General Board Prep Flashcards
What nerve is associated with the first branchial arch?
CN V3
What nerve is associated with the second branchial arch?
CN VII
What nerve is associated with the third branchial arch?
CN IX
What nerve is associated with the fourth branchial arch?
Superior laryngeal nerve (CN X)
What artery is associated with the first branchial arch?
Maxillary
What artery is associated with the second branchial arch?
Stapedial
What artery is associated with the third branchial arch?
Common and internal carotid arteries
What artery is associated with the fourth branchial arch?
Subclavian on the right
Arch of the aorta on the left
What nerve is associated with the sixth branchial arch?
Recurrent laryngeal nerve (CN X)
What artery is associated with the sixth branchial arch?
Pulmonary artery on the right
Ductus arteriousus on the left
Are type 1 or 2 first branchial arch anomalies more common?
2: ectodermal AND mesodermal elements
Ends inferior to the EAC or into the bony cartilaginous junction
What is the most common type of branchial anomaly?
Second: external opening along the ant border of the SCM, internal opening in the tonsillar fossa
**penetrates the platysma
Which branchial anomaly runs between the ICE and the ECA?
Second
What is the 10 point grading system for aural atresia?
Jahrsdoerfer system: 2 pts for stapes, 1 for mastoid pneumatization, oval windo status, round window status, malleus, incus, facial nerve course, status of middle ear, external appearance
What structure from the nose?
Frontonasal process
Bilateral nasal placodes
What is the origin of the inferior turbinate?
Maxilloturbinal
What is the origin of the agger nasi cell or uncinate process?
First ethmoturbinal
What is the origin of middle turbinate?
second ethmoturbinal
What is the origin of superior turbinate?
third ethmoturbinal
What is the origin of the supreme turbinate?
Fourth ethmoturbinal
What is the Furstenburg test?
Expansion of a nasal mass with compression of the IVJs
What is the most common midline nasal mass?
Dermoid cyst
Where on the nost is a glimoa usually found?
Glabella
What is torticollis post-tonsillectomy concerning for?
Grisel’s syndrome: rare cause of torticollis that involves subluxation of atlanto-axial joint from inflammatory ligamentous laxity following an infectious process in the head and neck, usually a retropharyngeal abscess.
Croup like sx in a child <6 mo whould be concerning for possible:
Subglottic hemangioma
What are the 2 major criteria for chronic pediatric sinusitis
Nasal obstruction
Purulent nasal discharge
Chandler classification
I: Pre-septal cellulitis II: Orbital cellulitis III: Sub-periosteal abscess IV: Orbital abscess V: Cavernous sinus thrombosis
What is the most common pattern of velopharyngeal closure?
Coronal
Followed by sagittal, circular, and circular with Passavant’s ridge
MCC of croup (laryngotracheitis)?
Parainfluenza
What does AP Xray show with Croup?
Steeple sign–subglottic narrowing
Rx croup:
Supportive care: humidification, racemic epi, steroids
NO intubation
AP xray finding with epiglottitis
Thumbprint sign
Rx for epiglottitis
OR intubation, extubate when air leak is present
What is the major blood supply of the adenoids?
Pharyngeal branch of the internal maxillary artery
What does inflammation do to the histopathological composition of the adenoids?
Increases squamous epithelium and decreases the respiratory proportion
What is the rule for tonsillectomy based on number of infections a year
7/yr for 1 yr
5/yr for 2 yr
3/yr for 3 yr
What is PFAPA syndrome
Periodic high fevers Aphthous stomatitis Pharyngitis Cervical adenitis 3-5 times per week for 6 mo
What microbe causes cat scratch fever?
Bartonella henselae
What is the most common pediatric salivary gland mass?
Hemangioma
What is the most common pediatric salivary gland neoplasm?
Pleomorphic adenoma
What is the most common pediatric salivary gland malignancy
Mucoepidermoid carcinoma
Are parotid gland neoplasms more commonly malignant in children vs adults?
Children 50%
Adults 20%
Three basic kinds of small blue cell malignancies of children?
Lymphoma
Sarcoma
Rhabdomyosarcoma
What is the most common site of rhabdomyosarcoma?
Orbit
What is the most common histopathology of rhabdomyosarcoma in infants and children?
Embryonal 75%: spindel shaped cells with eosinophilic cytoplasm, best prognosis
What is the most common histopathology of rhabdomyosarcoma in adolescents?
Alveolar: small round cells seperated by fibrous septae into alveolar groups
What syndrome is characterized by hemangioblastomas of the CNS and retina, renal cysts/carcinoma, pheochromocytoma, pancreatic cysts, and papillary cystadenomas of the epididymis
von Hippel Lindau syndrome
Classic triad of retrognathia, glossoptosis, and cleft palate
Pierre robin
What causes the cleft palate in Pierre robin sequence?
Retrognathia prevents descent of the tongue into the oral cavity, preventing palate fusion
MCC short limb dwarfism
Achondroplasia
What mutation causes achondroplasia?
AD mutation in FGFR-3
What is VATER syndrome? What is added in VACTERL?
Vertebral/vascular anomolies Anal atresia Tracheal anomalies Esophageal anomalies Renal/radial bone anomalies **Cardiac anomalies **Limb anomalies
What are the AD syndromes?
WANTBCS Waardenburg Apert Neurofibromatosis Treacher Collins Branchio-oto-renal Crouzon Stickler
What presents with pigmentary abnormalities, craniofacial anomalies, and unilateral or bilateral SNHL
Waardenburg
What is the key difference in phenotype between type I and type II Waardenburg syndrome?
Type I has dystopia canthorum
What is the key feature of type III and type IV Waardenburg syndromes?
III: skeletal dysplasias and muscular hypotonia
IV: Hirschsprung megacolon
What two syndromes are due to a mutation of the FGFR-2 gene?
Apert
Crouzon
What syndrome is similar to Apert’s syndrome but has digital broadening rather than syndactyly
Pffeiffer’s: associated with tracheal sleeve
Cafe au lait spots, Lisch nodules
Neurofibromatosis type I: mutation on chrom 17
Bilateral acoustic neuromas
Neurofibromatosis type II: mutation on chrom 22
Malformation of the 1st and 2nd branchial arches secondary to TCOF1 gene mutation on chrom 5
Treacher Collins: 50% have hearing impairment from EAC and/or middle ear malformations
Mutation in COL2A1 on chrom 12 (type II collagen) leadingto myopia with retinal detachment and cataracts, hypermobility with enlarged joints, early onset arthritis, SHNL in 80%
Stickler
Char by branchial cleft anomalies, otologic malformation, and renal dysplasia
Branchio-oto-renal
SNHL associated with iodine metabolism defect leading to euthyroid goiter, associated with Mondini’s dysplasia and enlarged vestibular aqueduct
Pendred
What was Pendred historically diagnose with?
Perchlorate discharge test: now test for the pendrin gene mutation
Hearing loss, vestibular deficits, ataxia, retinitis pigmentosa
Usher syndrome
What is the most common type of Usher syndrome?
Type I: most common, profound deafness with RP by the age of 10, absent vestibular response
Unilateral facial asymmetry, upper lid colobomata, otologic abnormalities, underdevelopment of the mandible
Goldenhar syndrome
Profound bilateral SNHL, cardiac defects, syncopal episodes
Jervell-Lange-Nielsen syndrome
X linked regressive syndromes
Alport
Norrie
Otopalatodigital
Wildervaank
X linked mutations in the NDP gene often leading to blindness, early onset SNHL
Norrie
Widely spaced first and second toes, CHL due to ossicular malformation, x linked recessive
Otopalatodigital