Transcript Congenital Disorder

Congenital Disorder
Although present at birth masses may not
become clinically apparent until childhood or
even adulthood
Congenital neck mass
Branchial system
Thyroid gland
Dermoid
Teratoid
vascular
Important criteria
Age of presentation
Location of the mass
Associated symptom
Thyroglossal duct cyst
The most common congenital neck
mass
M=F
Majority before age 12
Thyroid gland descent begin in the
third week & complete by the eight
week
As it descent it is intimately
associated with the hyoid bone which
is in the process of fusing in the
midline
It is the failure of thyroglossal
duct to involute that causes
thyroglossal duct cysts
The majority of the cysts present at
or below the level of the hyoid bone
in the midline of the neck
Thyroid arrest ( ectopic thyroid )
Lingual thyroid
As far as superior mediastinum
Thyroglossal duct cyst
( physical examination )
Smooth , nontender
Rise with swallowing
Cyst infection :
acute ↑ in size
skin erythema
tenderness
spontaneous drainage
connection with the pharynx :
polymicrobial infection
oral pathogen
Determination of the location of
normal thyroid tissue is essential
prior to the excision of any suspected
cyst or ectopic thyroid .
US is the preferred mode of imaging
In uncooperate child or dense cyst
thyroid scan should be considered
Treatment
preop. Antibiotic for infected cyst
Sistrunk excision
Rarely papillary adenoarcinoma
Reccurence
10% in Sistrunk
Failure of hyoid removal
Failure of remove section of tongue
Rupture of the cyst
Resurgery
Teratoma & dermoid are true
developmental neoplasm
Arises from pluripotent cells
at anatomic sites where they
are not normally found
Dermoid cyst
Consist ectoderm & mesoderm
Lined by epidermis and contain hair
follicle & sebsceous glands
Smooth nontender mass in
submental region
Surgical removal
teratoma
Three germ layers
Disorganized teratoid cyst → true
teratoma ( epignathi )
Cervical region
Firm & mobile
Cystic and solid composition
Surgical removal
Branchial arch anomaly
Present at birth , clinically apparent
at childhood
Develop during third to 7th embryonic
week
Six pairs arches,four paired groove
externally,four paired pouch
internally
First arch
Meckel‘s cartilage
Maxilla, malleus , incus , mandible
Sphenomandibular ligament
Mylohyoid , ant. Belly of digastric,
tensor tympani , TVP , masseter ,
temporalis , medial & lateral
pterygoids
Trigeminal nerve
Maxillary artery
Second arch
Reichert's cartilage
Upper body of hyoid bone lesser
cornu , stylohyoid ligament , styloid
process , stapes
Muscle of facial expression ,
platysma , stylohyoid, post. Belly of
digastric , stapedius muscle
Facial nerve
Stapedial artery
Third arch
Greater cornu & lesser portion of
hyoid bone
Stylopharyngeous , super and middle
constrictor of the pharynx
Glossopharyngeal nerve
Part of the internal carotid artery
Fourth arch
Thyroid cartilage
Cricothyroid muscle
vagus nerve
arch of the aorta
Sixth arch
Cricoid and arytenoid cartilage
Corniform & corniculate cartilage
RLN
Inferior constrictor muscle
Ductus arteriosus
Pharyngeal pouch
First pouch : form ET & middle ear
cleft
Second pouch : palatine tonsil
Third pouch : inf parathyroid gland &
thymic duct
Fourth pouch : sup parathyroid gland
Sixth pouch : ultimobranchial body
Pharyngeal groove
First groove : external auditory
meatus
The remaining grooves are
obliterated by the caudal overgrowth
of the second branchial arch
A cyst is a collection of fluid in
an epithelium – lined sac
Groove cyst : line with squamous
epithelium
Pouch cyst : line with respiratory
epithelium
Sinus tract : from epithelial surface
to the deeper tissue
Fistula : a tract between skin to the
pharynx or larynx internally
Branchial cleft anomaly
At birth or shortly there after
Small opening along the anterior
border of SCM
Mocoid discharge with URI
A complete fistula is uncommon with
most ending before the pharynx is
reached
Cyst is more common than fistula or
sinuses
Cont.
Majority arises from second branchial
cleft
Usual course is recurrent infection
Early surgical excision
Recurrent cases : preopertive
fistulogram
First branchial cleft anomaly
Uncommon
First category :
absent external auditory canal
Second category :
Type I : Duplication of membranous EAC
Type II : Duplication of membranous & bony
EAC
At angle of mandible
Fistulous tract at bony & membranous junction
Drainage may occur from EAC by
palpation of angle or preauricular
mass
Preauricular sinus
Malformation of six hillocks that form
the auricle .
Excision
Second branchial cleft anomalies
Most common anomaly
Opening : lower half of the neck
Anterior border of SCM
Internal opening : If present is in the
tonsillar fossa
Surgical removal
First branchial sinuses open anterior
to the SCM , above the hyoid bone
Second branchial sinuses open
anterior to the SCM below the
thyroid .
The tract runs between the
internal & external carotid
arteries and passes lateral to the
IX & XII and continues inferior to
the posterior belly of the digastric
, opening into the tonsillar fossa
If the fistula opens into the tonsillar
fossa , the tonsil should be removed
to obtain complete exposure .
Third branchial cleft cyst
Very rare Courses posterior and
lateral to the ICA and CN XII ,
terminate its course at the level of
the piriform sinus
Lymphangiomas
Abnormal lymphatic development
along the jugular lymphatic sac
50% present by 1year
90% preent by age 2
Occurs in the sixth week of
emberyonic development .
Thin-walled endothelial-lined cysts
infiltrating into the surrounding
tissue
Lymphangioma simplex
Thin-walled lymphatic channel
Cavernous lymphangioma
Large lymphatic channel
Cystic hygroma
Large lymphatic dilatation
The lesion present most often in
the posterior cervical triangle of
the neck and are soft , nontender
poorly defined mass that
transilluminate
Surgical excision is the treatment of
choice .
The timing is dependent on the surgeon
philosophy .
Early excision for possibility of infection ,
rapid growth , and potential airway
compromise .
Waiting until 3-4 years of age because of
involution and technical ease of operation
Hemangiomas
The most common tumor of infancy
Majority was recognized by the age
of 6 months of age .
Defective embryonic development of
the peripheral vessel .
Arrested development at the
endothelial stage gives rise to these
subcutaneous vascular mass
Type of hemangiomas
Capillary
Cavernous
Juvenile
Soft
Compressible
Nonpulsatile bluish mass
Involvement :
parotid
Neck
Tongue
Skin
Rapid growth in neonatal period
Involution at 5 years : 50%
Involution at 7years : 70%
Lesions that not involve critical
structure are usually observed
Imaging
CT scanning outline the lesion
Angiography :
diagnosis
embolization
Symptomatic lesion :
corticosteroid
interferon-α2a
Surgical excision
Critical area
Spontaneous bleeding
Recurrent infection
consumption coagulopathy
Fibromatosis colli
Congenital tumor of SCM
Often detected 2-3 weeks after birth
Firm nontender
Involved with the underlying muscle
Torticollis
Difficult vaginal delivery and traction
on the H&N
Usually resolved by 18 months
US is diagnostic
Physical therapy prevent long term
difficulty
Permanent lesion : surgery