing surgeries involving the aortic arch, carotid duplex scanning is also routinely
instituted in order to access for carotid stenosis
[125]. If TAA is detected, it is recommended to
image the abdominal aorta to screen for an
abdominal aortic aneurysm.
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201
9.10.7 Dierential Diagnosis
Other aortic arch conditions causing dilation of
the vessel segment, including pseudo-aneurysm,
intra-mural hematoma, and aortic dissection, can
manifest with similar symptoms to those of aortic
arch aneurysm. Pseudo-aneurysms usually
develop secondary to deceleration injury or torsional trauma from accidents and falls. The chest
pain from aortic dissection is usually very severe
and described as tearing and sharp in nature
[122].
9.10.8 Management
9.10.8.1 Medical Treatment
The primary step in the management of AAA
is to control the risk factors of atherosclerosis, to slow the rate of expansion, and to
lower the likelihood of development of the
complications, including dissection or rupture [132, 133]. Stringent control of hypertension, optimization of the lipid profile,
smoking cessation, and other atherosclerosis
risk-reduction measures should be implemented [132, 133].
Other important conservative treatment
strategies include (1) patient education regarding the signs and symptoms indicating the
development of complications, (2) serial imaging of aneurysm to evaluate for expansion, (3)
screening for aneurysms at other locations, and
(4) counseling for those suspected of having an
associated genetic disorder. It is reasonable to
re-image using CT scan or MRI at 12-month
intervals for isolated AAAs <4cm in diameter,
and at 6-month intervals for AAAs >4 cm in
diameter, in order to detect enlargement of the
aneurysm.
9.10.8.3 Candidates forOperative
Management
The 2014 European Society of Cardiology (ESC)
guidelines recommend that surgery should be
considered in patients who have an isolated AAA
with a maximal diameter of >5.5cm. Aortic arch
repair may also be considered in patients with
AAA who are already going to have surgery of an
adjacent aneurysm located in the ascending or
descending aorta [131]. In general, surgical treatment is often recommended for all symptomatic
patients, patients with aneurysm size >5.5 cm,
and any patient of TAA with a growth rate
exceeding 0.5cm/year [134].
9.10.8.4 Operative Procedures
Advancements in open surgical techniques, safer
anesthetic practices, and improvised methods of
maintaining cerebral perfusion have revamped
the most widely used conventional open repair
procedure. The newer endo-vascular and hybrid
modalities have also emerged. These newer technologies have made surgical treatment possible
even for a larger spectrum of patients, including
those at high risk and with multiple comorbidities. Each of these techniques has its own
merits and demerits, and the choice is tailored
considering peri-operative risks, co-morbidities,
and anatomy of the lesion.
9.10.9 Prognosis
The prognosis of AAA largely depends on the
size of the aneurysm and rate of expansion, which
are the most signicant predictors of the rupture.
Prognosis is usually good if timely intervention is
instituted before rupture, which can have mortality as high as 80% [130, 135, 136].
9.10.8.2 Surgical Treatment
Surgical intervention for treatment of AAAs
raises a particular concern due to high rates of
mortality, the need for creating a bloodless eld
with circulatory arrest, and challenges in maintaining perfusion to the head, neck, and upper
extremities [130].
t.me/Dr_Mouayyad_AlbtousH
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