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Contributors
AlfredMarcIloreta, MD, MS Department of Otolaryngology and Neurosurgery,
Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
Ameya A. Jategaonkar, MD Department of Otolaryngology and Skull Base
Surgery, Barrow Neurological Institute, Phoenix, AZ, USA
Anatoli F. Karas, MD Department of Otorhinolaryngology - Head and Neck
Surgery, Rush University Medical Center, Chicago, IL, USA
Department of Pediatrics, Rush University Medical Center, Chicago, IL, USA
VivianF.Kaul, MD Department of Otolaryngology - Head and Neck Surgery,
Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
MohemmedKhan, MD Department of Otolaryngology - Head and Neck Surgery,
Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
EliezerC.Kinberg, MD Otolaryngology - Head and Neck Surgery, Singing River
Premier Network, Pascagoula, MS, USA
JaclynAlycia Klimczak, MD Department of Otolaryngology - Head and Neck
Surgery, Mount Sinai Hospital, The Icahn School of Medicine at Mount Sinai, New
York, NY, USA
Benjamin M. Laitman, MD, PhD Department of Otolaryngology - Head and
Neck Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New
York, NY, USA
JanetWaiminLee, MD Department of Head and Neck Surgery & Communications
Sciences, Duke University Medical Center, Durham, NC, USA
FredY.Lin, MD Department of Otolaryngology - Head and Neck Surgery, Icahn
School of Medicine at the Mount Sinai Hospital, New York, NY, USA
Kelly Magliocca, DDS, MPH Department of Pathology, Emory University
Hospital Midtown– Pathology, Atlanta, GA, USA
Brett A. Miles, DDS, MD Otolaryngology Head and Neck Surgery, Oral and
Maxillofacial Surgery, Northwell Health System, New Hyde Park, NY, USA
Lucia S. Olarte, MD Surgery, Division of Otolaryngology – Head and Neck
Surgery, Cedars Sinai Medical Center, Los Angeles, CA, USA
ZaraM.Patel, MD Otolaryngology- Head and Neck Surgery, Stanford University
School of Medicine, Palo Alto, CA, USA
StanleyPelosi, MD Donald and Barbara Zucker School of Medicine at Hofstra/
Northwell, Long Island Jewish Medical Center, New Hyde Park, NY, USA
Noel M. Phan, MD Department of Otolaryngology - Head and Neck Surgery,
Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
Katie Phillips, MD Otolaryngology Head and Neck Surgery, University of
Cincinnati College of Medicine, Cincinnati, OH, USA
Contributors
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xv
Eitan Prisman, MD, MA, FRCSC Otolaryngology Head and Neck Surgery,
Microvascular Reconstruction, Trans-Oral Robotic Surgery, Endocrine Surgery,
Vancouver General Hospital, Vancouver, BC, Canada
Anaïs Rameau, MD, MSc, MPhil Department of Otolaryngology - Head and
Neck Surgery, Weill Cornell Medical College, New York, NY, USA
AnsleyM.Roche, MD Department of Surgery, Division of Otolaryngology - Head
and Neck Surgery, Yale School of Medicine, New Haven, CT, USA
Joshua D. Rosenberg, MD Department of Otolaryngology - Head and Neck
Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
VictorJ.Schorn, MD Head and Neck Surgery, Kaiser Permanente– West Los
Angeles, Los Angeles, CA, USA
Zachary G. Schwam, MD Department of Otolaryngology - Head and Neck
Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA
Arash Shahangian, MD Head and Neck Surgery, Kaiser Permanente San Jose
Medical Center, San Jose, CA, USA
Robert N. Sharobiem, DDS, MD Advanced Dentistry of Alhambra,
Alhambra, CA, USA
AnthonyDel Signore, MD, PharmD Department of Otolaryngology - Head and
Neck Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New
York, NY, USA
Department of Otolaryngology, Icahn School of Medicine at the Mount Sinai
Hospital, New York, NY, USA
ToddSpock, MD Department of Otolaryngology- Head and Neck Surgery, Icahn
School of Medicine at the Mount Sinai Hospital, New York, NY, USA
ChazL.Stucken, MD Department of Otolaryngology - Head and Neck Surgery,
University of Michigan, Ann Arbor, MI, USA
C. Kwang Sung, MD, MS Department of Otolaryngology - Head and Neck
Surgery, Stanford University School of Medicine, Stanford, CA, USA
Hailun Wang, MD Department of Otolaryngology, University of Connecticut,
Farmington, CT, USA
Todd R. Wentland, DDS, MD Department of Oral and Maxillofacial Surgery,
John Peter Smith Hospital, Fort Worth, TX, USA
AnniWong, MD Department of Otolaryngology- Head and Neck Surgery, Icahn
School of Medicine at the Mount Sinai Hospital, New York, NY, USA
KevinWong, MD Department of Otolaryngology- Head and Neck Surgery, Icahn
School of Medicine at the Mount Sinai Hospital, New York, NY, USA
Part I
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Pediatrics
Chapter 1
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Embryology
AnatoliF.Karas andJanetWaiminLee
Pearls
• A child who presents with a midline nasal mass requires imaging prior to
intervention.
• Imaging is usually not part of the initial workup of a child presenting with iso-
lated microtia or canal atresia; the initial management step is to amplify; imaging
is delayed until later when surgical intervention could be considered.
Branchial Arch Derivatives
See Table1.1.
A. F. Karas
Department of Otorhinolaryngology, Head & Neck Surgery, Rush University Medical Center,
Chicago, IL, USA
Department of Pediatrics, Rush University Medical Center, Chicago, IL, USA
e-mail: anatoli_f_karas@rush.edu
J. W. Lee (*)
Department of Head and Neck Surgery & Communications Sciences, Duke University
Medical Center, Durham, NC, USA
e-mail: janet.w.lee@duke.edu
© Springer Nature Switzerland AG 2023
F. Y. Lin, Z. M. Patel (eds.), ENT Board Prep,
https://doi.org/10.1007/978-3-031-26048-3_1
3
4
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Table 1.1 Branchial arch derivatives
Arch/Name Nerve Muscle/ligament Cartilage derivative Artery
1
Mandibular
2 Hyoid Facial (VII) Muscles of facial
3 Glossopharyngeal
4 Superior laryngeal
6 Recurrent laryngeal
Trigeminal (V) Muscles of
mastication
Mylohyoid Body, short process
Anterior digastric Mandible
Tensor veli palatini
Tensor tympani
Sphenomandibular
ligament
Anterior malleolar
ligament
expression
Posterior auricular Long process of
Stapedius Stapes superstructure
Posterior digastric Lesser cornu of hyoid
Stylohyoid Upper body of hyoid
Stylopharyngeus Greater cornu of
(IX)
Pharyngeal
(branch of X)
(branch of X)
constrictors
Cricothyroid
Cricopharyngeus Cuneiform Aorta (left)
Intrinsic laryngeal
muscles
Head, neck of
malleus
of incus
Manubrium of
malleus
incus
(stapes footplate
comes from otic
capsule)
hyoid
Lower body of hyoid
Thyroid Subclavian
Cricoid Pulmonary
Arytenoid Ductus
Corniculate
Trachea
A. F. Karas and J. W. Lee
Maxillary
Stapedial
(involutes)
Common and
internal
carotid
(right)
(right)
arteriosus
(left)
Branchial Cleft Anomalies: Cysts, Sinus, Fistulae
• Run deep to artery and vein in named arch, supercial to artery and vein of next arch.
• Consider branchio-oto-renal syndrome, especially in child with additional head
and neck anomaly.
• First branchial cleft
– 5–25% of all branchial cleft anomalies
– Work Classication
1 Embryology
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Type I
• Epidermoid elements only (no cartilage or adnexal structures)
• Duplication anomaly of the external auditory canal (EAC)
• Medial to concha, extends to postauricular crease
• Lateral to facial nerve, parallel to EAC, may run under annulus to umbo
• Present as draining ear, recurrent infection, may have pit or cyst in EAC
Type II
• More common than type I
• Ectodermal and mesodermal elements
• Variable relationship to the facial nerve
• End inferior to EAC or into bony/cartilaginous junction, may be
cyst or pit
• May present externally in the neck, typically at the angle of mandible
• Second branchial cleft
– Most common type of branchial anomaly, 40–95%
– Pathway
External opening along anterior border of sternocleidomastoid (SCM) in
the lower third of the neck
Internal opening found in tonsillar fossa, associated with posterior pillar
Runs between external carotid artery (ECA) and internal carotid
artery (ICA)
Runs lateral to cranial nerve (CN) IX and XII on ascent into oropharynx
More common on the right
5
• Third/fourth branchial cleft
– Rare anomalies, 2–8%. Existence of true fourth branchial anomaly controver-
sial as complete tract has not been reported
– Pathway
Also present lower in the neck anterior to SCM.
Deep to third arch structures—CN IX, ICA.
Supercial to fourth arch structures—CN X.
Enters the pharynx at thyrohyoid membrane or pyriform sinus.
Theoretically, third ends at base of pyriform, while fourth ends at apex.
Can be closely associated with thyroid, may present as thyroid abscess or
suppurative thyroiditis.
More common on the left.
– Treatment: controversial, but may include endoscopic approach with cauter-
ization of opening into pyriform sinus/hypopharynx and excision of skin
lesion OR if open approach, may need to include hemithyroidectomy with
resection of tract
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A. F. Karas and J. W. Lee
Thyroglossal Duct Cyst
• Embryologic remnant of the tract from descent of thyroid gland from foramen
cecum to natural anatomic position in the neck.
• Evaluate for the presence of a normal thyroid gland using ultrasound prior to
surgical management.
• Tx: Sistrunk procedure—excision of cyst, surrounding tissue, and central portion
of hyoid, variable tract path
Otologic Development
• Six Hillocks of His (1–3=rst arch; 4–6=second arch)
– 1 tragus, 2 helical root/crus, 3 helix
– 4 antihelix, 5 antitragus, 6 lobule
• Adult conguration and location at birth, 85% of adult size at 5years old, adult
size at 9years old
• EAC and tympanic membrane—product of rst branchial cleft
• Eustachian tube—50% adult length at birth, moves from horizontal to move vertical position by 5–7years old
• Ossicles—adult size at birth
• Mastoid—increases in size and pneumatization from birth to ~3years old (note
that facial nerve is located more supercially at birth and is medialized with
mastoid development)
• Microtia
– Class I: all structures present, auricle mildly decreased in size
– Class II: all major structures present but with tissue deciency
– Class III: rudimentary soft tissue without recognizable structures
(most common)
– Anotia/Class IV: complete absence
• Aural Atresia—See Chap. 5
• Management of microtia/atresia
– Early bone-conducting hearing aids for bilateral atresia.
– Defer surgical management until 5–8years; no imaging indicated unless sen-
sorineural component of hearing loss present or suspect cholesteatoma (middle ear or canal). Otherwise, usually obtain around 3–5 years old, if/when
planning surgical intervention.
– Autologous rib graft reconstruction requires multiple stages and generally not
performed at least until age 5. Uses autologous tissue; long-term outcomes
are good.
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– Cadaveric rib graft reconstruction also requires multiple stages but no donor
site morbidity. Resorption of framework is more common.
– Porous polyethylene implants require fewer stages and can be performed ear-
lier (age 3), but there is concern for implant extrusion and infection.
– Prostheses include least amount of surgery and can be adhesive or based on
osseointegrated implants but can be dislodged, resulting in potential psycho-
social trauma and expense of replacement.
– Microtia repair before atresiaplasty to preserve vascularity of tissue aps.
– Canal cholesteatoma may be associated with atresia and requires prompt
intervention.
• Syndromes associated with microtia/atresia: CHARGE, Crouzon, Goldenhar,
hemifacial microsomia, Pierre Robin sequence, Treacher Collins, VACTERL
Association
7
Midline Nasal Masses
• Nasal development
– Nose formed from frontonasal process and bilateral nasal placodes
– Origin of intranasal structures
Maxilloturbinal → inferior turbinate
First ethmoturbinal → agger nasi cell, uncinate process
Second ethmoturbinal → middle turbinate
Third ethmoturbinal → superior turbinate
Fourth ethmoturbinal → supreme turbinate
– Erroneous closure of embryologic spaces may lead to persistent communica-
tion and/or trapped neural or epithelial tissue elements and resultant congenital midline nasal pathology:
Anterior neuropore: most distal end of the ectoderm-derived neural tube,
vulnerable to developmental errors
Foramen cecum: pathway between frontal and ethmoid bones that usually
obliterates itself, continuous with prenasal space
Fonticulus nasofrontalis: embryonic space between the frontal and
nasal bones
Prenasal space: potential space during development between the nasal
bones and the cartilaginous precursors of the septum
• Imaging must be performed prior to intervention to evaluate for intracranial
extension and to characterize type of anomaly. Computed tomography (CT) scan
may show bid crista galli with intracranial communication but can be indeterminate due to incomplete ossication of skull base. Magnetic resonance imaging
(MRI) is recommended for more specic soft tissue evaluation.
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• Differential diagnosis:
– Dermoid cyst (most common): rare dural connection, rarely transilluminate,
negative Fustenberg test (expansion of a nasal mass with compression of the
internal jugular veins)
Can be associated with meningitis when intracranial
Found as a midline uctuating cyst with a sinus tract leading to the skin;
epithelium lines (look for hair at opening), contains skin appendages, may
penetrate deep to the nasal bone
Treatment: surgery; neurosurgical consultation, manage any intracranial
portion rst
Surgical approaches for nasal component: vertical midline dorsal excision,
external rhinoplasty, bicoronal
– Neurogenic: glioma, encephalocele, neurobroma
Glioma: trapped neural tissue without persistent dural connection, do not
transilluminate, negative Furstenberg test, not associated with meningitis,
a solid mass of glial tissue with a brous stalk
• Usually found at the glabella, can also present as lateral nasal mass
Encephalocele: always has a dural connection, transilluminates, positive
Furstenberg test, associated with meningitis, histologically an ependymal
lined sac that communicates with the cerebrospinal uid (CSF) spaces
A. F. Karas and J. W. Lee
– Hemangioma
Choanal Atresia
• Incidence 1:5000–8000 births, F/M 2:1.
• 50% have other congenital anomalies (75% of bilateral cases associated with
other anomalies).
• 30% bony, 70% mixed bony-membranous.
• 65–75% unilateral, rest are bilateral.
• Results from persistence of buccopharyngeal membrane.
• Severity of presentation depends on whether unilateral or bilateral; bilateral atresia presents with immediate cyclical cyanosis (cyanosis interrupted by crying
spells); unilateral atresia can remain hidden for years and present with unilateral
nasal obstruction and rhinorrhea.
• Four parts of the anatomic deformity:
– Narrow nasal cavity
– Lateral bony obstruction from pterygoid plate
– Medial bony obstruction from vomer
– Membranous obstruction
1 Embryology
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• General management approach:
– Unilateral atresia: non-urgent repair, can wait until ~1year of age.
– Bilateral atresia: establish temporary airway and feeding pathway (McGovern
nipple, oropharyngeal airway; intubation not necessary unless mechanical
ventilation required), and prepare for surgical correction.
• Surgical repair approaches:
– Transnasal
– Transpalatal (reserved for older children d/t orthodontic growth)
– Transantral
– Transseptal
• Syndromes associated with choanal atresia (mnemonic—“ACT TV”): Apert,
Crouzon, Treacher Collins, Trisomy 18, velocardiofacial syndrome
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Congenital Pyriform Aperture Stenosis
• Central megaincisor.
• Cerebral malformations—holoprosencephaly; obtain MRI.
Velopharyngeal Insufciency
• Four patterns of velopharyngeal closure
– Coronal (55%, most common)
– Sagittal (10–15%, least common)
– Circular (10–20%)
– Circular with Passavant’s ridge (15–20%)
• Management
– Medical
Speech therapy
Prosthesis: palatal lift or obturator
Biofeedback with nasometry
– Surgical
Sphincter pharyngoplasty—use when good anterior-posterior motion,
poor lateral motion.
Pharyngeal ap—use when good lateral motion, poor anteriorposterior motion.
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