Congenital Neck Mass

CONGENITAL NECK CONGENITAL NECK MASSMASS

Richel L. Cavas, MDRichel L. Cavas, MD

July 2011July 2011

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYST

►CLASSIFICATIONCLASSIFICATION

a. TYPE I ANOMALYa. TYPE I ANOMALY

- ectodermal in origin - ectodermal in origin

- duplication of the - duplication of the membranous membranous EACEAC

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYST►CLASSIFICATIONCLASSIFICATION

b. TYPE II ANOMALYb. TYPE II ANOMALY

- mesodermal and - mesodermal and ectodermal in ectodermal in originorigin

- duplication of the - duplication of the membranous membranous EAC EAC and pinna that contains skin and pinna that contains skin and and cartilagecartilage


FIRST BRANCHIAL CLEFT FIRST BRANCHIAL CLEFT CYST: 8%CYST: 8%

A.A. TYPE I TYPE I

- pre-auricular region - pre-auricular region (anterior or posterior (anterior or posterior to the pinna)to the pinna)

- parallel to the EAC - parallel to the EAC and lateral to CN VIIand lateral to CN VII

- In the pre-auricular - In the pre-auricular area: embedded in the area: embedded in the parotid gland.parotid gland.


FIRST BRANCHIAL FIRST BRANCHIAL CLEFT CYSTCLEFT CYST

A.A. TYPE II TYPE II - posterior or - posterior or

inferior to the angle inferior to the angle of the mandibleof the mandible- related to the - related to the parotid gland and parotid gland and may lie lateral, may lie lateral, medial or b/w medial or b/w branches of CN VII.branches of CN VII.

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYSTSECOND BRANCHIAL CLEFT SECOND BRANCHIAL CLEFT

CYSTCYST- majority of branchial - majority of branchial anomaliesanomalies- cyst or external - cyst or external opening found along opening found along the anterior border of the anterior border of the SCMthe SCM- epithelial tracts pass - epithelial tracts pass superiorly, lateral to the superiorly, lateral to the carotid arterial system carotid arterial system and CN IX & CN XIIand CN IX & CN XII- end close to the - end close to the middle constrictor ms middle constrictor ms or tonsillar fossaor tonsillar fossa


THIRD BRANCHIAL THIRD BRANCHIAL CLEFT CYSTCLEFT CYST

- tract ascends - tract ascends lateral to the CCA, lateral to the CCA, passes posterior to passes posterior to the ICA, superior to the ICA, superior to CN XII and inferior to CN XII and inferior to CN IX and medially CN IX and medially piercing the lateral piercing the lateral aspect of the thyro-aspect of the thyro-hyoid membrane into hyoid membrane into the pyriform sinus.the pyriform sinus.

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYSTFOURTH BRANCHIAL CLEFT CYSTFOURTH BRANCHIAL CLEFT CYST

- LEFT: begin at the apex of the pyriform - LEFT: begin at the apex of the pyriform sinus. sinus. - Descend to exit the pharynx caudal to the - Descend to exit the pharynx caudal to the superior laryngeal nerve, cricothyroid muscle superior laryngeal nerve, cricothyroid muscle and thyroid cartilageand thyroid cartilage- courses inferiorly, lateral to the trachea and - courses inferiorly, lateral to the trachea and recurrent laryngeal nerverecurrent laryngeal nerve- descends in to the thorax and loops - descends in to the thorax and loops posterior around the aortic arch before posterior around the aortic arch before ascending into the neck posterior to the CCAascending into the neck posterior to the CCA- crosses CN XII before descending to open to - crosses CN XII before descending to open to the skin at anterior aspect in the inferior the skin at anterior aspect in the inferior aspect of SCMaspect of SCM


HISTOPATHOLOGYHISTOPATHOLOGY

- stratified squamous epithelium- stratified squamous epithelium

- keratin, hair follicles, sweat & - keratin, hair follicles, sweat & sebaceous glands and cartilagesebaceous glands and cartilage

- presence of lymphoid aggregates.- presence of lymphoid aggregates.

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYSTCLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS► Non-tender, fluctuant massesNon-tender, fluctuant masses► Bilateral (2-3%)Bilateral (2-3%)► Inflammation, tender and develop abscessInflammation, tender and develop abscess► Depending on the location: dysphagia, Depending on the location: dysphagia,

dyspnea & stridordyspnea & stridor► 11stst branchial cyst: parotid masses; if branchial cyst: parotid masses; if

associated with the EAC: otorrhea and associated with the EAC: otorrhea and otalgia.otalgia.

► 33rdrd & 4 & 4thth: associated w/ recurrent acute : associated w/ recurrent acute suppurative thyroiditis; left paratracheal suppurative thyroiditis; left paratracheal fullness and tenderness with fever.fullness and tenderness with fever.

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYSTMANAGEMENTMANAGEMENT- Surgical excisionSurgical excision- Abscess: I & DAbscess: I & D

A. 1A. 1stst: dissected free to their termination : dissected free to their termination close to or at the EAC. close to or at the EAC.

- Superficial parotidectomy and CN VII Superficial parotidectomy and CN VII - EAC: portion of the canal skin & cartilage EAC: portion of the canal skin & cartilage

should be excisedshould be excised

BRANCHIAL CLEFT CYSTBRANCHIAL CLEFT CYSTMANAGEMENTMANAGEMENTA. 1A. 1stst: dissected free to their termination : dissected free to their termination

close to or at the EAC. close to or at the EAC. - Superficial parotidectomy and CN VII Superficial parotidectomy and CN VII - EAC: portion of the canal skin & cartilage EAC: portion of the canal skin & cartilage

should be excisedshould be excised

B. 3B. 3rdrd or 4 or 4thth: suppurative thyroiditis; : suppurative thyroiditis; hemithyroidectomy and closure of the hemithyroidectomy and closure of the internal pyriform sinus tract opening.internal pyriform sinus tract opening.

LYMPHANGIOMA (CYSTIC LYMPHANGIOMA (CYSTIC HYGROMA)HYGROMA)

► congenital malformations of lymphatic congenital malformations of lymphatic channelschannels

► presents in the posterior triangle presents in the posterior triangle (80%)(80%)

► 65% are present at birth65% are present at birth► 80 – 90% - 280 – 90% - 2ndnd year of life year of life


HISTOPATHOLOGYHISTOPATHOLOGY► Lymphangioma simplex: capillary Lymphangioma simplex: capillary

sized, thin walled lymphaticssized, thin walled lymphatics► Cavernous lymphangiomas: dilated Cavernous lymphangiomas: dilated

lymphatic spaces; infiltrative; lymphatic spaces; infiltrative; recurrencerecurrence

► Cystic hygroma: containing lymphatic Cystic hygroma: containing lymphatic cysts cysts


CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS Painless, soft, single or multiloculated Painless, soft, single or multiloculated

masses masses occupy one side of the neck and extend occupy one side of the neck and extend

to the skull base and thoracic cavityto the skull base and thoracic cavity compression of adjacent structures: compression of adjacent structures:

dysphagia, dyspnea or stridordysphagia, dyspnea or stridor transilluminatetransilluminate


MANAGEMENTMANAGEMENT► Surgical excisionSurgical excision► surgery must be postponed until 3 or surgery must be postponed until 3 or

4 years old4 years old► recurring infection, symptoms of recurring infection, symptoms of

compressioncompression

HEMANGIOMASHEMANGIOMAS

► developmental vascular abnormalitiesdevelopmental vascular abnormalities► 96% evident by 6 mos96% evident by 6 mos► disappears by 7 years olddisappears by 7 years old


HISTOPATHOLOGYHISTOPATHOLOGY► CapillaryCapillary► CavernousCavernous► MixedMixed► Juvenile (proliferative)Juvenile (proliferative)


CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS► diffuse skin lesionsdiffuse skin lesions► soft cystic masses in the oral cavity, soft cystic masses in the oral cavity,

pharynx , parotid gland and neckpharynx , parotid gland and neck► CompressibilityCompressibility► bluish discoloration of the skinbluish discoloration of the skin► BruitsBruits► change in size during crying or change in size during crying or

strainingstraining


MANAGEMENTMANAGEMENT► ConservativeConservative► Infants: phase of rapid growth Infants: phase of rapid growth

followed by slow resolution to the next followed by slow resolution to the next 3 or 4 years3 or 4 years

► Surgery is for causing functional or Surgery is for causing functional or cosmetic impairment.cosmetic impairment.

TERATOMAS AND DERMOID TERATOMAS AND DERMOID CYSTSCYSTS

► occur in 1:4,000 birthsoccur in 1:4,000 births► < 10% in the head and neck< 10% in the head and neck► most commonly involved are orbital most commonly involved are orbital

region, nose, nasopharynx, oral cavity region, nose, nasopharynx, oral cavity and neckand neck


► DERMOID CYSTSDERMOID CYSTS

- most common form- most common form

- composed of ectoderm & mesoderm- composed of ectoderm & mesoderm

- covered with skin and epidermal - covered with skin and epidermal appendages appendages


► TERATOID CYSTSTERATOID CYSTS

- composed of ectoderm, mesoderm & - composed of ectoderm, mesoderm & endodermendoderm

- simple stratified squamous - simple stratified squamous epithelium to ciliated respiratory epithelium to ciliated respiratory epithelium.epithelium.

- poorly differentiated- poorly differentiated


► TERATOMASTERATOMAS

- - composed of 3 germ layerscomposed of 3 germ layers

- well differentiated- well differentiated


► EPIGNATHIEPIGNATHI

- development of fetal organs and - development of fetal organs and limbs may occur.limbs may occur.

- not compatible with life- not compatible with life


► DERMOID CYSTSDERMOID CYSTS

- midline; occur in the submental - midline; occur in the submental region above the hyoid boneregion above the hyoid bone

- painless mass- painless mass

- Tx: surgical excision- Tx: surgical excision

LARYNGOCELESLARYNGOCELES

► abnormal dilatations of the laryngeal abnormal dilatations of the laryngeal ventricle and saccule.ventricle and saccule.

► most common in the 5most common in the 5thth and 6 and 6thth decade of life. decade of life.

► males > femalesmales > females


► saccule arises vertically off the saccule arises vertically off the anterior end of the ventricle and the anterior end of the ventricle and the pouch passes superiorly b/w the false pouch passes superiorly b/w the false vocal cord, base of the epiglottis and vocal cord, base of the epiglottis and inner surface of thyroid cartilage.inner surface of thyroid cartilage.


a.a. Internal laryngocele: confined to the Internal laryngocele: confined to the interior of the larynx and tends interior of the larynx and tends posterosuperiorly in the false vocal posterosuperiorly in the false vocal cord and the aryepiglottic foldcord and the aryepiglottic fold

b.b. External laryngocele: extends External laryngocele: extends superiorly to appear laterally in the superiorly to appear laterally in the neck through the opening in the neck through the opening in the thyrohyoid membrane for the thyrohyoid membrane for the superior laryngeal nerve and vessels.superior laryngeal nerve and vessels.


► CLINICAL MANIFESTATIONCLINICAL MANIFESTATION

- masses in the neck- masses in the neck

- swelling occurs during increased - swelling occurs during increased intralaryngeal pressureintralaryngeal pressure

- gurgling sensation in the neck - gurgling sensation in the neck

- hoarseness, cough, dyspnea & - hoarseness, cough, dyspnea & dysphagiadysphagia

THYROGLOSSAL DUCT CYSTSTHYROGLOSSAL DUCT CYSTS

► Failure of complete obliteration of the Failure of complete obliteration of the thyroglossal ductthyroglossal duct

► S/Sx: midline neck mass with cystic S/Sx: midline neck mass with cystic and solid components that elevates and solid components that elevates with tongue protrusionwith tongue protrusion

►Treatment: Sistrunk ProcedureTreatment: Sistrunk Procedure

ENCEPHALOCELEENCEPHALOCELE

► congenital herniation of CNS tissue congenital herniation of CNS tissue through a skull base defectthrough a skull base defect

►May contain meninges (meningocele); May contain meninges (meningocele); meninges and brain meninges and brain (meningoencephalocele) or meninges, (meningoencephalocele) or meninges, brain and part of the ventricular brain and part of the ventricular system (mengingoencephalocystocele)system (mengingoencephalocystocele)


HISTOPATHOLOGYHISTOPATHOLOGY► meningeal sac containing glial tissuemeningeal sac containing glial tissue


► Anterior: 20- 25%Anterior: 20- 25%

a. sincipital – nasofrontal, a. sincipital – nasofrontal, nasoethmoidal & naso-orbitalnasoethmoidal & naso-orbital

b. basal – sphenomaxillary, spheno-b. basal – sphenomaxillary, spheno-orbital and sphenopharyngealorbital and sphenopharyngeal

► Posterior: 75 -80%Posterior: 75 -80%►


CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS► external encephaloceles present at or external encephaloceles present at or

near the glabella as soft compressible near the glabella as soft compressible masses.masses.

► intranasal lesions present as bluish, intranasal lesions present as bluish, pedunculated masses medially, and high pedunculated masses medially, and high in the nasal cavityin the nasal cavity

► compressible and pulsatile, may enlarge compressible and pulsatile, may enlarge with crying or internal jugular vein with crying or internal jugular vein compression (Positive Furstenberg sign)compression (Positive Furstenberg sign)


► surgical resection surgical resection

Congenital Neck Mass

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