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Branchial apparatus
anomalies
R3 林哲儀
Branchia: gills
z 30% of congenital neck masses
z Present as cyst, sinus, or fistula
z
z Cyst:
without external opening
z Sinus: with external opening
z Fistula: persistent of branchial grooveÆ
pharyngocutaneous fistula
z
Male : Female = 1:1
Embryology
z
Branchial arches (apparatus)
z End
of 4th gestation, 4 well defined pair of arches
and 2 rudimentary arches developed
z Ectoderm
z Mesoderm: artery, nerve cartilage, muscle
z Endoderm
z Clefts,
externally, ectoderm
z Pouches, internally, endoderm
z Obliterate to form head and neck structure
z Incomplete obliteration: branchial anomalies
Pathology
z
Branchial anomalies lined with respiratory
or squamous epithelium
z Cyst:
squamous epithelium
z May
mixed with follicular-lymphoid tissue,
sebaceous gland, salivary tissue, cholesterol
crystals
z Sinus:
ciliated or columnar epithelium
z Nonfollicular
z Squamous
adults
lymphoid tissue
cell carcinoma: rare, but most in
Diagnosis
z
Upper airway endoscopy
z
z
z
z
Fine needle aspiration
z
z
z
z
z
Pharyngeal opening
Tonsillar fossa
Pyriform sinus
For rule out metastatic carcinoma
Avoid incision biopsy
CT is first choice
MRI, sonography
Esophagography: 80% sensitivity for 3rd and 4th
fistula
Treatment
Complete surgical excision
z Timing of operation
z
z Early
z
resection for prevent infection
Acute infection
z Antibiotic
treatment first
z Incision and drainage
z Complete resection after resolution
First branchial cleft anomaly
z
Rare, 1% of branchial cleft malformation
z
z
z
z
z
z
z
Female > male
May involve EAC, tympanic membrane or middle ear
Close to the parotid gland, superficial lobe
Traveling through above, or below the facial nerve
branchesÆ opening at mandible angle
Symptom: Otorrhea, parotid swelling, mandible pit
discharge……
Histology: most commonly lined by stratified
squamous epithelium
2 types, Arnot, 1971, base on anatomic location
z
Type I
z
z
z
Lower pole of the
parotid gland
Closely to lower
branches of facial
nerve
Passing between the
mandible and the
stylohyoid ligament
z
Type II
z
z
Anterior and below the
mandible angle
Ending at bony part of
EAC
z
Type I (Work, 1972)
z
z
z
z
duplications of the membranous
external auditory canal, parallelly
Composed of ectoderm only
Within parotid gland, lower pole
Superior to the facial nerve, and
between the mandible and
stylohyoid ligament
z
Type II (Work, 1972)
z
z
z
z
z
Composed of ectoderm and
mesoderm, contain cartilage,
hair, or glands
End at bony part of EAC
Subtance within parotid gland
and close to the facial nerve
present as preauricular,
infraauricular, or postauricular
inferior to the angle of the
mandible, anterior to SCM
z
Treatment
z Standard
cervico-mastoid-facial
parotidectomy incision with dissection of the
facial nerve and superficial parotidectomy
z Assisted by injection of methylene blue or
probe guide to identified the tract
z
Recurrence: common, average receive 2.4
procedure
Second cleft anomalies
z
z
z
z
~90% of all branchial cleft malformation,
Incidence: cyst > fistula
Present as recurrent unilateral tonsillitis or
parapharyngeal absecss
Cyst:
z
z
z
z
Most diagnosed at age of 30~ 50
Acute enlargement after a URI
Lead to airway compromised, torticollis, dysphagia
Fistula
z
z
Most diagnosed at infant
Present with chronic discharge along anterior SCM in lower 1/3
neck
z
Traverse deeply to second arch
structures and superficially to
third arch structures
z
z
external openingÆ pierce
platysmaÆ run superiorly along the
carotid sheathÆ turn medially to
hypoglossal nerveÆ beneath
digastric muscle (post belley)Æ
course between the internal and
external carotid arteriesÆ cross
glossopharyngeal nerveÆ internal
opening in the tonsillar fossa
most common: lateral to internal
jugular vein (carotid bifurcation)
z
Type I
z
anterior to the SCM,
not contact the carotid
sheath
z
Type II
z
z
the most common
deep to the SCM,
either anterior or
posterior to the carotid
sheath, lateral to IJV
z
Type III
z
between the internal
and external carotid
arteries, adjacent to
the pharynx
z
Type IV
z
medial to the carotid
sheath, close to the
pharynx, adjacent to
the tonsillar fossa.
z
Treatment
z Transverse
incision over skin fold
z Exploration of an associated fistula with
complete excision
z Monofilament or probe for cannulating the
fistula tract
z Finger assisted to identified the internal
opening in tonsillar fossa
z Cysts lying medial to the carotid sheath may
be more easily removed transorally
z
Recurrent rate, Mayo Clinic
z 21%
when there was a history of prior surgery
z 14% when there was a history of infection
z 3% for cases with no history of prior surgery
or infection
z Infection and prior surgery may cause
adhesions and distortion of tissue planes,
which may lead to higher recurrence rates
and an increased risk of damage to nearby
nerves and vessels
Third and fourth branchial
anomalies
z
Sandborn, 1972, the first description
z
z
Takai, 1979
z
z
z
sinus tract originating from the piriform fossa might
represent a branchial anomaly
Persistent sinus of pyriform fossa with acute
suppurative thyroiditis
Rare, 3rd branchial anomalies representing 2%
to 8% and 4th branchial anomalies representing
1% to 2%
Pouch: from pharynx to hypoid boneÆ open in
to pyriform sinus
z
Third branchial anomaly
z
pyriform fossaÆ pierce
thyrohyoid membraneÆ passing
cranially to the superior
laryngeal n. Æ pass superficial
to the hypoglossal n., deep to
the glossopharyngeal n. and the
ICA Æ platysmaÆ anterior
border of SCM at lower neck
z
Fourth branchial cleft anomaly
z
Pyriform sinusÆ circothyroid jointÆ
between sup. laryngeal nerve and
recurrent laryngeal nerveÆ
z
z
z
Left site: Æ descending along
trachea and esophagus to
mediastinumÆ looping aorta
anteriorly
Right site: Æ descending along
trachea and esophagusÆ looping
subclavian artery anteriorly
Ascending the neckÆ posterior to
the internal and common carotid
arteriesÆ exit anterior to SCM in
the lower neck
z
Distinguish from 3rd from 4th branchial anomaly
z
relationship of the anomalous tract to the superior and
recurrent laryngeal nerves
z
z
z
Histology finding
z
z
inferior to the superior laryngeal nerve and superior to the
recurrent laryngeal nerveÆ 4th pouch origin
pass cranial to the superior laryngeal nerve and inferior
constrictor muscleÆ 3rd pouch orogin
Ectopic thymic tissue(+), inferior parathyroid tissue(+),
superior parathyroid gland involvement(-)Æ 3rd arch anomaly
Case of 4th pouch sinus desecending below clavicle is
rare
z
4th branchial pouch anomaly
z
z
most in children, female predominant
97% left predominant,
z
z
z
z
z
Related to asymmetric development of 4th arch vascular
structure
In neonatal: present as lateral neck cyst or abscess with
obstructive airway symptoms
In children or adult: recurrent lateral cervical abscess and
recurrent suppurative thyroiditis
Retrograde migration of infective material from hypopharynx
May present as recurrent retropharyngeal abscess
z
Special exam
z Palin
film: air in cyst
z Flexible nasopharyngoscope
z Pharyngoesophagogram with barium study
z may
be performed at least 6 week later than acute
infection resolution
z CT
scan, FNA
z Thyroid scan
z Reflux of pus under laryngoscope
z
Hae-EL G, 1991:
z Suggest
an immediate search for an internal
pharyngeal sinus after a child's first episode of
acute thyroiditis
z
Treatment
z External
approach
z Excision
the tract with cannaulation under
endoscopy assisted
z ligating and dividing the tract
z Ipilateral hemithyroidectomy with partial ressection
of thyroid cartilage for 4th pouch anomaly
z Internal
approach
z Endoscopic
electrocauterization, 2004, D J Verret
z Endoscopic chemical cauterization, 2008, K D
Pepeira
z
Chemical cauterization by AgNO3
z
Complications
z Permanent
recurrent laryngeal nerve
paralysis
z Post operative esophagocutaneous fistula
z Hypoglossal palsy
z
z
z
z
z
Cystic malformations of the neck in children Pediatr Radiol (2005)
35: 463–477
Congenital Cervical Cysts, Sinuses and Fistulae Otolaryngol Clin N
Am 40 (2007) 161–176
Endoscopic chemical cautery of piriform sinus tracts: A safe new
technique Kevin D. Pereira International Journal of Pediatric
Otorhinolaryngology (2008) 72, 185—188
Management of anomalies of the third and fourth branchial pouches
Kevin D. Pereiraa,*International Journal of Pediatric
Otorhinolaryngology (2004) 68, 43—50
Head and Neck Anomalies Related to the Branchial Apparatus
David L. Mandell MD Otolaryngology clin N am 33(6)