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Congenital Cystic Masses of

the Neck

Marie Grobbelaar
Department of Radiology
Tygerberg Academic Hospital

Embryology

Pharyngeal / Branchial arches, clefts and


pouches. In Radiology literature = Branchial
Branchial arches develop 4-5th weeks.
Consist of bars of mesenchymal tissue separated
by clefts branchial clefts.
Branchial pouches appear along lat wall of
pharyngeal gut.

Branchial Arches

Each arch is characterized by its own muscular


components, their own cranial nerve component
and arterial component.

Branchial arches
Arch
1

Nerve
Mandibula
r division
(V3)

Muscles

Skeleton

Muscles of
mastication,
mylohyoid, ant belly
digastricus, tensor
palatini, tensor
tympani

Incus, malleus,
maxilla, zygomatic
bone, part of temp
bone, part of
mandible

VII

Muscles of facial
expression, post
belly digastricus,
stylohyoid, stapedius

Stapes, styloid
process, lesser horn
+ upper body hyoid

IX

Stylopharyngeus

Greater horn + lower


body hyoid

4-6

Cricothyroid,
contrictors of
pharynx, intrinsic
laryngeal muscles

Laryngeal cartilages

Branchial pouches

5 pairs (nr 5 considered part of 4).


1st: middle ear cavity, tympanic membrane,
auditory tube.
2nd: palatine tonsils
3rd: dorsal wing inferior parathyroid glands
ventral wing thymus
4th: dorsal wing superior parathyroid glands
5th: considered to be part of 4th, gives rise to
ultimobranchial body parafollicular / C-cells of
thyroid

Branchial clefts

4 pairs
Only 1st cleft contributes to a definitive structure
external auditory meatus.
Proliferation of mesenchymal tissue of 2nd arch
causes it to overlap 3rd + 4th.
Merges in lower part of neck and 2,3,4th clefts
loose contact with outside.
Temporary cavity cervical sinus forms.

Thyroid - embryology

Develops in the 3rd week of fetal life as an


outgrowth from the floor of the primitive pharynx
(originates at the level of the foramen cecum).
The primitive thyroid descends in the neck and
pass anterior to the developing hyoid bone and
laryngeal cartilages and reaches its final position
in the inferior part of the neck by the 7th week.
During its migration, the thyroid gland is
connected to the tongue by the thyroglossal duct
which involutes by the 8th10th week.
The site of the original opening of the thyroglossal
duct persists as the foramen cecum of the tongue,
with the inferior end of the canal becoming the
pyramidal lobe of the thyroid gland.

Congenital Cystic neck masses


Thyroglossal

duct cysts
Branchial cleft anomalies
Cystic hygromas
Dermoid and Epidermoid cysts
Thymic cysts
Parathyroid cysts
Laryngoceles
Bronchogenic cysts
Gastrointestinal duplication cysts

Congenital cystic neck masses


Manifest

during childhood / early


adulthood.
Slow growing masses.
Only symptomatic because of enlargement
or infection.
Clinically painless soft cervical mass.
Cystic features on ultrasound, CT, MRI
Treatment surgical.

Congenital cystic neck masses


Cysts

Location

Thyroglossal duct cysts


Branchial cleft anomalies

anterior neck, midline


1st parotid space
2nd submandibular space
3rd post cervical space

Cystic hygromas

neck, lower face

Dermoid and Epidermoid


cysts
Thymic cysts

floor of mouth

thoracic inlet, anterior/deep


to SCM

Parathyroid cysts
Laryngoceles

inferior to the thyroid

Cervical bronchogenic cysts

suprasternal notch or
supraclavicular area

Gastrointestinal duplication
cysts

mediastinal masses / couls


extend into lower neck

paralaryngeal space

Thyroglossal duct cyst

Most common congenital neck mass (70% of


congenital neck anomalies) and the second most
common benign neck mass, after benign
lymphadenopathy.
50% of patients present before 20 years of age.
M=F
Mass in midline (75%) / within 2cm of midline
anterior neck.
80% at / below level of hyoid.
Moves upward with tongue protrusion.
Complete excision to decrease recurrence.
1% of thyroglossal duct abnormalities associated with
thyroid carcinoma from ectopic thyroid rests in the
duct or duct itself.

Thyroglossal duct cyst

US - anechoic mass with a thin outer wall in


characteristic location (42%), more commonly
hypoechoic.
CT - smooth, well-circumscribed mass along the
vertical course of thyroglossal duct, thin wall and
homogeneous attenuation, 1018 HU. Usually
unilocular, septations occasionally.
MRI uncomplicated cyst T1 low, T2 high. With
infection variable signal intensity due to
proteinaceous debris.

CT with contrast in
fistula

Axial CECT
MRI T1

Axial CECT

Axial

MRI Sagittal T2

MRI Coronal T2

Branchial cleft anomalies

Manifest as any combination of sinus, fistula, or


cyst.
Cysts - 75%
Sinuses open externally on the side of the neck.
Fistula manifests as a patent abnormal canal,
opening externally on the neck surface and
internally within the pharyngeal mucosa.
Patients with branchial cleft cysts are usually
older children or young adults in contrast to
patients with fistulas, who are usually infants or
young children.
Unilateral (97%).

Second Branchial Cleft Defects

Majority (95%) of branchial cleft anomalies arise from the


second cleft.
75% - cysts, which occur between 10 and 40 years of age
25% - fistulas or sinuses, which manifest during the 1st
decade of life.
M=F
Bailey classified these cysts into 4 types.
Clinically situated in the submandibular space, but can
occur anywhere along a line from the oropharyngeal
tonsillar fossa to the supraclavicular region of the neck
(relation to the cervical sinus).
Slow-growing, painless, masses in the lateral portion of the
neck.
Surgical excision.

Drawing shows the path of second branchial cleft anomalies, which can occur
anywhere along a line from the supraclavicular region to the oropharyngeal mucosa.
The path travels lateral to the common carotid artery, then heads medially between
the external carotid (EC) and internal carotid (IC) arteries under the
glossopharyngeal nerve (IX) and above the hypoglossal nerve (XII). If the tract
continues further along this course, it will enter the parapharyngeal space and
pierce the middle constrictor muscle before ending as an opening within the tonsillar
fossa.

Second Branchial Cleft Defects

US - sharply marginated, round / ovoid, centrally anechoic


mass with a thin peripheral wall that displaces the
surrounding soft tissues. The mass is compressible and shows
distinct acoustic enhancement.
CT - well-circumscribed, homogeneously hypodense mass with
a uniformly thin wall.
MRI T1 variable signal, T2 high
The "classic" location (CT or MRI): at the anteromedial border
of the SCM muscle, lateral to the carotid space, and at the
posterior margin of the submandibular gland. The cyst
typically displaces the sternocleidomastoid muscle posteriorly
or posterolaterally, pushes the vessels of the carotid space
medially or posteromedially, and displaces the submandibular
gland anteriorly.
CT and MRI - "beak sign" represents a curved rim of tissue or
"beak" pointing medially between the internal and external
carotid arteries. It is considered a pathognomonic imaging
feature of a second branchial cleft cyst.

Submand
gland

Carotid space

MRI Axial T1

SCM

MRI Axial T2

CECT

MRI Axial T1 + Gad

MRI Coronal T1

First Branchial Cleft Defects

5-8% of all branchial cleft defects.


Cysts (68%), fistulas (16%) and sinuses (16%).
Commonly seen in middle aged woman, but both
children and adults may be affected.
Manifests as recurrent abscesses / inflammation
(sinus tract) around the ear or at the angle of the
mandible.
Typical history of recurrent parotid abscesses
unresponsive to antibiotic therapy and drainage.
Mimics the clinical characteristics of parotid
neoplasms.
Otorrhea commonly occurs if the cyst drains into the
external auditory canal.
Surgical excision is the only curative therapy.
Work classified 2 types.

First Branchial Cleft Defects

CT - cystic mass within, superficial to, or deep to


the parotid gland. Cyst wall thickness and
enhancement are variable and increase with
recurrent infections.
Neither CT nor MR imaging appearance of these
cysts is characteristic enough to allow
differentiating a first branchial cleft cyst from any
other cystic mass of the parotid gland.
May extend into the adjacent fat-containing
parapharyngeal space.

Axial CT

Axial CT

Coronal CT

Axial MRI
Axial MRI

Third Branchial Cleft Defects

Rare.
Children and young adults.
Second most common congenital lesion of the posterior
cervical space of the neck after cystic hygroma.
Most located in the posterior cervical space, posterior to the
SCM and posterior to the common / internal carotid artery.
Superior to the superior laryngeal nerve.
Majority on the left.
If a fistula present, will pierce thyrohyoid membrane en route
to the pyriform fossa.
May be associated with ectopic parathyroid gland.
Painless mass in posterior triangle of neck.
Surgical excision.
CT and MRI unilocular, cystic mass in posterior cervical
space.
T1 signal variable, T2 high.

Fourth Branchial Cleft Defects

Rare.
Children and young adults.
Majority on the left.
Manifest as a sinus tract rather than a cyst / fistula.
Arises from the pyriform fossa, pierces the
thyrohyoid membrane, and descends into the
mediastinum, following the path of the
tracheoesophageal groove. If the tract is long
enough, a left-sided lesion will eventually travel
under the aortic arch before ascending into the
neck along the ventral surface of the common
carotid artery. Most of these lesions are short and
thus sinus tracts
Inferior to the superior laryngeal nerve.
May be associated with ectopic parathyroid gland.

Cystic Hygroma

Most common form of lymphangioma - 5% of all benign tumors of


infancy and childhood.
Majority (about 80%90%) detected by age 2.
Arise from an early sequestration of embryonic lymphatic channels
or may also arise from a failure of the juguloaxillary lymphatic sac
to drain into the internal jugular vein, producing a congenital
obstruction of lymphatic drainage.
Association with Turner syndrome
75%80% of all cystic hygromas involve the neck and the lower
portion of the face.
Children - commonly the posterior cervical space, followed by the
oral cavity.
Adults - commonly seen in the sublingual, submandibular, and
parotid spaces.
Infiltrative in nature and do not respect fascial planes.
May extend inferiorly from the posterior cervical triangle into the
axilla and mediastinum or anteriorly into the floor of the mouth
and the tongue, it may extend across the midline.
Asymptomatic, painless, soft masses in the neck, variable size.

Cystic Hygroma

US multilocular, cystic mass with septa of


variable thickness. The echogenic portions of the
lesion correlate with clusters of small, abnormal
lymphatic channels.
CT - poorly circumscribed, multiloculated,
hypodense masses. They have characteristic
homogeneous fluid attenuation.
MRI T1 low / intermediate signal intensity, T2
high. May be hyperintense on T1 when associated
with clotted blood or high lipid (chyle) content. In
the case of hemorrhage, fluid-fluid levels may be
observed.

CECT

MRI Axial T1

MRI Coronal T1

CECT

MRI Coronal T1

MRI Axial

Dermoid and Epidermoid Cysts

Teratomas are common congenital neoplasms


consisting of tissues of all three germ cell layers.
The spectrum of teratomas includes dermoid
cysts, epidermoid cysts and teratoid cysts.
The essential difference between a dermoid cyst
and an epidermoid cyst lies in the presence of
skin appendages (eg, sebaceous glands, hair
follicles) within the wall of the dermoid cyst and
the absence of these features in the epidermoid
cyst.
A teratoid cyst may also contain tissue of other
major organ systems (eg, nervous,
gastrointestinal, respiratory).

Dermoid and Epidermoid Cysts

Dermoid cysts manifest during the 2 nd - 3rd decades of life.


M=F
Most common cervical location - floor of the mouth.
Appear clinically as a midline, suprahyoid, slowly growing
mass, which is soft, mobile, and unattached to overlying skin.
No association with the hyoid bone and do not move with
tongue protrusion. Variable size.
It may also arise within the tongue or from the palate. When
inferior to the mylohyoid muscle - submental swelling visible
and the cyst is externally palpable. If the mass is located
between the geniohyoid and mylohyoid muscles in the
sublingual space, only a small external swelling is seen. This
information is crucial for optimal preoperative planning.
5% of dermoid cysts undergo malignant degeneration into
squamous cell carcinoma.
Epidermoid cysts of the neck are rare congenital lesions, they
appear earlier than dermoid cysts, with most lesions evident
during infancy.

Dermoid and Epidermoid Cysts

CT - central cavity is filled with a homogeneous,


hypodense (018 HU) fluid material. It may appear
to be filled with "marbles," due to the coalescence
of fat into small nodules within the fluid matrix.
This "sack-of-marbles" appearance is virtually
pathognomonic for a dermoid cyst in this location.
Alternatively, the cyst may be heterogeneous on
because of the various germinal components. The
rim of these cysts often enhances after
administration of contrast.
MRI - dermoid cysts have variable signal intensity
on T1 and hyperintense on T2.
Epidermoid cysts have fluid density on CT and are
hypointense on T1 and hyperintense on T2.

Axial CECT

Coronal CECT

Axial CECT

US

MRI Coronal T1

Axial CECT

Thymic cysts

Cervical thymic cysts are uncommon.


Detected in 1st decade of life.
Present with a slowly enlarging, painless mass in
the lateral portion of the neck near the thoracic
inlet, either anterior or deep to the SCM muscle.
US - large, usually unilocular cystic mass
extending downward, parallel to the
sternocleidomastoid muscle.
CT - uniloculated or multiloculated, hypodense
cystic mass adjacent to the carotid space. The
mass may extend into the mediastinum.
MRI - T1 low and T2 high.

NECT

NECT

Parathyroid Cysts

Most commonly inferior to the thyroid.


Because of their proximity to the thymic
primordium can be situated anywhere a thymic
anomaly can be found.

Laryngocele

The laryngeal ventricle is a slitlike cavity, the orifice located


between the false and true vocal cords.
Along the roof of this ventricle arises a small, blind, mucosalined pouch known as the laryngeal saccule or appendix.
It extends superiorly between the false vocal cord and
aryepiglottic fold medially and the thyroid cartilage laterally.
The saccule is relatively large in newborns and children but
starts to involute by the end of the 6th year of life.
A laryngocele is a dilated laryngeal saccule.
3 types: internal, external, and mixed.
Internal (40%): confined to the larynx, do not pierce the
thyrohyoid membrane.
External (26%): extend through the thyrohyoid membrane. The
component superficial to the thyrohyoid membrane is dilated,
and the saccular portion inside the membrane is normal in size.
Mixed (44%): have abnormal dilatation of the saccule on both
sides of the thyrohyoid membrane.

Laryngocele

Manifests in adults.
Hoarseness, dysphagia, and stridor may occur with internal
laryngoceles.
A compressible soft-tissue mass is seen with the external type
which may expand when a Valsalva maneuver is performed.
Increased frequency of laryngoceles in patients with laryngeal
carcinoma, best appreciated on T2-weighted images on which
the neoplasm is lower in signal intensity compared with that of
the fluid within the laryngocele
CT - well-defined, smooth mass in the lateral aspect of the
superior paralaryngeal space. Internal laryngoceles will be
limited by the thyrohyoid membrane. External and mixed
laryngoceles lie superficial to the thyrohyoid membrane at the
point of insertion of the superior laryngeal nerve and vessels.
MRI - best appreciated on T2-weighted images.

Axial CT

Cervical bronchogenic cysts

Extremely rare.
In infants as well as adults.
Occur in males about three times as often as in
females.
Present as swelling or draining sinus, usually
located in the suprasternal notch or
supraclavicular area.

Gastrointestinal duplication cysts

Manifest during the 1st year of life.


Esophageal duplications may appear as
mediastinal masses.

MRI Coronal T1+Gad+FS

MRI Axial T2

References

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