Congenital Neck Masses

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Congenital Neck Masses
James Ridgway MD4/6/2006
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Case review

• Patient was found to have a left sided neck mass
  on fetal ultrasound at the age of 16 weeks
  gestation. EXIT (Ex-utero Intrapartum Treatment)
  procedure was planned due to high level of
  concern for CHAOS (Congenital High-Airway
  Obstruction Syndrome) based on high resolution US
  studies. Three days prior to planned procedure,
  the mother presented in preterm labor, EXIT
  procedure was performed with competent airway
  observed.

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Case review

• Patient subsequently admitted to CHOC due to
  increased size of the cystic lesion with
  development of mild inspiratory stridor. Surgical
  excision performed on day of life 10 through left
  neck incision only. The remaining hospital
  course was uneventful.

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Neck Masses - Considerations

• Age
• Location
• Lateral branchial cleft cysts and laryngoceles
• Midline thyroglossal duct cyst, dermoid cyst,
  thymic cyst, and teratoma
• Exceptions hemangiomas and vascular
  malformation lesions

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Vascular Lesions - Classification

• Mulliken and Glowacki simple biologic
  classification
• Hemangiomas and vascular malformations
• Hemangioma not evident at birth, rapid
  endothelial proliferation followed by slow
  involution.
• Vascular malformation present at birth, normal
  rate of endothelial turnover, lesion grows with
  the child, progressive dilation of vessels

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Hemangiomas

• Most common pediatric tumor.
• Rapid proliferation of endothelium, slow
  progressive involution.
• Less than 33 present at birth
• 90 of lesion involute
• CT w/ contrast or MRI w/ Gadolinium.
• If associated w/ stridor, must rule out
  subglottic hemangioma.
• Kasabach-Merritt syndrome relation?

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Vascular malformations

• Nevus flammeus vs. port wine stain
• Sturge-Weber Syndrome
• Venous Malformations
• Lips and cheeks
• Expand with jugular venous congestion
• Intraosseous soap bubble appearance
• AVM
• High flow CHF
• Thrill/bruit
• Pain, ulceration, bleeding and pulsatile tinnitus

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Lymphangioma

• Microcystic and macrocystic
• Large, soft, compressible masses
• 60 presenting in 1st year, 90 by three years of
  age
• Type I and Type II
• Anterior/OC/FOM vs. Posterior triangle
• 40 presenting with airway compromise
• Centrifugal vs. Centripetal theory
• MRI
• Spontaneous regression is rare (8-15) and
  surgical excision is the treatment of choice.
• Recurrence is 10-52

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Branchial System

• Six pairs of mesodermal arches separated
  externally by ectodermally-lined clefts and
  internally endodermally-lined pouches
• Each arch consists of a nerve, artery, muscle
  rudiment and cartilaginous skeleton
• Neck musculature gains contributions from
  cervical somites.

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Branchial System

• First Branchial arch
• Maxillary and mandibular (Meckels) process
  regress to leave the malleus and incus.
• Ossification around Meckels cartilage gives rise
  to the mandible, sphenomandibular ligament, and
  anterior mallear ligaments.
• Muscles- temporalis, masseter, pterygoids,
  mylohyoid, ant belly of digastric, tensor
  tympani, tensor veli palatini

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Branchial System

• First Branchial Cleft
• persists as the external auditory canal, and
  tympanic membrane
• First Branchial Arch
• Nerve- 5th cranial nerve
• Artery- maxillary artery
• First Branchial Pouch
• persists as the Eustachian tube, middle ear,
  portions of the mastoid bone.

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Branchial System

• Second Branchial Cleft Cervical sinus of His
• Second Branchial Arch
• Reicherts cartilage contributes to the
  superstructure of the stapes, the upper body and
  lesser cornu of the hyoid, the styloid process
  and stylohyoid ligament.
• Muscles- platysma, muscles of facial expression,
  posterior belly of digastric, stylohyoid, and
  stapedius
• Nerve- 7th cranial nerve
• Artery- stapedial artery

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Branchial System

• Third Branchial Cleft Cervical sinus of His
• Third Branchial Arch
• Lower body of the hyoid and greater cornu.
• Muscles- stylopharyngeus, superior and middle
  pharyngeal constrictors.
• Nerve- 9th cranial nerve
• Artery- common carotid and proximal portions of
  the internal and external carotid.
• Third Branchial Pouch
• Inferior parathyroids
• Thymus gland and thymic duct

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Branchial System

• Fourth Cleft Cervical sinus of His
• Fourth Arch
• Muscles- cricothyroid, inferior pharyngeal
  constrictors
• Nerve- Superior Laryngeal Nerve
• Artery- Right Subclavian, Aortic arch
• Fourth Pouch- superior parathyroid glands and
  parafollicular thyroid cells
• Fourth and Sixth Branchial arches fuse to form
  the laryngeal cartilages.

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Branchial System

• Sixth Branchial Arch
• Muscles- remaining laryngeal musculature
• Nerve- Recurrent Laryngeal Nerve
• Artery- Pulmonary Artery and ductus arteriosus

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First Branchial Cleft Cysts

• Type I
• Ectodermal duplication anomaly of the EAC with
  squamous epithelium only
• Fistulous tracts near the lower portion of the
  parotid gland
• Parallel to the EAC
• Pretragal/ postauricular sulcus
• Surgical Excision

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First Branchial Cleft Cysts

• Type II
• Represents anomalous EAC and rudimentary pinna
  (epithelium, mesoderm)
• Cyst/ tract below angle of mandible and through
  the parotid in variable position to CN VII
• Tract runs from the neck to the EAC or middle ear
• Surgical excision- superficial parotidectomy

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Second Branchial Cleft Cysts

• Most Common (90) branchial anomaly failure of
  obliteration of cervical sinus of His
• Painless, fluctuant mass in anterior triangle
• Can occur at carotid bifurcation or
  parapharyngeal space
• Inferior-middle 2/3 junction of SCM, deep to
  platysma, lateral to IX, X, XII, between the
  internal and external carotid and terminate in
  the tonsillar fossa
• Surgical treatment may include tonsillectomy

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Third Branchial Cleft Cysts

• Patients present with recurrent infections of the
  lower lateral neck
• Masses low in the anterior neck, more often on
  the left side
• Sinus tract starting at the piriform fossa,
  through the thyrohyoid membrane, tracking under
  CN XII and carotid, but anterior to CN X
• Often track through the upper pole of the thyroid

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Fourth Branchial Cleft Cysts

• May have opening located near the apex of the
  pyriform sinus, fistula or sinus tract that
  travels between the superior and inferior
  laryngeal nerves, or an external opening along
  the anterior border of the sternocleidomastoid
  muscle in the lower neck.
• Very rare, first reported by Sanborn in 1972

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Thyroglossal Duct Cyst

• Most common congenital midline mass
• Elevates with tongue protrusion
• Commonly at the level of the hyoid
• Ectopic thyroid tissue vs. thyroglossal duct cyst
• Ultrasound
• Radioisotope scan

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Cervical Thymic Cysts

• Commonly in the lower neck, but anywhere from the
  pyriform sinus to the chest
• Failure of involution of the cervical
  thymopharyngeal ducts.
• Firm, mobile masses found in the lower aspects of
  the neck.
• CXR, CT scan

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Dermoid Cysts

• Tissue from all three germinal layers
• Sweat and sebaceous glands
• Midline mass that does not elevate with tongue
  protrusion
• Misdiagnosed as thyroglossal duct cysts
• Total surgical excision to prevent recurrence

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Teratoma

• All three germ cell layers, but foreign to the
  site of presentation
• Mature vs. immature
• Rarely present after the first year of life
• 20 associated maternal polyhydramnios
• Unlike adult teratomas, they rarely demonstrate
  malignant degeneration.
• Surgical excision.

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Laryngoceles

• Enlarged laryngeal saccule
• Classified as internal, external, or both
• Internal
• Confined to larynx, involves FC and AE fold
• Hoarseness/ respiratory distress vs. neck mass
• External and Combined Laryngoceles
• Compressible, lateral neck mass that distends
  with increases in intralaryngeal pressures
• Through the thyrohyoid membrane at the entrance
  of the Superior Laryngeal Nerve.
• CT scan

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Plunging Ranula

• Simple - unilateral OC cystic lesion
• Plunging - though mylohyoid
• Cyst aspirate- high protein, amylase levels
• CT scan/MRI
• Treatment is intra-oral excision to include the
  sublingual gland of origin

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Fibromatosis colli

• Torticollis with firm mass on the SCM
• Noted at birth or within 1st few weeks
• Inflammatory lesion of unknown etiology with
  muscle replacement by fibrosis
• Range of motion exercises
• Myoplasty of the SCM only if refractory to PT

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Case Revisited

• What was the diagnosis?

• Branchial Cleft Cyst