Congenital DisorderCongenital Disorder

Although present at birth masses may not Although present at birth masses may not become clinically apparent until childhood or become clinically apparent until childhood or

even adulthood even adulthood

Congenital neck massCongenital neck mass

Branchial systemBranchial system

Thyroid gland Thyroid gland

DermoidDermoid

Teratoid Teratoid

vascularvascular

Important criteria Important criteria

Age of presentation Age of presentation

Location of the massLocation of the mass

Associated symptomAssociated symptom

Thyroglossal duct cyst Thyroglossal duct cyst

The most common congenital neck The most common congenital neck massmass

M=FM=F

Majority before age 12Majority before age 12

Thyroid gland descent begin in the Thyroid gland descent begin in the third week & complete by the eight third week & complete by the eight week week

As it descent it is intimately As it descent it is intimately associated with the hyoid bone which associated with the hyoid bone which is in the process of fusing in the is in the process of fusing in the midline midline

It is the failure of thyroglossal It is the failure of thyroglossal duct to involute that causes duct to involute that causes

thyroglossal duct cysts thyroglossal duct cysts

The majority of the cysts present at The majority of the cysts present at or below the level of the hyoid bone or below the level of the hyoid bone in the midline of the neck in the midline of the neck

Thyroid arrest ( ectopic thyroid ) Thyroid arrest ( ectopic thyroid )

Lingual thyroid Lingual thyroid

As far as superior mediastinum As far as superior mediastinum

Thyroglossal duct cyst Thyroglossal duct cyst ( physical examination ) ( physical examination )

Smooth , nontender Smooth , nontender

Rise with swallowing Rise with swallowing

Cyst infection : Cyst infection :

acute acute ↑ in size ↑ in size

skin erythema skin erythema

tenderness tenderness

spontaneous drainage spontaneous drainage

connection with the pharynx :connection with the pharynx :

polymicrobial infectionpolymicrobial infection

oral pathogen oral pathogen

Determination of the location of Determination of the location of normal thyroid tissue is essential normal thyroid tissue is essential prior to the excision of any suspected prior to the excision of any suspected cyst or ectopic thyroid .cyst or ectopic thyroid .

US is the preferred mode of imaging US is the preferred mode of imaging

In uncooperate child or dense cyst In uncooperate child or dense cyst thyroid scan should be considered thyroid scan should be considered

Treatment Treatment

preop. Antibiotic for infected cyst preop. Antibiotic for infected cyst

Sistrunk excisionSistrunk excision

Rarely papillary adenoarcinoma Rarely papillary adenoarcinoma

ReccurenceReccurence

10% in Sistrunk 10% in Sistrunk

Failure of hyoid removal Failure of hyoid removal

Failure of remove section of tongue Failure of remove section of tongue

Rupture of the cyst Rupture of the cyst

Resurgery Resurgery

Teratoma & dermoid are true Teratoma & dermoid are true developmental neoplasm developmental neoplasm

Arises from pluripotent cells Arises from pluripotent cells at anatomic sites where they at anatomic sites where they

are not normally found are not normally found

Dermoid cyst Dermoid cyst

Consist ectoderm & mesoderm Consist ectoderm & mesoderm

Lined by epidermis and contain hair Lined by epidermis and contain hair follicle & sebsceous glands follicle & sebsceous glands

Smooth nontender mass in Smooth nontender mass in submental regionsubmental region

Surgical removalSurgical removal

teratomateratoma

Three germ layers Three germ layers

Disorganized teratoid cyst Disorganized teratoid cyst → true → true teratoma ( epignathi ) teratoma ( epignathi )

Cervical region Cervical region

Firm & mobile Firm & mobile

Cystic and solid compositionCystic and solid composition

Surgical removalSurgical removal

Branchial arch anomalyBranchial arch anomaly

Present at birth , clinically apparent Present at birth , clinically apparent at childhoodat childhood

Develop during third to 7Develop during third to 7thth embryonic embryonic week week

Six pairs arches,four paired groove Six pairs arches,four paired groove externally,four paired pouch externally,four paired pouch internally internally

First archFirst archMeckel‘s cartilage Meckel‘s cartilage Maxilla, malleus , incus , mandible Maxilla, malleus , incus , mandible Sphenomandibular ligamentSphenomandibular ligamentMylohyoid , ant. Belly of digastric, Mylohyoid , ant. Belly of digastric, tensor tympani , TVP , masseter , tensor tympani , TVP , masseter , temporalis , medial & lateral temporalis , medial & lateral pterygoids pterygoids Trigeminal nerve Trigeminal nerve Maxillary artery Maxillary artery

Second arch Second arch Reichert's cartilage Reichert's cartilage Upper body of hyoid bone lesser Upper body of hyoid bone lesser cornu , stylohyoid ligament , styloid cornu , stylohyoid ligament , styloid process , stapes process , stapes Muscle of facial expression , Muscle of facial expression , platysma , stylohyoid, post. Belly of platysma , stylohyoid, post. Belly of digastric , stapedius muscledigastric , stapedius muscleFacial nerve Facial nerve Stapedial arteryStapedial artery

Third archThird arch

Greater cornu & lesser portion of Greater cornu & lesser portion of hyoid bone hyoid bone

Stylopharyngeous , super and middle Stylopharyngeous , super and middle constrictor of the pharynxconstrictor of the pharynx

Glossopharyngeal nerve Glossopharyngeal nerve

Part of the internal carotid artery Part of the internal carotid artery

Fourth archFourth arch

Thyroid cartilage Thyroid cartilage

Cricothyroid muscle Cricothyroid muscle

vagus nerve vagus nerve

arch of the aorta arch of the aorta

Sixth arch Sixth arch

Cricoid and arytenoid cartilage Cricoid and arytenoid cartilage

Corniform & corniculate cartilage Corniform & corniculate cartilage

RLN RLN

Inferior constrictor muscle Inferior constrictor muscle

Ductus arteriosus Ductus arteriosus

Pharyngeal pouch Pharyngeal pouch

First pouch : form ET & middle ear First pouch : form ET & middle ear cleft cleft

Second pouch : palatine tonsil Second pouch : palatine tonsil

Third pouch : inf parathyroid gland & Third pouch : inf parathyroid gland & thymic ductthymic duct

Fourth pouch : sup parathyroid gland Fourth pouch : sup parathyroid gland

Sixth pouch : ultimobranchial body Sixth pouch : ultimobranchial body

Pharyngeal groove Pharyngeal groove

First groove : external auditory First groove : external auditory meatusmeatus

The remaining grooves are The remaining grooves are obliterated by the caudal overgrowth obliterated by the caudal overgrowth of the second branchial arch of the second branchial arch

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

Groove cyst : line with squamous Groove cyst : line with squamous epithelium epithelium

Pouch cyst : line with respiratory Pouch cyst : line with respiratory epitheliumepithelium

Sinus tract : from epithelial surface Sinus tract : from epithelial surface to the deeper tissue to the deeper tissue

Fistula : a tract between skin to the Fistula : a tract between skin to the pharynx or larynx internallypharynx or larynx internally

Branchial cleft anomaly Branchial cleft anomaly At birth or shortly there after At birth or shortly there after Small opening along the anterior Small opening along the anterior border of SCM border of SCM Mocoid discharge with URI Mocoid discharge with URI A complete fistula is uncommon with A complete fistula is uncommon with most ending before the pharynx is most ending before the pharynx is reached reached Cyst is more common than fistula or Cyst is more common than fistula or sinusessinuses

Cont.Cont.

Majority arises from second branchial Majority arises from second branchial cleft cleft

Usual course is recurrent infection Usual course is recurrent infection

Early surgical excision Early surgical excision

Recurrent cases : preopertive Recurrent cases : preopertive fistulogram fistulogram

First branchial cleft anomalyFirst branchial cleft anomalyUncommon Uncommon First category : First category :

absent external auditory canal absent external auditory canal Second category :Second category :

Type I : Duplication of membranous EAC Type I : Duplication of membranous EAC Type II : Duplication of membranous & bony EACType II : Duplication of membranous & bony EAC

At angle of mandible At angle of mandible Fistulous tract at bony & membranous junctionFistulous tract at bony & membranous junction

Drainage may occur from EAC by Drainage may occur from EAC by palpation of angle or preauricular palpation of angle or preauricular

massmass

Preauricular sinus Preauricular sinus

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

Excision Excision

Second branchial cleft anomalies Second branchial cleft anomalies

Most common anomaly Most common anomaly

Opening : lower half of the neck Opening : lower half of the neck

Anterior border of SCMAnterior border of SCM

Internal opening : If present is in the Internal opening : If present is in the tonsillar fossa tonsillar fossa

Surgical removal Surgical removal

First branchial sinuses open anterior First branchial sinuses open anterior to the SCM , above the hyoid bone to the SCM , above the hyoid bone

Second branchial sinuses open Second branchial sinuses open anterior to the SCM below the thyroid anterior to the SCM below the thyroid ..

The tract runs between the The tract runs between the internal & external carotid arteries internal & external carotid arteries and passes lateral to the IX & XII and passes lateral to the IX & XII

and continues inferior to the and continues inferior to the posterior belly of the digastric , posterior belly of the digastric , opening into the tonsillar fossa opening into the tonsillar fossa

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

Third branchial cleft cyst Third branchial cleft cyst

Very rare Courses posterior and Very rare Courses posterior and lateral to the ICA and CN XII , lateral to the ICA and CN XII , terminate its course at the level of terminate its course at the level of the piriform sinus the piriform sinus

LymphangiomasLymphangiomasAbnormal lymphatic development Abnormal lymphatic development along the jugular lymphatic sac along the jugular lymphatic sac 50% present by 1year 50% present by 1year 90% preent by age 290% preent by age 2Occurs in the sixth week of Occurs in the sixth week of emberyonic development . emberyonic development . Thin-walled endothelial-lined cysts Thin-walled endothelial-lined cysts infiltrating into the surrounding infiltrating into the surrounding tissue tissue

Lymphangioma simplexLymphangioma simplex

Thin-walled lymphatic channel Thin-walled lymphatic channel

Cavernous lymphangiomaCavernous lymphangioma

Large lymphatic channel Large lymphatic channel

Cystic hygromaCystic hygroma

Large lymphatic dilatationLarge lymphatic dilatation

The lesion present most often in The lesion present most often in the posterior cervical triangle of the posterior cervical triangle of

the neck and are soft , nontender the neck and are soft , nontender poorly defined mass that poorly defined mass that

transilluminatetransilluminate

Surgical excision is the treatment of Surgical excision is the treatment of choice .choice .

The timing is dependent on the surgeon The timing is dependent on the surgeon philosophy . philosophy .

Early excision for possibility of infection , Early excision for possibility of infection , rapid growth , and potential airway rapid growth , and potential airway compromise .compromise .

Waiting until 3-4 years of age because of Waiting until 3-4 years of age because of involution and technical ease of operation involution and technical ease of operation

HemangiomasHemangiomas

The most common tumor of infancy The most common tumor of infancy

Majority was recognized by the age Majority was recognized by the age of 6 months of age .of 6 months of age .

Defective embryonic development of Defective embryonic development of the peripheral vessel . the peripheral vessel .

Arrested development at the Arrested development at the endothelial stage gives rise to these endothelial stage gives rise to these subcutaneous vascular mass subcutaneous vascular mass

Type of hemangiomasType of hemangiomas

Capillary Capillary

Cavernous Cavernous

Juvenile Juvenile

Soft Soft Compressible Compressible Nonpulsatile bluish massNonpulsatile bluish massInvolvement :Involvement :

parotid parotid NeckNeck Tongue Tongue SkinSkin

Rapid growth in neonatal period Rapid growth in neonatal period

Involution at 5 years : 50%Involution at 5 years : 50%

Involution at 7years : 70%Involution at 7years : 70%

Lesions that not involve critical Lesions that not involve critical structure are usually observed structure are usually observed

ImagingImaging

CT scanning outline the lesionCT scanning outline the lesion

Angiography :Angiography :

diagnosisdiagnosis

embolization embolization

Symptomatic lesion : Symptomatic lesion :

corticosteroidcorticosteroid interferon-interferon-αα2a2a

Surgical excision Surgical excision

Critical area Critical area

Spontaneous bleeding Spontaneous bleeding

Recurrent infection Recurrent infection

consumption coagulopathyconsumption coagulopathy

Fibromatosis colliFibromatosis colli

Congenital tumor of SCM Congenital tumor of SCM

Often detected 2-3 weeks after birthOften detected 2-3 weeks after birth

Firm nontender Firm nontender

Involved with the underlying muscle Involved with the underlying muscle

Torticollis Torticollis

Difficult vaginal delivery and traction Difficult vaginal delivery and traction on the H&Non the H&N

Usually resolved by 18 months Usually resolved by 18 months

US is diagnostic US is diagnostic

Physical therapy prevent long term Physical therapy prevent long term difficulty difficulty

Permanent lesion : surgeryPermanent lesion : surgery