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