Branchial Cleft Cyst Anomalies 4_2

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    Branchial Cleft Cyst Anomalies: A

    Pictorial Review of Their Embryologic,

    Anatomical and Radiologic

    Appearance Along with Treatment

    Options

    Alex Chau, MD

    Vinh Nguyen, MD

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    Disclosure

    I do not now have and have not within the

    past 12 months had a financial interest or

    other relationship with a commercial

    organization that may have an interest in

    the content of this educational activity.

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    Branchial Cleft Cyst

    Introduction

    Embryology

    Anatomy Cases Branchial Cleft Cysts

    Summary

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    Introduction Branchial Cleft Cyst:

    Benign congenital epithelial cyst

    20% of cervical mass children1

    Cause:

    Incomplete involution of branchial cleft2

    Buried epithelial cell rests

    Location:

    Lateral neck

    Morbidity:Super infection

    Mass effect

    Population:

    Older children and young adults

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    Embryology: Branchial Apparatus

    Pouch

    Arch Cleft

    I

    II

    III

    IV

    I

    II

    III

    IV

    Endo Meso Ecto

    Pharyngeal apparatus at 4th week

    Branchial Apparatus:

    Arch mesoderm

    Pouch endoderm

    Cleft ectoderm

    Eye

    Olfactory PitMaxillary processMandibular archHyoid archThird arch

    Magnus Manske. (March 24, 2007). Retrieved 4/15/2011 from http://en.wikipedia.org/wiki/File:Gray41.png

    V, VI

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    Pharyngeal Apparatus Development

    Proliferation of 2nd arch caudally

    7th week

    Clefts involutes

    Obliteration of sinus of His

    Failure of involution

    Cyst

    Sinus Fistula

    Pouch

    Arch CleftI

    II

    III

    IV

    I

    II

    III

    IV

    Sinus of His

    I

    II

    III

    IV

    Pouch CleftArch

    I

    II

    III

    IV

    Cyst

    Pouch CleftArch

    Sinus

    Fistula

    V, VI

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    Pharyngeal Apparatus Derivative3

    Pouch Arch Cleft Nerve

    I Eustachian tube,tympanic cavity,

    mastoid air cells

    Mandible, muscles ofmastication, malleus and

    incus

    Externalear

    canal

    Trigeminal (V)

    II Palatine tonsil Lesser horns of hyoid,muscles of facial expression,

    buccinator, platysma,stapedius, stylohyoid,

    posterior belly of digastric

    Sinus of

    His

    Facial (VII)

    III Inferior parathyroid,thymus, piriform

    fossa

    Hyoid (greater horn & body), Sinus of

    His

    Glossopharyngeal

    (IX)

    IV Superiorparathyroid

    Thyroid cartilage Sinus of

    His

    Vagus (X),

    superior laryngeal

    VI Parafollicular cellsof thyroid gland

    Muscle of larynx, cricoid

    cartilage

    None Vagus (X),

    recurrent

    laryngeal

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    Pharyngeal Apparatus Derivative

    Arch I

    Malleus

    & Incus Stapes

    Meckels CartilageStylohyoid

    Cartilage

    HyoidArch II

    Arch IIIThyroid

    CartilageArch IV

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    Radiographic

    Nonenhanced CT

    Low attenuation

    Contrast Enhanced CT

    Well circumscribed, non-enhancing mass

    Superinfected: Peripheral enhancement

    MRI

    T1WI: Low signal

    T1WI + contrast: No enhancement

    Superinfected T1WI: Peripheral enhancement

    T2WI: High signal cystic lesion

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    Type I BCCEpidemiology

    1% of branchial cleft defect4

    Middle-aged women

    Clinical

    Recurrent otorrhea or parotid

    gland abscess

    Location

    Near external auditory canal

    (EAC): usually posterior &

    inferior

    Parotid or angle mandible

    Submandibular gland

    Treatment

    Complete surgical excision

    Endoscopic cauterization

    Possible

    locations of

    Type I BCC

    Patrick Lynch. 12/23/2006. Facial Nerve Branch. Retrieved 4/15/2011 from http://en.wikipedia.org/wiki/File:Head_facial_nerve_branches.jpg

    http://en.wikipedia.org/wiki/File:Head_facial_nerve_branches.jpghttp://en.wikipedia.org/wiki/File:Head_facial_nerve_branches.jpg
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    Type I

    History: 5- year old p/w right parotid lump

    Finding:

    Periparotid mass, with central fluid attenuation, mild adjacent fat

    stranding and peripheral enhancement anterior to the EAC.

    Impression:

    Type I Branchial Cleft Cyst.

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    Type I Imaging

    T1. Right periparotidcystic mass

    T1 + contrast:Peripheral

    enhancement

    T2: Cystic masswithout tract to EAC

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    Type 1

    T1 + contrastT1 T2 fat sat

    DX:Infected Left Type I BCC.

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    Branchial Cleft Cyst Type II

    Epidemiology

    5

    95% of Branchial cleft cysts

    Age 10-40

    Gender nonspecific

    Clinical Painless cystic mass

    2nd infection: tender

    Recurrent submandibular infection

    SCM

    Patrick Lynch. 12/23/2006. Head sagittal mouth. Retrieved 4/15/2011 from http://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpg

    http://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpghttp://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpg
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    Location for Type II BCCBaileys Four Subgroups

    Type I:Superficial and anterior tosternocleidomastoid (SCM) muscle.

    Type II:Most commonClassically anterior to SCM, lateral tocarotid space and posterior to

    submandibular glandType III:

    Medially between ICA and ECA, underglossopharyngeal nerve (IX) and abovehypoglossal nerve (XII)

    Type IV:

    Pharyngeal mucosal space

    CN IX

    CN XII

    ICA

    ECA

    Path

    Possible locationof a

    Type II BCC

    Path:Green path from supraclavicular

    to oropharyngeal mucosa

    Lateral to common carotid

    In between ICA and ECA

    Below CN IX and above CN XII

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    Case I: Type II BBC.

    U/S: Sagittal right cervical

    region with diffuse fine

    echoe mass

    CT Axial: Thick walled cystic

    lesion anterior-medial to SCM,

    lateral to carotid space

    CT Sagittal: Cystic

    lesion is posterior to

    submandibular gland

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    Case II: Type II BBC.

    Cystic mass with internal septation and peripheral soft tissue suggestive of previous

    infection.

    SCM

    Lesion

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    Type III

    Epidemiology6-7

    3% of Branchial cleft cysts

    Childhood

    Preference upper 1/3 left posterior

    triangle (97%)

    2nd most common lesion of posteriortriangle after lymphatic malformation

    Clinical

    Painless cystic mass

    2nd infection: tender

    Difficult to differentiate 4th BCC

    SCM

    Trapezius

    Patrick Lynch. 12/23/2006. Head sagittal mouth. Retrieved 4/15/2011 from http://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpg

    http://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpghttp://commons.wikimedia.org/wiki/File:Head_sagittal_mouth.jpg
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    Type III

    CN IX

    CN XII

    Thyroid

    Cartilage

    ECA

    Path

    Hyoid

    ICA

    Location Start pyriform sinus

    Posterior to common or internal

    carotid

    Below CN IX and above CN XII

    Pierces thyrohyoid membrane

    Superior to laryngeal nerve

    Terminate around aorta on left side

    and subclavian artery on the right

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    Type IV

    Epidemiology6,8

    Rarest,1%-2% of BCC

    Mostly presents as tracts

    Usually infants and childhood

    Mainly left sided (90%)

    Associated thyroid lobe and

    thyroiditis

    Difficult to differentiate 3rd BCC

    Clinical

    Painless cystic mass

    Airway compromise

    Recurrent neck abscess

    Thyroiditis

    Hyoid

    Thyroid C.

    Thyroid G.

    Path

    Path

    Starts at pyriform sinus through

    thyrohyoid membrane into left

    thyroid.

    Inferior to superior laryngeal nerve

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    Case I of Type III vs IV BCC

    Multiloculated fluid collection Involves left hemithyroid

    Extends to left piriform sinusMass effect hypopharynx

    Case I: 9 year old male presents with recurrent neck abscess

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    Case I of Type III vs IV BCC

    Endoscopic survey demonstrates aTract in the ipsilateral pyriform sinus.

    Endoscopic cauterization with silver nitrate.

    Type III vs IV ????Unclear because both type III and IV arise

    from piriform sinus and an surgicalexploration wasnt performed to show

    relationship to superior laryngeal nerve.

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    Case II of Type III vs IV BCC

    Case II: 5 day old male born with increasing neck mass and airway compromise.Finding:

    Cystic mass with internal septation associated with the thyroid.

    Mass effect with rightward deviation of airway, oropharynx and

    hypopharynx

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    Case II of Type III vs IV BCC

    Piriform sinus tract with post-endoscopic electrocauterization

    DX: Type III vs IV BCC

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    Case III of Type III vs IV BCC

    Axial T1 Post-contrast:

    Homogenous tubular enhancement lateral to left thyroid extending to skin

    Medial to SCM

    Cyst not visualized

    Consistent with recurrent infection of tract

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    Case III of Type III vs IV BCC

    Axial T2 fat suppressed:

    Homogenous tubular enhancement lateral to left thyroid extending to skin.

    Cyst and extension to piriform sinus not visualized

    Consistent with recurrent tract infection

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    Case IV of Type III vs IV BCC

    Case IV: 18 year old female w/ history of I & D of neck mass presents with

    recurrent neck mass, pain and fever.CT: Left hemithyroid multiloculated abscess extending to

    anterior skin

    U/S: Complex fluid

    collection

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    Case IV of Type III vs IV BCC

    Barium Swallow:

    Blind ending sinus from left piriform sinus

    Connection with neck abscess not visualized

    Post-endoscopic cauterization

    Closure of sinus tract

    T t t

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    Treatment

    Traditionally9-12:

    Complete surgical excision for all 4 types ofBCC

    +/- thyroid lobectomy for type 3 & 4 BCC.

    Morbidity: Infection, reoccurence, andrecurrent laryngeal nerve injury.

    Novel approach for type 3 & 4 BCC

    Endoscopic cauterization

    Electrocauterization, low power diode laser,chemical cauterization (trichloroacidic acid), andsilver nitrate

    Reduce morbidity, hospital stay and cost.

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    Conclusion Branchial cleft cyst is a differential for lateral

    neck mass. There are four types of branchial cleft cyst.

    Type I: Near EAC, parotid and angle of mandible

    Type II: Lateral to carotid sheath, anterior to SCM and posterior to

    submandibular gland

    Type III & IV: Type III is in posterior compartment, posterior to SCM and

    ICA. Both involves the piriform sinus.Depends on relationship

    to superior laryngeal nerve. Understanding embryologicalorigin and the anatomical presentation will aid in accuratediagnosis

    Treatment: Incision and dissection, & novelendoscopic cauterization.

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