PETER CLARKE
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]
adulthood, with over 30 percent presenting before ten present suprahyoid and just over 10 percent related to the
years of age. There is an equal male-to-female incidence thyroid. Because of the hyoid attachments, these masses
(Figure 140.1). move both on swallowing and protrusion of the tongue.
Cysts may become infected and incision and drainage
Embryology may result in the development of a sinus from the cyst to
the skin.
During the development of the branchial apparatus in a
four- to five-week-old embryo, the thyroid enlage arises in Pathology
an invagination of endoderm in the floor of the pharynx
and develops caudally, with the descent of the great
Thyroglossal cysts are usually lined by columnar epithe-
vessels. It enlarges and becomes bilobed, coming to rest at
lium with small glands frequently containing thyroid
the root of the neck as the thyroid gland by the end of the
colloid. Decalcification of the hyoid bone will often
seventh week. The tract that is left usually atrophies and
confirm that the tract passes through it.4
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 Management
expand into a cyst because of an accumulation of
secretions. The hyoid bone, developing later, may entrap
The diagnosis is clinical and may be confirmed with a fine
a portion of the thyroid duct or draw it caudally, leaving
needle aspirate if the mass is suprahyoid to help
the duct dorsal to the bone.3
differentiate it from a dermoid cyst or submental lymph
node. Further investigations are not necessary for
Clinical features diagnosis, but should be undertaken to ensure that a
normal thyroid gland is present. Ultrasound scanning is
Most present as midline masses 23 cm in diameter just accurate, cost-effective and would seem to be the
inferior to the hyoid bone (Figure 140.1). A quarter 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.
Figure 140.1 Large thyroglossal cyst presenting in late There have been a large number of case reports and
adulthood with symptoms of obstructive sleep apnoea. Elevation small series of thyroid carcinoma presenting in a
of cyst on tongue protrusion demonstrated. thyroglossal cyst. Up to 90 percent of these are papillary.
Chapter 140 Benign neck disease: infections and swellings ] 1779
A recent review from Slone Kettering6 suggests treatment Pharyngeal Pharyngeal Pharyngeal
should be with Sistrunks operation and thyroid suppres- clefts arches pouches
sion with thyroxine. Thyroidectomy and postoperative
radioactive iodine, although revealing malignancy in a
quarter of patients, did not confer a survival advantage I
and they conclude that this should be reserved for high-
risk groups. [Grade D] 1 1. Eustachian tube
and middle ear
II cleft
LATERAL CERVICAL CYSTS AND BRANCHIAL
ABNORMALITIES 2. Palatine tonsil
III
Lateral cervical or branchial cysts occur equally in the 2
sexes and present most commonly in young adults, 3. Inferior
3
although they can occasionally present at any age. There is parathyroid thymus
debate about the precise aetiology of the cysts and fistulae 4 IV
and there may be a number of different aetiologies 4. Superior
resulting in a similar clinical presentation. parathyroid
ultimobranchial
body
Branchial fistulae
Figure 140.2 Development of the pharyngeal (branchial)
Branchial fistulae usually present in childhood as a apparatus. The second arch grows over the third and fourth
weeping defect along the anterior border of sternoclei- pharyngeal arches so burying the second, third and fourth
domastoid, or occasionally as an acute infection. pharyngeal clefts. The remnants of these clefts form the cervical
sinus of His.
EMBRYOLOGY
ultimobranchial body (Figure 140.2) and hence extend
During the fourth week of intrauterine life, six branchial from the pharynx to the thyroid. The fact that fourth
(or pharyngeal) arches develop as neural crest cells branchial fistulae only seem to occur on the left would
migrate into the head and neck region. During the fifth suggest that the timing of the descent of the aorta allows a
week, the second branchial arch grows over the third and fistula to remain on the left whilst descent of the
fourth branchial clefts which form a cervical sinus (Figure subclavian artery may obliterate potential fistulae on the
140.2). Failure of the cervical sinus to close may therefore right. Although fistulae undoubtedly exist running from
potentially communicate with the second branchial the piriform fossa to the skin and are presumably related
pouch (and therefore the tonsil fossa), the third branchial to the branchial apparatus, the exact mechanism of their
pouch in the area of the larynx or the fourth branchial formation cannot be certain. Often described in the
pouch opening in the piriform fossa. 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
PRESENTATION usually present in children though may be asymptomatic
Clinically, second branchial cleft fistulae are the most until adult life. [**/*]
common. They have a cutaneous opening along the
anterior border of the sternocleidomastoid, usually at the
MANAGEMENT
junction of the middle and lower thirds, and track up
through the neck to run between the internal and external Fistulae may be investigated with a sinogram. Pharyngo-
carotid arteries and end in the tonsillar fossa (Figure scopy with careful examination of the tonsil fossa and
140.3). piriform fossa mucosa, depending on presentation,
Third and fourth branchial fistulae are more rare, should be undertaken prior to excision.
opening low in the neck and ending in the piriform fossa. Surgical excision should be started with an elliptical
The third arch fistula should pass over the hypoglossal excision of the fistula opening. Dissection of the track can
nerve and the fourth arch fistula should run down into be helped by careful injection of dye into the opening
the chest, running below the arch of the aorta on the left before commencement. A second branchial fistula will
or subclavian archway on the right. It is doubtful whether need one or more further skin incisions to allow safe
these exist as clinical entities, however. A recent paper7 dissection to the carotid. Excision of the tonsil fossa
suggests that fourth branchial cleft fistulae arise from the 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.
mucosa from the tonsil fossa does, however, infection, attempts should be made to aspirate the
suggest an origin from this area.9 contents and treat the patient with intravenous anti-
2. Origin from the cervical sinus: This theory biotics, though incision and drainage may be necessary.
proposes the branchial cleft rather than pouch Imaging is not usually necessary to make the diagnosis.
forms cysts. Overgrowth of the second arch Lateral cervical cysts should be surgically removed.
produces the cervical sinus of His (Figure 140.2) After a transverse skin crease incision over the cyst is
which closes to leave the cervical vesicle, which, made, dissection is followed along the anterior border of
it is postulated, results in the lateral cervical the sternocleidomastoid. Blunt dissection close to the cyst
cyst. This is an attractive theory is usually simple and allows removal of the cyst. If there
embryologically, although does not fully has been previous infection or the cyst is very adherent to
explain why cysts usually do not appear local tissues, dissection should continue as for a neck
clinically until the third or fourth decade and it dissection with identification of the spinal accessory
might be expected that more cysts are found nerve, carotid sheath and hypoglossal nerve with resection
with tracts or sinuses running to the skin. of the cyst and surrounding nodes and fibrous tissue.
3. Origin from lymph node degeneration: Proponents of a branchial sinus origin suggest looking
Originally postulated in the nineteenth century, for a cord or tract extending between the internal and
King10 in 1949 studied a large number of cysts external carotid and possibly examination of the tonsil
and concluded that cystic transformation of fossa prior to excision of the cyst. If cysts have presented
lymph nodes was the likely origin of lateral with acute infection and been treated with antibiotics, the
cervical cysts. Certainly most cysts have lymphoid cyst will occasionally not be apparent after resolution and
tissue similar to lymph nodes in their walls and unless radiology either ultrasound scan or CT scan
few, if any, have tracts to the skin or pharynx. It shows an obvious cyst, these cases can be treated
might be expected, however, if this theory were expectantly. [Grade D]
true that the position in the neck may be more
variable.
Branchiogenic carcinoma
PRESENTATION Opinion on the existence of this entity varies from the
In early adulthood, a cystic, often tender oval mass suggestion that it is underdiagnosed to denial of its
46 cm in diameter anterior to the upper third of the existence.
sternocleidomastoid develops fairly quickly. A recent Following initial descriptions and the coining of the
upper respiratory tract infection is often described. term branchiogenic carcinoma, it became clear that the
Swelling may be intermittent and occasionally the cyst majority of these cases were actually metastatic deposits
is overtly infected with overlying erythema and pain at from primary squamous cell carcinomas in the head and
presentation. The main differential diagnosis is from neck. In 1950, Martin et al.11 therefore suggested that to
reactive lymphadenopathy. In children, a dermoid cyst or diagnose a branchiogenic carcinoma, four criteria had to
rhabdomyosarcoma should also be considered. In young be met.
adults, lymphoma, tuberculosis (TB) or nerve sheath 1. The cervical tumour occurs along the line
tumours need to be excluded. In patients over 35 years of extending from the tragus to the clavicle, along
age, a cervical metastasis from a head and neck primary the anterior border of sternocleidomastoid.
must be excluded. 2. The histological appearance must be consistent
with an origin from tissue known to be present in
branchial vestiges.
PATHOLOGY 3. No primary source of the carcinoma should be
Lateral cervical cysts are usually lined with squamous discovered after at least five years of follow-up.
epithelium with transitional type pseudostratified epithe- 4. There is histologic demonstration of cancer
lium. Cysts presenting in the younger age group are more arising in the wall of an epithelial-lined cyst.
likely to be lined with respiratory epithelium. Even apparent fulfilment of these criteria, however,
cannot exclude a metastatic squamous cell carcinoma. A
cystic metastasis may have many similar features,
MANAGEMENT
histologically, to a branchial cyst and the addition of
The differential diagnosis can be simplified considerably radiotherapy to the excision of these masses will treat
by fine needle aspiration. Occasionally straw-coloured small tonsil or tongue base tumours, so making even
fluid is obtained, though more often, greasy yellow thick adherence to Martins criteria meaningless.12
mucoid material is aspirated, probably representing Most metastatic nodes with unknown primary are
recent inflammation. Should they present with acute solid and most cystic nodes have primary tumours that
1782 ] PART 14 THE NECK
can be demonstrated in the tongue base or tonsil. Several and about a quarter in the neck. They are uncommon
series, including 136 cases of cystic squamous cell with only five cases per 3000 admissions to a paediatric
carcinoma,13 without an obvious primary site of origin hospital and only account for four of 152 benign tumours
at initial diagnosis, have shown that in the vast majority a of the neck. These lesions are congenital, arising from
primary tumour can be found. Most are in Waldeyers localized lymphatic stasis caused by congenital blockage
ring, but other sites found include lung, larynx, thyroid, of regional lymphatic drainage or developing from
palate, sinuses and salivary gland. sequestration of the primitive embryonic endothelial
There is no innate reason why the epithelium of a budding that arises from the lymphatic sacs.
lateral cervical cyst should not become malignant and
1. Capillary lymphangioma: Capillary or simple
certainly there are case reports of tumours that fit
lymphangiomas are comprised of capillary-like
Martins criteria, but estimates suggest that the incidence
lymphatic vasculature and are usually
of branchiogenic carcinoma, if they exist, is in the region
asymptomatic and confined to the superficial skin
of 0.3 percent of all head and neck carcinomas.14 [**/*]
or oral cavity. They are pale, small vesicle-like
lesions and no treatment is necessary.
2. Cavernous lymphangioma: Cavernous
Other cystic lesions lymphangiomas are composed of dilated
lymphatic channels and typically occur in the
DERMOID CYSTS tongue, cheeks and lips, causing diffuse swelling.
These originate from ectoderm and mesoderm, and the These account for 40 percent of lymphangiomas.
head and neck is the most common site to find them. 3. Cystic hygroma: Cystic hygromas are
They can present at any age, but the majority present in histologically similar to cavernous lymphangiomas
patients aged under six years. Sex distribution is equal with thin-walled sinuses and cysts that are lined
and they present along lines of embryonic fusion of the by flat endothelial cells and contain eosinophilic,
facial processes predominately in the midline or lateral acellular lymph fluid. In cystic hygromas, there
to the submandibular gland. They occur in the sub- are larger cystic masses which may communicate
cutaneous tissues and contain skin appendages such as or be isolated.
hair follicles, sebaceous glands and sweat glands, so
differentiating them from epidermoid cysts. Dermoid
PRESENTATION
cysts may also occur from implantation secondary to
puncture wounds. Complete surgical excision is required Lymphangiomas occur predominately in the neck, often
and is usually straightforward. 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-
BRONCHIAL CYSTS stinum or axilla. In the neck they are fluctuant, soft, cystic
These are uncommon congenital lesions that can occur in diffuse masses with indistinct margins. Classically they
the suprasternal notch, but are more common within the transilluminate.
thoracic cavity or mediastinum. They are more common in The majority of lymphangiomas are present at birth and
males and one-third are associated with a discharging may now be diagnosed prenatally. It is extremely rare for
sinus. The cysts are lined by ciliated pseudostratified these to present after the age of two years, but recurrence
columnar epithelium and the sinus with stratified squa- after previous treatment may occur after many years. They
mous epithelium. Complete surgical excision is curative. are usually asymptomatic, but are a distressing cosmetic
Cervical or plunging ranulae are considered with problem. Cystic hygroma and cavernous lymphangioma
salivary glands (Chapter 147, Non-neoplastic salivary either grow progressively or remain static and there have
gland diseases) and laryngocoeles are considered with the also been many reports of involution. Bleeding or infection
larynx (Chapter 88, Congenital disorders of the larynx, may, however, cause rapid growth and symptoms of pain
trachea and bronchi). or airway compromise which may be life-threatening. The
largest cystic hygromas may also cause airway problems
without infection or bleeding.14[**/*]
BENIGN TUMOURS
MANAGEMENT
Cystic hygroma and lymphangioma Magnetic resonance imaging (MRI) delineates the extent of
the lesion well and will facilitate accurate diagnosis and
These benign lymphatic lesions can be divided into three pretreatment planning. Prenatal diagnosis may be made
morphological types: capillary, cavernous and cystic. with ultrasound scanning and potential airway compromise
One-third of cases are found in the oral cavity and cheek assessed with prenatal MRI. There are case reports of
Chapter 140 Benign neck disease: infections and swellings ] 1783
infants being treated ex utero to avoid airway problems at develop from lymph nodes, may develop in any of the
birth. In this country, the majority are not diagnosed until cystic masses described above or may spread from local
birth and treatment depends on the size and anatomical abscess formation around teeth, tonsil or from trauma.
location of the lesion. Treatments used for lymphangioma Tuberculosis, infectious mononucleosis, human im-
include surgical excision and intralesional injections of such munodeficiency virus (HIV) infection, cat scratch fever
sclerosants as bleomycin, tetracycline and alcohol. Surgery and toxoplasmosis may all produce generalized cervical
is challenging and should be undertaken in specialist lymphadenopathy.
centres. With time and expertise, results can be excellent
and surgery remains the treatment of choice. Resection is
Tuberculosis
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 Cervical lymphadenopathy is the most common head and
the lymphatic spaces. A staging system has been used to neck manifestation of TB and can occur in any age group,
predict surgical outcome (Table 140.1).15, 16 though most series suggest the most common presenta-
Injection of sclerosants may lead to scarring and make tion is in young adults. Most series suggest only 1020
subsequent surgery more difficult, but a new intralesional percent to have associated pulmonary disease or to have
injection of OK-432 (picinabil) has shown promising had a history of contact with TB. Up to half of these
results. It is thought to produce an inflammatory reaction patients will have systemic symptoms. It has been
leading to adhesions or to increased permeability of the suggested that the bacillus enters via the tonsils as
endothelial lining, increasing the rate of drainage from tonsillectomy specimens will often show evidence of TB
the lesion. Pooled results from a group of series show infection in these patients. Nodes tend to be tender and
marked shrinkage in 72 out of 116 children. It may be less may have overlying erythema or occasionally a dischar-
effective in cavernous lymphangioma and lymphangioma ging sinus. Suspicion should be raised in immunocom-
with haemangiomatous elements.17 [**/*] 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 [**/*]
Paragangliomas and nerve sheath tumours
results in a mild self-limiting illness characterized by syndrome (AIDS) or a poor prognosis. Enlarging or
fever, pharyngitis, tonsillitis and lymphadenopathy. How- tender nodes are not typical of PGL and in a series of HIV
ever, in teenagers and young adults, it may present with patients with enlarging or tender nodes none had PGL
significant tonsillar enlargement causing airway compro- and a diagnosis of TB was made in eight, opportunistic
mise and rarely death. Suppuration of lymph nodes can nocardial infection in two, lymphoma in one and
occasionally occur and awareness of the possibility of metastatic carcinoma in one.25
splenic rupture is important. The enlarged tonsils have a
characteristic grey fibrinous exudate and patients often
have a typical hot potato voice.
Cat scratch disease
Actinomycosis
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
Figure 140.6 Actinomycosis. Note multiple weeping punctae. must be considered. Needle aspiration under radiological
control with either should be considered. Small loculated
fourth decade. There is a dental origin in 40 percent of abscesses may be treated with intravenous antibiotics
patients and other aetiologies are tonsillitis with or for 1224 hours and drainage reserved for those that are
without peritonsillar abscess, TB, intravenous drug abuse not improving or with large collections.31, 32 A signifi-
following injection into the internal jugular vein, man- cant abscess that cannot be substantially drained under
dibular fracture and foreign body ingestion. In up to 20 imaging control should be treated with surgical
percent of cases, no obvious aetiological factor is found.29 drainage.33 Retropharyngeal abscesses or parapharyngeal
Most bacterial cultures are polymicrobial and Streptococcus abscesses medial to the carotid sheath may be drained
viridans is the most common organism isolated. Staphy- intraorally though care must be taken not to rupture the
lococcus aureus and Staphylococcus epidermidis are also abscess before the endotracheal tube is safely in the
important. Anaerobes probably play a significant role in trachea. Submandibular and most parapharyngeal
these abscesses, but are not always cultured. abscesses are best drained externally.
If there is significant airway compromise, endotracheal
incubation with gas induction is usually possible. Twenty-
Clinical features four to 48 hours of intubation may be necessary or the
insertion of a tracheostomy following drainage will allow
These patients present acutely unwell with pain in the a return to the ward. Local anaesthetic tracheotomy
throat and neck, fever, a raised white cell count and raised should be undertaken if the airway cannot be maintained
inflammatory markers. The clinical picture depends on the for a gaseous induction. [**/*]
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 CONCLUSION
present with dysphagia, odynophagia and airway compro-
mise. The most common deep neck abscess is a lateral Any chapter on neck swellings should probably conclude
pharyngeal (parapharyngeal) space infection which causes with the advice to put a needle in if there is any doubt or
a diffuse, tender, indurated swelling which is usually potential doubt about a diagnosis. In the presence of
Chapter 140 Benign neck disease: infections and swellings ] 1787
infection, this may also be therapeutic. The addition of [ For deep neck space infections:
ultrasound to the fine needle aspirate is probably best start intravenous antibiotics immediately;
practice and the availability of this and a cytologist in a image and consider draining under ultrasound or
one-stop clinic for neck swellings is ideal and should be CT control;
becoming commonplace. consider endotracheal intubation or tracheostomy
Any neck lump in the over 40-year-old must be treated early.
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 Deficiencies in current knowledge and
a branchial cyst even if supported by histology should be areas for future research
treated with healthy scepticism and a search for the
primary. 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
KEY POINTS unless multicentred or even national. For the majority of
these conditions this is probably not practical. The
! Thyroglossal cysts may present at any age, are author would hope to see the tertiary referral of cystic
midline, associated with the hyoid bone and hygromas to a small number of surgeons so that
so move on tongue protrusion and progress in their management can be focused and
swallowing. Excision must include the body controlled trials may be possible.
of the hyoid. It would be encouraging if a database of all cystic
! Branchial cysts in the over 35 age group should neck nodes with squamous carcinoma and no identifiable
be investigated and treated as metastatic primary could be developed to plan a trial of positron
squamous cell carcinoma. Excision should be emission tomography (PET) scanning and bilateral
undertaken only after fine needle aspiration, tonsillectomy versus tonsil biopsy to prove best practice.
panendoscopy with biopsies of the tongue base, A current European Organisation for Research and
postnasal space and bilateral tonsillectomy. Treatment of Cancer (EORTC) trial may help decide
! Injection of OK-432 is a promising new whether the mucosal sites should be included in
treatment for lymphangioma. Where possible, radiotherapy fields when treating these tumours.
surgical resection remains the treatment of A randomized trial of ultrasound-guided aspiration of
choice. neck abscesses versus open drainage versus antibiotics
! TB lymphadenopathy may be caused by alone may struggle to recruit sufficient patients as the
atypical Mycobacteria and therefore excision author would predict there would be a large number of
biopsy of the node may be required for exclusions and would certainly need to be multicentred.
culture and antimicrobial sensitivities. A study looking at CT and ultrasound accuracy in the
! Many early or small deep neck space detection of pus in deep neck abscesses would be easy
infections can be treated with aspiration to plan, but choice will often depend on local availability
under ultrasound or CT control, together and expertise.
with intravenous antibiotics.
! Ludwigs angina has a high incidence of
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