Congenital Disorder. Although present at birth masses may not become clinically apparent until...
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Transcript of Congenital Disorder. Although present at birth masses may not become clinically apparent until...
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Congenital DisorderCongenital Disorder
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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
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Congenital neck massCongenital neck mass
Branchial systemBranchial system
Thyroid gland Thyroid gland
DermoidDermoid
Teratoid Teratoid
vascularvascular
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Important criteria Important criteria
Age of presentation Age of presentation
Location of the massLocation of the mass
Associated symptomAssociated symptom
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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
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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
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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
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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
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Thyroid arrest ( ectopic thyroid ) Thyroid arrest ( ectopic thyroid )
Lingual thyroid Lingual thyroid
As far as superior mediastinum As far as superior mediastinum
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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
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connection with the pharynx :connection with the pharynx :
polymicrobial infectionpolymicrobial infection
oral pathogen oral pathogen
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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
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Treatment Treatment
preop. Antibiotic for infected cyst preop. Antibiotic for infected cyst
Sistrunk excisionSistrunk excision
Rarely papillary adenoarcinoma Rarely papillary adenoarcinoma
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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
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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
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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
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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
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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
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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
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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
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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
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Fourth archFourth arch
Thyroid cartilage Thyroid cartilage
Cricothyroid muscle Cricothyroid muscle
vagus nerve vagus nerve
arch of the aorta arch of the aorta
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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
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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
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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
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A cyst is a collection of fluid in A cyst is a collection of fluid in an epithelium – lined sacan epithelium – lined sac
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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
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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
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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
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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
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Drainage may occur from EAC by Drainage may occur from EAC by palpation of angle or preauricular palpation of angle or preauricular
massmass
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Preauricular sinus Preauricular sinus
Malformation of six hillocks that form Malformation of six hillocks that form the auricle . the auricle .
Excision Excision
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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
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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 ..
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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
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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 .
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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
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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
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Lymphangioma simplexLymphangioma simplex
Thin-walled lymphatic channel Thin-walled lymphatic channel
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Cavernous lymphangiomaCavernous lymphangioma
Large lymphatic channel Large lymphatic channel
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Cystic hygromaCystic hygroma
Large lymphatic dilatationLarge lymphatic dilatation
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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
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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
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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
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Type of hemangiomasType of hemangiomas
Capillary Capillary
Cavernous Cavernous
Juvenile Juvenile
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Soft Soft Compressible Compressible Nonpulsatile bluish massNonpulsatile bluish massInvolvement :Involvement :
parotid parotid NeckNeck Tongue Tongue SkinSkin
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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%
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Lesions that not involve critical Lesions that not involve critical structure are usually observed structure are usually observed
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ImagingImaging
CT scanning outline the lesionCT scanning outline the lesion
Angiography :Angiography :
diagnosisdiagnosis
embolization embolization
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Symptomatic lesion : Symptomatic lesion :
corticosteroidcorticosteroid interferon-interferon-αα2a2a
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Surgical excision Surgical excision
Critical area Critical area
Spontaneous bleeding Spontaneous bleeding
Recurrent infection Recurrent infection
consumption coagulopathyconsumption coagulopathy
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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
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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