140

Benign neck disease: infections and swellings

PETER CLARKE

Introduction 1777 Key points 1787

Cystic lesions 1777 Best clinical practice 1787
Lateral cervical cysts and branchial abnormalities 1779 Deficiencies in current knowledge and areas for future
Benign tumours 1782 research 1787
Infections 1783 References 1787
Deep neck space abscesses 1785 Further reading 1788
Conclusion 1786

SEARCH STRATEGY AND EVIDENCE-BASE

A PubMed search revealed only reviews and case reports. Searches for the following were undertaken: branchial cysts,
branchiogenic carcinoma, lateral cervical cysts, thyroglossal cysts, dermoid cysts, bronchial cysts, lymphangiomata, cystic
hygroma and thymic cysts. A search combining neck and cervical and the following was also undertaken: TB, HIV,
AIDS, infectious mononucleosis, cat scratch disease, toxoplasmosis, brucellosis, Kikuchis disease, actinomycosis,
necrotizing fasciitis, abscess and infection. There are no Cochrane Reviews, and no level II or I evidence for the
management of these conditions. Management recommendations are supported only by retrospective reviews and expert
opinion. [Grade D]

INTRODUCTION may also be related to cystic structures, the most common

Benign neck swellings may be nodal or cystic, lateral or
central, neoplastic, inflammatory or congenital. Thyroid
and salivary gland swelling will be considered elsewhere.
Maisel1 suggests that 80 percent of nonthyroid neck
masses are neoplastic. Of these, 80 percent are metastatic
and three-quarters are from primaries above the clavicle.
Although the statement above might be prefaced by a
suggestion that this applies to masses over 2 cm in
diameter in those over 35 years of age, it is a useful rule of
thumb especially for those not regularly seeing patients
with neck masses. This compares with 90 percent of neck
masses in children representing benign conditions of
which 55 percent were congenital.2 [**/*]
Neck masses are often enlarged lymph nodes, which
may be reactive to either local or generalized infection.
Nodal swellings may be suppurative or nonsuppurative.
Pus may also spread from a local focus of suppuration
related to, for example, the tonsil or a tooth. Swellings
of which are branchial cysts and thyroglossal cysts. These
may present as infections or become infected. Benign
tumours, particularly neurogenic tumours may present as
neck swellings.
Initial investigation should include history, physical
examination and fine needle aspiration for cytology or
microbiology and culture. This, with knowledge of the
anatomy and physiology should allow an accurate
diagnosis in most cases. Ultrasound scanning, computed
tomography (CT) and magnetic resonance (MR) images
will often be helpful in difficult cases.
CYSTIC LESIONS
Thyroglossal cysts
Most authors suggest these are the most common cystic
lesions in the neck. Up to 50 percent will present in
1778 ] PART 14 THE NECK
adulthood, with over 30 percent presenting before ten
years of age. There is an equal male-to-female incidence
(Figure 140.1).
Embryology
During the development of the branchial apparatus in a
four- to five-week-old embryo, the thyroid enlage arises in
an invagination of endoderm in the floor of the pharynx
and develops caudally, with the descent of the great
vessels. It enlarges and becomes bilobed, coming to rest at
the root of the neck as the thyroid gland by the end of the
seventh week. The tract that is left usually atrophies and
disappears, although the caudal end often remains as a
pyramidal lobe. Failure of the tract to involute may leave
epithelial remnants or an open area of duct, which may
expand into a cyst because of an accumulation of
secretions. The hyoid bone, developing later, may entrap
a portion of the thyroid duct or draw it caudally, leaving
the duct dorsal to the bone.3
Clinical features
Most present as midline masses 23 cm in diameter just
inferior to the hyoid bone (Figure 140.1). A quarter
Figure 140.1 Large thyroglossal cyst presenting in late
adulthood with symptoms of obstructive sleep apnoea. Elevation
of cyst on tongue protrusion demonstrated.
present suprahyoid and just over 10 percent related to the
thyroid. Because of the hyoid attachments, these masses
move both on swallowing and protrusion of the tongue.
Cysts may become infected and incision and drainage
may result in the development of a sinus from the cyst to
the skin.
Pathology
Thyroglossal cysts are usually lined by columnar epithe-
lium with small glands frequently containing thyroid
colloid. Decalcification of the hyoid bone will often
confirm that the tract passes through it.4
Management
The diagnosis is clinical and may be confirmed with a fine
needle aspirate if the mass is suprahyoid to help
differentiate it from a dermoid cyst or submental lymph
node. Further investigations are not necessary for
diagnosis, but should be undertaken to ensure that a
normal thyroid gland is present. Ultrasound scanning is
accurate, cost-effective and would seem to be the
investigation of choice and is the most common
investigation undertaken in the UK. Radionuclide scan-
ning may be reserved for patients with normal thyroid
glands that cannot be localized.
Thyroglossal cysts should be treated surgically. An
infected cyst may be aspirated for microbiological culture
and to avoid incision into the cyst. A horizontal skin
crease incision is made over the cyst and skin flaps raised
superiorly and inferiorly. Care should be taken through-
out the dissection to avoid rupturing the cyst. For
infrahyoid cysts, the sternohyoid and thyrohyoid muscles
must be reflected laterally and stripped off the hyoid to
reveal the cyst. The cyst is dissected from surrounding
tissues up to the body of the hyoid. The hyoid is then
divided with heavy scissors just medial to the lesser horns
on both sides. A cuff of suprahyoid and intrinsic tongue
muscles is then taken with the body of the hyoid. A
specific tract is often not found, but if a convincing tract
is seen, this should be followed into the musculature of
the tongue base and removed. There is no need to resect
epithelium of the tongue base. Sistrunk described this
operation in 1920.5 Recurrence rates are significantly less
after Sistrunks operation than after simple cyst excision.
If a sinus is present, an ellipse of skin around the sinus is
removed in continuity. Surgery for recurrent cysts should
include excision of the hyoid and a cuff of tongue base
muscle if the hyoid has not previously been excised, or
excision of the cyst with a surrounding cuff of muscle if
Sistrunks operation has previously been performed.
There have been a large number of case reports and
small series of thyroid carcinoma presenting in a
thyroglossal cyst. Up to 90 percent of these are papillary.

A recent review from Slone Kettering6 suggests treatment
should be with Sistrunks operation and thyroid suppres-
sion with thyroxine. Thyroidectomy and postoperative
radioactive iodine, although revealing malignancy in a
quarter of patients, did not confer a survival advantage
and they conclude that this should be reserved for high-
risk groups. [Grade D]
LATERAL CERVICAL CYSTS AND BRANCHIAL
ABNORMALITIES
Lateral cervical or branchial cysts occur equally in the
sexes and present most commonly in young adults,
although they can occasionally present at any age. There is
debate about the precise aetiology of the cysts and fistulae
and there may be a number of different aetiologies
resulting in a similar clinical presentation.
Branchial fistulae
Branchial fistulae usually present in childhood as a
weeping defect along the anterior border of sternoclei-
domastoid, or occasionally as an acute infection.
EMBRYOLOGY
During the fourth week of intrauterine life, six branchial
(or pharyngeal) arches develop as neural crest cells
migrate into the head and neck region. During the fifth
week, the second branchial arch grows over the third and
fourth branchial clefts which form a cervical sinus (Figure
140.2). Failure of the cervical sinus to close may therefore
potentially communicate with the second branchial
pouch (and therefore the tonsil fossa), the third branchial
pouch in the area of the larynx or the fourth branchial
pouch opening in the piriform fossa.
PRESENTATION
Clinically, second branchial cleft fistulae are the most
common. They have a cutaneous opening along the
anterior border of the sternocleidomastoid, usually at the
junction of the middle and lower thirds, and track up
through the neck to run between the internal and external
carotid arteries and end in the tonsillar fossa (Figure
140.3).
Third and fourth branchial fistulae are more rare,
opening low in the neck and ending in the piriform fossa.
The third arch fistula should pass over the hypoglossal
nerve and the fourth arch fistula should run down into
the chest, running below the arch of the aorta on the left
or subclavian archway on the right. It is doubtful whether
these exist as clinical entities, however. A recent paper7
suggests that fourth branchial cleft fistulae arise from the
Chapter 140 Benign neck disease: infections and swellings ] 1779
Pharyngeal Pharyngeal Pharyngeal
clefts arches pouches
I
1 1. Eustachian tube
and middle ear
II cleft
2. Palatine tonsil
III
2
3. Inferior
3
parathyroid thymus
4 IV
4. Superior
parathyroid
ultimobranchial
body
Figure 140.2 Development of the pharyngeal (branchial)
apparatus. The second arch grows over the third and fourth
pharyngeal arches so burying the second, third and fourth
pharyngeal clefts. The remnants of these clefts form the cervical
sinus of His.
ultimobranchial body (Figure 140.2) and hence extend
from the pharynx to the thyroid. The fact that fourth
branchial fistulae only seem to occur on the left would
suggest that the timing of the descent of the aorta allows a
fistula to remain on the left whilst descent of the
subclavian artery may obliterate potential fistulae on the
right. Although fistulae undoubtedly exist running from
the piriform fossa to the skin and are presumably related
to the branchial apparatus, the exact mechanism of their
formation cannot be certain. Often described in the
literature as fourth branchial pouch fistulae, they present
on the left, often involve the thyroid gland and may
present as a suppurative thyroiditis (Figure 140.4). They
usually present in children though may be asymptomatic
until adult life. [**/*]
MANAGEMENT
Fistulae may be investigated with a sinogram. Pharyngo-
scopy with careful examination of the tonsil fossa and
piriform fossa mucosa, depending on presentation,
should be undertaken prior to excision.
Surgical excision should be started with an elliptical
excision of the fistula opening. Dissection of the track can
be helped by careful injection of dye into the opening
before commencement. A second branchial fistula will
need one or more further skin incisions to allow safe
dissection to the carotid. Excision of the tonsil fossa
opening or dissection in the fossa will allow excision in
1780 ] PART 14 THE NECK
Figure 140.3 Second branchial cleft fistula. (a) Dissection followed up through neck and second incision to allow safe dissection
between internal and external carotid. (b) Opening of fistula in tonsil fossa demonstrated by appearance of dye.
Figure 140.4 Fourth branchial arch fistula. Young adult
presenting with anterior neck abscess and fistula
communicating with left pyriform fossa. Lifetime history of
intermittent weeping from small punctum in left neck.
continuity (Figure 140.3). Removal of a fourth branchial
arch fistula should include excision of the thyroid tissue
as necessary to allow removal of the whole tract. Ligation
of the tract at the pharyngeal mucosa can then be
undertaken. [Grade D]
Lateral cervical (or branchial) cysts
The cervical sinus normally closes by six weeks leaving a
cervical vesicle and it is this trapped ectoderm which, it is
postulated, forms the lateral cervical or branchial cysts
commonly seen in young adults.
There are a number of theories of origin for these
lateral cervical cysts, which have a constant clinical
presentation anterior to the upper third of sternocleido-
mastoid in young adults. They usually appear fairly
quickly often in relation to an upper respiratory tract
infection. There is a slight left-sided predominance. All
three main theories of their aetiology have some merit.8
1. Origin from a branchial pouch remnant: A tract
from lateral cervical cysts running between the
internal and external carotid would bolster this
theory and is sometimes described but often not
proven histologically and may be a surgically
produced aggregation of tissue. Certainly, in
common with several eminent surgeons, the
author has never seen a tract from a branchial
cyst. Detailed histological mapping of the
cytokeratin profile of lateral cervical cysts and
 Chapter 140 Benign neck disease: infections and swellings
mucosa from the tonsil fossa does, however,
suggest an origin from this area.9
2. Origin from the cervical sinus: This theory
proposes the branchial cleft rather than pouch
forms cysts. Overgrowth of the second arch
produces the cervical sinus of His (Figure 140.2)
which closes to leave the cervical vesicle, which,
it is postulated, results in the lateral cervical
cyst. This is an attractive theory
embryologically, although does not fully
explain why cysts usually do not appear
clinically until the third or fourth decade and it
might be expected that more cysts are found
with tracts or sinuses running to the skin.
3. Origin from lymph node degeneration:
Originally postulated in the nineteenth century,
King10 in 1949 studied a large number of cysts
and concluded that cystic transformation of
lymph nodes was the likely origin of lateral
cervical cysts. Certainly most cysts have lymphoid
tissue similar to lymph nodes in their walls and
few, if any, have tracts to the skin or pharynx. It
might be expected, however, if this theory were
true that the position in the neck may be more
variable.
PRESENTATION
In early adulthood, a cystic, often tender oval mass
46 cm in diameter anterior to the upper third of the
sternocleidomastoid develops fairly quickly. A recent
upper respiratory tract infection is often described.
Swelling may be intermittent and occasionally the cyst
is overtly infected with overlying erythema and pain at
presentation. The main differential diagnosis is from
reactive lymphadenopathy. In children, a dermoid cyst or
rhabdomyosarcoma should also be considered. In young
adults, lymphoma, tuberculosis (TB) or nerve sheath
tumours need to be excluded. In patients over 35 years of
age, a cervical metastasis from a head and neck primary
must be excluded.
PATHOLOGY
Lateral cervical cysts are usually lined with squamous
epithelium with transitional type pseudostratified epithe-
lium. Cysts presenting in the younger age group are more
likely to be lined with respiratory epithelium.
MANAGEMENT
The differential diagnosis can be simplified considerably
by fine needle aspiration. Occasionally straw-coloured
fluid is obtained, though more often, greasy yellow thick
mucoid material is aspirated, probably representing
recent inflammation. Should they present with acute
] 1781
infection, attempts should be made to aspirate the
contents and treat the patient with intravenous anti-
biotics, though incision and drainage may be necessary.
Imaging is not usually necessary to make the diagnosis.
Lateral cervical cysts should be surgically removed.
After a transverse skin crease incision over the cyst is
made, dissection is followed along the anterior border of
the sternocleidomastoid. Blunt dissection close to the cyst
is usually simple and allows removal of the cyst. If there
has been previous infection or the cyst is very adherent to
local tissues, dissection should continue as for a neck
dissection with identification of the spinal accessory
nerve, carotid sheath and hypoglossal nerve with resection
of the cyst and surrounding nodes and fibrous tissue.
Proponents of a branchial sinus origin suggest looking
for a cord or tract extending between the internal and
external carotid and possibly examination of the tonsil
fossa prior to excision of the cyst. If cysts have presented
with acute infection and been treated with antibiotics, the
cyst will occasionally not be apparent after resolution and
unless radiology either ultrasound scan or CT scan
shows an obvious cyst, these cases can be treated
expectantly. [Grade D]
Branchiogenic carcinoma
Opinion on the existence of this entity varies from the
suggestion that it is underdiagnosed to denial of its
existence.
Following initial descriptions and the coining of the
term branchiogenic carcinoma, it became clear that the
majority of these cases were actually metastatic deposits
from primary squamous cell carcinomas in the head and
neck. In 1950, Martin et al.11 therefore suggested that to
diagnose a branchiogenic carcinoma, four criteria had to
be met.
1. The cervical tumour occurs along the line
extending from the tragus to the clavicle, along
the anterior border of sternocleidomastoid.
2. The histological appearance must be consistent
with an origin from tissue known to be present in
branchial vestiges.
3. No primary source of the carcinoma should be
discovered after at least five years of follow-up.
4. There is histologic demonstration of cancer
arising in the wall of an epithelial-lined cyst.
Even apparent fulfilment of these criteria, however,
cannot exclude a metastatic squamous cell carcinoma. A
cystic metastasis may have many similar features,
histologically, to a branchial cyst and the addition of
radiotherapy to the excision of these masses will treat
small tonsil or tongue base tumours, so making even
adherence to Martins criteria meaningless.12
Most metastatic nodes with unknown primary are
solid and most cystic nodes have primary tumours that
1782 ] PART 14 THE NECK
can be demonstrated in the tongue base or tonsil. Several
series, including 136 cases of cystic squamous cell
carcinoma,13 without an obvious primary site of origin
at initial diagnosis, have shown that in the vast majority a
primary tumour can be found. Most are in Waldeyers
ring, but other sites found include lung, larynx, thyroid,
palate, sinuses and salivary gland.
There is no innate reason why the epithelium of a
lateral cervical cyst should not become malignant and
certainly there are case reports of tumours that fit
Martins criteria, but estimates suggest that the incidence
of branchiogenic carcinoma, if they exist, is in the region
of 0.3 percent of all head and neck carcinomas.14 [**/*]
Other cystic lesions
DERMOID CYSTS
These originate from ectoderm and mesoderm, and the
head and neck is the most common site to find them.
They can present at any age, but the majority present in
patients aged under six years. Sex distribution is equal
and they present along lines of embryonic fusion of the
facial processes predominately in the midline or lateral
to the submandibular gland. They occur in the sub-
cutaneous tissues and contain skin appendages such as
hair follicles, sebaceous glands and sweat glands, so
differentiating them from epidermoid cysts. Dermoid
cysts may also occur from implantation secondary to
puncture wounds. Complete surgical excision is required
and is usually straightforward.
BRONCHIAL CYSTS
These are uncommon congenital lesions that can occur in
the suprasternal notch, but are more common within the
thoracic cavity or mediastinum. They are more common in
males and one-third are associated with a discharging
sinus. The cysts are lined by ciliated pseudostratified
columnar epithelium and the sinus with stratified squa-
mous epithelium. Complete surgical excision is curative.
Cervical or plunging ranulae are considered with
salivary glands (Chapter 147, Non-neoplastic salivary
gland diseases) and laryngocoeles are considered with the
larynx (Chapter 88, Congenital disorders of the larynx,
trachea and bronchi).
BENIGN TUMOURS
Cystic hygroma and lymphangioma
These benign lymphatic lesions can be divided into three
morphological types: capillary, cavernous and cystic.
One-third of cases are found in the oral cavity and cheek
and about a quarter in the neck. They are uncommon
with only five cases per 3000 admissions to a paediatric
hospital and only account for four of 152 benign tumours
of the neck. These lesions are congenital, arising from
localized lymphatic stasis caused by congenital blockage
of regional lymphatic drainage or developing from
sequestration of the primitive embryonic endothelial
budding that arises from the lymphatic sacs.
1. Capillary lymphangioma: Capillary or simple
lymphangiomas are comprised of capillary-like
lymphatic vasculature and are usually
asymptomatic and confined to the superficial skin
or oral cavity. They are pale, small vesicle-like
lesions and no treatment is necessary.
2. Cavernous lymphangioma: Cavernous
lymphangiomas are composed of dilated
lymphatic channels and typically occur in the
tongue, cheeks and lips, causing diffuse swelling.
These account for 40 percent of lymphangiomas.
3. Cystic hygroma: Cystic hygromas are
histologically similar to cavernous lymphangiomas
with thin-walled sinuses and cysts that are lined
by flat endothelial cells and contain eosinophilic,
acellular lymph fluid. In cystic hygromas, there
are larger cystic masses which may communicate
or be isolated.
PRESENTATION
Lymphangiomas occur predominately in the neck, often
in the posterior triangle where tissue planes are looser
than in the lips, tongue and cheek. As these enlarge, they
may involve the cheek, parotid, oral cavity and media-
stinum or axilla. In the neck they are fluctuant, soft, cystic
diffuse masses with indistinct margins. Classically they
transilluminate.
The majority of lymphangiomas are present at birth and
may now be diagnosed prenatally. It is extremely rare for
these to present after the age of two years, but recurrence
after previous treatment may occur after many years. They
are usually asymptomatic, but are a distressing cosmetic
problem. Cystic hygroma and cavernous lymphangioma
either grow progressively or remain static and there have
also been many reports of involution. Bleeding or infection
may, however, cause rapid growth and symptoms of pain
or airway compromise which may be life-threatening. The
largest cystic hygromas may also cause airway problems
without infection or bleeding.14[**/*]
MANAGEMENT
Magnetic resonance imaging (MRI) delineates the extent of
the lesion well and will facilitate accurate diagnosis and
pretreatment planning. Prenatal diagnosis may be made
with ultrasound scanning and potential airway compromise
assessed with prenatal MRI. There are case reports of
 Chapter 140 Benign neck disease: infections and swellings
infants being treated ex utero to avoid airway problems at
birth. In this country, the majority are not diagnosed until
birth and treatment depends on the size and anatomical
location of the lesion. Treatments used for lymphangioma
include surgical excision and intralesional injections of such
sclerosants as bleomycin, tetracycline and alcohol. Surgery
is challenging and should be undertaken in specialist
centres. With time and expertise, results can be excellent
and surgery remains the treatment of choice. Resection is
often limited to the neck initially and recurrence rates can
be high and may occur rapidly or after many years. Surgical
excision may be helped by the injection of tissue blue into
the lymphatic spaces. A staging system has been used to
predict surgical outcome (Table 140.1).15, 16
Injection of sclerosants may lead to scarring and make
subsequent surgery more difficult, but a new intralesional
injection of OK-432 (picinabil) has shown promising
results. It is thought to produce an inflammatory reaction
leading to adhesions or to increased permeability of the
endothelial lining, increasing the rate of drainage from
the lesion. Pooled results from a group of series show
marked shrinkage in 72 out of 116 children. It may be less
effective in cavernous lymphangioma and lymphangioma
with haemangiomatous elements.17 [**/*]
Paragangliomas and nerve sheath tumours
These neoplasms of neural crest origin arise from the
paraganglionic cells in the carotid body, vagal ganglia and
jugulotympanum and, less commonly, other areas
including the larynx, nose and orbit. Most present in
the parapharyngeal space with swelling of the pharynx
and fullness in the upper neck. These are considered
in more detail in Chapter 191, Tumours of the para-
pharyngeal space.
INFECTIONS
Infections may be suppurative or nonsuppurative and
related to lymph nodes or neck spaces. Suppuration may
Table 140.1 Staging system.
Stage Disease Percentage surgical
complications
Stage 1 Unilateral infrahyoid 17
disease
Stage 2 Unilateral suprahyoid 41
disease
Stage 3 Unilateral infrahyoid and 67
suprahyoid disease
Stage 4 Bilateral suprahyoid 80
disease
Stage 5 Bilateral supra and 100
infrahyoid disease
] 1783
develop from lymph nodes, may develop in any of the
cystic masses described above or may spread from local
abscess formation around teeth, tonsil or from trauma.
Tuberculosis, infectious mononucleosis, human im-
munodeficiency virus (HIV) infection, cat scratch fever
and toxoplasmosis may all produce generalized cervical
lymphadenopathy.
Tuberculosis
Cervical lymphadenopathy is the most common head and
neck manifestation of TB and can occur in any age group,
though most series suggest the most common presenta-
tion is in young adults. Most series suggest only 1020
percent to have associated pulmonary disease or to have
had a history of contact with TB. Up to half of these
patients will have systemic symptoms. It has been
suggested that the bacillus enters via the tonsils as
tonsillectomy specimens will often show evidence of TB
infection in these patients. Nodes tend to be tender and
may have overlying erythema or occasionally a dischar-
ging sinus. Suspicion should be raised in immunocom-
promised patients, those from regions with high levels of
TB, such as the Indian subcontinent and parts of the
former Soviet Union, as well as parts of Africa.18, 19 [**/*]
MANAGEMENT
Ultrasound scanning of the nodes will often show
multiple matted nodes most commonly in the upper
deep jugular chain or supraclavicular region.
A fine needle aspiration (FNA) should be undertaken
and will often reveal mycobacteria. With an increased
number of multiresistant strains of TB, however, an
excision biopsy is often needed to establish sensitivities.20
This may become less necessary as the application of DNA
probes and polymerase chain reaction (PCR) becomes
more widespread in the future. When FNA and PCR were
used together, a specificity of 84 percent and sensitivity of
100 percent were found.21 If possible, total excision of the
node or nodes is suggested rather than incisional biopsy to
reduce the chance of developing a discharging sinus. This
may mean utilizing neck dissection techniques to under-
take a selective neck dissection often with some excision of
the sternocleidomastoid, which is commonly involved if
the nodes are very inflamed and matted. Antituberculous
chemotherapy should be started immediately after surgery
and adjusted according to sensitivities. [**/*]
Infectious mononucleosis
Although generally diagnosed and seen in the ear, nose
and throat (ENT) setting due to the acute tonsillitis,
generalized lymphadenopathy is often present. Epstein
Barr virus infection often occurs in childhood and usually
1784 ] PART 14 THE NECK
results in a mild self-limiting illness characterized by
fever, pharyngitis, tonsillitis and lymphadenopathy. How-
ever, in teenagers and young adults, it may present with
significant tonsillar enlargement causing airway compro-
mise and rarely death. Suppuration of lymph nodes can
occasionally occur and awareness of the possibility of
splenic rupture is important. The enlarged tonsils have a
characteristic grey fibrinous exudate and patients often
have a typical hot potato voice.
MANAGEMENT
Nasopharyngeal endoscopy may be a useful diagnostic tool
showing the typical fibrinous membrane in the nasophar-
yngeal cavity of over 90 percent of patients with infectious
mononucleosis and none of 24 patients with acute
tonsillitis.22 Diagnosis is made by Paul Bunnell monospot
serology, detection of a viral capsid antigen-IgM or the
presence of lymphocytosis with characteristic apoptotic
lymphocytes present in 89 percent of blood smears of
patients with acute infectious mononucleosis and only 4
percent of controls. Elevated liver function tests are usual.
More recent quantitative polymerase chain reaction may
allow prediction of fulminant infections. Treatment is
supportive with hydration, if swallowing is a significant
problem, and pain relief. Nonampicillin antibiotics are
usually prescribed to prevent secondary bacterial infection.
Upper airway compromise should be treated early with
steroids and there are case reports of antiviral treatment
with acyclovir or famcyclovir which may be of help.
Tracheostomy should be avoided by the prompt use of
steroids. Acute tonsillectomy has been advocated for upper
airway obstruction. Only a small proportion of patients go
on to have recurrent tonsillitis and so interval tonsillect-
omy is not indicated. A prospective cohort of 250 primary
care patients with infectious mononucleosis or ordinary
upper respiratory tract infections suggested postviral
fatigue syndrome at two months was most reliably
predicted by cervical lymphadenopathy and initial bed rest
and at six months was predicted by a positive monospot
test and lower physical fitness.23 [***/**]
HIV infection
Persistent generalized lymphadenopathy (PGL) is a
syndrome of diffuse lymphadenopathy involving two or
more extrainguinal sites for greater than three months. It
may be an early sign of HIV infection with up to 70
percent of patients infected developing diffuse lympha-
denopathy within the first few months after seroconver-
sion. Fine needle aspiration will usually show a reactive
picture, so excision biopsy of a node or panendoscopy is
usually not indicated. Histology, if the excision is
undertaken, will usually show a reactive follicular
hyperplasia.24 A lymphocyte depletion pattern may be
seen in patients with acquired immunodeficiency
syndrome (AIDS) or a poor prognosis. Enlarging or
tender nodes are not typical of PGL and in a series of HIV
patients with enlarging or tender nodes none had PGL
and a diagnosis of TB was made in eight, opportunistic
nocardial infection in two, lymphoma in one and
metastatic carcinoma in one.25
Cat scratch disease
This is an infection caused by Bartonella henselae, a
fastidious Gram-negative bacillus acquired from exposure
to an infected kitten or cat, either directly or via cat fleas.
Symptoms are of fever, arthralgia and lymphadenopathy.
The presence of cat scratches or a history of a cat scratch
should guide the diagnosis which is confirmed by indirect
immunofluorescence antibody assay with high antibody
titres which settle over several weeks. More recent
polymerase chain reaction RNA of Bartonella henselae
RNA has been used to confirm the diagnosis. The
organism is sensitive to many antibiotics and so it is
likely that many cases are never diagnosed. There is some
evidence that azithromycin is associated with rapid
resolution, but many infections will settle without the
need for antibiotic therapy.26
Toxoplasmosis
This is a parasitic infection with Toxoplasma gondii which
may be asymptomatic or produce cervical lymphadeno-
pathy, fever and malaise. The main reservoir for
toxoplasma in the UK is the cat, but transmission may
also occur from cysts in undercooked meat. A fine needle
aspiration will often show the microorganism on micro-
scopic examination. Diagnosis can be confirmed by
serology. A retrospective review of 731 patients who had
reactive lymph node hyperplasia revealed features
supporting a diagnosis of toxoplasmosis in 15 percent
of cases.27 Treatment is not usually indicated.
Brucellosis
The brucella group of pathogens occur in domesticated
animals and brucellosis is caught from contaminated
meat or dairy products or via direct contact through
broken skin. An undulating fever, malaise and cervical
lymphadenopathy in 20 percent of patients is typical. The
diagnosis is confirmed serologically and treatment is with
doxycycline and rifampicin for six weeks. Prevention by
animal vaccination is the ideal.
Kikuchis disease
Kikuchis disease, previously known as subacute necrotiz-
ing lymphadenitis, has been increasingly recognized in the
 Chapter 140 Benign neck disease: infections and swellings ] 1785

West, having been described more commonly in Asia. It is

a histiocytic necrotizing lymphadenitis without granular
cell infiltration, mainly affecting young women. It
presents with malaise, mild fever and painless posterior
triangle cervical lymphadenopathy. The course is benign
and resolves in weeks or months. There may be an
association with systemic lupus erythematosus (SLE)
which may develop subsequently up to three years later.
Monitoring should therefore be considered. Fine needle
aspiration will usually be nonspecific, but diagnosis has
been made with cytology. Excisional biopsy is usually
required to rule out a lymphoma or TB. Histology shows
areas of patchy necrosis and a paucity of neutrophils.

Cervical necrotizing fasciitis

This is a rare, but life-threatening, infection that causes

progressive necrosis of the subcutaneous fat and fasciae
and secondary necrosis of the overlying skin. It may
complicate deep neck space infections or result directly
from odontogenic or tonsillar infection. Streptococcus
milleri or S. viridans and mixed anaerobes are commonly
found. The clinical picture is of cellulitis with dispropor-
tionate pain. Reduced skin sensation may be a useful early
sign of affected areas. CT may show oedema and air
pockets in the deep and superficial neck spaces and
necrotic patches of skin are diagnostic. Untreated,
necrotizing fasciitis can be rapidly fatal and so early
diagnosis and aggressive treatment with intravenous
penicillin and metronidazole and surgical debridement
of all necrotic areas are the keys to success (Figure 140.5).
Skin that has not been affected should be preserved to
reduce cosmetic deformity.

Actinomycosis

Actinomycosis species are facultative anaerobes whose

normal habitat is the oral cavity but they can cause a
chronic lesion mimicking malignancy or TB, usually
following surgery or trauma to the mouth. The anterior
cervical triangle is most often affected and they present as
a slow growing mass or as an abscess or group of abscesses
with sinus tracts to the skin (Figure 140.6). These
manifestations are probably polymicrobial. The organism
is difficult to culture but the characteristic colonies,
known as sulphur granules, are often seen histologically.
Fine needle aspiration may also show sulphur granules. A
two-month course of penicillin or cephalosporin and
removal of carious teeth is usually curative. Longer
courses of antibiotics may be necessary in resistant
cases. Infection with Nocardia species may give a similar
picture, especially in immunocompromised patients.
Growth of the organism may therefore be important as
treatment should be with imipenem or sulphonamides for
Nocardia.28
Figure 140.5 Necrotizing fasciitis. (a) Gross oedema and
diagnostic collections of subcutaneous gas on CT. (b) Outcome
following debridement and secondary skin grafting.
DEEP NECK SPACE ABSCESSES
These include parapharyngeal or lateral pharyngeal,
retropharyngeal and submandibular space abscesses and
although less common than in the past, partially because
of better oral care and antibiotic treatment, may still
present as life-threatening infections. Some series suggest
there are slightly more men than women presenting with
these infections and the peak ages are in the third and
1786 ] PART 14 THE NECK
Figure 140.6 Actinomycosis. Note multiple weeping punctae.
fourth decade. There is a dental origin in 40 percent of
patients and other aetiologies are tonsillitis with or
without peritonsillar abscess, TB, intravenous drug abuse
following injection into the internal jugular vein, man-
dibular fracture and foreign body ingestion. In up to 20
percent of cases, no obvious aetiological factor is found.29
Most bacterial cultures are polymicrobial and Streptococcus
viridans is the most common organism isolated. Staphy-
lococcus aureus and Staphylococcus epidermidis are also
important. Anaerobes probably play a significant role in
these abscesses, but are not always cultured.
Clinical features
These patients present acutely unwell with pain in the
throat and neck, fever, a raised white cell count and raised
inflammatory markers. The clinical picture depends on the
space affected. Retropharyngeal abscesses are more com-
mon in children and in adults are most commonly caused
by TB of the spine. These may cause no neck swelling but
present with dysphagia, odynophagia and airway compro-
mise. The most common deep neck abscess is a lateral
pharyngeal (parapharyngeal) space infection which causes
a diffuse, tender, indurated swelling which is usually
anterior to the superior half of the sternocleidomastoid.
Submandibular space infections will have localized swelling
below the mandible and may be associated with significant
oedema of the floor of the mouth, with elevation of the
floor of mouth and tongue when it is described as Ludwigs
angina. In this situation, airway compromise is significant
and in one series, 75 percent of these patients required a
tracheostomy.30 Airway compromise is the most common
complication of deep neck space abscess, but internal
jugular vein thrombosis characterized by spiking fevers,
tenderness along the sternocleidomastoid and prostration,
or mediastinitis are potential complications which should
be anticipated. [**/*]
MANAGEMENT
Antibiotics should be commenced before culture and
sensitivities are available. Intravenous penicillin or amox-
ycillin with the addition of clavulanic acid or metronida-
zole to treat anaerobic bacteria should be the first choice.
The history and examination should highlight the
possibility of an abscess but investigations should include
the assessment of the white cell count, inflammatory
markers and radiologic assessment of the neck. A plain
soft tissue lateral x-ray will often show a retropharyngeal
abscess but should be interpreted with caution in children
and an orthopantomogram (OPG) may show a tooth root
infection. Ultrasound scanning or CT scanning will
usually delineate between cellulitis and abscess formation,
but the possibility of false-negatives with either technique
must be considered. Needle aspiration under radiological
control with either should be considered. Small loculated
abscesses may be treated with intravenous antibiotics
for 1224 hours and drainage reserved for those that are
not improving or with large collections.31, 32 A signifi-
cant abscess that cannot be substantially drained under
imaging control should be treated with surgical
drainage.33 Retropharyngeal abscesses or parapharyngeal
abscesses medial to the carotid sheath may be drained
intraorally though care must be taken not to rupture the
abscess before the endotracheal tube is safely in the
trachea. Submandibular and most parapharyngeal
abscesses are best drained externally.
If there is significant airway compromise, endotracheal
incubation with gas induction is usually possible. Twenty-
four to 48 hours of intubation may be necessary or the
insertion of a tracheostomy following drainage will allow
a return to the ward. Local anaesthetic tracheotomy
should be undertaken if the airway cannot be maintained
for a gaseous induction. [**/*]
CONCLUSION
Any chapter on neck swellings should probably conclude
with the advice to put a needle in if there is any doubt or
potential doubt about a diagnosis. In the presence of
 Chapter 140 Benign neck disease: infections and swellings
infection, this may also be therapeutic. The addition of
ultrasound to the fine needle aspirate is probably best
practice and the availability of this and a cytologist in a
one-stop clinic for neck swellings is ideal and should be
becoming commonplace.
Any neck lump in the over 40-year-old must be treated
as a metastatic squamous cell carcinoma from a head and
neck source until another definite diagnosis is made. The
diagnosis of branchial cyst in the over 40-year-old should
be treated with caution and the pathologist asked to do
multiple sections. Similarly, the diagnosis of carcinoma in
a branchial cyst even if supported by histology should be
treated with healthy scepticism and a search for the
primary.
KEY POINTS
! Thyroglossal cysts may present at any age, are
midline, associated with the hyoid bone and
so move on tongue protrusion and
swallowing. Excision must include the body
of the hyoid.
! Branchial cysts in the over 35 age group should
be investigated and treated as metastatic
squamous cell carcinoma. Excision should be
undertaken only after fine needle aspiration,
panendoscopy with biopsies of the tongue base,
postnasal space and bilateral tonsillectomy.
! Injection of OK-432 is a promising new
treatment for lymphangioma. Where possible,
surgical resection remains the treatment of
choice.
! TB lymphadenopathy may be caused by
atypical Mycobacteria and therefore excision
biopsy of the node may be required for
culture and antimicrobial sensitivities.
! Many early or small deep neck space
infections can be treated with aspiration
under ultrasound or CT control, together
with intravenous antibiotics.
! Ludwigs angina has a high incidence of
airway compromise and tracheostomy should
be considered early.
Best clinical practice
[ If history and examination does not lead to a clear
diagnosis, neck swellings should have a fine needle
aspirate for cytology and microbiology ideally with
ultrasound imaging as part of a one-stop clinic.
[ Lymphangiomas should be treated surgically in a
specialist unit.
[ Fine needle aspiration and polymerase chain reaction
should be utilized for the diagnosis of TB.
] 1787
[ For deep neck space infections:
start intravenous antibiotics immediately;
image and consider draining under ultrasound or
CT control;
consider endotracheal intubation or tracheostomy
early.
Deficiencies in current knowledge and
areas for future research
Many of the conditions in this chapter are relatively rare
and any prospective data collection or randomized trial
of management options would therefore be very difficult
unless multicentred or even national. For the majority of
these conditions this is probably not practical. The
author would hope to see the tertiary referral of cystic
hygromas to a small number of surgeons so that
progress in their management can be focused and
controlled trials may be possible.
It would be encouraging if a database of all cystic
neck nodes with squamous carcinoma and no identifiable
primary could be developed to plan a trial of positron
emission tomography (PET) scanning and bilateral
tonsillectomy versus tonsil biopsy to prove best practice.
A current European Organisation for Research and
Treatment of Cancer (EORTC) trial may help decide
whether the mucosal sites should be included in
radiotherapy fields when treating these tumours.
A randomized trial of ultrasound-guided aspiration of
neck abscesses versus open drainage versus antibiotics
alone may struggle to recruit sufficient patients as the
author would predict there would be a large number of
exclusions and would certainly need to be multicentred.
A study looking at CT and ultrasound accuracy in the
detection of pus in deep neck abscesses would be easy
to plan, but choice will often depend on local availability
and expertise.
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FURTHER READING
Enepekides DJ. Management of congenital anomalies of the
neck. Facial Plastic Surgery Clinics of North America. 2001;
9: 13145.
Gidley PW, Ghorayeb BY, Stiernberg CM. Contemporary
management of deep neck space infections. Otolaryngology
and Head and Neck Surgery. 1997; 116: 1622.
McGuirt WF. The neck mass. Medical Clinics of North America.
1999; 83: 21934.
Soh KB. Branchiogenic carcinomas: do they exist? Journal of the
Royal College of Surgeons of Edinburgh. 1998; 43: 15.