Pediatric ENT Flashcards
1st branchial arch nerve + derivatives
CN V
Mandible, Meckel’s cartilage –> malleus (except manubrium), body + short process incus, tensor tympani, mastication muscles, mylohyoid, anterior belly digastric, tensor palati muscle
2nd branchial arch nerve + derivatives
CN VII
Reichart’s cartilage –> Manubrium malleus, long process incus, all of stapes except footplate (all stapedial artery, styloid process, lesser cornu of hyoid, stapedius muscle, facial muscles, posterior belly of digastric
3rd branchial arch nerve + derivatives
CN IX
Greater cornu of hyoid, stylopharyngeus muscle, sensory to posterior 1/3 of tongue, superior + middle constrictors, CC a., ICA
Pouch: Inferior parathyroids, thymus
4th branchial arch nerve + derivatives
Superior laryngeal nerve
Thyroid cartilage, inferior pharyngeal constrictor, cricopharyngeus, cricothyroid muscles, left aorta, subclavian on right
Pouch: Superior parathyroids, ultimobranchial body (C cells)
6th branchial arch nerve + derivatives
Recurrent laryngeal nerve
Cricoid, arytenoids, corniculate, trachea, intrinsic laryngeal muscles, ductus arteriosis
6 Hillocks of His
1st Arch
1 - Tragus
2 - Helical root/crus
3 - Helix (rim)
2nd Arch
4 + 5 - Antihelix
6 - Antitragus
*Preauricular cyst arises from partial failure of involution of epithelium between 1st and 2nd hillocks
Chandler classification (1-5)
- Preseptal involvement (EOMI, no visual changes)
- Orbital Cellulitis (NO abscess)
- Subperiostial abscess (between orbital periostium and bony orbital wall). Displaces orbit down and lateral (proptotic). Associated with limited EOM and vision changes
- Orbital abscess
- Cavernous Sinus Thrombosis - BILATERAL SX
Complications of persistent sinus disease
-Orbital (chandler classification)
-Intracranial (meningitis, epidural abscess, subdural abscess, acute and chronic brain abscess) –> meningitis is the most common!
What is Cidofovir and what is it used for?
DNA polymerase inhibitor, used off label for respiratory papillomatosis
What is Bevacizumab and what is it used for?
VEGF inhibitor, used off label for respiratory papillomatosis
What HPV subtypes cause respiratory papillomatosis?
HPV 6 and 11
Most common cause of pediatric otitis media (bugs)
Strep pneumo, H inluenza, Moraxella, Group A Strep
Indications for T tube placement
-Chronic OME > 3 months bilaterally or >6 months unilaterally
-Earlier if severe, significant HL, speech delay, severe retraction, vestibular SX, has complications from OM
Top 4 bugs for acute mastoiditis
S. pneumo, H influenza, Strep pyogenes, S. Aureus
Indications for bone conducting hearing aids
Atresia or microtia (can’t use traditional), CHL with AB gap > 30 dB, Mixed HL (BAHA can compensate for up to 65 dB HL), SS deafness, inability to fit traditional hearing aid because of skin allergy or chronic draining ears
Features of nose in cleft lip
-Posterior septum deviated TOWARD the cleft
-Nasal tip, anterior septum and base of columella deviate AWAY from the cleft
-Nostril on the cleft side is flattened and stretched inferiorly
and laterally (nostril is horizontal)
-Cleft side has SHORTER medial crus + LONGER lateral crus (lateral steal phenomenom)
-Cleft side lower lat cartilage is displaced inferolaterally + posteriorly
-Incomplete orbicularis oris sphincter
Timeline for infantile hemangiomas
Appear in first few weeks of life –> grow rapidly over a few weeks –> continue to grow over 6-9 months –> then involute over the next few years.
What factors are present during proliferation of infantile hemangiomas?
-Always GLUT-1 positive
-VEGF, type IV collegenase, insulin like GF, fibroblast GF all increase during proliferation. Everything but fibroblast gf decrease during involution.
Two type of congenital hemangiomas?
- Rapidly involuting (RICH)
- Noninvoluting (NICH)
What is PHACES?
Posterio fossa abnormalities (Dandy-walker), hemangioma (congenital, segmental), arterial abnormalities, cardiac and aortic abnormalities, eye abnormalities (cataract), sternal clefts.
What percent of beard distribution hemangioma are associated with airway involvement?
65%
Treatment for hemangiomas
Propanolol - 1st line
-Steroid - only if fail propanolol or for local injection
-Surgical excision (if non involuting, ulcerating, pedunculated so unlikely to involute, or disfiguring/causing distress)
-CO2 vs Yag laser (though overuse can cause subgottic stenosis)
What is Kasabach-Merrit Syndrome?
-Associated with vascular hemangiomas (kaposiform or tufted hemangioma).
-Rare, tumor traps and destroys platelets + other coagulopathies.
-TX - Chemotherapy (vincrystine)
Timeline of vascular malformations
Present at birth and grow with age of child (so not rapid).
Features of Sturge Weber syndrome?
-Vascular malformation
-Facial port wine stain in trigeminal nerve distribution, glaucoma/eye abnormalities, ipsilatral intracranial malformation.
-Children often develop seizures, migraines, stroke, hemiparesis, vision issues, etc.
-TX: PDL to help with port wine stain, Yag laser if deeper, surgical debulking also an option + medical management for the health issues.
What is PFAPA + treatment?
Periodic fever, apthous stomatitis, pharyngitis, adenitis.
Adenoidectomy is highly effective at resolving this condition. Otherwise NSAIDS, consider steroids.
Criteria for abnormal sleep study for pediatric patients?
AHI 1-5: Mild
AHI 6-10: Moderate
AHI > 10: Severe
O2 desat to below 92%
Peak end tidal CO2 > 53 mmHg
Elevated end tidal CO2 > 50 mmHg > 25% of total sleep time.
Indications for tonsillectomy?
Recurrent infections (7 in 1 year, 5 per year for 2 years, 3 per year for 3 years), PTA, chronic tonsillitis, obstruction, sleep disordered breathing, OSA, PFAPA, craniofacial growth abnormalities, dysphagia, Cor Pulmonale
Indications for post op sleep study?
Persistent symptoms, AHI > 20, pre op complication of OSA (pulm HTN), Age < 1 year
Max dosage for lidocaine (without epi) in kids?
3-5 mg/kg
Treatment for prominauris?
-Mustart technique to recreate antihelical fold (three horizontal mattress sutures to secure auricular cartilage to itself, no incisions). Each horizontal mattress suture is placed 2 mm apart and involved 16 mm distance across antihelical fold with 1 cm bite width.
-Furnas technique to reduce conchomastoid angle (secure conchal cartilage to mastoid periosteum)
Issue = large auricocephalic angle (ear sticks out more than 25-35 degrees), abnormal distal insertion of anti tragus muscle.
SX, TX and mortality rate for untreated Kawasaki?
SX - strawberry tongue, conjunctivitis, coronary artery aneurysms, cervical lymphadenopathy, fever.
TX - IVIG + Aspirin
Mortality untreated - 1%
What is mobius syndrome? What is a potential surgical treatment?
-Incomplete development of FN 6 and 7 (and absence of FN 7 in the IAC).
-Bilateral facial nerve paralysis
-TX - Temporalis muscle sling (can’t do any nerve grafts)
Crouzon
-AD, FGFR mutation
-Craniosynostosis
-Midface hypoplasia
-Ocular proptosis, parrot beak nose
-CHL (due to stenosis and stapes fixation
Apert Syndrome
Apert: AD, FGFR mutation, all of Crouzon PE + limb syndactyly.
Pfeifer: All of Crouzon PE + broad thumb + toes.
Stains for rhabdomyosarcoma?
Z bands
Stains positive for vimenting, myoglobin, desmin
Microcystic vs macrocystic lymphatic malformations (location, characteristics).
Microcystic - usually suprahyoid. More difficult to treat (more extensive infiltration of surrounding soft tissues). More likely to recur. TX - excision or sclerotherapy
Macrocystic - thick walled cysts, less infiltrative. Easier to excise. Less likely to recur. Infrahyoid.
Features of choanal atresia
F>M
Unilateral > Bilateral
Usually BONY defect (90%)
Associated with CHARGE, Apert, Treacher Collins, Trisomy
First Branchial Cleft Cyst
Three types?
When to get hearing test?
Excision steps?
I - Inferoposteromedial to pinna (ectoderm only)
II - Between angle of mandible and EAC (ectoderm and mesoderm)
III - Periparotid
Tract runs in close proximity to facial nerve (medial or lateral) –> would need FN dissection
*Don’t forget formal hearing test for bilateral or family history even if not planning for surgical excision.
Excision: Ellipse around the cyst, dissect down to helical root and INCLUDE perichondrium/cartilage to help reduce risk of recurrence.
2nd Branchial Cleft Cyst
What side is it most common on?
Course?
Most common (more common on RIGHT)
Anterior to SCM, BETWEEN ICA and ECA, deep to FN, superficial to CN 9 and 12, opens into tonsillar fossa
*Monitoring is appropriate if asymptomatic.
3rd Branchial Cleft Cyst
What side is it most common on?
Course?
External opening at lower anterior neck –> Passes posterior to ICA and ECA, superficial to CN X and common carotid artery, between CN IX (deep to it) and CN XII –> pierces thyrohyoid membrane staying above superior laryngeal nerve –> internal opening at APEX piriform sinus
*Fourth branchial cleft cyst is similar but STARTS as the BASE of the piriform sinus, ends @ anterior border of SCM. Very rare.
*Both are more common on the LEFT
Thymic cyst
Remnant of third pharyngeal pouch between angle of mandible and midline neck
SSx: unilateral (usually left) neck mass
Dx: biopsy, serum calcium (associated parathyroid disorders, DiGeorge syndrome/22q11), CT/MRI
Rx: excision
Atypical mycobacterium
Slowly enlarging, nontender, indurated neck mass with progressive skin discoloration (purplish), skin fixation, and possible fistulization
M. avium intracellulare (most common), M. scrofulaceum, M. kansasii
Dx: culture (requires 2–4 weeks for growth), PCR, PPD
TX: triple antibiotic therapy (clarithromycin, rifampin, ethambutol for 6 months); full surgical excision vs I&D (high risk of fistula)
What is worse acid or alkali esophageal injury and what type of injury is caused by alkali?
Alkali is worse - liquifactive necrosis to oral and upper esophageal area.
Acid - Coagulative necrosis (think milk goes sour/coagulates)!
Ex of alkali - lye
Suture lines and associated defomities due to premature closure of each one?
Scaphocephaly - saggital suture (boat shaped)
Brachycephaly - coronal suture
Plagiocephaly - coronal and lamboid
Trigoncephaly - metopic suture line (triangular forehead)
Most common site for pediatric mandible fractures?
Condyle, followed by symphysis
Rule of repair for cleft lip?
10, 10, 10!
-Hgb 10
-Weighs 10 lbs
-10 weeks old
Goal: Restore function of orbicuaris oris
Cotton Meyer grading for subglottic stenosis?
I: 0-50%
II: 51-70%
III: 71-99%
IV: Complete occlusion
At what week do these occur?
Common cavity
Cochlear agenesis
Cochlear hypoplasia
Incomplete partition
CAHP
-Common cavity - 4th week
-Cochlear agenesis - 5th week
-Cochlear hypoplasia - 6th week
-Incomplete partition - 7th week, most common (type II)
Cochlear apparatus fully developed by 8th week
Branchio-oto renal syndrome
AD, EYA1 gene mutation
Ear: pinna deformity, pre auricular pits, mixed HL, ossicular/cochlear malformations, +/- mondini malf.
Other: Renal deformity (may be a-sx), lacrimal duct stenosis
Main blood supply to adenoid pad?
Pharyngeal branch of IMAX
Top three most common salivary gland malignancies in children?
Most common benign tumor?
- Mucoepidermoid
- Acinic cell carcinoma
- Adenoid cystic
Benign: pleomorphic adenoma (in both adults and kids, most common in parotid gland).
Differential for a cystic external nasal mass (3) and embryology?
Dermoid, glioma, encephalocele
A temporary foramen cecum is present between nasal bone and inferior frontal bone (through 8 weeks of fetal life) and a dural projection extends through this. Failure of involution results in dermoid, glioma or encephalocele (depending on what remains).
*Do not biopsy - risk of CSF leak!
Eosinophilic Esophagitis Criteria for DX
-Endoscopic findings of rings, furrows, exudates, narrowing, crepe paper should increase suspicion.
-Concomitant atopic sx should increase suspicion
->15 (or =) eos/hpf on biopsy
Gorlin Syndrome Features
-“Nevoid basal cell carcinoma syndrome” - rare, AD
-Frontal bossing
-Keratocystic odontogenic tumors
-Ocular hypertelorism
What syndrome is enlarged vestibular aqueduct associated with?
Pendred (SCL26A4) - also thyroid dysfunction
What is pierre robin sequence and what syndrome is it most associated with?
Micrognathia, cleft palte, macroglossia.
Most commonly associated with PRS is Stickler syndrome, followed by 22q11 syndrome.
What two other features are associated with central mega incisor?
-Congenital nasal piriform aperture stenosis, holoproscencephaly –> get MRI!
What is CULLP?
Congenital unilateral lower lip palsy.
Most common inherited cause for congenital facial palsy (most common aquired = birth trauma).
Absence of depressor anguli oris
Associated with cardiac defects and 22q11 deletion.
What two branchial arches are the 6 Hillocks of His derived from?
1st and 2nd
Definition of pediatric hypopnea and apnea?
Pediatric hypopnea - reduction of airflow by 50% for two respiratory cycles accompanied by reduction of saturation by 3% or more, arousal or awakening from sleep.
Apnea - Reduction of more than 90%
For children, apnea and hypopnea events are based on respiratory cycles (2) unlike the adults (10 seconds)
What is the CPG recommendation for kids with recurrent AOM but clear middle ear on exam?
CPG recommends AGAINST ear tube placement in children with recurrent ear infections but clear middle ears at the time of the exam –> watchful waiting (see them in clinic every 3-6 months)
Characteristic MRI findings of a hemangioma?
Characteristic MRI findings of pleomorphic adenoma?
Hemangioma: T1 HYPOintense, T2 HYPERintensse
Pleo: Well defined mass with central necrosis!
Biggest risk of intracapsular tonsillectomy vs extracapsular?
10% risk of tonsil regrowth
Indications for pediatric DISE?
Children with persistent sleep apnea after adenotonsillectomy, those with OSA but without tonsillar hypertrophy, those with risk factors predisposing to multiple sites of obstruction, and when sleep-state dependent laryngomalacia is suspected.
Most common symptom of laryngeal cleft and workup?
Aspiration
-Video fluorscopic swallow study –> If confirmed, proceed with DL/Bronch to assess/diagnose
Main blood supply to adenoid pad?
Pharyngeal branch of IMAX
Jervell/Lange-Nielsen Syndrome
AR
Bilateral severe SNHL
Long QT syndrome (get EKG)
Issue with potassium channels
Where are the majority of congenital glottic webs and what are they associated with?
Majority (over 90%) are ANTERIOR and 65% of those are associated with 22q11.2 deletion syndrome (so do comprehensive workup)
What is propanalol and what is it used for?
Non selective beta adrenergic antagonist. Used for hemangiomas.
Side Effects: GERD, hypoglycemia
What is a pulmonary sling associated with?
Complete tracheal rings (will have symptoms of airway compression with this)
Features of thyroglossal duct cyst?
Failure of thyroglossal duct to involute (foramen cecum –> anterior or posterior to hyoid –> final position at mid neck.
DD (midline neck mass): Dermoid cyst, TGDC, Lymph node
Imaging: Solid = more likely to be LN. Consider US to reduce radiation risk for kids.
TX: Sistrunk procedure (excision of mass, mid third of hyoid, up to part of base of tongue)
Definition of recurrent acute otitis media?
Recurrent acute otitis media is defined as three or more well-documented and separate episodes of otitis media during the last 6 months, or at least four well documented and separate episodes of otitis media during the last 12 months.
-Place tubes bilaterally if noted to have unilateral or bilateral middle ear effusion during time of exam
Most common site of pediatric mandible fractures?
Condylar (and deviation is AWAY from fracture site unlike in adults where it’s opposite (contralateral side if more open)
What are the 4 Chandler staging classes for JNA?
I: Nasopharynx
II: Nasal cavity or sphenoid extension
III: Extension to rest of sinuses, infratemporal fossa, orbit, cheek, cavernous sinus
IV: Intracranial extension
What is the course of a pulmonary sling?
Left pulmonary artery arises from right pulmonary artery and travels over right bronchus and passes through the tracheoesophageal groove
-Associated with complete tracheal rings
Which muscle is most important to reconstruct during cleft palate surgery/palatoplasty?
Levator Veli Palatini (improves VPI)
What is Mitomycin C used for an mechanism?
(Mighty C fights fibroblasts)!
-Alkylating agent that inhibits DNA synthesis and prevents fibroblast proliferation
-Derived from streptomyces c.
-Used for subglottic stenosis
Acute tonsillitis top 3 bugs?
Group A strep, Moraxella, H. Flu
What virus and what diagnostic lab findings is seen in infectious mono?
EBV
Heterophile Abs in serum (mono spot test)
Atypical lymphocytes
Remember penicillin with mono may cause severe rash!
Pediatric definitions for Apnea, Hypopnea, RERA, hypoventilation syndrome?
Apnea: Drop in airflow > 90% over 2 breaths
Hypopnea: Nasal air pressure decreases by >30% over 2 breaths AND associated with 3% O2 desat or arousal
RERA: Decrease by LESS than 30% (and associated with other sx such as snoring, etc.)
Hypoventilation: Arterial or end tidal CO2 . 50 mmHg for >25% of total sleep time
What are the muscles of the palate innervated by?
CN X EXCEPT tensor veli palatini (innervated by V3)
Prevelance of cleft lip +/- palate? How often associated with syndromes? M vs F?
Cleft Lip: 30% associated with syndromes, M>F
Isolated Cleft Palate: Less common, 50% associated with syndromes, F>M
80% of clefts are UNIlateral
2/3 of cleft lips are LEFT sided
FUL CLEFT PALATE!
What syndromes are cleft palate/lip associated with?
-Van der Woude (AD, MOST COMMON syndromic cause of cleft lip +/- palate)
-PRS
-Stickler (AD) - cleft lip as part of PRS with this syndrome
-22q11.2 (AD)
-Other: Treacher Collins, Down, CHARGE, Apert, etc.
Timing of cleft palate repair?
Before 18 months (usually between 6-16 months)
most common parotid masses (pediatric)?
Pleomorphic adenoma, hemangioma
Mucoerpidermoid Ca (most common malignant)
Features of rhabdomyosarcoma in pediatric population (most common location, subtype, metastatic sites, TX)
-Most common sarcoma of H&N
-Broken into parameningeal i.e. nasopharynx, middle ear (worse prognosis) vs non parameningeal (parotid, submandublar region, etc).
-Primary site: Orbit (non parameningeal)
-Mets: Lungs, bone, BM
-DC: PET CT
-TX: Multimodal (surger/chemo/RT)
-Embryonal - most common in infants, botryoidal + pleo variants
-Alveolar: Adolescents, poorer prognosis w/ PAX3/FOX01 mutations, resembles pulmonary alveoli
What is velocardiofacial syndrome (DIGEORGE)?
Inheritance
Gene/mutation
Features
-AD
-22q11 deletion
-Abnormal facies (long, malar depression), VPI, cleft lip/palate, cardiac anomalies
-Medialized carotid arteries - get imaging before pharyngeal surgery!
CATCH-22 mnemonic for DiGeorge?
Cardiac anomalies
Abnormal Face
Thymic hypoplasia
Cleft Palate
Hypoparathyroidism (hypocalcemia)
What syndromes is choanal atresia associated with?
CHARGE, Crouzon, Apert, Treacher Collins, DiGeorge
General management for microtia (hearing and reconstruction options/timing)? What syndromes is it associated with?
Hearing:
-Soft band can be used as early as 6 months
-BAHA (ages 5 + in US)
Reconstruction:
-Prosthetic
-Reconstruction (ages 5-10) with Medpor vs rib cartilage.
-Associated with Goldenhar, Treacher Collins, BOR, Crouzon
Occult cleft palate vs submucous cleft findings?
Occult: absence of the musculus uvulae.
Submucous cleft palate: bifid uvula, zona pellucidum of the soft palate, and palpable notch in the posterior hard palate. Disruption dehiscence of LVP!
Most common cause of Croup?
Parainfluenza virus
Criteria for overnight stay after T&A?
CPG Guidelines:
<3 years
AHI > 10 (severe OSA)
O2 Nadir < 80%
Comorbidities (syndromic, etc.)
Mechanism of propanolol and side effects?
Non selective beta adrenergic antagonist.
Side effects: Rash, somnolence, GERD, hypoglycemia, bronchospasm
Beckwith-Wiedemann Syndrome?
Chromosome 11
Macroglossia, macrosmia, hemihyperplasia, hypoglycemia, auricular abnormalities, risk of Wilm’s tumors + other malignancies.
Most common vascular ring and key finding on swallow study?
Most common: Double aortic arch
Barium swallow shows POSTERIOR indentation of esophagus (vs abberent inominate would show ANTERIOR compression during bronchoscopy)
4 stages of Cormack and Lehane system for larynx visualization?
Grade 1: Full view of glottis.
Grade 2a: Partial view of glottis.
Grade 2b: Only posterior portion of glottis or arytenoid cartilages seen.
Grade 3: Only epiglottis seen, none of glottis seen.
Grade 4: Neither glottis or epiglottis seen.
Stickler Syndrome Features?
-AD
-Progressive sensorineural HL, cleft palate, abnormal development of the epiphysis, vertebral anomalies, and osteoarthritis.
Type 1: WITH retinal detachment
Type 2: Without retinal detachment
Type 3 has similar findings to type 1 WITH facial abnormalities.
-COL2A1 gene
Pfeiffer Syndrome features?
AD!!
Crouzon SX (brachiocephaly, midface hypoplasia, parrot beak nose, ocular proptosis), CHL PLUS broad thumbs/toes
Type 1: Compatible with life + mild intellectual disability
Type 2: Cloverleaf skull
Type 3: Poor prognosis, TRACHEAL CART. SLEEVE
What is the Brent and Nagata techniques for microtia reconstruction?
Brent (4 stage)
1. Costal cartilage construct
2. Lobule transposition
3. Auricle elevation
4. Tragal construction + conchal bowl
Nagata (2 stage)
1. Costal cartilage construct, including a tragus and incorporating transposition of the lobule
2. Elevation of the auricle + placement of a temporoparietal fascia flap and split-thickness skin graft.
How to determine ETT size for peds?
(Age + 16)/4
What is the most common vascular anomaly to cause stridor?
What is the most common arch anomaly?
Double aortic arch - right 4th BRANCHIAL arch persists
Most common arch anomaly: Retroesophageal right subclavian artery (R subclavian originates as last branch from descending aorta)
Most common type of tracheoesophageal fistula?
Type C - Esophageal atresia with DISTAL TEF (85%)
Which HPV vaccine can help prolong periods between surgery for respiratory papillomatosis?
Quadrivalent (Gardasil)!
Congenital syphilis findings (SNUFFLES)?
DX? TX?
Sinonasal drainage, saddle nose, saber shins, Hutchinson teeth, mulberry molars
DX: VDRL, RPR tests for SCREENING, do FTA-ABS to CONFIRM
TX: Penicillin G!
