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10 Lateral Cervical Swellings
227
increase in size if it becomes infected. Discharge
may be reported if the lesion is associated with a
sinus tract. In some instances, branchial cleft cyst
patients may present with locally compressive
symptoms. A family history of branchial cleft
cysts may be present [147].
On examination, the cyst is usually found to
lie deep to the upper third of the anterior border
of the SCM muscle, below the angle of the mandible, partially under cover of the muscle and
partially projecting into the carotid triangle. It is
usually unilateral. On examination, most branchial cysts are 5–10cm wide, rounded or oval,
with its long axis running forward and downwards (Fig.10.15).
The cyst has a smooth surface and may feel
hard if tense, or soft if lax. The overlying skin
is mobile. It is mobile from side-to-side but
may be tethered to surrounding structures. It
cannot be reduced or compressed. It is transopaque because it contains desquamated epithelial cells that make the contents thick.
Sometimes, aspiration reveals uid that is
golden-yellow, rich in fat globules and cholesterol crystals (seen under the microscope)
secreted by the sebaceous glands in the epithelial lining. Such cysts may trans- illuminate.
Rarely, branchial cleft anomalies occur in association with biliary atresia and congenital cardiac anomalies, an association that is referred
to as Goldenhar’s complex [148, 149].
10.5.2.4 Investigations
Imaging Studies
Ultrasonography (US)
Ultrasonography (US) helps to delineate the cystic nature of these lesions [88, 150]. On US imaging, second branchial cleft cysts are typically
well-circumscribed, thin-walled, and anechoic
(Fig.10.16) with evidence of compressibility and
posterior acoustic enhancement [151]. They may
contain internal echoes compatible with internal
debris.
Computed Tomography (CT) Scan
A contrast-enhanced CT scan clearly shows the
characteristics of the branchial cyst with its anatomical site and relation to the SCM muscle. It
may aid preoperative planning and identify compromise of local structures. On CT imaging,
branchial cysts are well-circumscribed, lowdensity cystic masses with a thin wall [152]
(Fig. 10.17). If they become infected, this may
become thick-walled with evidence of mural
enhancement, localized inammatory change,
and peri-lesional fat stranding.
Magnetic Resonance Imaging (MRI)
On T1-weighted imaging, branchial cysts may
turn from low- to-high signal depending on the
proteinaceous content of the cyst but are typically
Fig. 10.15 (a) Left
second branchial arch
cyst. (a) Anterior view.
(b) Lateral view
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228
Fig. 10.16 Ultrasonography of the neck showing the
branchial cyst, typically well-circumscribed, thin-walled
and (arrows) anechoic with posterior acoustic
enhancement
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Histologically, a branchial cyst is lined by
cubical or stratied squamous epithelium, covered by a brous tissue capsule containing lymphoid tissue, which may cause repeated attacks
of inammation due to its communication with
other lymph channels in the neck. In a small
number, the cyst is lined with respiratory (ciliated
columnar) epithelium. The cyst is lled with a
turbid yellow uid in the cyst is rich in “cholesterol” and is made of mucoid material simulating
tuberculous pus.
10.5.2.5 Dierential Diagnosis
A branchial cyst should be differentiated from
Schwannoma, carotid body tumor, other lateral
cysts of the neck particularly lymphatic cyst, vascular neoplasms, and malformations and pyogenic or cold abscess, rhabdomyo-sarcoma, and
lymphadenopathy (reactive, neoplastic, lymphoma, metastasis). Other lesions to consider
also include ectopic thyroid tissue, ectopic salivary tissue, and Hydatid cyst of the neck [155].
Fig. 10.17 Computed tomography (CT) scan of the neck
showing the branchial cyst as a well-circumscribed, lowdensity cystic mass with a thin wall
hyper-intense on T2-weighted imaging [153]. As
with CT imaging, mural thickening and enhancement vary with inammatory change and typically occur in the setting of infection.
Cytology/Histopathology
FNA may be helpful to distinguish branchial cleft
cysts from malignant neck masses [154]. FNA
and culture may also help guide antibiotic therapy for infected cysts.
10.5.2.6 Complications
Most branchial cysts are asymptomatic. However,
they may become tender, enlarged, or inamed,
or they may develop abscesses. Spontaneous rupture of the abscess may result in the formation of
an acquired branchial stula. Depending on the
size and the anatomical extension of the mass,
local symptoms, such as dysphagia, dysphonia,
dyspnea, and stridor, may occur. The most serious complication, yet very rare, is the development of a “branchogenic carcinoma,” which is,
histopathologically, a squamous cell carcinoma
(SCC).
10.5.2.7 Treatment
Surgical management involves complete surgical
excision [149] encompassing the external sinus
opening with dissection of the sinus tract, if present [156].
Conventional (Open) Technique
Via a transverse incision in the neck running
across one of the creases, if possible, the cyst is
completely excised, under general anesthesia, by
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10 Lateral Cervical Swellings
229
careful dissection to preserve the important
nearby structures, as the track passes through the
carotid fork and here it is supercial to the glossopharyngeal and hypoglossal nerves. The cyst
should be removed intact (Fig.10.18), otherwise,
the part left may cause the formation of a branchial stula [156].
Denitive branchial cleft cyst surgery should
not be attempted during an episode of acute
infection or if an abscess is present. In such a
case, I&D of the abscess under an antibiotic
cover should be performed. The result will be
either re-formation of the cyst later on, or the formation of an acquired stula, which should be
excised completely.
Endoscope-Assisted Technique
The endoscope-assisted dissection technique for
cervical branchial cleft anomalies enables an
excellent view of surrounding structures [157,
158]. As compared to classical surgery, the
endoscopic technique allowed a smaller incision
(2cm), reduced edema and pain, shortened hospitalization time, improved the general wellbeing of the patient after surgery, and nally,
limited the risk of complications [159, 160]. In
addition, endoscopy provides better illumination and magnication of the surgical eld pro-
Fig. 10.18 Branchial cyst delivered and completely
removed via a transverse neck incision
viding superior visualization of the mass and
surrounding vital structures such as spinal
accessory nerve, hypoglossal nerve, and the
carotid sheath [160]. Furthermore, several
authors suggested that an endoscopic “retroauricular” approach may provide good surgical
clearing of second branchial cleft cysts with
minimal scarring [161, 162].
Robotic-Assisted Surgery
Trans-oral robotic-assisted surgery was used in
the management of patient with branchial cleft
anomalies and allowed a minimally invasive
approach with excellent visualization of the pharyngeal component. Robot-assisted excision of
branchial cysts in three patients via a postauricular facelift approach has been reported by
Song etal. (2015). All operations were performed
successfully without complications. Postoperative
cosmesis was excellent and all patients were satised with their scars, which were concealed by
hair and auricle [163].
10.5.2.8 Branchial Fistula
Congenital Type
A congenital branchial stula presents earlier in
life than the cyst (at 3–6months). It is usually
diagnosed with drainage of secretions or purulent
material from an opening at the anterior border of
the SCM muscle within the lower third of the
neck (Fig.10.19). It may be bilateral in approximately 30% of the patients. A track, lined by
squamous epithelium, extends from the opening
upwards between the ICA and ECA to the supratonsillar fossa in the oro-pharynx.
It is treated by “step-ladder operation” via 2–3
small transverse incisions in a “step-ladder” fashion (more cosmetic than a long oblique incision).
A ureteric catheter or ne lacrimal duct probe
may be inserted inside the track to facilitate dissection. Injection of a small amount of Methylene
blue dye into the tract may also be useful. Care
should be taken to avoid injury of the carotid
fork, glossopharyngeal, and hypoglossal nerves.
Robotic-assisted surgery for the treatment of
branchial stula has been reported by Rassekh
etal. in 2016. The robotic telescope can provide
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230
Fig. 10.19 Bilateral congenital second branchial stulae.
Dotted lines denote the location of the stula openings
and the anterior borders of the sternocleidomastoid
muscles
excellent visualization and allow removal of the
entire stula tract without a tonsillectomy thus
limiting the potential morbidity to the patient
[164].
Acquired Type
The acquired type of branchial stula is less common than the congenital type, and the opening is
higher in the neck. It results from rupture or incision of an infected branchial cyst or incomplete
removal of a branchial cyst. It is treated by complete excision along its whole length to avoid
recurrence.
10.5.3 Laryngocele
10.5.3.1 Denition
A laryngocele is an abnormal cystic dilation of
the laryngeal saccule that extends upward within
the false vocal fold, is lled with air, and communicates with the laryngeal lumen [165, 166].
The term laryngocele should be used only when
the lesion is symptomatic, palpable, or visible
during laryngoscopy or when it extends above
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the upper border of thyroid cartilage [165]. It is
usually unilateral but may be bilateral [167].
10.5.3.2 Classication
Laryngoceles are classied clinically according
to their relationship with the thyrohyoid membrane into three types namely, (1) internal laryngocele, which is conned inside the larynx within
the thyrohyoid membrane, (2) external laryngocele, which dissects superiorly outside the larynx
through the thyrohyoid membrane into the subcutaneous tissues of the neck, and (3) combined
or mixed laryngocele, which contains both parts
[168, 169].
10.5.3.3 Etiology/Pathophysiology
There are currently three main theories regarding
the etiology of laryngoceles: congenital factors,
increased laryngeal pressure, and mechanical
obstruction [170, 171].
Developmentally, the saccule develops as an
outpouching of the laryngeal cavity during the
second month of intra-uterine life. The saccule is
lined by pseudo-stratied ciliated columnar epithelium. It also contains numerous mucous
glands that keep the vocal cords moist and lubricated; hence the saccule is known as the “oil can”
of the larynx [172].
Acquired causes may be due to raised intraluminal laryngeal pressure for prolonged period as
in glass blowers and wind instrument players due
to continual forced expiration producing
increased pressures in the larynx. The use of a
laryngeal mask during general anesthesia can
have the same effect [173–175]. Gradual weakening of the laryngeal tissues due to aging also
plays a role in the pathophysiology of development of laryngocele. In addition, laryngeal
tumors causing mechanical obstruction increase
the intra-laryngeal pressure, which may lead to
the formation of laryngocele [170, 173, 174,
176]. Other causes may be attributed to amyloi-
dosis, chondroma, and scleroderma [177].
10.5.3.4 Clinical Presentation
Symptoms of laryngocele depend on its type,
size, location, and age of the patient. The two
common presenting symptoms are hoarseness of
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10 Lateral Cervical Swellings
Fig. 10.20 A large “external” laryngocele on the left side
of the anterior triangle of the neck in a 64-year-old
gentleman
voice and an asymptomatic cervical mass (external or combined laryngocele); however, it may be
associated with cough, dyspnea, and foreign
body sensation in the throat [178]. Large internal
laryngoceles/combined laryngoceles may cause
stridor due to airway obstruction [179].
Classically, physical examination of external
or combined laryngocele reveals a tense, resonant, translucent swelling (Fig. 10.20), which
empties on compression. It is characterized by
increase in size on Valsalva maneuver, coughing,
or straining. It is located in the anterior triangle of
the lateral side of the neck but may extend in
front of the trachea. Laryngoceles are usually
nontender and soft. If the neck mass is tender and
tense, then infected laryngocele/pyo-laryngocele
is a distinct possibility.
10.5.3.5 Investigations/Diagnosis
The diagnosis of laryngocele is essentially clinical, but imaging is required to provide the denitive diagnosis.
Plain X-Ray
Plain radiograph of the soft tissue of the neck
shows an air-lled sac protruding from the soft
231
tissues of neck. When X-ray is repeated on
Valsalva’s maneuver, the size of the mass shows
an increase in size.
Computed Tomography (CT) Scan
The CT scan is the most accurate imaging modality. It clearly denes external laryngocele and the
spatial relationship with the mass and the larynx.
It also helps in differentiating laryngocele from
other lateral cervical cystic lesions and can detect
any concomitant pathology, if present it helps in
ruling out coexistent laryngeal malignancy [180].
Magnetic Resonance Imaging (MRI)
MRI gives detailed information about the boundaries of the laryngocele and its relation to the thyrohyoid membrane, distinguishing the internal
from the external or the mixed component. In
case of laryngocele and laryngo-pyocele, MRI is
the imaging technique of choice and may distinguish obstructed mucous and inammation from
neoplastic disease [181].
Ultrasonography (US)
Internal laryngoceles have been described on US
to be echo-free, well-dened structures inside the
thyroid cartilage. Combined laryngocele has an
additional cystic mass outside the laryngeal skeleton, at the thyrohyoid membrane [182].
Laryngoscopy
Indirect laryngoscopy is diagnostic. Combined
laryngocele appears as submucosal mass in the
region of the false vocal cord. If a beroptic
laryngoscope is used, these masses can be seen to
enlarge during a Valsalva maneuver. In pure
external laryngoceles, endo-laryngeal examination will be normal.
10.5.3.6 Treatment
The treatment of choice of laryngocele is surgical
excision. It is performed via external (open)
approach, mainly for external and combined
laryngocele, and endo-laryngeal approach mainly
for internal laryngocele. Initially, excision of all
the three types of laryngocele was done using an
external approach [183]. Complications of this
procedure include (1) airway compromise due to
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mucosal edema, (2) laryngo-cutaneous stula,
(3) SC emphysema, and (4) injury to the superior/internal laryngeal nerve. Microlaryngoscopic surgery and the CO2 laser have
gained popularity during the last two decades as
the endo-laryngeal technique for internal and
mixed laryngoceles [174, 184]. In 2013, Ciabatti
et al. [185] reported the rst endo-laryngeal
resection of a combined laryngocele using
“robotic surgery.”
infection, arteritis, Behcet’s disease, bromuscular dysplasia, and medial degeneration.
The commonest causes of false or pseudo ECAAs
are iatrogenic, post-carotid end-arterectomy,
postradiation, post-trauma, and post-dissection.
The two most common ECAAs are atherosclerotic aneurysms (35–66%) and pseudo- aneurysms
(12.5–82%) [191–194]. Bilateral ECAAs have
been noted in up to 13% of patients [195].
10.5.4.4 Clinical Presentation
Men are more affected with ECAA than women
10.5.4 Extra-cranial Carotid Artery
Aneurysm (ECCA)
10.5.4.1 Denition
Aneurysm means “permanent localized dilatation of blood vessel more than 1.5 times of its
original diameter.” It is derived from the Greek
word “aneuryn,” which means “dilatation.”. It
may affect arteries (more common) or veins. If
generalized, it is known as “ectasia.” Extracranial carotid artery aneurysm (ECAA) refers to
all aneurysms located in the internal carotid
artery (ICA) or in the common carotid artery
(CCA) [186].
10.5.4.2 Classication
Five different types of ECCA were dened based
on anatomy of an aneurysm [2]: Type I: isolated
and short aneurysms of the ICA above the carotid
bulb; Type II: long aneurysms of the ICA, ranging from the carotid bulb up to the line of Blaisdell
(the line between the mastoid process and angle
of the mandible); Type III: aneurysms of the
proximal ICA and the carotid bifurcation; Type
IV: aneurysms involving the CCA and ICA as in
Type III, but extending far more distally and
proximally; and Type V: isolated aneurysms of
the CCA.
10.5.4.3 Etiology/Pathogenesis
CCA is rare; it accounts for only 0.4–4% of all
peripheral artery aneurysms [187–189]. Similar
to aneurysms elsewhere in the body, ECAAs can
either be “true” or “false.” True aneurysms constitute <10% of ECCAs [190]. They are mainly
caused by atherosclerosis (commonest cause),
with a male to female ratio of 2:1 [196].
Depending on the etiology of the aneurysm, age
of the patients ranges from 19 to 95years; however, most are in the sixth or seventh decade of
life [196].
location, and size of the aneurysm. Patients present with a pulsatile mass in the lateral side of the
neck in 12–93% of cases. It may be painful due to
tension of the dilated carotid arteries and reex
muscle spasm in the surrounding muscles.
Inammation in the carotid sheath can be another
cause of pain. Between 12 and 51% of patients
present with a transient ischemic attack (TIA)/
stroke due to embolism or aneurysm thrombosis
[197–199]. Other less frequent presenting symptoms include cranial nerve decit or dysfunction
(direct compression), infection, dysphagia, tinnitus, bruit, hemorrhage, hoarseness of voice (RLN
compression, direct laryngeal compression), tracheal obstruction (stridor), Horner’s syndrome
(sympathetic nerve compression), and dizziness.
Aneurysm rupture is rare [191–193].
anterior triangle of the lateral side of the neck,
with expansile pulsation and a bruit or thrill. The
mass is mobile across, not along, the axis of the
related artery. Proximal and distal compression
on the affected artery cause reduction and
increase in the size of the aneurysm,
respectively.
10.5.4.5 Investigations/Diagnosis
The purpose of imaging is to (1) conrm diagnosis, (2) classify the nature of the ECAA (true/
false) and identify its likely etiology, and (3) to
Clinical presentation depends on etiology,
Physical examination reveals a mass in the
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10 Lateral Cervical Swellings
233
assess its extent and anatomy in order to plan
management [200]. Most aneurysms are diagnosed using duplex US; however, corroborative
CT angiography (CTA) or MR angiography
(MRA) is essential as it can provide valuable
information on extent and whether an intervention might be appropriate. Limitations of CTA
include a relative lack of sensitivity for lesions
involving the carotid artery at the skull base or
within the contrast-lled cavernous sinuses.
Particularly in these cases, MRA might be preferable [201].
Adequacy of cerebral circulation of the patient
can be investigated by simple external digital
compression of the carotid artery with neurological monitoring (Matas test) or can be combined
with MRA for enhancing accuracy [202].
10.5.4.6 Management
There are multiple treatment options that include
medical therapy, open surgical therapy, and endovascular therapy [203–205]. Treatment of ECAA
should be tailored to the individual patient. The
goal of therapy is to prevent local mass effect,
rupture, and neurological decits from either
embolization or thrombosis. Naturally, in the
case of rupture, there is an acute indication for
intervention. However, rupture is very unusual in
ECAAs, and most surgeons may only intervene
in patients with thromboembolic symptoms or
proven progressive ECAA growth [186].
Medical Therapy
Medical treatment is appropriate in selected
patients (e.g., small asymptomatic aneurysms,
truly inoperable cases, and patients with severe
life-limiting co-morbidities). Medical therapy of
ECAAs has mostly been derived from the Mayo
Clinic experience. Fankhauser etal. reported that
75 aneurysms out of 141 (53.2%) were treated
non-operatively over a 15-year period in Mayo
Clinic [194]. Treatment included antiplatelet
therapy, anti-coagulation, or serial imaging per
the treating clinician. Most of the patients were
asymptomatic and were in patients with prior
imaging showing aneurysm stability. None of the
patients died or suffered major morbidity related
to the aneurysm. One had signicant enlargement
but nonsurgical treatment was elected due to the
patients’ age [194].
Surgical (Invasive) Treatment
Invasive treatment for ECAAs pertains to only
0.6–3.8% of all extra-cranial carotid interventions [206–215]. Traditional (open) surgical
treatment, which is the current treatment of
choice of symptomatic or growing ECAAs, consists of open resection of the entire aneurysm
with or without arterial replacement with an
inter-position graft [216, 217]. However, this
approach has been associated with the risk of
stroke and cranial nerve damage [218].
Nowadays, small case series advocating an endovascular approach with a stent to treat ECAA
have reported favorable procedural results but
with a limited number of cases and no mid- or
long-term follow-up [218, 219].
Open Surgical Approach
Open surgical approaches vary depending on
anatomy (location and accessibility) and
underlying pathology. These can include ligation,
resection with primary repair, resection with
interposition graft, and resection with patch
repair of the artery [187, 191–193]. Ligation of
the ICA is primarily reserved for emergency situations, such as arterial rupture. This is especially
true when infection is considered the primary etiology. Most typically, these patients are placed
on anti-coagulation (for 2weeks to 3months) to
prevent embolization as the ICA progressively
lls with thrombus [220, 221].
Open surgical results nearly always achieve a
technical success but do vary in surgical risk.
Early risk of mortality ranges between 0 and 7%,
peri-operative stroke 0.7–11%, cranial nerve
injury 0–66%, hematoma 0–5%, acute renal failure 0–1.5%, thrombosis 0–6%, myocardial
infarction 0–1.7%, and infection 0–1.7%. Cranial
nerve injuries include facial, vagus, spinal accessory, hypoglossal, and glossopharyngeal nerves
[187, 191–193].
Endo-Vascular Approach
Li etal. recently performed a systematic review
of the endo-vascular management of ECAAs of
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234
M. Sakr
224 patients with nearly half of the aneurysms
being attributed to trauma. The average diameter
of the aneurysms was 26.3mm, and the ICA was
the most common site. At the time of treatment,
43% of patients had neurological dysfunction.
Covered stents were used in 68% of the time,
being used in 83% of true aneurysms and 67% of
pseudo-aneurysms. Procedural success was
noted in 100% of true aneurysms and 92.4% of
pseudo- aneurysms. Average duration follow-up
was 15.4 months. Overall occlusion rate was
6.3%. Stroke rate was 2.5% with covered stents
and none seen with the bare metal stents (0/37)
[218].
10.6 Solid Swellings
ofthePosterior Triangle
10.6.1 Cervical RIB
10.6.1.1 Denitions
A “cervical rib” in humans is an extra (supernumerary) rib, which arises from the seventh cervical vertebra (C7). However, according to Tubbs
etal. (2012), a cervical rib could also originate
from the 6th (C6) or the 5th (C5) cervical vertebrae [222]. Cervical rib is also known as “Eve’s
rib” [223]. Its presence is a congenital anomaly,
located above the normal rst rib.
The term “thoracic outlet syndrome” (TOS)
(also known as scalene syndrome=superior thoracic aperture syndrome) refers to a heterogeneous group of disorders that compress one or
more of the neurovascular elements within the
thoracic outlet (superior aperture of the thoracic
cavity) on their way to the axilla. Cervical ribs
are known to cause TOS or brachial plexopathy
in up to 10% of the affected individuals [224]
10.6.1.2 Varieties ofCervical Rib
Varieties of cervical rib reported in the literature
can be summarized as follows:
– A complete rib: It may contain a false joint
(pseudoarthrosis) in its length and articulates
anteriorly with the manubrium sterni or rst
rib [225].
– The free end of the rib expands into a large
bony mass [226].
– A rib ending into a tapering point connected
by a brous band to the scalene tubercle of the
rst rib [227].
– A brous band only: It does not appear on
plain X-ray [228].
10.6.1.3 Pathology
The subclavian artery and the trunks of the brachial plexus pass in front of the rst rib and
behind the clavicle between the scalenus anterior
and medius. The presence of a cervical rib can
cause a form of TOS due to compression of the
lower trunk of the brachial plexus or subclavian
artery [229]. These structures become encroached
upon by the cervical rib as it forms the new oor
instead of the rst rib and scalene muscles. The
cervical rib may be unilateral or bilateral, complete or incomplete, and bony, cartilagenous or
brous. The main pathology is post-stenotic dilatation of blood vessels, and angulation and brosis of nerves.
Histopathological studies, on human cadavers, had demonstrated the structural changes in
the lower trunk of the brachial plexus due to cervical rib compression. These changes included
epi- and peri-neural brosis leading to thickening
with intersecting brous bands, vascular hyalinization, focal mucinous degeneration of nerve
fasciculi, and intra-neural collagenous nodules.
None of the control cadavers showed such histological ndings [230].
10.6.1.4 Etiology ofCervical Rib
Syndrome
According to Sandring (2005) [231], excessive
growth of the anterior tubercle of C7 is responsible for the development of cervical ribs. It has
been reported that all fetuses have cervical ribs,
which disappear before childbirth [232]. Thus, a
cervical rib represents a persistent ossication of
the C7 lateral costal element [229]. During early
development, this ossied costal element typically becomes re-absorbed. Failure of this process results in a variably elongated transverse
process or complete rib that can be anteriorly
fused with the rst thoracic rib below [233].
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10 Lateral Cervical Swellings
235
Clinically, compression of brachial plexus or vascular (subclavian) elements results in manifestations of TOS. Anomalies in musculoskeletal
structures that may be responsible for TOS other
than cervical rib include prolonged transverse
process of the C7 vertebra, anomalous rst rib,
and clavicle fractures [224].
10.6.1.5 Clinical Picture ofCervical Rib
Syndrome
Although a cervical rib can contribute to thoracic
outlet syndrome (TOS) [229, 234] and cause serious neurological and vascular symptoms in the
upper arm [235, 236], it usually (90% of cases)
does not cause symptoms, and clinical examination of the neck usually reveals no abnormalities
[229]. The abnormal rib is generally discovered
incidentally, most often during X-rays and CT
scans [228, 237, 238].
10.6.1.6 Symptoms
Only 5–10% of patients have symptoms, which
may include a swelling or fullness at the root of
the neck (clinical diagnosis is difcult), neuro-
logical symptoms (common) in the form of pain
in the C8,T1 dermatomes, wasting and weakness
of the small muscles in the hand due to compression of the brachial plexus [239], as well as vas-
cular symptoms (uncommon) such as Raynaud’s
phenomenon, trophic changes, even rest pain and
gangrene, particularly of the tip of the index nger [240]. It may cause post-stenotic dilatation
(subclavian artery aneurysm) leading to the formation of a mural thrombus, which may result in
showers of emboli to the extremities [241].
breathing test” (Adson’s sign) on examination,
where the radial pulse in the arm is lost during
abduction and external rotation of the shoulder
toward the affected side and taking a deep breath
and holding it [229]. However, a positive Adson’s
sign is nonspecic for the presence of a cervical
rib as many individuals without a cervical rib will
have a positive test [239]. Compression of the
sympathetic chain may cause Horner’s syndrome
[224].
10.6.1.7 Investigations
Plain X-Ray
Most commonly, cervical ribs are detected in
X-ray lms of the chest or the cervical part of the
vertebral column. Plain X-ray of the neck will
show the abnormal cervical rib(s) whether unilateral or bilateral (Fig.10.21). On imaging, the cervical rib can be distinguished because its
transverse processes are directed infero-laterally,
whereas those of the adjacent thoracic spine are
directed antero-laterally [241].
Computed Tomography (CT) Scan
The CT scan can delineate the anatomical relationships of the cervical rib [224]; however,
according to Viertel etal. (2012), 74.5% of cervi-
Physical Examination
A bony swelling, which is hard and xed, may be
felt in the supraclavicular fossa. It may be bilateral or unilateral being more on the right side.
Physical examination may also reveal “pulsation”
at the base of the neck due to displacement of the
subclavian artery [228, 242, 243]. Compression
of the brachial plexus may be identied by weakness of the muscles around the muscles in the
hand, near the base of the thumb [224].
Compression of the subclavian artery is often
diagnosed by nding a positive “Adson’s deep
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Fig. 10.21 Plain XR showing of the neck bilateral cervical ribs (arrows)
236
Fig. 10.22 Helical computed tomography (CT) scan
showing a three-dimensional view of a left cervical rib
cal ribs are overlooked in cervical spine CT
examinations [244]. On the other hand, helical
CT (Fig. 10.22) provides a three-dimensional
view of the thoracic outlet and is thus valuable
for the detection of anatomical variations [245]
and it could be considered the “gold standard” for
cervical rib detection.
Magnetic Resonance Imaging (MRI)
An alternative to CT scan is MRI, which, due to
its excellent spatial resolution, allows the detection of the brous band, which may connect the
distal end of cervical rib with the rst thoracic rib
[228].
Ultrasonography (US)
Demonstration of the fetal ribs by twodimensional (2D) US is not a simple study.
However, increasingly in the last decade, threedimensional (3D) US has been used as part of the
fetal organ examination [246, 247]. Hershkovitz,
in 2008, recommended using 3D US with maximal mode rendering for detection of the cervical
ribs because it is not time-consuming and is very
simple [247]. In children, musculo-skeletal
sonography may be a reliable method for diagnosis of cervical ribs without the need for ionizing
radiation [248].
M. Sakr
10.6.1.8 Dierential Diagnosis
A cervical rib should be differentiated from other
causes of a “solid swelling” in the lateral side of
the neck, as well as from other causes of pain and
parasthesia of the shoulder, arms, and hands. The
latter includes the following
– Cervical causes: cervical disc protrusion, cer-
vical arthritis, cervical cord tumors.
– Shoulder–Hand Syndrome: The primary
lesion is sub-deltoid bursitis or coronary
occlusion. It causes atrophy and then sympathetic overactivity (cold, sweaty, painful
hands), and later on joints become brosed
and stiff.
– Vascular causes: Angina, Raynaud’s disease,
thrombangitis obliterans, migratory thrombophlebitis, diffuse vasculitis.
– Nervous causes: Herpes Zoster, peripheral
neuritis, syringomyelia.
10.6.1.9 Treatment
The presence of cervical ribs is usually “asymptomatic” and does not require their removal.
Patients with “mild symptoms” require physiotherapy (muscle strengthening and postural exercises), but some patients may also need to change
their profession or life-style [230]. Patients with
“severe symptoms” will need scalenotomy (division of the muscle) or sclenectomy (excision of
the muscle to avoid recurrence) in addition to
removal of the cause (cervical rib or cartilage).
Different surgical approaches are available for
the treatment of TOS (supra-clavicular or transaxillary) [249].
10.6.2 Lipoma/Liposarcoma
10.6.2.1 Epidemiology
A lipoma is a benign tumor made of mature adipocytes. It is most common benign soft tissue tumor as
it accounts for about 80% of all benign soft tissue
tumors [250]. It affects ~2% of the general population occurring in men more than women [251].
Lipomas can occur at any age but are most common
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