Congenital Disorder

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Congenital Disorder
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Although present at birth masses may not become
clinically apparent until childhood or even
adulthood
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Congenital neck mass

• Branchial system
• Thyroid gland
• Dermoid
• Teratoid
• vascular

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Important criteria

• Age of presentation
• Location of the mass
• Associated symptom

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Thyroglossal duct cyst

• The most common congenital neck mass
• MF
• Majority before age 12

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• 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

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It is the failure of thyroglossal duct to
involute that causes thyroglossal duct cysts
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• The majority of the cysts present at or below the
  level of the hyoid bone in the midline of the
  neck

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Thyroid arrest ( ectopic thyroid )

• Lingual thyroid
• As far as superior mediastinum

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Thyroglossal duct cyst ( physical examination )

• Smooth , nontender
• Rise with swallowing
• Cyst infection
• acute ? in size
• skin erythema
• tenderness
• spontaneous
  drainage

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• connection with the pharynx
• polymicrobial infection
• oral pathogen

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• 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

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Treatment

• preop. Antibiotic for infected cyst
• Sistrunk excision
• Rarely papillary adenoarcinoma

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Reccurence

• 10 in Sistrunk
• Failure of hyoid removal
• Failure of remove section of tongue
• Rupture of the cyst
• Resurgery

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Teratoma dermoid are true developmental
neoplasm

• Arises from pluripotent cells at anatomic sites
  where they are not normally found

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Dermoid cyst

• Consist ectoderm mesoderm
• Lined by epidermis and contain hair follicle
  sebsceous glands
• Smooth nontender mass in submental region
• Surgical removal

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teratoma

• Three germ layers
• Disorganized teratoid cyst ? true teratoma (
  epignathi )
• Cervical region
• Firm mobile
• Cystic and solid composition
• Surgical removal

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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

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First arch

• Meckels cartilage
• Maxilla, malleus , incus , mandible
• Sphenomandibular ligament
• Mylohyoid , ant. Belly of digastric, tensor
  tympani , TVP , masseter , temporalis , medial
  lateral pterygoids
• Trigeminal nerve
• Maxillary artery

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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

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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

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Fourth arch

• Thyroid cartilage
• Cricothyroid muscle
• vagus nerve
• arch of the aorta

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Sixth arch

• Cricoid and arytenoid cartilage
• Corniform corniculate cartilage
• RLN
• Inferior constrictor muscle
• Ductus arteriosus

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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

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Pharyngeal groove

• First groove external auditory meatus
• The remaining grooves are obliterated by the
  caudal overgrowth of the second branchial arch

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A cyst is a collection of fluid in an epithelium
lined sac
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• 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

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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

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Cont.

• Majority arises from second branchial cleft
• Usual course is recurrent infection
• Early surgical excision
• Recurrent cases preopertive fistulogram

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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

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Drainage may occur from EAC by palpation of angle
or preauricular mass
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Preauricular sinus

• Malformation of six hillocks that form the
  auricle .
• Excision

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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

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• First branchial sinuses open anterior to the SCM
  , above the hyoid bone
• Second branchial sinuses open anterior to the SCM
  below the thyroid .

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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
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• If the fistula opens into the tonsillar fossa ,
  the tonsil should be removed to obtain complete
  exposure .

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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

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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

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Lymphangioma simplex

• Thin-walled lymphatic channel

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Cavernous lymphangioma

• Large lymphatic channel

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Cystic hygroma

• Large lymphatic dilatation

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The lesion present most often in the posterior
cervical triangle of the neck and are soft ,
nontender poorly defined mass that
transilluminate
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• 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

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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

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Type of hemangiomas

• Capillary
• Cavernous
• Juvenile

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• Soft
• Compressible
• Nonpulsatile bluish mass
• Involvement
• parotid
• Neck
• Tongue
• Skin

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• Rapid growth in neonatal period
• Involution at 5 years 50
• Involution at 7years 70

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Lesions that not involve critical structure are
usually observed
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Imaging

• CT scanning outline the lesion
• Angiography
• diagnosis
• embolization

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• Symptomatic lesion
• corticosteroid
• interferon-a2a

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Surgical excision

• Critical area
• Spontaneous bleeding
• Recurrent infection
• consumption coagulopathy

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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
  HN

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• Usually resolved by 18 months
• US is diagnostic
• Physical therapy prevent long term difficulty
• Permanent lesion surgery