Sleep disorders, Adenotonsillar

Question 1

What is a “tonsil”?
Name the components of Waldeyer’s Ring. Compare and contrast their following properties:
1. Location
2. Presence of capsule
3. Epithelium type
4. Presence and types of crypts

Answer 1

Tonsil = lymphoid tissue from surface epithelium enveloped by mesenchymal stroma, populated by lymphocytes + immunologic mediators

A. PALATINE TONSILS (FAUCIAL TONSILS; LATERAL)
1. Location: Lateral aspect of oropharynx between palatoglossus and palatopharyngeus
2. Capsule: Incomplete
3. Epithelium: Non-keratinized stratified squamous epithelium
4. Crypts: Long, branched

B. LINGUAL TONSILS (ANTEROINFERIOR)
1. Location: Posterior to sulcus terminalis of the tongue
2. Capsule: Incomplete
3. Epithelium: Non-keratinized stratified squamous epithelium
4. Crypts: Long, unbranched

C. TUBAL TONSILS (GERLACH’S TONSILS; POSTEROSUPERIOR)
1. Location: Lateral aspect of nasopharynx, at eustachian tube
2. Capsule: None
3. Epithelium: Ciliated pseudostratified columnar
4. Crypts: Minimal

D. ADENOIDS (NASOPHARYNGEAL TONSILS; POSTEROSUPERIOR)
1. Location: Superior aspect of nasopharynx
2. Capsule: Not encapsulated (or incomplete)
3. Epithelium: Predominantly ciliated pseudostratified columnar (respiratory epithelium)
4. Crypts: Minimal

*Can also include: Posterior pharyngeal wall tonsils, lateral pharyngeal bands

Question 2

Describe the Brodsky Grading and Friedman grading of tonsils

Answer 2

BRODSKY
1. Grade 0: Tonsillectomy
2. Grade 1: ≤25% airway occluded
3. Grade 2: 26-50% airway occluded
4. Grade 3: 51-75% airway occluded
5. Grade 4: >75% airway occluded

FRIEDMAN
1. Grade 0: Absence of tonsillar tissue
2. Grade 1: Tonsil within the pillars
3. Grade 2: Tonsil extends to the pillars
4. Grade 3: Tonsil extends past the pillars
5. Grade 4: Tonsils extend to the midline

Vancouver 503

Question 3

Regarding the palatine tonsils, discuss:
1. Embryology
2. Histology
3. Muscles of the tonsillar fossa
4. Anatomic relationships
5. Blood supply
6. Innervation
7. Lymphatics

Answer 3

EMBRYOLOGY:
- From endoderm of ventral portion of 2nd pharyngeal pouch
- Capsule = condensation of mesenchyme

HISTOLOGY:
- Encapsulated mass of lymphoid tissue with deep crypts (which are formed by cell apoptosis)
- HPV takes residence in basal cell layer at base of crypts
- Stratified squamous epithelium covers the palatine tonsil

MUSCLES OF TONSILLAR FOSSA:
1. Palatoglossus (anterior; pharyngeal branch of CNX)
2. Palatopharyngeus (posterior; pharyngeal branch of CNX)
3. Superior constrictor (lateral, pharyngeal plexus from CNX)

ANATOMIC RELATIONSHIPS:
- CNIX runs just deep to superior constrictor
- ICA runs 2cm posterolateral to deep surface
- In 1% (e.g. Velocardiofacial) the ICA runs just deep to superior constrictor (< 5mm from the surface)

ARTERIAL SUPPLY:
1. UPPER POLE
- Ascending pharyngeal –> tonsillar branch of ascending pharyngeal
- IMAX –> descending palatine –> lesser palatine
2. LOWER POLE
- Facial artery –> ascending palatine branch
- Facial artery –> tonsillar branch of facial artery (largest artery)
- Lingual artery –> Dorsal lingual artery –> tonsillar branch of dorsal lingual artery

VENOUS PLEXUS:
1. Tonsillar branch of lingual vein —> drains to lingual vein
2. Accessory tonsillar vein –> drains to pharyngeal plexus
3. External palatine vein (upper part) –> pierces through superior constrictor muscle (Main source of bleeding post-tonsil)

INNERVATION:
1. Lesser palatine nerve branches (descending branches)
2. CNIX –> damage causes referred otalgia via Jacobsen’s nerve

LYMPHATICS:
- Superior cervical and jugular digastric

Question 4

Regarding the adenoids, discuss:
1. Histology of adenoids & types of surface epithelium
2. Anatomical relationships
3. Blood supply
4. Innervation
5. Clinical course of adenoid

Answer 4

HISTOLOGY:
- Unencapsulated lymphoid tissue with deep crypts
- 60% B cells: in the Mantle zone (naive cells) & Follicular/Germinal Centres (site of maturation/differentiation
- 40% T-cells (CD4): Extra-follicular zone (Marginal/Lymphoid zone)
- Crypts create increased surface area for antigen exposure
- Antigen presented to T-cells –> induce B-cell differentation to IgA –> Local immunity

Three types of surface epithelium:
1. Ciliated pseudostratified columnar (respiratory epithelium)
2. Stratified Squamous (predominantly in chronically infected adenoids
3. Transitional epithelium

ANATOMICAL RELATIONSHIPS:
- Along midline posterior nasopharyngeal wall
- Just inferior to sphenoid rostrum
- Just superior to superior margin of superior constrictor muscle (Passavant’s ridge)
- Fossa of Rosenmuller: Space between adenoid bed & ET orifice (torus tubarius)
- Gerlach’s Tonsil: lymphoid tissue in fossa of Rosenmuller that extends into ET orifice

ARTERY BLOOD SUPPLY:
1. Ascending pharyngeal artery
2. Facial artery –> Ascending palatine branch + tonsillar branch of facial artery
3. IMAX –> pharyngeal artery –> artery of pterygoid canal

INNERVATION:
1. Pharyngeal plexus

CLINICAL COURSE:
- Increase in size until 6-7yo, diminish mid-puberty

Kevan Peds Question 22

Question 5

What is the adenoid’s role in immunity?

Answer 5

A. LOCAL & SECRETORY IMMUNITY
- Crypts trap foreign material & are surrounded by numerous aggregates of cellular mediators of the immune system
- Antigen presetned to T-cells in adenoid peri-follicular zones –> induce B cell differentation to IgA in follicular centres

B. SYSTEMIC IMMUNITY
- Crypts take up airborne antigens for exposure to immune system in lyphoid tissue

*Most active between ages 4-10 years
* No immune deficiency documented if removed

Question 6

Describe the clinical presentation of acute adenoiditis

Answer 6

1. Purulent rhinorrhea
2. Nasal obstruction
3. Otitis media
4. Fever

Question 7

Describe the clinical presentation of chronic adenoiditis

Answer 7

1. Chronic rhinorrhea
2. Chronic nasal congestion/obstruction (open-mouth breathing, snoring)
3. Hyponasal speech
4. Halitosis

Question 8

What is the definition of recurrent acute adenoiditis?

Answer 8

4+ episodes within 6 months (with symptom resolution between episodes)

Question 9

What are 7 features of “Adenoid Facies”? What causes this characteristic appearance?

Answer 9

Adenoid Facies:
1. Incompetent lip seal
2. Narrow upper dental arch
3. Increased anterior face height
4. Steep mandibular plane angle
5. Retrognathic mandible
6. Increased total and inferior anterior heights of the face
7. Increased anterior and inferior position of the hyoid

Caused Secondary to:
1. Changes in head and tongue position
2. Changes in muscular balance secondary to open mouth breathing (From nasal obstruction)

Question 10

List 4 absolute indications, 5 relative indications, and 4 contraindications for adenoidectomy

Answer 10

Absolute Indications:
1. Adenoid hyperplasia resulting in SDB or OSA, associated with cor pulmonale
2. Nasal obstruction associated with orofacial abnormalities
3. FTT (not attributable to other causes)
4. Suspected malignancy

Relative Indications:
1. Recurrent acute adenoiditis (5-7 infections in 1 year, 5infx/year x 2 years, 3 infx/year x 3 years, or >2 weeks of missed school or work in 1 year)
2. Chronic adenoiditis with persistent sore throat, halitosis, or cervical adenitis
3. Swallowing difficulties (not attributable to other causes)
4. Drooling within the context of adenoid hyperplasia
5. Recurrent or chronic otitis media (second set of tubes by ≥ 4 years old) or rhinosinusitis

Contraindications:
1. Cleft palate / submucous cleft - if SDB or OSA can consider superior partial adenoidectomy
2. Velopharyngeal insufficiency (submucous cleft palate, hypernasal speech, nasal regurgitation, any neurologic/muscular impairment causing impaired palate function)
3. Bleeding risk: Coagulation abnormalities (should be addressed prior to surgery)
4. Acute infection (impair ability to maintain adequate hydration or safely undergo general anesthesia)

Question 11

What are the risk factors for VPI after adenoidectomy?

Answer 11

1. History of nasal fluid regurgitation
2. Occult submucous cleft
3. Family history of clefts
4. Neuromuscular problems/hypotonia (CNS)

Question 12

What are the microbial components of normal oral flora?

Answer 12

1. Actinomyces
2. Bacteroides
3. Candida (30-60% carriers)
4. Clostrium
5. Diphtheroids
6. Eubacterium
7. Eikenella
8. Fusobacterium
9. Haemophilus
10. Lactobacillus
11. Leptotrichia
12. Neisseria
13. Nocardia
14. Peptococcus
15. Peptostreptococcus
16. Porphyromonas
17. Prevotella (bacteroides)
18. Propionibacterium
19. Streptococcus
20. Staphylococcus
21. Veillonella

Letters: ABCDEF HLLNNPPPPPSSV

*Routine surface cultures have limited utility for identifying causative organisms (especially in chronic inflammation)

Question 13

What is the most common microbial causing pharyngitis? What are other common pathogens?

Answer 13

Most common = Group A Strep (GAS) = Strep pyogenes
- Especially B-hemolytic strains (15-30% of bacterial cases of acute tonsillopharyngitis)

Other pathogens:
1. Group C beta-hemolytic streptococci
2. Neisseria gonorrhea
3. Corynebacterium diphtheria
4. Chlamydia Pneumoniae
5. Mycoplasma pneumoniae
6. Haemophilus influenza
7. Streptococcal pneumoniae
8. Moraxella catarrhalis
9. Staphylococcus aureus
10. Bacteriodes
11. Peptostreptococcus

Question 14

What are symptoms typical of acute tonsillitis?

Answer 14

2 or more needed for diagnosis:
1. Fever > 38.5
2. Erythematous or exudative tonsils
3. Tender cervical lympadenopathy > 2cm
4. Positive GABHS culture
5. Note: can also include dysphagia

Question 15

What are the symptoms of chronic tonsillitis?

Answer 15

1. Chronic sore throat
2. Halitosis
3. Tonsilliths
4. Peritonsillar erythema
5. Persistent tender cervical lymphadenopathy

Question 16

What are two diagnostic investigations for pharyngitis? What is the sensitivity/specificity/PPV of these?

Answer 16

1. Rapid Strep Test
   - Sensitivity 72-92% low
   - Specificity 89-96% high
   - PPV 68-89%
   - If rapid test negative –> throat culture; culture takes 24-48 hours, therefore treat empirically (rapid test = 10-15 minutes)
   - 20% children chronic carriers, 10% false negative rate
   - Gold standard = throat culture on sheep blood agar (95% sensitivity)
2. Monospot + blood count

Question 17

What are 4 indications for screening for Group A Beta-hemolytic streptococcus carrier state?
How do you distinguish this positive test from an active infection?

Answer 17

Screening indications:
1. Patients with family history or history of rheumatic fever
2. During a community outbreak of rheumatic fever, poststreptococcal glomerulonephritis, or invasive GAS infection
3. When tonsillectomy is being considered
4. When symptomatic GAS spreads among household members

Distinguishable from active infections by:
1. A rise in antistreptolysin-O-titer ≥ 0.2log10 between the acute and convalescent phase (2-4 weeks after presentation)
2. Relevant history of symptoms and signs during episode
3. Documented response to antibiotics

Question 18

What are the complications of GAS pharyngitis/untreated tonsillitis?

Answer 18

Suppurative complications:
1. Cervical lymphadenitis
2. Peritonsillar abscess (most common DNSI)
3. Parapharyngeal abscess (extension through superior constrictor muscle from tonsil)
4. Retropharyngeal abscess (spread via retropharyngeal lymph nodes)
5. Mediastinitis (spread via danger space behind the retropharyngeal space, before prevertebral fascia)

Nonsuppurative complications:
1. Rheumatic Fever/heart disease: generally follows after an episode of pharyngeal GABHS
- Polyarthritis
- Carditis (cardiomegaly, heart murmur - MVP/aortic valve disease, pericardial rub/effusion, CHF)
- Sydenham chorea (jerky, uncontrollable movements/purposeless)
- Truncal rash
- Treatment: refer to cardiologist, Penicillin prophylaxis

2. Scarlet Fever (strep endotoxin)
   - Variation of GABHS pharyngitis
   - Descending erythematous rash from neck to trunk and extremities
   - Strawberry tongue
   - Develops within first 24 hours of illness, fades within a week
3. PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder Associated with Strep): Phenomenon against neural cells
   - Presence of OCD symptoms and/or tics (sudden repetitive movements or sounds) which interfere with normal function
   - Age range: 3 years to puberty
   - Symptoms appear abruptly and dramatically with relapsing remitting course
   - Association with worsening of GAS infections (positive swab or anti-GAS titre)
   - Often no complaint of a sore throat
   - Underlying cause unknown, though to be abnormal immune response
   A. Diagnostic Criteria:
   - Obsessive-Compulsive disorder and/or tic disorder with onset between 3 years of age and puberty
   - Episodic course with abrupt onset of neuropsychiatric symptoms or dramatic exacerbations
   - Well-documented temporal relationship between symptom exacerbations and CABHS infections with culture confirmation
   - Abnormal neurologic exam with motoric hyperactivity and adventitious movements without frank chorea
   B. Treatment: Unclear evidence; steroids, plasma exchange, IVIg, tonsillectomy, antibiotics, behavioural therapy ± medication for neuropsychiatric symptoms
4. Glomerulonephritis: 1-2 weeks after streptococcal infection
   - No evidence that antibiotic therapy affects the natural history
5. Grisel syndrome
6. Airway obstruction

Question 19

What is the treatment regimen for GABHS pharyngitis?

Answer 19

1. Penicillin or amoxicillin x 10 days
2. Tylenol and/or NSAIDs for symptom management
3. Avoid ASA
4. Steroids not indicated
5. Failure –> Penicillin fails 7-37% of the time

Penicillin allergy: First generation cephalosporin, clindamycin, clarithromycin, azithromycin

Question 20

What are the possible causes of penicillin failure in treating GAS Strep pharyngitis?

Answer 20

1. Carrier state
2. Masked concomitant viral infection
3. Lack of compliance to medication
4. Protective effect for GABHS by beta-lactamase-producing bacteria (treat with Clavulin instead)
5. Lack of other bacteria that inhibit GABHS virulence
6. Poor pentration of pharyngeal tissues by penicillin
7. Antimicrobial resistance to penicillin
8. Recurrent exposure (re-infection)
9. Contaminated toothbrush (re-infection)

Question 21

What are the Centor Criteria for strep tonsillitis? What are the indications to use it?

Answer 21

Centor Criteria = Correlates with risk of positive throat culture for GAS

Indications to use: Children with pharyngitis (recent onset ≤ 3 days acute pharyngitis)
- Risk of GAS decreases with age into adulthood

CRITERIA:
1. Exudate or swelling of tonsils (Yes +1)
2. Tender/swollen anterior cervical lymph nodes (Yes +1)
3. Temperature > 38 degrees celcius (Yes +1)
4. Cough (No +1)
5. Age modification: < 15 years +1, >44 years -1

Total Score:
-1 to 0: Observe, no antibiotics, no throat culture
2 to 3: Throat culture, treat if positive
4 to 5: Rapid strep testing ± culture (no longer recommendations for empiric treatment; based on sympatomatology)

Question 22

What is the clinical significance of a unilateral enlarged tonsil?
What is the DDx in solid organ transplant patients?
When should NH lymphoma be considered?

Answer 22

Usually nothing! Tonsil is usually actually the same size, just more deeply recessed into the fossa

New onset unilateral tonsil enlargement: Likely due to infection, but should consider malignancy

Differential of unilateral symmetric enlargement in solid organ transplant:
1. Atypical mycobacteria
2. Mycobacteria (tuberculosis)
3. Actinomycosis
4. Fungal, toxoplasmosis
4. Lymphoproliferative disease

Sudden enlargemnet of Waldeyer ring = concern for lymphoma; suspect NH lymphoma if:
1. Not resolving > 2 weeks
2. B-symptoms
3. Other bodily lymphadenopathy

Question 23

What is the differential for a congenital tonsillar mass?

Answer 23

1. Teratoma
2. Hemangioma
3. Lymphatic malformation

Question 24

Regarding peritonsillar abscesses, discuss:
1. What are the borders of the peritonsillar space?
2. What are the possible etiologies of a peritonsillar abscess?
3. What are the treatment strategies?
4. What are the possible complications of a PTA?

Answer 24

BORDERS OF PERITONSILLAR FOSSA:
1. Anterior: palatoglossus
2. Posterior: palatopharyngeus
3. Lateral: Superior constrictor

POSSIBLE ETIOLOGIES:
1. Contiguous spread from tonsil parenchyma
2. Secondary infection of minor salivary gland at superior pole (Weber glands) - causing abscess to form in potential space between tonsil capsule + superior constrictor

TREATMENTS:
1. IV fluids
2. Analgesia: Tylenol, Narcotic PRN
3. Anti-inflammatory: Toradol (NSAID), Ibuprofen, Steroid (Dex 0.5-1mg/kg, max 10mg)
4. I&D (intraoral) - 75% effective
5. Antibiotics (IV, PO, or combined)
6. If unsuccessful I+D, CT scan, repeat I+D, consider hot Quinsy tonsillectomy (especially if child already undergoing GA)

COMPLICATIONS:
1. Dehydration
2. Airway obstruction
3. Deep neck space infections (spread to adjacent spaces)
4. Carotid artery erosion
5. IJV thrombophlebitis (Lemierre’s syndrome)
6. Sepsis

15% recurrence rate

Question 25

Regarding Lemierre’s syndrome, discuss:
1. What is the most common pathogen?
2. Explain the pathophysiology
3. What are the typical symptoms?
4. How is it diagnosed?
5. What is the treatment?
6. What are the possible complications?

Answer 25

Most common pathogen: Fusobacterium Necrophorum

PATHOPHYSIOLOGY:
- Throat infection –> PTA –> Spread to internal jugular vein through tonsil veins –> thrombosis (from bacterial endotoxin that induces platelet aggregation) –> septic emboli, septicemia

SYMPTOMS:
- Severe neck pain/stiff neck
- Spiking fevers
- SOB, chest pain (pulmonary and systemic emboli)
- Tender swelling along the SCM and angle of the jaw
- Lethargy in context of throat infection

DIAGNOSIS:
- CT: Ring enhancement and filling defect of the IJV (secondary to clot or purulence)

TREATMENT:
1. Beta-lactamase-resistant antibiotics (ceftriaxone, clindamycin/flagyl)
2. Heparin anticoagulation in the presence of thrombus progression or septic emboli
3. Worsening symptoms or abscess formation –> surgical ligation of IJV

COMPLICATIONS:
1. Retrograde spread of the thrombophlebitis –> cavernous sinus thrombosis (manage with IV antibiotics and critical care support)

Question 26

List at least 5-10 complications of chronic adenoid/tonsil hypertrophy

Answer 26

ADENOID HYPERTROPHY:
1. Nasal obstruction
2. Chronic or recurrent nasal discharge
3. Snoring
4. SDB
5. Recurrent otitis media
6. Eustachian tube dysfunction
7. Mouth breathing - dry mouth, dental caries
8. Hyponasal voice
9. Adenoid facies

TONSILLAR HYPERTROPHY:
1. Dysphagia
2. SDB
3. Voice changes
4. Dental malocclusion
5. Oropharyngeal fullness

Question 27

What are the symptoms and signs of obstructive tonsillar hyperplasia?

Answer 27

1. Enlarged tonsils
2. Snoring
3. Obstructive disturbances
4. Dysphagia and voice changes

Question 28

Define obstructive Sleep Disordered Breathing

Answer 28

Sleep disordered breathing (obstructive):
- Obstructive abnormality of the respiratory pattern or adequacy of oxygentation/ventilation during sleep
- Abnormal respiratory pattern during sleep that includes snoring, mouth breathing, and pauses in breathing

Spectrum:
Snoring (10%) –> Upper airway resistance syndrome –> obstructive hypoventilation –> obstructive sleep apnea (1-3%)

Question 29

List the absolute and relative indications for tonsillectomy (as many as you can think of)

Answer 29

ABSOLUTE INDICATIONS:
1. Suspected malignancy
2. Persistent or recurrent tonsillar hemorrhage (e.g. uncontrollable hemorrhagic tonsillitis)
3. Tonsillar hyperplasia resulting in SDB or OSA (AHI > 5 commonly used)
4. OSA or SDB associated with complications (FTT, Cor Pulmonale, pHTN)
4. FTT (not attributable to other causes)
5. Tonsillitis resulting in febrile convulsions (may require Quinsy tonsillectomy)

RELATIVE INDICATIONS:
1. Recurrent acute tonsillitis per PARADISE criteria (strong rec to watchful wait until this criteria is met)
- 7 episodes/year x 1 year
- 5 episodes/year x 2 years
- 3 episodes/year x 3 years
- An episode requires at least sore throat plus one of: fever >38.3, LAD, exudates, or +GABHS test
2. Recurrent tonsillitis not meeting above criteria, but with multiple antibiotic allergy/intolerances, or complications or tonsillitis (PANDAS, PFAPA, at least 2 PTAs)
3. OSA/SDB causing poor school performance, growth retardation, enuresis, asthma, and behavioural problems
4. Chronic tonsillitis with persistent sore throat, halitosis, or cervical adenitis
5. Dysphagia (not attributable to other causes)
6. Drooling within the context of severe tonsillar hyperplasia
7. Tonsillolithiasis
8. Orofacial or dental disorders (results in a narrow upper airway)
9. Streptococcus carrier unresponsive to medical management
10. Recurrent or chronic otitis media

Poorly validated indications:
1. PANDAS
2. PTA
3. Chronic tonsillitis
4. Febrile seizure
5. Muffled (“hot potato”) voice
6. Halitosis
7. Malocclusion of teeth
8. Tonsillar hypertrophy
9. Cryptic tonsillitis
10. GABHS carrier

Question 30

What are contraindications to tonsillectomy?

Answer 30

Absolute:
1. Leukemia
2. Hemophilia
3. Agranulocytosis
4. Uncontrolled systemic disease (e.g. diabetes, TB)

Relative contraindications:
1. Cleft palate
2. Acute infection

Question 31

What are the long term effects of tonsillectomy with respect to immunity?

Answer 31

• Decrease in serum IgA
• Possibly a decreased response to the polio vaccine
• Generally, there are no noticeable effects

Question 32

What medication does the AAO-HNS 2019 guidelines strongly recommend administering intraoperatively for tonsillectomy, and the dose?

Answer 32

Dexamethasone 0.5mg/kg to reduce post-operative nausea and vomiting (NNT = 4)

Question 33

What medications does the AAO-HNS 2019 guideline strongly recommend for post-tonsillectomy management of pain?

Answer 33

1. Ibuprofen 5-10mg/kg/dose q6-8h
2. Acetaminophen 10-15mg/kg/dose q4-6h

Question 34

What medications should be avoided peri-op for tonsillectomy according to AAOHNS 2019 guidelines?

Answer 34

1. Antibiotics
2. Injection of local anesthesia
3. Ketorolac (other NSAIDs okay)
4. Codeine (do not prescribe if < 12 years old)

Question 35

Why is codeine problematic in children according to the AAO HNS 2019 guidelines?

Answer 35

Substantial variation in the activity of cytochrome p450 enzyme CYP2D6 that converts codeine into the active metabolite
- Slow metabolism - can render codeine ineffective
- Fast metabolism - risk of respiratory suppression

Question 36

Define the Paradise criteria for tonsillitis

Answer 36

Frequency
- 7 episodes in 1 year
- 5 episodes / year x 2 years
- 3 episodes / year x 3 years

Clinical features: Must have sore throat plus one of:
- Fever > 38.3
- Tonsillar exudates
- Cervical lymphadenopathy (tender or size >2cm)
- Positive culture for GABHS

Documentation of episodes:
- If episodes not documented, observe two episodes of throat infection if frequency and clinical features consistent with initial history (observe over a 12 month period)

CPG GUIDELINES: STRONG recommendation for watchful waiting until this criteria is met before considering tonsillectomy

Question 37

Regarding PFAPA, discuss:
1. What does it stand for?
2. What are the symptoms? How is it diagnosed?
3. What lab tests should be done?
4. What is the pathophysiology?
5. What is the natural history
6. What is the differential for recurrent fevers?
7. What is the etiology?
8. What is the treatment options?

Answer 37

PFAPA = Periodic Fever, Aphthous ulcers, pharyngitis, and adenitis

DIAGNOSIS:
1. Prodromal phase of malaise, irritability, and fatigue precedes onset of fever
2. Fevers (38.5 - 41) last 3-6 days
3. Episodes occur every 2-8 weeks with asymptomatic intervals
4. Strict criteria - association of periodic fevers with at least one of the following:
- Aphthous stomatitis
- Cervical adenitis
- Pharyngitis

OTHER SYMPTOMS:
1. Headaches
2. Diarrhea
3. Arthralgia
4. Rash
5. Chills
6. Abdominal Pain

LAB TESTS:
1. Mild leukocytosis
2. Elevated ESR

PATHOPHYSIOLOGY:
- Etiology unknown
- Immunologic dysregulation
- Activation of proinflammatory cytokines - hyperactive Th1 IL1 cytokines
- Suppression of anti-inflammatory responses

NATURAL HISTORY:
- Age of onset ~2-5 years of age
- Some experience resolution of symptoms after months - years
- Majority persistent symptoms with increased length of intervals over time
- Typically lasts > 4 years

DDX OF RECURRENT FEVERS:
1. Hyper-IgD syndrome (autosomal recessive dx of recurring fevers/chills attacks in first year of life, possible triggered by vaccine or mild trauma)
2. Cyclic neutropenia
3. Familial Mediterranean fever - recurrent fever, serositis; autoinflammatory (genetic)
4. Hereditary periodic fevers
5. Bechet disease (blood vessel inflammation through body - mouth sores, eye inflammation, rashes, genital sores)

ETIOLOGY: UNKNOWN

TREATMENT OPTIONS:
1. Corticosteroids (reduce symptoms, resolve fevers, reduce intervals between episodes) - single high dose of prednisone at the onset of fever
2. Cimetidine (Colchicine - antiinflammatory) - H2-receptor antagonist: for prophylaxis, outcomes variable
3. Definitive treatment: Tonsillectomy for severe pharyngitis symptoms
- Significant improvement in chance of total resolution or longer duration between episodes (Meta-analysis 2010 with 2 RCTs)

Question 38

What factors predict malignancy in unilateral tonsillar enlargement?

Answer 38

1. Rapid enlargement that doesn’t go down (if this symptom alone, re-check in 2 months)
2. B-symptoms
3. Nodes
4. Pain
5. Dysphagia
6. Mucosal abnormality
7. Concomitant immunosuppression

Question 39

What is the current guideline for perioperative steroid and antibiotic administration for tonsillectomy?

Answer 39

Intraoperative Steroids Statement 11:
STRONG recommendation - Clinicials should administer a singel intraoperative dose of IV dexamethasone to children undergoing tonsillectomy

Pre-operative antibiotics Statement 10:
STRONG recommendation AGAINST - Clinicals should NOT administer or prescribe perioperative antibiotics to children undergoing tonsillectomy

AAO CPG: Tonsillectomy in children 2019 update

Question 40

What are the indications for admission post-T+A? Discuss the AAO indications, as well as a full list

Answer 40

Statement 12 Recommendation - Inpatient monitoring for children after tonsillectomy
1. < 3 years old
2. Severe OSA (AHI ≥10, oxygen saturation nadir < 80%, or both)
- “REM rebound” may occur in severe OSA patients after 18 hours, which may cause hypoventilation and hypoxia
- “REM rebound” = increased frequency, depth, and intensity of REM sleep following sleep deprivation or significant stressors (ie. from having OSA)

Additional Indications for admission:
1. Syndromic / craniofacial anomalies
2. Comorbidities (e.g. DM, seizure disorders, sickle cell disease, neuromuscular disorders)
3. Obesity (> 99th percentile BMI), especially if no PSG to quantify severity
4. Live > 1 hour from hospital
5. Unreliable parents
6. Bleeding disorders
7. Postoperative recalcitrant emesis
8. Intraop airway obstruction
9. Post-obstructive pulmonary edema
10. Sickle cell (risk of pain crisis)
11. Current respiratory infection

Indications for ICU admission:
1. Severe OSA
2. Comorbidities that cannot be managed on ward
3. Significant airway obstruction
4. Oxygen desaturation in the recovery room that is unresponsive to repositioning and oxygen

AAO CPG: Tonsillectomy in children 2019 update

Question 41

Regarding post-obstructive pulmonary edema, discuss:
1. What is the clinical presentation
2. What is the etiology and pathophysiology of POPE I vs. POPE II?
3. What is the treatment?

Answer 41

CLINICAL PRESENTATION:
1. Tachypnea, tachycardia, rales, ronchi
2. Pink, frothy secretions
3. Hypoxemia
4. Bilateral expiratory wheeze
5. CXR –> increased pulmonary markings/ fluid overload

ETIOLOGY:
1. POPE I = Occurs after sudden, acute severe upper airway obstruction
- Forceful attempts to inhale against an obstruction causes negative intrathoracic pressure
- Leads to increased venous return
- Decreased cardiac output
- Fluid transudation into alveolar space (exuding/slow escape of liquids from vessels through pores/breaks in membrane)

2. POPE II = Occurs after surgical relief of chronic upper airway obstruction
   - Obstruction lesion produces modest PEEP
   - Increases end-expiratory volumes
   - Removing obstruction removes the PEEP returning lung volumes and pressures to normal
   - The interstitial fluid does not resolve immediately
   - Sudden removal of the PEEP causes transudation of interstitial fluid (fluid goes out into less pressured alveolus)

TREATMENT:
1. Positive end expiratory pressure (PEEP) - CPAP/BIPAP/ETT - “push fluid back out of alveolus”
2. Lasix (pediatric dose = 1mg/kg IV)
3. Fluid restriction

Question 42

List all the possible complications of tonsillectomy + adenoidectomy (at least 15). Discuss the etiology of why each occurs, and treatment options for them.
What is the rate of mortality post-T+A?

Answer 42

Mortality: 1: 16000-35000 (anesthetic complications and hemorrhage)

A. GENERAL COMPLICATIONS
1. Throat pain, otalgia, post-op nausea/vomiting, delayed feeding, voice changes, hemorrhage, death, halitosis
2. TRAUMATIC complications: injury to eyes, lips, tongue, teeth, nerves, local vascular structures, larynx, pharyngeal wall, soft palate; jaw dislocation, and fracture of the mandibular condyle

B. HEMORRHAGIC COMPLICATIONS (most common serious complication 0.5-5%)
Types:
1. INTRAOPERATIVE BLEEDING: arterial injury such as aberrant carotid artery, retained tonsillar tissue, tears in posterior pharyngeal wall
2. IMMEDIATE POST-OPERATIVE BLEEDING (< 24 hours; 0.2-2.2%): May be due to inadequate hemostasis during surgery, caused by same reasons as intraoperative
3. DELAYED POST-OPERATIVE BELEDING (> 24 hours; 0.1-3%): Usually 5-10 days post-op, due to eschar sloughing

Treatment of hemorrhagic complications:
1. Intraoperative pressure packing
2. Hemostatic agents (e.g. thrombin, cellulose, microfibrillar collagen)
3. Suction cautery
4. Sutures
5. Posterior nasopharyngeal pack (for recalcitrant intraoperative bleeding)
6. External carotid artery ligation (life-threatening bleeding)
7. Embolization
8. Very mild delayed post-operative bleeding may sometimes be addressed in the treatment room with a cooperative patient with topical hemostatic agents, if unsuccessful proceed to operating room

C. NONHEMORRHAGIC COMPLICATIONS
1. ANESTHETIC COMPLICATIONS
- Airway fire (treatment: avoid airway leaks, remove ETT, water irrigation)
- Laryngospasm
- Difficult intubation
- Endotracheal tube kinking
- Iatrogenic premature extubation
- Postoperative opioid overmedication

2. POST-OPERATIVE AIRWAY COMPROMISE (Rare)
   - May occur from dislodged clot, dislodged adenotonsillar tissue, postoperative oropharyngeal edema, or retropharyngeal hematoma
   - Treatment: manage airway (reintubation, surgical airway), corticosteroids, address retropharyngeal hematoma
3. DEHYDRATION
   - Secondary to pain
   - Treatment: Analgesics, parenteral hydration
4. PULMONARY EDEMA (POPE) - see card on POPE
5. VELOPHARYNGEAL INSUFFICIENCY
   - Due to incompetent velopharyngeal inlet
   - Increased risk: submucosal cleft palate, history of nasal regurgitation, pre-operative hypernasality
   - Treatment: Speech therapy (typically resolves) pharyngeal flap or palatal lengthening for persistent problems, testing for 22q11 deletion if persistent
6. NASOPHARYNGEAL STENOSIS
   - Rare complication from scarring
   - Treatment: Difficult (high rate of recurrence), may consider pharyngeal flaps or stents
7. EUSTACHIAN TUBE DYSFUNCTION
   - From scarring and stenosis of the eustachian tube orifice
   - Treatment: Pressure equalization tubes, prevent by avoiding curretage or cautery near torus tubarius
8. ASPIRATION PNEUMONIA
   - Occurs from aspiration of blood clots of adenotonsillar tissue
9. ATLANTOAXIAL (C1-C2) SUBLUXATION (Grisel Syndrome)
   - Spread of inflammation from the oropharyngeal to the cervical ligaments resulting in laxity and spinal cord compression
   - Increased risk: Down syndrome
   - Treatment: Orthopedic consult, may need antibiotics, cervical collar
10. EAGLE SYNDROME
    - Long styloid process previously covered by the tonsils or new osteitis and reactive styloid calcification
    - Symptoms: Unilateral neck pain, sore throat, dysphagia
    - Treatment: NSAIDs, pain control, local steroid injection, partial styloidectomy
11. SICKLE CELL CRISIS
    - Consider pre-operative CBC and sickle cell screen in African American Children
    - See Sickle cell card (under Misc) for further sickle cell crisis management detials
12. NONRESOLUTION OF SYMPTOMS
    - Parental counsellinig on this possibility is important
    - Increased risk for syndrome (e.g. T21) or obesity (60-80% persistent SDB)

Question 43

What are 7 possible causes of desaturation post T+A?

Answer 43

1. Upper airway edema
2. Laryngospasm (usually on emergence)
3. Post-obstruction pulmonary edema
4. Central apnea
5. Hypoventilation secondary to loss of hypercapnic drive
6. Blood aspiration
7. Narcotic overdose

Question 44

Regarding Grisel’s syndrome, discuss:
1. What is it?
2. What are the possible etiologies? What are the typical causative organisms?
3. What is the epidemiology and risk factors?
4. What is the clinical presentation?
5. Discuss the pathophysiology.
6. What are the radiographic features?
7. What is the treatment?

Answer 44

DEFINITION:
- Non-traumatic atlantoaxial subluxation, usually after head/neck infections
- Rare cause of torticollis

ETIOLOGY: Infection or inflammation of the anterior transverse ligament of cervical spine, causing laxity
1. Retropharyngeal abscess
2. URTI
3. Tonsillectomy/T+A
4. Otitis media

ORGANISMS:
1. Staphylococcus aureus
2. Group B Strep
3. Oral flora

PATHOPHYSIOLOGY:
- Adenodiectomy breaches pre-vertebral fascia –> C1-2 infection/inflammation –> inflammation causes vertebral body decalcification and higher flexibility of ligaments and increases anterior transverse ligament laxity –> neck pain and stiffness –> subluxation between axis and atlas of the cervical spine/skull base
- May manifest up to 1 week post-op
- When inflammation heals, the child may experience neck fixation in a rotated position

EPIDEMIOLOGY/RISKS:
1. Infants or young children
2. Down Syndrome
3. Marfan syndrome

CLINICAL PRESENTATION:
1. Torticollis
2. Cervical pain with active or passive rotation
3. Symptoms related to underlying infection
4. Neurological complications from radiculopathy to death from medullary compression

RADIOGRAPHIC FEATURES:
1. Best investigation is CT (Vancouver says best is MRI to assess ligaments - depending what you’re looking for)
2. Abnormal lateral placement of C1 on C2 and of occipital facets on C1
3. MRI useful for evaluating surrounding tissues

TREATMENT:
1. Neck immobilization: soft collar
2. Conservative: Anti-inflammatories, muscle relaxants, bed rest
3. Broad-spectrum antibiotics
4. Consider spine service consult for assessment (may need traction if very severe or unresponsive to conservative management)
5. Surgical: If no spontaneous reduction with conservative treatment = closed reduction in OR; if irreducible subluxation = craniocervical fusion
6. Residual subluxation after 8 weeks of treatment or neurological symptoms may require operative treatment

Question 45

Regarding Atlantoaxial subluxation, discuss:
1. What is atlantoaxial subluxation?
2. List a complete differential for etiologies of atlantoaxial subluxation.
3. What are the mechanisms for how this occurs? List a few possibilities
4. How is Atlantoaxial Subluxation/Dislocation diagnosed?

Answer 45

DEFINITION:
- Disorder of C1-C2 causing impairment in rotation of the neck
- The anterior facet of C1 is fixed on the facet of C2
- It may be associated with dislocation of the lateral mass of C1 on C2

ETIOLOGIES:
A. CONGENITAL
1. Os Odontoideum (anatomic variant of odontoid process of C2 - hypoplastic dens)
2. Down Syndrome (20%)
3. Morquio Syndrome (genetic condition that affects child’s bones/spine)
4. Spondyloepiphyseal Dysplasia
5. Osteogenesis imperfecta (inherited brittle bone disease)
6. Marfan disease (affects connective tissue)
7. Neurofibromatosis Type 1 (NF1 - associated with decreased bone mineral density)
8. Hurler’s / Mucopolysaccharoidosis MPS Type 1 (Lysosomal storage condition also affects bone function
9. Achondroplasia / Dwarfism (ossification of cartilage)

B. INFLAMMATORY
1. Rheumatoid arthritis
2. Psoriatic arthritis
3. Reiter Syndrome (reactive arthritis) - caused by infection
4. Ankylosing spondylitis - causes vertebrae to fuse overtime
5. Systemic Lupus Erythematosus (SLE) - Causes lupus myelitis/transverse myelitis (inflammation of spinal cord)

C. ACQUIRED
1. Trauma
2. Grisel Syndrome (and its infectious causes)
3. Surgery
4. URTI

MECHANISMS OF SUBLUXATION:
1. Congenital fusion of atlas & occiput
a. Weakens transverse ligament holding odontoid process to atlas, causing instability and can result in foramen magnum compression
2. Anteroposterior Subluxation
3. Rotatory Subluxation (aka. Atlantoaxial Rotatory Fixation AARF), characterized by 4 types:
a. Type I: The atlas is rotated on the odontoid with no anterior displacement
b. Type II: The atlas is rotated on one lateral articular process with 3-5mm of anterior displacement
c. Type III: Comprises a rotation of the atlas on both lateral auricular processes with anterior displacement greater than 5mm
d. Type IV: Characterized by rotation and posterior displacement of the atlas
4. Vertical subluxation
5. Lateral subluxation

RADIOGRAPHIC FEATURES:
1. PLAIN RADIOGRAPH (x-ray)
- In a non-traumatic setting - Flexion and Extension views may be performed
- The expected distance between anterior arch of C1 and the dens in the fully flexed position should be < 3mm in an adult (5mm in a child)
- In a vertical subluxation, the dens is often above the McGregor line by over 8mm in men, and 9.7mm in women
- McGregor Line: Horizontal line drawn from posterior edge of the hard palate to most caudal point of occiput curve. If dens significantly above this line –> suggest vertical subluxation. Normal ~4.5mm

2. CT
   - On CT, C1 is not oriented in line with the head. Head may be pointed anteriorly, C1 is turned. If this is a fixed defect, C2 is rotated in conjunction with C1

Kevan Peds Question 43

Question 46

What are the indications for Polysomnography in kids prior to T+A?

Answer 46

Statement 5 (Recommendation): INDICATIONS FOR POLYSOMNOGRAPHY (Prior to Tonsillectomy)
1. < 2 years of age
2. Obesity
3. Down syndrome
4. Craniofacial abnormalitiies
5. Neuromuscular disorders
6. Sickle cell disease
7. Mucopolysaccharidoses

Statement 6 (Recommendations):
Clinician should advocate for PSG priior to tonsillectomy for OSA/SDB in children without any of the comorbidities listed in KAS5 for whom the need for tonsillectomy is uncertain or when there is discordance between the physical examination and reported severity of SDB

AAO CPG: Tonsillectomy in Children 2019 update

Question 47

Regarding Achondroplasia, discuss:
1. What are the genetics
2. What are the factors/features of achondroplasia that make them susceptible to OSA/sleep disorders
3. How should these kids be worked up prior to T+A?
4. What is the treatment for these patients with sleep disorders?

Answer 47

GENETICS:
- Autosomal Dominant syndrome
- Mutation in fibroblast growth factor receptor 3 gene (FGFR3) –> causes ossification of cartilage
- Most common form of dwarfism

FEATURES INCREASING SLEEP DISORDERS RISK:
1. May have mixed apnea syndrome
2. Compression of the brainstem at cervical junction may impact ventilatory drive –> central apnea
3. Cervicomedullary compression –> affects upper airway control –> OSA
4. Maxillary/midfacial hypoplasia
5. Dysplasia of the basiocciput
6. Foramen magnum stenosis
7. Thoracic cage restriction

WORKUP:
- PSG
- Neurology and Respirology consultations

TREATMENT:
1. T+A
2. Ventriculoperitoneal shunt
3. Foramen magnum decompression

Question 48

Regarding Down Syndrome, discuss:
1. What is the incidence of OSA?
2. What are the anatomic factors that cause OSA?
3. What is the treatment for OSA in Down Syndrome?

Answer 48

INCIDENCE:
- 57%
- All patients with Down Syndrome should have a PSG by 4 years of age

ANATOMIC CAUSES FOR OSA:
1. Midfacial and maxillary hypoplasia
2. Macroglossia
3. Narrow nasopharynx
4. Shortened palate
5. Generalized hypotonia
6. Obesity

TREATMENT:
1. T+A (persistent abnormal PSG in 80% post-op, but most will have at least 50% reduction in AHI)
2. Nasal CPAP
3. UPPP
4. Tongue reductiion
5. Genioglossus advancement (+ radiofrequency ablation = 58% successful rate)
6. Lingual tonsillectomy
7. Radiofrequency ablation of the tongue base (RFA)
8. Supraglottoplasty
9. Tracheostomy

Question 49

What are the symptoms and complications of OSA in children?

Answer 49

DAYTIME SYMPTOMS
1. Open-mouth breathing
2. Frequent nasal obstruction
3. Hyperactive behaviour
4. Aggressive behaviour
5. Attension deficit disorder
6. Poor school performance
7. Daytime somnolence

NIGHTTIME SYMPTOMS
1. Frequent loud snoring
2. Gasping or choking
3. Frequent nighttime sweating
4. Witnessed apneas
5. Paradoxical breathing
6. Frequent position changes/restless sleep
7. Hyperextension of the neck
8. Nocturnal enuresis

OTHER COMPLICATIONS:
1. Dental carries
2. Dental malocclusion
3. Poor brain development (REM necessary for neuropsych development)
4. Growth retardation (REM necessary for hormonal secretion, especially GH)
5. HTN
6. Pulmonary HTN
7. Cor Pulmonale (RV failure secondary to pHTN)
8. FTT

Question 50

How does OSA syndrome typically present in adults compared to in children?

Answer 50

In adults compared to kids:
- Snoring intermittent vs continuous
- Mouth breathing rare vs common
- Obesity common vs rare
- Failure to thrive & enuresis rare vs common
- Daytime somnolence common vs rare
- Hyperactivity, attention deficit, aggression rare vs common
- Nighttime arousals common vs rare
- Gender predilection male vs none
- CPAP mainstay vs selective (postop OSA,
craniofacial anomaly, where surgery is
contraindicated)
Surgery selective vs mainstay

Question 51

What is the pathophysiology behind enuresis in OSA?

Answer 51

1. Negative intrathoracic pressure –> cardiac stretch –> release ANP (smooth muscle relaxant) –> inhibits ADH –> increases urination
2. Increases SNS bladder stimulation - increase bladder tone, decrease volume capacity (because OSA increases sympathetic response by increasing catecholamines)
3. Decreased arousal to micturition stimulus

Question 52

Define OBSTRUCTIVE APNEA and HYPOPNEA in PEDIATRICS. What are some other concerning findings on PSG?

Answer 52

Apnea: Near-complete cessation of airflow for at least 2 respiratory cycles (resp cycle = inhale exhale) despite ongoing respiratory effort
1. ≥ 90% drop in pre-event baseline of airflow
2. Lasting ≥ 2 consecutive breaths
3. Associated respiratory effort

Hypopnea = partial upper airway obstruction results in reduction of airflow > 30% for at least 2 respiratory cycles with either an arousal or a desaturation > 3%
1. ≥ 30% drop in pre-event baseline of airflow
2. Lasting ≥2 consecutive breaths
3. ≥ 3% desaturation OR arousal

Mild = 1-4
Moderate = 5-10
Severe = > 10

Other concerning findings on PSG:
- O2 < 92% (aka. Hypoxemia)
- Desaturations > 4% x 3+ /hour with HR change (up or down) > 25%
- End-tidal CO2 > 45mmHg for >60% of sleep, or >50mmHg for >8% of sleep, or >53mmHg at all

Question 53

Describe the OSA snoring scale for pediatrics

Answer 53

AHI: Apnea Hypopnea index
- Number of obstructive apneas and hypopneas per hour of sleep

1. Normal: AHI ≤ 1, SpO2 nadir > 92%
2. Mild OSA: AHI >1, ≤5
3. Moderate OSA: AHI >5 and ≤ 10
4. Severe OSA: AHI > 10, SpO2 nadir < 80%

Question 54

What are the abnormal polysomnogram criteria for pediatric OSA?

Answer 54

1. AHI ≥ 1
2. Oxygen desaturation to < 92%, >4% x > 3 times/hour, or associated with a change in heart rate > 25%
3. End tidal CO2 > 50mmHg for >8% of total sleep time (or >53 at any point), or > 45mmHg for > 60% of total sleep time
4. SpO2 levels:
   - >92% = normal
   - 86-91% = mild
   - 76-85% = moderate
   - < 75% = severe

Question 55

What is the definition of pediatric CENTRAL apnea?

Answer 55

1. ≥90% drop in pre-event baseline of airflow
2. Absence of respiratory effort
3. One of the following is met:
   - ≥20 seconds
   - ≥ 2 breaths AND either: associated with arousal OR ≥3% desaturation

If < 1 year old:
- ≥2 breaths AND either: heart rate decreased < 50bpm for 5sec, OR < 60bpm for 15 sec

Question 56

Define Respiratory Event Related Arousal

Answer 56

1. Lasting ≥ 2 consecutive breaths; AND
2. Increased respiratory effort; OR either:
   - Flattening of the inspiratory portion of the flow waveform
   - Snoring
   - Elevation in end-tidal CO2
3. Causing arousal from sleep

Question 57

Define Hypoventilation

Answer 57

• > 25% of the total sleep time with a PaCO2 > 50mmHg

Question 58

What are indications of polysomnography post-T+A

Answer 58

1. Persistent snoring or other symptoms of sleep-related upper airway obstruction 4-6 weeks post-operatively
2. Significant complications from OSA (e.g. Cor pulmonale, FTT)
3. Severe OSA on initial sleep study (T+A not as effective if very severe, doesn’t decrease AHI all the way)
4. < 1 years old, to ensure resolution of nighttime breathing abnormalities

Question 59

What are the high risk groups for pediatric OSA syndrome?

Answer 59

1. Abnormal airway anatomy: Downs, PRS, AChondroplasia, Craniosynostoses, Treacher collins, Macroglossia, Klippel-Feil, Pyriform aperture stenosis, Laryngomalacia, Masses, clefts
2. Neuromuscular disease - CP, Down
3. Hypothyroidism
4. Muscular dystrophies
5. Seizures
6. Prader willi
7. Chiari malformations
8. CVS - pulmnonary/systemic HTN, congenital heart disease
9. Morbid obesity
10. Pectus excavatum/scoliosis
11. Male
12. < 6 years

Question 60

What are risk factors that T+A will not cure OSA? What is the typical success rate in normal kids?

Answer 60

Risks:
1. Severe OSA (RDI > 19.1)
2. Obesity
3. Craniofacial and mandibular anomaliies
4. Asthma
5. Age > 7 years (likely multifactorial problem)

Overall success rate in normal non-syndromic kids = 77-83%

Question 61

What are the indications for polysomnography post-operatively for pediatric OSA?

Answer 61

1. Persistent snoring
2. Preoperative severe OSA (AHI > 10)
3. Preoperative OSA complications
4. Age < 1 year old

Question 62

What are further work-up options and surgical options for persistent OSA following T+A?

Answer 62

WORKUP:
1. Repeat PSG
2. Muller maneuver with endoscopy (if child can cooperate) - forced inspiration against a closed nasal + oral airway; determiines the most likely sites of airway collapse
3. Cine MRI: Looking for base of tongue/lingual tonsil collapse to post-pharyngeal wall - 120 slices over 2 minutes while child is experiencing obstruction
4. Sleep endoscopy - DISE (VOTE criteria 0-2)

SURGICAL OPTIONS:
1. UPPP: Children at high risk for persistent obstruction secondary to neuromuscular dysfunction after T+A
2. Midline glossectomy - US tech should locate lingual arteries intra-operatively pre-procedure, then make midline incision and widen using coblator
3. BOT reduction (via radiofrequency needle probe)
4. Lingual tonsillectomy
5. Repose technique (suturing tongue to mandible) –> not often used (glossopexy)
6. Relieve other levels of obstruction (larynx: supraglottoplasty; Nose: FESS, septoplasty, nasal valve surgery, inferior turbinate reduction, etc.)
7. Manidbular advancement
8. Tongue-hyoid suspension
9. Tracheostomy

Question 63

Describe how a DISE is performed

Answer 63

DISE = Drug induced sleep endoscopy
- Assessment of the upper airway using a flexible endoscope while patients are in a pharmacologically-induced sleep to mimic natural sleep

Question 64

What are the indications for DISE?

Answer 64

1. Persistent OSA after T+A
2. Prior T+A for patients at high risk for persistent OSA (Obesity, Down syndrome, Craniofacial anomalies, Neurologic Impairment)
3. Significant symptoms of sleep disordered breathing /OSA with small tonsils and adenoids
4. Occult or sleep-state dependent laryngomalacia
5. Prior hypoglossal nerve stimulator treatment

Question 65

What are the advantages of cine MRI over DISE?

Answer 65

1. Simultaneously assess multiple levels of obstruction
2. Better to qualify the size of the lingual tonsils and distinguish base of tongue obstruction from lingual tonsillar hypertrophy
3. Better overall view of the airway and allows one to observe both primary and secondary causes of obstruction

Question 66

What are the key elements of peri-operative management for Down’s Syndrome kids undergoing T+A?

Answer 66

PREOPERATIVE:
- Lateral neck x-ray in extension and flexion (rule out atlantoaxial instability)

INTRAOPERATIVE:
- Use ETT 2 sizes smaller than usual (often SGS)
- Don’t suspend the mouth gag
- Be careful when intubating with laryngoscope (pulling forward motion that subluxes the skull)

POSTOPERATIVE:
- Greater risk for post-op obstruction, desaturations, and dehydration

Question 67

Regarding nasal corticosteroid use in kids, discuss:
1. Mechanism of action
2. Specific cellular effects that occurs with nasal corticosteroid use

Answer 67

MECHANISM OF ACTION:
1. Binds to steroid-receptor in cytoplasm to allow entry into nucleus
2. Enters the nucleus to bind with the steroid binding receptor on chromatin –> downregulates the transcription of mRNA –> downregulates the manufacture of inflammatory proteins

Reasons it takes minimum 30 days for nasal corticosteroids to work:
1. Inhibits mast-cell degranulation
2. Inhibits release of cytokines, ILs, chemotactic factors
3. Decreases mucous production

SPECIFIC CELLULAR EFFECTS:
1. Decrease stability of mRNA coding for & production of IL-1/2/6/8, TNF and GM-CSF
2. Reduce leukocyte adherence to vascular endothelium
3. Limits leukocyte exit from vascular compartment (therefore, get increased serum leukocytes)
4. Decrease number of criculating T-cells (reduce stimulatory creation by IL-2, increase apoptosis)
5. Decrease IL-4/5 secretion –> decrease eosinophil/basophil chemotaxis
6. Promote eosinophil apoptosis
7. Reduce degranulation of eosinophils, mast cells
8. Decrease clearance of opsonized bacteria
9. Decrease expression of Class II HLA molecules to macrophages (to prevent kick-off of inflammatory cascade)
10. Suppress COX-2 (therefore, decrease pro-inflammatory prostaglandin production)

Question 68

Regarding nasal corticosteroid use in kids, discuss:
1. Indications
2. Risks
3. Recommended formulas
4. Recommended Dosage
5. Pharmacokinetics

Answer 68

INDICATIONS:
1. Allergic rhinitis
2. OSA
3. Adenoid hypertrophy
4. Nasal obstruction

RISKS:
1. Growth suppression (warning label)
2. Adrenal suppression
3. Glaucoma
4. Skin thinning
5. Epistaxis

FORMULAS:
A. LOWER RISK (< 1% - high binding, low systemic availability)
- Mometasone (Nasonex), Fluticasone (Flonase, Avamys), Ciclesonide (Omnaris),
B. HIGHER RISK
- Budesonide (Rhinocort, Pulmicort nebs)

DOSAGES:
A. Kids < 12 years: 1 spray (50mcg) to each nostril daily - no increased benefit shown with higher doses
B. 12 years: 2 sprays (100mcg) to each nostril daily
C. 12 years for nasal polyps: 2 sprays (100mcg) to each nostril BID

PHARMACOKINETICS:
1. 20% swallowed - 1st pass metabolism (digested)
2. ~10% absorbed systemically - binds to albumin (varying degrees with different preparations)

Question 69

Discuss the Childhood Adenotonsillectomy trial (CHAT)

Answer 69

Research Question: Watchful waiting vs surgical treatment for the treatment of obstructive sleep apnea syndrome in school age children

Population:
- 464 children, 5 to 9 years of age, with obstructive sleep apnea syndrome
- Randomized to early adenotonsillectomy or a strategy of watchful waiting

Watchful waiting Results:
- 7 month follow up
- Did not significantly improve attention or executive function
- Reduced symptoms and improve secondary outcomes of behavior, quality of life, and polysomnographic findings
- Thus, providing evidence of beneficial effects of early adeno-tonsillectomy

Question 70

What is coblation tonsillectomy?

Answer 70

Dissociation of isotonic saline into sodium ions between electrodes, breaking molecular bonds (45-85 degrees)