PEDIATRICS ANSWERS • Congenital cysts

• Extrinsic compression, Vascular (innominate artery, right

1. Q: Ddx of Unilateral Pediatric Nasal Mass
sided aortic arch, aberrant left pulmonary artery), or Mass
A: Encephalocele
(teratoma,lymphatic malformation, hemangioma)
A: Glioma
A: Infectious/Inflammatory –
A: Neurofibroma
• Croup
A: Nasolacrimal duct cyst
• Tracheitis
A: FB/Rhinolith
• Epiglottitis
A: JNA
• Retropharyngeal abscess
A: Hemangiopericytoma
• GERD
A: Hemangioma
A: Traumatic –
A: AVM
• External compression
A: Polyp
• Foreign body
2. Q: Ddx of Neonatal Nasal Obstruction A: Neoplastic –
A: Pyriform aperture stenosis • Subglottic hemangioma
A: Midnasal stenosis • RRP
A: Choanal atresia 8. Q: Seven Infectious causes of laryngeal stenosis
A: Nasolacrimal duct cyst A: Epiglottitis
A: Tumors – Encephalocele, Glioma, Dermoid, Teratoma, A: Croup
Hemangioma A: RRP
A: Deep neck space infection
3. Q: Percentage of laryngeal anomalies with other airway
A: Severe Laryngitis
anomaly A: Bacterial Tracheitis
A: 50%
A: Infected Laryngocele/Saccular cyst
4. Q: Nasolacrimal Duct Cyst location, pathophysiology, and
9. Q: Discuss Junvenile Nasopharyngeal Angiofibroma
treatment (JNA)
A: Location – Below inferior turbinate anteriorly
A: Clinical – Males, second decade (rare beyond 25 yrs), unilateral
A: Pathophysiology – Proximal and distal obstruction of the
nasal obstruction and recurrent epistaxis, conductive hearing loss,
nasolacrimal duct with fluid accumulation and cyst formation; 85% dacrocystits, rhinolalia, hard and soft palate deformity,  facial
resolve by 9 months
swelling, proptosis, cranial neuropathy, and massive hemorrhage
A: Treatment – Medical = Nasal decongestants and feeding
A: Centered at the PPF (superior border of the sphenopalatine
modifications foramen-basisphenoid) and usually extends into nasopharynx +/-
A: Treatment – Surgical = Endoscopic transnasal marsupialization,
the Pterygomaxillary fissure (PMF), Infratemporal fossa (ITF),
CO2 laser may be used, ophthalmology consult for intraoperative
Skull base, or cavernous sinus and orbit
nasolacrimal duct probing stenting A: Path: Multiple thin walled vessels lacking smooth muscle in a
3: Indications for surgery (ION) – Infection, respiratory
fibrous connective tissue stroma with abundant mast cells,
Obstruction, Nutrition (feeding difficulties)
intensive immunostaining for vimentin on EM
5. Q: Ddx of Posterior Tongue Mass & investigations A: DO NOT BX due to the risk of bleeding and need to r/o other
A: Lingual thyroid causes by imaging
A: TGDC A: Tests – CT & MRI, do Angio with Embolization prior to
A: Cystic hygroma removal; If in a female, do Karyotyping to R/O androgen
A: Valecular cyst insensitivity/testicular feminization
A: Dermoid/Teratoma A: Holman-Miller sign: the characteristic anterior bowing of the
A: Granular cell tumor (epulis is a type on the alveolus not tongue) posterior maxillary wall due to the presence of a mass in the
3: Investigations – CT scan to define margins of the mass; TSH, pterygomaxillary space on CT scan
T3/T4 establish thyroid function; I-123 scan or ultrasound to rule
10. Q: Describe the blood supply of JNA?
out lingual thyroid, identify other foci of functioning thyroid tissue
A: The main supply comes from the internal maxillary artery
6. Q: Ddx Congenital Laryngeal Stridor
A: Laryngomalacia A: Others: ascending pharyngeal, vidian arteries, unnamed
A: VC paralysis branches from the internal carotid artery (rare)
A: Stenosis (subglottic, tracheal)
A: Subglottic Hemangioma 11. Q: Staging of JNA
A: Web A: Chandler I-IV (based on NPC)
A: Clefts A: Sessions I-III (lateral spread to PMF and ITF)
A: Cysts (valecular, saccular, subglottic) A: Radkowski I-IIIB (Sessions + extent of SB erosion)
A: Rings (complete tracheal, vascular) A: Fisch

7. Q: Ddx of Laryngotracheal stenosis 12. Q: Describe the Chandler staging of JNA 1984?
A: Congenital – A: Stage 1 Tumor confined to nasopharynx
• Tracheomalacia A: Stage 2 Tumor extends into nasal cavity or sphenoid
• Laryngomalacia A: Stage 3 Tumor involves the maxillary, ethmoids, infratemporal
• VC paralysis fossa, orbit, cheek, and cavernous sinus
• Laryngeal cleft A: Stage 4 Tumor is intracranial
13. Q: Describe the JNA classification according to Sessions A: XRT (30-35 Gy), generally reserved for larger and/or
1981? unressectable tumors with significant risks in a developing child
A: Embolization (24-72 hours prior to excision), significantly
A: IA - Tumor limited to posterior nares and/or nasopharyngeal
decreased intraoperative blood loss and facilitated resection of
vault larger tumors
A: IB - Tumor involving posterior nares and/or nasopharyngeal A: Surgery (mainstay),
 recurrence rates 30-50% but can
vault with involvement of at least 1 paranasal sinus spontaneously regress in some cases
A: IIA - Minimal lateral extension into pterygomaxillary fossa 18. Q: Ten possible Surgical approaches for JNA excision,
A: IIB - Full occupation of pterygomaxillary fossa with or without from least to most invasive
superior erosion of orbital bones A: Endoscopic transnasal
A: IIC – ITF with or w/o cheek invasion A: Transmaxillary (Transantral?)
A: III - intracranial extension A: Transpalatal
A: Lateral rhinotomy
14. Q: Describe the Radkowski staging of JNA 1996? A: Medial maxillectomy
A: IA – Limited to nose and/or nasopharyngeal vault A: Midfacial degloving +/– LeFort I
A: IB – Extension to one or more sinuses A: Facial translocation (Maxillary swing?)
A: IIA – Minimal extension to Pterygomaxillary Fossa (PMF) A: Infratemporal fossa (Fisch C?)
A: IIB – Full occupation of PMF with or without erosion of orbital A: Subcranial
bones 19. Q: Top 3 congenital laryngeal anomalies
A: IIC – Infratemporal fossa with/without cheek, or posterior to A: Laryngomalacia
pterygoid plates A: Vocal cord paralysis
A: IIIA – Erosion of skull base; minimal intracranial A: Subglottic stenosis
A: IIIB – Erosion of skull base; extensive intracranial with/without
cavernous sinus 20. Q: Seven structures that can be injured during a neonatal
tracheostomy
15. Q: Describe the Fisch Classification of JNA A: Carotid (and innominate) artery
A: I – Limited to nose and/or nasopharyngeal vault A: Jugular vein
A: II – Extension to one or more sinuses, or the Pterygomaxillary A: Recurrent laryngeal nerve
Fossa A: Esophagus
A: III – Invades the Infratemporal fossa, orbit, or parasellar areas A: Lung – Pneumothorax
A: IV – Extends into cavernous sinus, optic chiasm, or pituitary A: Thymus
fossa A: Larynx
A: Posterior tracheal wall
21. Q: Neonatal tracheostomy safety factors intraoperatively
and postoperatively
A: Perform the tracheostomy with the neck extended using a
shoulder roll
A: Stay sutures in tracheal incision
A: Placement of ETT before performing tracheotomy
*Classification systems of JNA A: Keep NG tube in situ to prevent mistaking esophagus for
trachea
16. Q: Routes of JNA spread? A: Postoperative observation in PICU
A: Medially: into the nasopharynx and the nasal cavity and along A: Tracheostomy set at the bedside
A: Flexion of neck while applying ties
the vidian nerve into the floor of the sphenoid sinus
A: Do not tack skin edges together to avoid subcutaneous
A: Laterally: through the pterygomaxillary fissure leads to the emphysema
infratemporal fossa A: CXR in recovery room to verify tube position and to R/O
A: Anteriorly: the posterior wall of the maxillary sinus is pneumothorax
progressively pushed forward A: Always keep at bedside – Trach set with Hemostat, Suction,
A: Superiorly (intracranial) 20-36%: Same size and smaller trach tubes
A: First tube change at 5-7 days
- From PPF through foramen rotundum 22. Q: Grading Firm Mature SGS, Myer & Cotton (1994)
- From PPF  IOF  orbital cavity  SOF A: Using cuffless pediatric ET tube
- From ITF through foramen ovale or spinosum A: Assess air leak. If < 10cm H20, upsize tube; if btwn 10-25
- Through sphenoid sinus (medial to IC & cavernous sinus) cmH20 (compare to expected ETT); if > 25 cmH20, downsize tube
- Through ethmoid sinuses (anterior cranial fossa) A: Comparing to expected size ET tube for patient age deduction
% of lumen obstruction from the above
17. Q: Treatment strategies for JNA 3: Usefulness in prognosis for decannulation, and number of
A: Hormonal therapy: flutamide (testosterone receptor blocker) or operations required to decannulation
estrogen, decreases size and vascularity of tumor but due to risks
and variable response not used 23. Q: Cotton-Myer grading of SGS
A: I) 1-50%
A: II) 51-70%
A: III) 71-99%
A: IV) 100%
24. Q: McCaffrey system classifies laryngotracheal stenosis
A: Stage I – confined to the subglottis or trachea, <1 cm long
A: Stage II – isolated to the subglottis, >1 cm long
A: Stage III – subglottic/tracheal lesions not involving the glottis
A: Stage IV – lesions involve the glottis
*McCaffrey grading system for SGS
25. Q: Bogdasarian classification of adult posterior glottic
stenosis
A: Type 1: Interarytenoid adhesion (with posterior sinus tract in
Cotton classification)
A: Type 2: Posterior commisure stenosis
A: Type 3: Posterior commissure stenosis with unilateral
cricoarytenoid ankylosis
A: Type 4: Posterior commissure stenosis with bilateral
cricoarytenoid Ankylosis
*Cotton classification of posterior GS
26. Q: Etiology/predisposing factors for acquired SGS in
adults (10)
A: Intubation-related (>90%, 1-8% incidence): oversized, repeated,
shearing motion (agitation), route, and duration
A: Iatrogenic trauma (laser surgery, high tracheotomy,
cricothyrotomy)
A: External laryngeal trauma
A: Burn (inhalational/thermal/chemical/radiation)
A: Gastroesophageal reflux
A: Infection (Primary or Superimposed bacterial or fungal
infection)
A: Neoplasms (Benign or Malignant, Intrinsic or Extrinsic)
A: Autoimmune (Wegeners, Sarcoidosis, SLE)
A: Inflammatory disease (Sarcoidosis, Relapsing Polychondritis)
A: Idiopathic SGS
27. Q: Four Preventative measures for avoiding SGS
A: Smaller ETT without compromising safe ventilation (air leak at
<25 cmH2O)
A: Diagnosing (pH probe) & treating LPR
A: Prophylactic Antibiotics when tracheotomy is performed
following prolonged/traumatic intubation
A: Prolonged intubation up to 6 months preferred over
tracheostomy in neonates
28. Q: Histopathologic classification of Congenital SGS
A: Soft tissue – Granulation tissue, submucosal gland hyperplasia,
submucosal fibrosis
A: Cartilaginous – Normal shape (cricoid small for infant’s size)
A: Cartilaginous – Abnormal shape (elliptical shape, large anterior
lamina, large posterior lamina, generalized thickening,
submucus/incomplete laryngeal cleft, other)
A: Cartilaginous – Trapped first tracheal ring
A: Combined stenosis
29. Q: Definition of Congenital SGS (vs Acquired SGS)
A: No history of ETT or laryngeal trauma
30. Q: Which has worse symptoms and prognosis: Congenital
or Acquired SGS?
A: Acquired
3: Congenital tends to improve with growth of the child
31. Q: Normal term subglottis
A: 4.5 – 5 mm (4 mm in premature; BB says 3)
A: Size 3 ETT
32. Q: SGS at term & premie?
A: <4 mm & <3.5 mm (BB says <3mm)
33. Q: Rule for choosing the appropriate ETT size
A: Age/4 + 4 or (Age+16)/4
34. Q: By what percentage will 1 mm of subglottic edema
reduce the airway in a neonate?
A: 67% (BB says ~60%)
35. Q: Pediatric bronchoscope sizes (outer diameter)
A: Premie - 2.5 (3.7)
A: 0-3 months (Term)- 3.0 (5.0)
A: 3-18 months - 3.5 (5.7)
A: 1-3 years - 3.7 (6.3)
A: 2-6 years - 4.0 (6.7)
A: 5-10 years - 5.0 (7.8)
A: 10-16 years - 6.0 (8.2)
3: Outer diameter (OD)= inner diameter (ID) + 0.8
3: ID is the same as the size of an ETT while they’re two different
values in bronchoscopes (see below)
*Bronchoscope and ETT pediatric size chart A: Exposure of cartilage during CO2 laser excision predisposing to
chondritis
36. Q: Pediatric esophagoscope/laryngoscope sizes
A: Loss of cartilaginous framework
A: Age Esophagoscope Laryngoscope 43. Q: Indications & Contraindications for Anterior Cricoid
Preemie 4 8 Split

 
0-3mos 4-5 8 A: Indications – Failure of extubation 2 times in neonate/young
3-18mos 5-6 9
child, congenital small cricoid in older child
37. Q: A: Contraindications – Short duration of extubation before
1-3yr 6 10.5
reintubation (hours), Peak airway pressure > 35 mm Hg
2-5yr 6-7 10.5-12
5-10yr 7 12 44. Q: Seven selection Criteria for Anterior Cricoid Split
A: Weight > 1500 gm
>10 yrs 8 16
A: Failed extubation twice 2ndry to laryngeal pathology
Adult
A: No acute respiratory tract infection
A: O2 requirement < 30%
Smallest bronchoscope able to accommodate peanut A: No ventilation support for at least 10 days
grasper A: No antihypertensive medications at least 10 days
A: 3.5 A: No CHF for at least 1 month
3 broad catagories: Airway, ventilatory, cardiac
38. Q: 2 alternatives to ETT in the airway management of H20 UVWX Heart failure, Hypertention, O2, URTI, Vent support,
known SGS cases Weight, Extubation
A: Laryngeal mask airway
A: Heliox 45. Q: How much distraction of the cricoid is required for a
A: TIVA cartilage graft to be placed in the anterior split
A: 3mm
39. Q: Management options for SGS
A: Observation – Grade I-II with minimal symptoms & reliable 46. Q: Most common techniques for laryngotracheal
follow up, especially congenital, repeat bronch q3-6 months reconstruction (LTR)
A: Medical – Anti-reflux A: Anterior cartilage graft + tracheotomy + no stent
A: Tracheotomy A: Short term stenting (4-6 weeks) + cartilage grafts (anterior &/or
A: Endoscopic procedures – Balloon dilatation, Laser, mitomycin posterior)
C A: Long-term stenting (several months) +/– cartilage graft
A: Open reconstructive procedures – A: Single stage LTR (SSLTR) – cartilage grafts + brief period of
• Expansion procedure (LTP/LTR, single stage or with trach nasotracheal intubation (7 - 10 days for ant graft, 10 - 14 days for
and Stent: Anterior w/wo Posterior w/wo Lateral cricoid split, post graft, older = shorter)
anterior and/or posterior cartilage Graft) 47. Q: Four indications for 2-Step LTR
• Segmental resection (Cricotracheal resection & anastomosis, A: Severe stenosis
primary, salvage, extended with expansion, arytenoid A: History of reactive airway
lateralization or arytenoidectomy, stents) A: Poor pulmonary function
40. Q: Contraindications to airway surgery A: Inadequate intensive care facilities
A: Absolute –
• A: Tracheotomy dependent (aspiration, severe BPD) 48. Q: Three indications for LTR with Division of the
• A: Severe GER refractory to surgical and medical therapy Posterior Cricoid lamina
• A: Unfit for GA A: Posterior Glottic/Subglottic stenosis
A: Relative – A: Complete Glottic/Subglottic stenosis
• A: Steroid use A: Significant Cricoid deformity
• A: Diabetes
49. Q: Four indications for Cartilage Grafting in the posterior
• A: Cardiac, renal or pulmonary disease
glottis and subglottis
A: Posterior Glottic/Subglottic stenosis
41. Q: Five Indications of Laser for SGS
A: Isolated Subglottic shelves
A: Early stenosis
A: Circumferential Subglottic stenosis
A: Grade I, II
A: Total or near total obstruction at the glottic or subglottic level
A: Granulation tissue
A: Thin webs 50. Q: Five indications for Long-term Stenting in pediatric
A: Crescent-shaped bands airway reconstruction
3: “Early mild soft thin crescents” A: Posterior cricoid split without cartilage grafting
A: Lack of airway wall Rigidity
42. Q: Eight Contra-indications of endoscopic laser for SGS
A: Keloid formation
A: Circumferential thick (cicatricial) scarring
A: Severely altered anatomy by stenosis or surgery
A: Length >1 cm
A: Unstable cartilage grafts
A: Laryngotracheal stenosis
3: “Posterior Rigid Keels are Severely Unstable”
A: Posterior glottic stenosis with arytenoid fixation
A: Previous failure
A: Previous severe bacterial infection associated with 51. Q: Five types of Stenting
tracheostomy
A: Aboulker or Cotton-Lorenz stent (rigid Teflon – polytef II, 58. Q: Ddx of Pediatric Lateral neck mass (6)
hollow lumen) A: Branchial anomaly
A: Montgomery T tube (hollow silicone) A: Laryngocele
A: Montgomery laryngeal stent (solid silicone) A: Pseudotumor of infancy
A: Single stage LTP (ETT used as alternative to stenting) A: Hemagioma
A: Finger cot A: Lymphatic malformation
A: Silastic sheet / Swiss roll A: Thymic cyst
52. Q: Four advantages of Cricotracheal Resections & 59. Q: Ddx of Pediatric Midline neck mass
Thyrotracheal Anastomosis A: TGDC
A: Safe effective treatment for Severe SGS A: Dermoid
A: Results are Superior to similar cases done by LTR techniques A: Teratoma
A: Voice quality results are better (preserves voice) A: Plunging ranula
A: No interference with normal growth of Larynx A: Thymic cyst
3: Contraindication of CTR: subglottic scarring within 3 mm of A: Hemagioma
vocal cords A: Lymphatic malformation
53. Q: Two disadvantages of Cricotracheal Resections & 60. Q: Histology of Thyroglossal duct epithelium
Thyrotracheal Anastomosis A: Squamous
A: Possibility of injury to the Recurrent Laryngeal Nerve (lateral A: Respiratory
cricoid dissection is performed in subperichondrial plane & lateral A: Thyroid follicles and colloid
resection is anterior to the Cricothyroid joint)
61. What are the most common H&N peds malignancies in
A: Possible partial Dehiscence at anastomotic site resulting in
general and rank by age?
Restenosis (laryngeal release only if >5 tracheal rings resected)
A: Bailey’s:

- Hodgkin disease 31%

- Non-Hodgkin lymphoma 26%
- Rhabdomyosarcoma 15%
- Other sarcomas 8%
- Thyroid carcinoma 8%
- Nasopharyngeal carcinoma 5%
- Neuroblastoma 4%
- Salivary gland carcinoma 2%
*Cricotracheal resection (Note the RLN)   - Malignant teratoma <1%
A: Cumming’s: Lymphomas (27%) (Hodgkin's 17%, NHL 10%),
54. Q: Three options for Post-operative airway support for
Glottic Edema following Cricotracheal Resections & Neural tumors (23%: retinoblastoma and neuroblastoma), Thyroid
Thyrotracheal Anastomosis malignancies (21%, PTC most common), Soft tissue sarcomas
A: Short term ET intubation (12%: rhabdomyosarcoma; 8%, nonrhabdomyosarcoma soft tissue
A: Montgomery T tube stenting for older child (4-6 weeks) sarcomas [NRSTSs])
A: Distal tracheotomy (4-6 weeks)
55. Q: Two indications for Four Quadrant Cricoid Split
A: Grade III & IV
A: Congenital elliptical cricoid
3: Procedure – Division of ant. + post. walls (+/- grafts) + lateral
walls of cricoid anterior to inf. cornu of thyroid &
extraperichondrial externally to avoid RLN, Aboulker or Cotton-
Lorenz Stent for 6 months (very unstable airway) *Ranking of H&N malignancies in peds by age (Cumming’s)

56. Q: Idiopathic subglottic stenosis (ISS) 62. Lymphoma pearls

A: Rare inflammatory process of unknown cause
A: Limited to subglottis & upper 2 tracheal rings
A: Young female >85% (estrogen altering wound healing
response?)
A: Surgery is the main treatment modality (endoscopic laser with
mitomycin-c for < 1cm, open laryngotracheal surgery (CTR) for
thicker complex scar)
57. Q: Safe length of time for intubation in adults, children,
and neonates
A: Adults – 5-10 days
A: Children – up to 50 days
A: Neonates – up to 6 months
A: 60% of pediatric lymphomas are NHL but Hodgkin’s is more
common in the H&N (see above)
A: Presentation:
- NHL in the H&N is seen in 5-10% of children and most
often extranodal involving Waldeyer’s ring, salivary
glands, larynx, sinuses, orbit and scalp that rapidly
progresses but can lead to asymptomatic cervical LN
- HD will present with asymmetric lymph node
enlargement above the diaphragm in about 90% of cases
A: EBV is associated with
 - Burkitt’s (90% of endemic BL & 20% of sporadic BL)
- Hodgkin’s lymphoma in 19-59%
A: Risk factors for NHL:
- AIDS
- Immunosuppressive therapy
- Chemotherapy
- Congenital immunodeficiency syndromes (Wiskott-
Aldrich syndrome, ataxia-telangiectasia, X-linked
lymphoproflerative disorders)
63. Q: Pathognomic cell in Hodgkin’s lymphoma
A: Reed Sternberg cell
*Reed Sternberg cell
64. Q: Rye classification of Hodgkin’s lymphoma
A: Nodular sclerosis (60%) – nodules of lymphoid infiltrates,
lacunar variants of RS cells
A: Mixed cellularity (30%) – pleomorphic lymphocytes, more
numerous RS cells
A: Lymphocyte depleted (6%) – paucity of lymphocytes, diffuse
fibrosis and bizarre RS cells; worse prognosis
A: Lymphocyte predominant (3%) – rare Reed-Sternberg cells,
favorable prognosis
65. Q: Ann Arbor staging of Hodgkin’s lymphoma
A: I – Single LN region or extralymphatic site (IE)
A: II – 2+ LN regions or EL sites (IIE) on same side of diaphragm
A: III – Nodal regions, EL sites (IIIE), or spleen involvement
(IIIS) on both sides of diaphragm
A: IV – Disseminated disease
A: A (absence) or B (presence) of unexplained weight loss >10%
of total body weight, unexplained fever >38, night sweats
66. Q: Treatment of Hodgkin’s lymphoma
A: Early disease (I & IIA) – XRT, 10 year survival 90%, 10 year
relapse free survival 75-80%, chemo given for mediastinal disease
A: Advanced disease – Combination chemotherapy +/- XRT:
  Rhabdomyosarcoma in descending order (ONES)?
- MOPP (nitrogen mustard, vincristine, procarbazine and
prednisone)
- ABVD (Adriamycin [doxorubicin], bleomycin,
vinblastine, dacarbazine)
- Stanford V (doxorubicin, vinblastine, mustard, bleomycin,
vincristine, etoposide, prednisone)
- Complete response rate 44-87%, long term disease free
survival rate 50%
A: Children – Combined modality therapy equally effective while
causing less growth impairment
67. Q: Working classification of non-Hodgkin’s
A: Low grade – Small lymphocytic, Follicular small cleaved,
Follicular mixed. (SMALL)
A: Intermediate grade – Follicular large, Diffuse small cleaved,
Diffuse mixed, Diffuse large cell (most common in H&N).
(DIFFUSE)
A: High grade – Small noncleaved (Burkitt), Immunoblastic
(Large cell), Lymphoblastic. (BURKETTS OR LARGE)
68. Q: Staging of non-Hodgkin’s disease
A: I – Single LN region or ES site with exclusion of mediastinum
and abdomen
A: II – Single ES site with regional node involvement; 2+ LN
regions or ES sites on same side of diaphragm
A: III – Nodal regions or ES sites on both sides of diaphragm; any
intrathoracic, paraspinal, epidural tumor
A: IV – Disseminated disease or any of above with initial
involvement of CNS, bone marrow, or both
69. Q: Describe the high grade NHL lesions
A: >90% of children have high grade lesions
A: Burkitt lymphoma (small noncleaved) – Diffuse B-cell
malignancy, classic starry sky pattern of phagocytic histiocytes
and tumor cells, Genetics: translocation of myc gene from
chromosome 8 to 14 in 80%
A: Lymphoblastic – Immature T-cell origin, small lymphoblasts
with round/convoluted nuclei, distinct nuclear membranes,
basophilic cytoplasm
A: Immunoblastic/Large cell – Heterogeneous group of
lymphocytic & histiocytic tumors; 80% of adults are B-cell in
origin; in children equal numbers originate from T-cell, B-cell or
indeterminate origin
70. Q: Discuss the clinical assessment for non-Hodgkin’s
lymphoma after LN biopsy
A: H&P with direct laryngoscopy
A: CBC/Diff, LFT’s, LDH
A: CT neck, chest, abdomen & pelvis
A: Barium swallow (3- 11% of patients with Waldeyer ring
lymphoma will have an associated GI lesion)
A: Bone marrow biopsy (18% of patients with extranodal H&N
lymphoma involved)
A: Lumbar puncture (at risk of CNS involvement: High grade
lymphoma, Intermediate grade lymphoma of the paranasal sinuses,
bone marrow, testes, paraspinal areas)
71. Discuss treatment of NHL?
A: Combination chemo: CHOP (cyclophosphamide, doxorubicin,
vincristine, and prednisone)
A: For extensive disease: methotrexate, ifosfamide and other
chemo agents maybe added +/- XRT +/- Surgery for airway issues
72. Q: Most common primary sites in the head and neck for
A: Orbit (25-30%)
A: Nasopharynx
A: middle Ear/mastoid
A: Sinonasal cavity
3: 35-40% of all cases occur in the head and neck
73. Q: Most common metastatic sites for head and neck
Rhabdomyosarcoma
A: Lungs
A: Bone
A: Bone marrow
74. Classification of rhabdomyosarcoma according to site in
the head and neck?
A: Orbital, most common, treated with CRTx (No Sx)
A: Parameningeal sites (nasopharynx/nasal cavity, the middle ear,
the paranasal sinuses, and the infratemporal fossa/pterygopalatine
space), worse prognosis due to skullbase/IC involvement in 65-
80%, Treated with CRTx +/- Sx
A: Non-parameningeal sites: Superficial sites: scalp, cheek, and
external ear, or Deep structures: parotid gland, larynx, oral cavity,
and oropharynx. More amenable to surgical excision
3: Orbit and non-PM are favorable sites while PM sites are
unfavorable
75. Q: Histologic classification of Rhabdomyosarcoma
A: Embryonal (60-70%)
- Most common in infants and children in the H&N
- Intermediate prognosis (Best prognosis in spindle cell and
botryoid subtypes)
- Histo: Small round to spindle shaped cells (strap cells)
with eosinophilic cytoplasm (blue)
- Botryoid subtype appears as a cluster of grapes & arise
under the mucosal surface of body orifices such as the
vagina, bladder, nasopharynx
- Spindle cell subtype usually involves paratesticular site
A: Alveolar (20%)
- Most common in adolescents in the extremities & trunk
- Poor prognosis
- Histo: clusters of small round cells with fibrous septae (
resembles lung alveoli) in > 50% of the tumor (if <50%
 embryonal)
A: Pleomorphic/Anaplastic (5%)
  A: PTLD usually manifests with B-cell proliferation induced by
- Almost exclusive in adults in the extremities & trunk
- Poor prognosis
- Histo: Greater degree of nuclear atypia, multinucleation
and pleomorphism.
- Some consider this a subtype of embryonal RS in peds
and a form of malignant fibrous histiocytoma in adults
and deleted it as a type on its own in the new
classifications
A: A fourth class may be Undifferentiated, poor prognosis
76. Immunohistochemical stains for rhabdomyosarcoma?
 Management options:
A: myogenin, muscle specific actin, and desmin
77. How to stage rhabmyosarcoma?
A: Pre-operative staging, using the TNM classification, is
commonly referred to as “stage”
A: Post-operative staging system is commonly referred to as
“group” depending on amount of residual disease post Sx
A: Then assign a Risk Group: Determined by Stage, Group, and
histology
78. Q: TNM Staging of rhabdomyosarcoma from IRS-IV
A: T staging
 Acid fast gram positive obligate aerobes that can be found in the
T1 – Confined to anatomic site of origin
T2 – Extension and/or fixation to surrounding tissue
- A ≤5cm in diameter
- B >5cm in diameter
A: N staging
 between
 ages 1-5 years
N0 – Not clinically involved
 > pre-auricular > parotid region
N1 – Clinically involved
Nx – Clinical status unknown
A: M staging
M0 – No distant metastases
M1 – Distant metastasis
A: Stages
I – Head and Neck (Orbit, non-PM); T1 or T2, A or B; any N; M0
II – Parameningeal; T1 or T2, A; N0 or Nx; M0
III – Parameningeal; T1 or T2, B; any N; M0
IV – All sites; T1 or T2, A or B; any N; M1
3: Groups and risk classification can be seen in staging pdf
79. Q: Discuss general treatment of Rhabdomyosarcoma
A: Surgical excision
- Should be done if feasible with 2 cm margin
- Not recommended if produces significant morbidity
(functional or cosmetic) or no increase in survival seen
post excision compared to CRTx (e.g orbit)
- Can be used for salvage
A: Induction chemo in all cases: vincristine and dactinomycin +/-
cyclophosphamide (VAC)
A: Radiation therapy (40-50Gy) is used in cases with gross or
microscopic disease after surgery or in cases in which surgery is
not feasible
A: Neck should NOT be treated (Sx or RT) unless N+
80. Discuss Post-transplantation lymphoproliferative disorder
(PTLD)?
A: Umbrella term for all abnormal proliferations of lymphoid
tissue in the transplant recipient, ranging from lymphoid
hyperplasia to NHL
EBV; this proliferation is left unopposed by the pharmacologically
suppressed T-cell system
A: Risk factors: degree & type of immunosuppression, EBV
seronegative status at time of transplant, young age, donor-
recipient mismatch & GVHD, T-cell depletion or use of anti T-cell
monoclonal antibodies
A: Tonsillar hypertrophy, adenoid enlargement +/- cervical
adenopathy is a common presentation in the H&N
A:
- Biopsy of tissue to diagnose & exclude lymphoma
- Excision of obstructing lymphoid tissue (T&A), can be
curative
- Reduction or cessation of immunosuppression
- Antiviral treatment with acyclovir or ganciclovir to
control Epstein-Barr virus (EBV) replication
- Interferon alpha
- IV immunoglobulin
- Immunotherapy with donor t-lymphocyte infusion
- Chemotherapy +/- RT for lymphoma cases
- Surgery to remove the transplanted organ
81. Q: Discuss Atypical mycobacterial infection
A:
environment in soil, water, vegetables, and even in domestic
animals and dairy products
A: Most common are M. Avium intracellulare, M. kansasii, M.
scrofulaceum
A: Childhood disease
A: Submandibular region
A: Nontender, slowly enlarging, skin fixation common with a
violaceous hue
A: Corneal ulceration is most common H&N manifestation
A: Few systemic effects, rare pulmonary involvement
A: PPD (5 units) intradermally  negative or weekly positive (If
strongly positive may suggest typical TB  CXR)
A: Ziehl-Neelson stain shows AFB & Lowenstein-Jensen medium
for c/s (2-8 weeks incubation period)
A: Antibiotics:
- Less cure rate compared to Sx, adjunct to Sx, if severe
adenopathy, residual/recurrent disease,
immunocompromised pt , disseminated disease
- mono, dual or triple therapy: Clarithromycin or
azithromycin +/- Ethambutol +/- Rifampin (or rifabutin)
for Disseminated disease
- Used for 6-12 months
A: Incision and drainage may cause fistulization
A: Curettage or excision with skin is the main modality of
treatment (Caution in regards to marginal mandibular nerve)
82. Q: Infantile Hemangioma age of appearance, Gender
predilection, percent Multiple, Cellular findings, site in the
airway and syndrome associated
A: First 6 weeks
A: Female: Male = 3-6:1
A: Percentages:
- 20% Multiple
- 50% with a Subglottic hemangioma will also have a
cutaneous lesion, but the converse in only true in 1-10%
- However, 63% of young children with hemangiomas in
four or more sites in the beard distribution have airway
lesions
A: Proliferating Endothelial cells and increased Mast cells, stains
positively for glucose transporter 1 (GLUT-1)
A: Predilection for the Left Posterolateral subglottis
A: PHACES syndrome (posterior fossa malformations (P),
segmental facial hemangiomas (H), arterial anomalies (A), cardiac
defects (C), eye abnormalities (E), and sternal defects (S))
3: usual size 0.5-5 cm (up to 20 cm)
83. Q: Stages of Infantile Hemangioma evolution
A: Proliferative – 6-12 months
A: Involuting – 50% by 5 years, 70% by 7 years
A: Involuted – Redundance, scarring, telangiectasias
3: Specific Markers of proliferation: Serum and urinary vascular
endothelial growth factor (VEGF), Urinary beta-fibroblast growth
factor (b-FGF), Urinary matrix metalloproteinases (MMPs)
84. Q: Indications for treatment of Hemangioma (VASCO)?
A: Impaired Vision or Hearing
A: Airway compromise
A: Impaired Swallowing
A: Cosmesis (massive, ulcerating, disfiguring)
A: High Output Cardiac failure
3: Complications of Hemangiomas are the above + Kasabach-
Merritt Sx (hemangioma-thrombocytopenia)
  A: Low flow (capillary, venous, lymphatic, combinations)
85. Q: Treatment options for Hemangioma
A: Observation
A: Standard of care = Tracheostomy and wait for involution
A: Oral steroids (2-3 mg/Kg/d PO x 7 days then R/A, if responsive,
taper over 4-6 weeks up to 10 months)
A: Intralesional steroids (Triamcinolone 40 mg or Betamethasone
6 mg q4-6 weeks x 1-5, avoid periorbital)
A: IFN-Alpha 2a or IFN-Alpha 2b (unresponsive to steroids, can
be daily S/Q x 6+ months)
A: Surgery (endo or open cold knife with cricoid split,
Microdebrider, CO2 /KTP /Nd:YAG / Pulse dye laser)
A: Photocoagulation (q4-6weeks, early proliferative phase,
superficial lesions)
A: Cryotherapy
A: Tracheostomy or Laryngotracheoplasty
A: Propranolol:
- Mode of action: BetaBlocker, vasoconstriction, down-
regulation of angiogenetic factors such as VEGF and
bFGF, up-regulation of apoptosis of capillary endothelial
cells, inhibiting the expression of MMP-9 & HBMEC
- Side effects: bradycardia, hypotension, hypoglycemia,
rash, gastrointestinal discomfort/reflux, fatigue and
bronchospasm
- CI: Large hemangiomas are at risk for high-output cardiac
compromise if used, renal or hepatic dysfunction,
underlying cardiovascular disease, asthma, diabetes,
glaucoma or allergy
- Dose: initiated with dose of 0.5-1 mg/kg/day (divided PO
TID) with cardiac & BS monitoring x 48 hrs, If tolerated
 increased to 2-3 mg/kg/day x 6-12 months then tapered
off
86. Q: Subglottic Hemangioma – which type is safe to biopsy
and use CO2 laser on
A: Capillary – Less colour to lesion, smaller vessel size, therefore
able to use laser
A: Cavernous – Dark red/blue, bleed with biopsy, and difficult to
control with CO2 laser
87. Q: Complication of laser of subglottic hemangioma
A: Subglottic stenosis
88. Other vascular neoplasms?
A: Rapidly involuting congential hemangioma (RICH):
- Present at birth and involute in the first yr, GLUT1 –ve
- Tx: observation
A: Non-involuting congential hemangioma (NICH):
- Present at birth and persist, GLUT-1 –ve
- Tx: Laser and surgical Tx
A: Lobular capillary hemangioma (pyogenic granuloma)
A: Kaposiform hemangioedothelioma
A: Tufted angioma
A: Angiosarcoma
89. Q: Kasabach-Merritt phenomenon
A: Kaposiform hemangioendothelioma or tufted angioma
A: Sequestration of platelets, ecchymoses
A: Heparin contraindicated
A: Tx: Supportive care, steroids, IFN-A2a, PRBCs for anemia,
limit blood products unless bleeding, RT or embolization maybe
used
90. Q: Classification of vascular malformations
A: High flow (arterial, arteriovenous)
91. Q: Discuss vascular malformations?
• Capillary malformations
o Dilated capillaries (Port-wine stain)
o If involving upper face & eyelid  R/O Sturge-
Weber Sx (MRI brain & ophtho consult); glaucoma,
sz, mental retardation
o Lesions progressively darken and thicken
o Serial laser therapy the management of choice (argon,
pulsed tunable dye)
o Surgical excision possible; watch for hypertrophy or
unpredictable pigmentation
• Venous malformations
o Abnormally tangled vessels with slow flow
o Compressible, enlarge with valsalva or gravity
 o CT may show calcified phleboliths 92. Q: De Serres Lymphatic malformation staging?
o May lead to consumptive coagulopathy
o Tx: Symptom management and surgical excision/ laser A: Stage I - Unilateral infrahyoid (17% Complication rate)
/ or sclerotherapy in selected lesions A: Stage II - Unilateral suprahyoid (41% Cx rate)
• Lymphatic malformations A: Stage III - Unilateral infrahyoid and suprahyoid (67% Cx rate)
o Divided into
o Macrocystic (>2cm3) A: Stage IV - Bilateral suprahyoid (80% Cx rate)
 Usually infrahyoid and lateral to canthus A: Stage V - Bilateral infrahyoid and suprahyoid (100% Cx rate)
 Resolve spontoneusly if not septated + 3: Modified Seattle classification added: Stage VI Bilateral
posterior neck + InfraH
infrahyoid, Stage VII Retropharyngeal, M for mediastinal
 Resolves with sclerotherapy or Sx otherwise
o Microcystic (<2cm3) or Mixed extension
 Usually suprahyoid and midface
 Involves oral & OP mucosa (infiltrative) 93. Q: Options for Sclerotherapy
 Often associated with complications: A/W, A: Effective for lymphatic and venous malformations
speech, feeding, bleeding A: Ethibloc (95% ethanol with starch)
 Responds poorly to Tx A: Sodium tetradecyl sulphate
o Associated issues: A: OK-432 (lyophilized low-virulence Strep Pyogenes,
 Sudden ↑ in size occur w/ trauma,
hemorrhage or infx of cystic spaces  broad
macrocystic = 92% response microcystic = 44%)
spectrum Abx A: Bleomycin
 Lymphopenia can occur A: Doxycycline
 Bony overgrowth or resorption (Gorham 94. MRI Characteristics of vascular anomalies?
Syndrome)
o Associated Syndromes: A:
 Turner syndrome, Down syndrome,
Klinefelter syndrome, and trisomy 18, 13,
  T1- T2- Contrast
Gradient
Noonan syndrome, Fryns syndrome, Weighted Weighted (gadolinium)
multiple pterygium syndrome, and Lobulated High-flow
achondroplasia Soft-tissue
soft-tissue vessels
o MRI findings: mass, Uniform
mass, within and
 septated masses w/ low intensity on T1 Hemangioma isointense or intense
increased around
 high intensity on T2 w/out flow voids hypointense, enhancement
signal, flow soft-tissue
o If detected on prenatal U/S with possible A/W issues flow voids
voids mass
 EXIT procedure
o Surgical resection mainstay of therapy +/- trach Septated
Isointense to
 Cold knife soft-tissue
muscle, Diffuse or No high-
 laser (CO2, Nd:YAG) if oral cavity & tongue Venous mass, high
possible inhomogeneous flow
 Considered if minimal morbidity and malformation signal,
high-signal enhancement vessels
unsuccessful other treatments signal voids
thrombi
 No consensus on timing (phleboliths)
o Sclerothrapy (e.g. OK-432): Soft-tissue
 Ineffective for microcystic disease & good Septated Rim
mass, high No high-
response rates for macrocystic disease Lymphatic soft-tissue enhancement
signal, flow
 Aspirate macrocyst then inject agent under malformation mass, low or no
fluid/fluid vessels
fluro signal enhancement
levels
o Other treatment: RT, RF ablation, Sildenafil (new
trials) High-flow
• AV malformations Soft-tissue Variable vessels
Arteriovenous Diffuse
o Abnormal communications b/w arteries & veins, thickening, increased throughout
malformation enhancement
bypass capillary bed, high flow flow voids flow voids abnormal
o Intracranial > extracranial tissue
o Characteristically a pulsatile mass w/ an assoc thrill or
 
bruit on auscultation or Dopler, warm, on cheek or
auricle
o Stages: dormancy, expansion, destruction & CHF 95. Differences between Hemangioma & vascular
o Dx with MR or CT angio malformations?
o MRI:
 no enhancement on T2 A:
 flow voids present on both T1 & T2
o Cx: Hemangioma Vascular Malformation
• skin necrosis
• ulceration Usually not seen at birth Always present at birth
• bleeding
• heart failure
o Tx:
Gender: Female > Male Equal gender distribution
 Nothing required for asymptomatic lesions
 symptomatic lesions require embolization Race: More common in
alone if in bone or preoperative embolization white Equal between all races
and surgical resection of nidus if involving
soft tissue
 Hemangioma 102. Q: Features of Stickler syndrome (hereditary progressive
Vascular Malformation
arthro-ophthalmopathy)?
Rapid growth and slow A: AD mutation of COL2A1 gene, chromosome 12, for type II
Grows proportionately with the child collagen
regression
A: Robin sequence, mid face hypoplasia
A: Eye – Myopia, cataracts, & retinal detachment
Firm and rubbery Compressible A: Joint – Hypermobility & enlarged joints, early arthritis, occ.
spondyloepiphyseal dysplasia
Rarely involves bone or May cause significant hypertrophy and A: SNHL or mixed HL in 80%
cartilage distortion of craniofacial skeleton

Proliferating Endothelial
cells and increased Mast Mature endothelium with normal
cells mitotic activity and no mast cells

     
96. Q: 9 anatomic relationships of a 2nd branchial arch * Features of Stickler Sx
anomaly
A: External opening along lower third of SCM 103. Q: Twelve Craniofacial features of Down syndrome
A: Internal opening associated with posterior pillar in tonsillar A: Brachycephaly/Flat occiput
fossa A: Small ears with Narrow EACs, low set
A: Deep to platysma, CN VII, external carotid A: Upslanting palpebral fissures
A: Superficial to stylopharyngeus, CN IX, X, XII, & internal A: Epicanthic folds, Brushfield spots on iris
carotid A: Midface hypoplasia, microgenia
A: Small nose
97. Q: Rule of branchial arch anomaly relationships A: Narrow nasopharynx
A: Run deep to own arch structures A: Large fissured lips
A: Run superficial to next arch structures A: Large fissured tongue
98. Q: Which cranial bones are formed by Endochondral A: Dental abnormalities
ossification (i.e. the others are all intramembranous) A: Short neck
A: Hyoid bone A: Subglottic stenosis
A: Inferior turbinate A: Small larynx
A: Styloid process A: Atlantoaxial instability & subluxation
A: Petrous Temporal
A: Occipital
A: Ethmoid
A: Mastoid
A: Sphenoid
A: “HIS POEMS”
99. Q: Triad seen in Pierre-Robin
A: Micrognathia *Down’s Sx Facial features
A: Cleft palate 104. Q: Downs Peds patient with OSA and pulmonary
A: Glossoptosis hypertension: Two treatments
3: If isolated (non-syndromic) mandible catch up growth happens A: T and A
in first year and attains normal profile in 5-6 yrs. If syndromic, this A: Bronchoscopy
persists A: ?rapid maxillary expansion
100. Q: Percent of Robin sequence associated with a syndrome 3: Use smaller ET tube in Down’s patients
A: 50-80% 105. Q: Eight Reasons why Downs are susceptible to OSA
A: Stickler A: Hypoplastic midface and cranium
A: VCFS 22q11 A: Narrow nasopharynx
A: Others: Treacher Collins, trisomy 11q syndrome, trisomy 18 A: Macroglossia
syndrome, Möbius syndrome, and CHARGE association A: Muscular hypotonia
A: Obesity
101. Q: Management options for respiratory distress in Pierre- A: Increased susceptibility to upper respiratory tract infections
Robin patient A: Small larynx
A: Medical – Prone position, McGovern nipple, Nasopharyngeal A: SGS
airway, NG tube, Intubation (difficult), NIPPV 3: UPPP may be useful in this patient population
A: Surgical – Tracheostomy, Subperiosteal Floor of mouth release, 106. Q: Treacher-Collins syndrome (mandibulofacial
Glossopexy, Tongue-lip adhesion (Routledge), Distraction dysostosis)
osteogenesis, CP repair A: AD, 60% sporadic
A: Mutation in TCOF1 gene, TREACLE protein, chromosome 5q
A: Malformation of 1st & 2nd branchial arches
A: Eye:
- Antimongoloid palpebral fissures (downslanting)
- Coloboma of the lower eyelids (upper lid in Goldenhar)
- Aplasia of lower lid lashes
A: Ear:
- Microtia, , EAC stenosis or atresia, ossicular
malformation, preauricular tags & fistulas, CHL in 30%,
occasional SNHL (Mondini)
A: Facial: * Crouzon (Lt) & Apert Syndrome (Rt) note syndactyly
- Malar hypoplasia with non-fusion of zygomatic arches
- Hypoplastic supraorbital rims
- Flat nasofrontal angle
- Narrow nares, hypoplastic alar cartilages
- Tongues of hair onto cheeks
A: Mandible & oral cavity:
- Mandibular hypoplasia (including condyle)
- Macrostomia
- High arched or cleft palate
- Dental abN *Pfeiffer Syndrome note digital broadening
A: May have choanal atresia
A: Normal IQ
109. Q: Branchiootorenal syndrome (Melnick-Fraser
syndrome)
A: AD, EYA1 gene, chromosome 8q
A: Branchial cleft anomalies (63%)
A: Otologic malformations – Hearing loss (89%), preauricular pits
(77%), auricle abnormalities (41%), ossicular & cochlear
malformations, lacrimal duct stenosis
A: Renal dysplasia (66%) – Agenesis, polycystic kidneys,
duplicated ureters
3: Renal abnormalities identifiable on IVP or renal U/S
110. Q: Goldenhar syndrome (oculoauriculovertebral
spectrum)
*Treacher-Collins Sx A: Most sporadic, some AD
107. Q: Discuss Achondroplasia A: Unilateral facial asymmetry, Hemifacial microsomia
A: Most common cause of short limb dwarfism A: Ocular – Upper lid coloboma, epibulbar dermoids
A: AD, most sporadic, mutation of FGFR-3 gene, chromosome A: Otologic – Mild deformity to Anotia, EAC atresia, ossicular
4p16.3 abnormalities, CHL>SNHL
A: Short limbs, genu varum, limited elbow extension, trident hand, A: Vertebral: Cervical fusion
long trunk, lumbar lordosis, frontal bossing, sunken nasal bridge, A: Others: Underdevelopment of Orbit, Facial muscles, Mandible
midface hypoplasia
A: Normal cognition
108. Q: Apert (Acrocephalosyndactyly), Crouzon (Craniofacial
dysostosis) and Pfeiffer syndromes
A: AD, mutations of FGFR-2 gene, chromosome 10q26 *Upper lid coloboma and epibulbar dermoid
A: Craniosynostosis (Brachycephaly), midface hypoplasia, low
nasal bridge, Parrotbeaked nose, choanal stenosis or atresia,
mandibular prognathism, high arched palate, bifid uvula, cleft
palate, and cervical fusion
A: Hypertelorism, exophthalmos, and strabismus
A: Cognitively normal to severe mental retardation
A: Apert specific – Syndactyly, Stapes fixation (CHL) and patent
Cochlear aqueduct
A: Pfeiffer specific – Digital broadening

*Goldenhar Syndrome
111. Q: Classification of hemifacial microsomia?
A: OMENS+ classification: A: CT scan
116. Q: Four parts to the anatomic deformity in Choanal
- O is for orbital distortion
Atresia
- M is for mandibular hypoplasia
A: Narrow nasal cavity
- E is for ear anomaly
A: Lateral bony obstruction from Pterygoid plate
- N is for nerve involvement
A: Medial bony obstruction from Vomer
- S is for soft tissue deficiency
A: Membraneous obstruction
- Plus is used to include the expanded spectrum: cardiac,
skeletal, pulmonary, renal, gastrointestinal, and limb 117. Q: General management approach of Choanal Atresia
anomalies. A: Unilateral – Nonurgent repair, ~1 year of age
A: Bilateral – Establish airway & feeding pathway (McGovern
112. Q: Maffucci syndrome nipple, Oropharyngeal airway; intubation not necessary unless
A: Multiple Cavernous Hemangiomas, occasional visceral vascular mechanical ventilation required)
lesions A: Surgical repair approaches (SPAN = transSeptal, transPalatal,
A: Dyschondroplasia & shortening/deformity of involved bones transAntral, transNasal)
A: Chondrosarcoma in 25% A: Postop care includes – ICU monitoring, frequent Suctioning,
Antibiotics, PPI
113. Q: Describe von Hippel Lindau syndrome (HIPPEL)
A: AD, mutation in the VHL gene, chromosome 3p25 118. Q: Syndromes are associated with Choanal Atresia (50%
A: Hemangioblastomas of CNS & retinas of all cases, CAT CTV)
A: renal cysts/carcInoma A: Crouzon syndrome
A: Pheochromocytoma A: Apert syndrome
A: Pancreatic cysts A: Treacher-Collins syndrome
A: Epididymal papillary cystademonata A: CHARGE syndrome
A: endoLymphatic sac tumors in 11% A: Trisomies 18, 21
A: Dx criteria: A: Velocardiofacial syndrome
• Family history of von Hippel-Lindau (VHL) disease
119. Q: Describe CHARGE syndrome
PLUS a tumour (CNS/retinal haemangioblastoma or clear
A: AD, CHD7 gene, chromosome 8q12
cell renal cell carcinoma (RCC)); OR
A: Coloboma
• If no family history, ≥2 CNS/retinal haemangioblastomas
A: Heart disease (endocardial cushion defect)
plus visceral tumour (RCC, phaeochromocytoma or
A: Atresia (choanal)
pancreatic tumour).
A: Retardation of growth, or mentation
A: Genital defects (in males)
A: Ear anomalies & deafness (CHL>SNHL)
120. Q: Embryologic spaces/structures of note in
Glioma/Encephalocele formation
A: Anterior neuropore
A: Foramen Cecum (between frontal and ethmoid)
A: Prenasal Space (between nasal bones and cartilaginous septum)
A: Fonticulus Nasofrontalis (between frontal and nasal bones)
121. Q: Ddx of pediatric midline nasal mass
A: Dermoid cyst (most common)
A: Neurogenic – Glioma, Encephalocele, Neurofibroma
A: Hemangioma
122. Q: Dermoid
A: Epithelium lined, contains skin appendages, sinus tract leading
to the skin
A: Contains ectoderm and mesoderm
A: Pathognomonic sign: Protruding hair (seen in a minority)
*Manifestations of VHL syndrome A: Highly sensitive IC extension findings on imaging: Bifid crista
galli and enlarged foramen caecum
114. Q: Epidemiology of Choanal Atresia A: Dural connection in 30%
A: Incidence 1:5000-8000 births 123. Q: Glioma
A: F/M = 2/1 A: Solid mass of Glial tissue with a fibrous stalk
A: 50% have other anomalies (75% of bilateral cases) A: Dural connection in 15%
A: 60% mixed bony-membranous, 30% bony, 10% membranous A: 60% external (glabella), 30% internal (lateral nasal wall), 10%
A: 70% unilateral (60% of which are right-sided) combined
115. Q: Four methods of evaluating for Choanal Atresia A:  Path: dysplastic, neuroglial and fibrovascular tissue with NO
A: Using cotton or mirror to detect airflow ependymal tissue
A: Inability to pass a small suction catheter A: Manage any intracranial portion first; surgical excision through
A: Flexible scope vertical midline dorsal excision, external rhinoplasty, or bicoronal
approach
124. Q: Classification of congenital Encephaloceles amoxicillin component), Cefpodoxime proxetil or cefuroxime
A: Occipital – Most common, ~75% of cases axetil.
A: Sincipital/frontoethmoidal, ~15% – A: Severe ARS with Abx in the past 4-6 weeks:
- nasoFrontal (most common subtype)
Amoxicillin/clavulanate or combination therapy (amoxicillin or
- nasoEthmoidal
- nasoOrbital clindamycin plus cefpodoxime or cefixime)
A: Basal, ~10% –
- Transethmoidal (most common subtype) 129. Q: Viruses most commonly associated with acute
- Sphenoethmoidal rhinosinusitis
- Transsphenoidal A: Rhinovirus
- Sphenoorbital A: Influenzae
3: Path: glial component with astrocytes surrounded by collagen, A: Parainfluenza
submucosal glands and sometimes septal cartilage with ependymal A: Adenovirus
tissue (not present in gliomas) 3: Others may include coronavirus, and RSV
3: Surgical excision needed within the first few months of life to 130. Q: Bacteriology of acute pediatric sinusitis
minimize the risk of meningitis and cosmetic deformity open or A: Streptococcus pneumonia
endo A: Moraxella catarrhalis
125. Q: Describe Furstenburg’s sign A: Haemphilus influenzae
A: Expansion of a nasal mass with compression of the both IJV’s, 131. Q: Bacteriology of chronic pediatric sinusitis
associated with encephalocele, but not glioma or dermoid A: Aerobes: S. pneumonia, M. catarrhalis, H. influenzae, S. aureus,
126. Q: Parson’s major criteria (7) for chronic pediatric α-hemolytic Strep, P. aeruginosa
sinusitis A: Anaerobes: Peptococcus, Peptostreptococcus, Bacteroides
A: Chonic nasal obstruction 132. Q: Indications for CT scanning for pediatric rhinosinusitis
A: Nasal discharge A: Severe illness or Toxic condition
A: Postnasal drainage A: Immunocompromise
A: Chronic cough A: Acute RS that does not improve with medical therapy in 48-72
A: Halitosis hours
A: Headache A: Suppurative Complication
A: Behavioral change
133. Garcia & Harris indications of draining an orbital
127. Q: How to diagnose peds acute ARBS according to AAP subperiosteal abscess?
guidelines 2001?
A: Age>9
A: Infection of the paranasal sinuses lasting less than 30 days that A: Large size >10 mm
presents with either persistent or severe symptoms A: Acute optic nerve or retinal compromise
A: Persistent symptoms are those that last longer than 10 to 14, but A: frontal sinusitis
less than 30, days. Such symptoms include nasal or postnasal A: Non-medial subperiosteal abscess
discharge (of any quality), daytime cough (which may be worse at A: Chronic sinusitis
night), or both. A: Odontogenic source
A: Severe symptoms include a temperature of at least 102°F (39C) A: Suspicion of anaerobic subperiosteal infection (e.g., presence of
and purulent nasal discharge present concurrently for at least 3 to 4 gas within the abscess space as visualized on CT scan)
consecutive days in a child who seems ill. A: Recurrent/prior I&D
3: Subacute 4-12 weeks, chronic > 12 weeks, recurrent acute A: Others that are not included: Worsening despite medical Tx,
bacterial sinusitis defined as having had 3 episodes in 6 months or lack of improvement in 48 hrs.
4 episodes in 12 months
134. Q: 5 indications for pediatric maxillary sinus aspirate
128. Abx in ABRS according to  Antimicrobial guidelines for A: Severe Toxic child
the treatment of ABRS in immunocompetent children, A: Immunocompromise
2002? A: Unresolving symptoms after 72 hours
A: Abx given for 10-14 days. If no improvement in Sx after 72 hrs A: Suppurative complications
A: Work up for fever of unknown origin
consider an alternative Abx. If pt. is NOT aSx after completing the
course of Abx  cont. on Abx for 7-10 days 135. Q: Absolute Indications for FESS in children
A: Mild ARS & no Abx in the past 4-6 weeks: Amoxicillin (45–90 A: Massive polyps in CF
A: Antrochoanal polyp
mg/kg per day), Amoxicillin/clavulanate (45–90 mg/kg per day),
A: Fungal sinusitis
Cefpodoxime proxetil, Cefuroxime axetil, If allergic to β –lactams: A: Mucocele
TMP/SMX, Azithromycin, clarithromycin, or erythromycin. A: Intracranial complication
A: Mild ARS with Abx in the past 4-6 weeks OR A: Orbital abscess
Severe ARS with no Abx in the past 4-6 weeks: High-dose A: Traumatic injury to optic nerve
amoxicillin (90 mg/kg/day), Amoxicillin/clavulanate (high-dose A: dacrocystorhinitis due to sinusitis and resistant to medical Tx
A: Meningoencephaloceles and neoplasms
3: Relative indication = CRS exacerbation despite maximal
medical management
136. Q: Immune workup for recurrent sinusitis 141. Q: Three diagnostic signs of submucous cleft palate
A: IgG subclasses A: Bifid uvula
A: IgM A: Muscular diastasis of the soft palate (zona pellucida)
A: IgA A: Notched hard palate
A: IgE 142. Q: Environmental factors contributing to cleft palate
A: Ability to respond to polysaccharide antigens of S. pneumoniae, A: Drugs – Phenytoin, Thalidomide, Vitamin A derivatives, Folic
and H. flu acid antagonists, steroids in 1st trimester
137. Q: Lab finding with common variable hypoglobulinemia A: Smoking & Alcohol use in 1st trimester
A: Consistently low total immunoglobulins A: Amniotic band syndrome, maternal diabetes
138. Q: 2 ways pediatric allergic fungal sinusitis is different 143. What is SIMONART’S BAND?
from adult A: In an incomplete CL, bridge or bar of lip tissue of varying size
A: More likely to facial skeleton abnormalities
that bridges the cleft gap
A: More likely unilateral
139. Q: Bacteriology of pediatric Acute Sialadenitis
A: Staphylococcus aureus
A: Streptococcus viridans
A: Streptococcus pneumoniae
A: Streptococcus micros
A: Esherichia coli
A: Bacteroides melaninogenicus
140. Q: Epidemiology & classification of Cleft Lip and Palate
A: Second most common malformation after club foot
A: CL +/- P in 1/1000 births, more in native Americans, M:F 2:1
A: Isolated CP occur in 1/2000 births, does NOT vary among
ethnic groups, M:F 1:2
A: 70% of CL+/-P nonsyndromic, 50% of CP nonsyndromic 144. Things to follow in Cleft L&P patients?
A: Risk of inheritance in non syndromic CL&P and classification
systems below (there are a few more) A: CLP team consultation
A: Growth & Feeding: haberman/mead johnson/pigeon bottles
A: Hearing screening & F/U
A: SLP referral & F/U
A: Genetic counselling
A: Psych/social issues
A: Orthodontic evaluation

145. Q: Lip adhesion

A: If necessary, done @ 2-4 weeks with definitive repair at 4-6
months of age
A: For unilateral, bilateral, or asymmetric wide complete cleft lip
and palate
146. Q: Five options for Cleft Lip repair
A: Rule of 10’s = 10 weeks, 10 pounds, 10 g of hemoglobin
A: Straight line closure (rarely used anymore)
A: Millard rotation advancement technique
A: Millard bilateral cleft repair
A: Tennison-Randall or Skoog techniques (single) triangular flap
interdigitation
A: Bardach (double) triangular flap interdigitation
*Iowa classification of CL&P (Bailey’s)

VEAU CLASSIFICATION, 1931 147. Q: Cleft Palate repair

•Group 1: cleft of the soft palate only A: Performed at 5-15 months
•Group 2: cleft of the soft and hard palate as A: Restoration of soft palate sling incorporating tensor and levator
far forward as the incisive foramen. palate muscles
•Group 3: complete unilateral alveolar cleft, A: Soft palate repair only:
usually involving the lip - Schweckendiek (primary veloplasty), obturate HP until
•Group 4: complete bilateral alveolar cleft, older
usually associated with bilateral clefts of the A: Secondary CP repair:
lip.
 - If isolated 2ndry CP:
o Von Langenbeck (bipedicled flap palatoplasty)
- If primary + secondary CP: (but just fixing secondary P)
o Wardill-Kilner-Peet V-Y pushback technique
o Bardach uni-pedicle two flap palatoplasty
A: Repair of complete cleft of primary & secondary palate or 2ndy
cleft palate alone or SM cleft: Furlow double opposing Z-plasty
* Schweckendiek
*
 Von Langenbeck
* Wardill-Kilner-Peet V-Y pushback technique (Lt) & Bardach
two flap palatoplasty (Rt)
*
 Furlow double opposing Z-plasty
148. Q: Characteristics of the unilateral Cleft Lip Nasal
deformity
A: Nasal tip deflected to noncleft side with short medial crus and
long lateral crus on cleft side
A: Columella lies on noncleft side 2ndary to unopposed action of
intact orbicularis oris
A: Septum deflected to noncleft side
A: Nasal dorsum to tilt to the cleft side due to underdeveloped
nasal bones and nasal process of the maxilla
A: Lat crus of LLC caudally displaced on cleft side
A: Nostril on cleft side horizontally oriented (rather than vertical)
A: Alar base on cleft side displaced lat/inf/post
A: Deficiency of maxillary bone on cleft side (nasal floor often
absent)
3: Bil. CL nasal deformity: lack of adequate columellar tissue (Alar
cartilage & skin deficient), tip broad and flat, ala laterally displaced
with horizontal oriented nostrils
*Unilateral cleft lip nasal deformity
149. Q: Outline the timeline of Cleft Lip and Palate repair
A: 3 months – Cleft lip repair, PET insertion
A: 1 year – Cleft palate repair
A: 5 years – Columellar lengthening (for bilateral cleft lip)
A: 8-16 years – Orthodontics
A: 10 years – Alveolar cancellous bone graft
A: 14 years – Definitive rhinoplasty and orthognatic surgery
150. Q: List five syndromes associated with Cleft Palate (TDP
CAVS)
A: Stickler
A: Treacher-Collins
A: Pierre-Robin sequence
A: Apert
A: Velocardiofacial
A: Down
A: Crouzon’s
151. Q: Passavant’s ridge
A: Prominence on the posterior wall of the nasopharynx from
contraction of the superior constrictor during swallowing
152. Q: Management of velopharyngeal insufficiency (VPI)
A: Non-surgical – Speech therapy, Prosthetics (palatal lift or
obturator), Biofeedback with nasometry
A: Surgical – Pharyngoplasty, Pharyngeal flaps, Posterior
pharyngeal wall augmentation
153. Q: Name all 6 components of Walderyer’s ring
A: Lingual tonsils
A: Palatine tonsils
A: Adenoid
A: Gerlach’s tonsils (Tubal, posterior to ET opening)
A: Lateral bands
A: Posterior pharyngeal wall
154. Q: Adenoid blood supply (5)
A: Pharyngeal branch of the internal maxillary (major supply)
A: Ascending pharyngeal artery
A: Ascending palatine branch of the facial artery
A: Ascending cervical branch of thyrocervical trunk
A: Artery of the pterygoid canal
155. Q: Adenoid innervation 165. Q: Five symptoms of Acute Tonsillitis
A: CNs IX & X A: Erythematous exudative tonsils
A: Sore throat
156. Q: Adenoid histology
A: Dysphagia
A: Ciliated pseudostratified columnar epithelium
A: Tender cervical adenopathy
A: Stratified squamous epithelium
A: Fever
A: Transitional epithelium
3: Inflammation increases specialized squamous epithelium 166. Q: Recurrent Acute Tonsillitis
proportion and decreases respiratory proportion A: 7 infections in a year
A: 5 infections for 2 consecutive years
157. Q: Four zones of antigen processing in adenotonsillar
A: 3 infections for 3 consecutive years
tissue
A: Specialized squamous epithelium (Dendritic cells) 167. Q: Five symptoms/signs of Chronic Tonsillitis
A: Extrafollicular area (T-cells) A: Chronic sore throat
A: Mantle zone of lymphoid follicle (Mature B-lymphocytes) A: Halitosis
A: Germinal center of follicle (Active B-cells) A: Tonsilloliths
A: Peritonsillar erythema
A: Persistent tender cervical adenopathy
168. Q: Four symptoms/signs of Obstructive Tonsillar
hyperplasia
A: Enlarged tonsils
A: Snoring
A: Obstructive disturbances
A: Dysphagia and voice changes
169. Q: Seven Etiologies of pseudomembranous tonsillitis
A: Epstein-Barr virus (mononucleosis)
A: Group A ß-hemolytic Streptococcus
A: Corynebacterium diphtheriae
A: Neisseria gonnorheae
A: Syphilis
158. Q: Tonsil blood supply A: Candidiasis
A: Lower pole: A: Vincent’s angina
- Facial artery (Tonsillar & Ascending Palatine branches)
- Dorsal lingual 170. Define Centor criteria for likelihood of acute GABHS
A: Upper pole: tonsillitis?
- Internal maxillary artery (lesser +/- greater palatine art) A: Four criteria:
- Ascending pharyngeal artery
- History of fever
159. Q: Describe efferent lymphatics of the tonsils and - Tonsillar exudates
adenoids - Tender anterior cervical adenopathy
A: Upper deep cervical nodes (T+A) - Absence of cough
A: Retropharyngeal LN (A only) A: Each criteria = 1 point
3: No afferent lymphatics for T and A A: Management:
160. Q: Major immunologic product of tonsils and adenoids
- 0-1 points - No antibiotic or throat culture necessary (Risk
A: Secretory IgA
of strep. infection <10%)
161. Q: Four symptoms of Acute Adenoiditis - 2-3 points - Throat culture and treat with an antibiotic if
A: Purulent rhinorrhea culture is positive (Risk of strep. infection 32% if 3
A: Nasal obstruction criteria, 15% if 2)
A: Fever - 4 points - Treat with antibiotic (Risk of strep. infection
A: Otitis media 56%)
162. Q: Recurrent acute adenoiditis
171. Q: Indications for Adenoidectomy (ION)
A: 4+ episodes in a 6 month period
A: Infection – Recurrent acute/chronic Adenoiditis, recurrent
163. Q: Four symptoms of Chronic Adenoiditis acute/chronic Otitis Media with or without Effusion (kids >4 years
A: Persistent nasal discharge benefit from adenoidectomy with the Second set of PETs), chronic
A: Chronic congestion Sinusitis
A: Postnasal drip A: Obstruction – Adenoid hyperplasia with chronic Nasal
A: Halitosis Obstruction or obligate Mouth Breathing, Sleep-related disordered
breathing (OSAS, OSHS, UARS), Cor pulmonale, FTT, Orofacial
164. Q: Obstructive adenoid hyperplasia triad growth/dental/speech/swallowing abnormalities
A: Chronic nasal obstruction (obligate mouth breathing, snoring) A: Neoplasia – Suspected, benign or malignant,
A: Rhinorrhea Lymphoproliferative disorder
A: Hyponasal speech 3: Absolute indications for adenoidectomy (Darrow, 2002)
 - OSA - Dysphagia
- Suspected Malignancy - Speech impairment
- FTT - Halitosis
- Abnormal dentofacial growth - Recurrent or chronic pharyngotonsillitis, peritonsillar
Relative: abscess
- UAO (SDB) - Streptococcal carriage
- Dysphagia Clinical indicators for tonsillectomy as recommended by the
- Speech impairment AAOHNS in 2000 are:
- Halitosis
- Otitis media - Patient with 7 or more episodes of tonsillitis in the
- Recurrent or chronic rhinosinusitis or adenoiditis preceding year, OR 5 or more episodes in each of the
Clinical indicators for adenoidectomy as recommended by the preceding 2 y, OR 3 or more episodes in each of the
AAO-HNS in 2000 are: preceding 3 y despite adequate medical therapy (with
- Four or more episodes of recurrent purulent rhinorrhea in documentation in the medical record for each episode of
prior 12 months in a child <12. sore throat and one or more of the following: temperature
- One episode documented by intranasal examination or >38.3°C, cervical adenopathy, tonsillar exudate, or
diagnostic imaging. positive test for GABHS)  Paradise criteria
- Persisting symptoms of adenoiditis after 2 courses of - Hypertrophy causing dental malocclusion or adversely
antibiotic therapy. affecting orofacial growth documented by orthodontist.
- One course of antibiotics should be with a betalactamase - Hypertrophy causing upper airway obstruction, severe
stable antibiotic for at least 2 weeks. dysphagia, sleep disorders, or cardiopulmonary
- Sleep disturbance with nasal airway obstruction persisting complications.
for at least 3 months. - Peritonsillar abscess unresponsive to medical management
- Hyponasal or nasal speech and drainage documented by surgeon, unless surgery
- Otitis media with effusion >3 months or second set of performed during acute stage.
tubes - Persistent foul taste or breath due to chronic tonsillitis not
- Dental malocclusion or orofacial growth disturbance responsive to medical therapy.
documented by orthodontist. - Chronic or recurrent tonsillitis associated with the
- Cardiopulmonary complications including cor pulmonale, streptococcal carrier state and not responding to beta-
pulmonary hypertension, and right ventricular lactamase resistant antibiotics.
hypertrophy associated with upper airway obstruction. - Unilateral tonsil hypertrophy presumed neoplastic
- Otitis media with effusion over age 4. 175. Q: Four contraindications for Tonsillectomy (LASH CPA)
172. Q: Four contraindications for Adenoidectomy A: Leukemias
A: Cleft palate A: Agranulocytosis
A: Submucous cleft A: Systemic disease that is uncontrolled (DM, TB)
A: Hypernasal speech A: Hemophilias
A: Nasal regurgitation 3: Relative contraindications = Cleft Palate, and Acute infection
3: All are signs of VPI
176. Q: Eight Criteria which should suggest an overnight stay
173. Q: Risk factors for VPI after adenoidectomy posttonsillectomy
A: History of nasal fluid regurgitation A: Under 3 years of age
A: Occult submucus cleft A: OSA
A: Family history of clefts A: Craniofacial abnormalities
A: Neuromuscular problems (CNS) A: Neuromuscular dz – CP, duchenne, down’s
174. Q: Indications for Tonsillectomy (ION) A: Mucopolysaccharidosis – hunter’s, hurler’s
A: Infection – Recurrent acute/chronic Tonsillitis, Halitosis, A: Medical comorbidity (diabetes, seizures, asthma, cardiac
Complications (Cervical Abscess, Airway obstruction Cardiac disease, etc)
valve disease, recurrent febrile Seizures, tonsillar Hemorrhage), A: Peritonsillar abscess
streptococcus Carrier unresponsive to medical treatment, recurrent A: Emesis or hemorrhage
or unresponsive Peritonsillar Abscess A: Patient lives greater than 60 minutes away from hospital
A: Obstruction – Tonsillar hyperplasia with obstruction, A: Poor socioeconomic class predisposing to neglect
Sleeprelated disordered breathing (OSAS, OSHS, UARS), Cor 3: Indications for overnight monitoring according to CPG 2011:
pulmonale, FTT, Orofacial growth/dental/speech/swallowing Age <3 and severe OSA (AHI>10 or O2 sat nadir <80%)
abnormalities 177. Indications for PSG prior to Tonsillectomy (CPG 2011)?
A: Neoplasia – Suspected, benign or malignant,
Lymphoproliferative disorder A: Need for surgery is uncertain
3: Absolute indications for tonsillectomy (Darrow, 2002) A: When there is discordance between tonsillar size on PE and the
- Suspected Malignancy reported severity of SDB
- FTT A: Obesity
- OSA A: Down syndrome
- Abnormal dentofacial growth
A: Craniofacial abnormalities
- hemorrhagic tonsillitis
Relative: A: Neuromuscular disorders
- UAO (SDB) A: Sickle cell disease
A: Mucopolysaccharidoses Muscular dystrophies, Seizures, Prader-Willi, Chiari
malformations
178. Q: Complications of Adenotonsillectomy A: Cardiovascular disease – Pulmonary/systemic HTN, Congenital
A: Halitosis (most common) heart disease
A: Dehydration A: Morbid obesity
A: Postoperative hemorrhage – 0.5-1% A: Pectus excavatum/Scoliosis
A: Pulmonary – postoperative edema & hypoxemia (loss of auto-
186. Q: Ten Differences in adult vs pediatric OSAS
PEEP, hypercapneic respiratory drive)
A: Snoring intermittent vs continuous
A: Airway obstruction
A: Mouth breathing rare vs common
A: Velopharyngeal insufficiency
A: Obesity common vs rare
A: Nasopharyngeal stenosis
A: Failure to thrive & enuresis rare vs common
A: Atlantoaxial subluxation (Grisel’s syndrome) – Down syndrome
A: Daytime somnolence common vs rare
at higher risk
A: Hyperactivity, attention deficit, aggression rare vs common
A: Eagle syndrome
A: Nighttime arousals common vs rare
A: Death – 1/25,000
A: Gender predilection male vs none
179. Q: Six reasons for post-adenotonsillectomy desaturation A: CPAP mainstay vs selected (postop OSA, craniofacial anomaly,
A: Post-obstructive pulmonary edema C/I to surgery)
A: Loss of hypercapnic respiratory drive A: Surgery selected vs mainstay
A: Airway swelling & obstruction
187. Q: Criteria for abnormal polysomnogram in pediatric
A: Aspiration of blood clots
OSAS
A: Laryngospam
A: Apnea index ≥1 (lasting longer than two consecutive breaths)
A: Narcotic overmedication
A: Oxygen desaturation to <92%, >4% x >3 times/hour, or
180. Q: Define Grisel’s syndrome associated with a change in heart rate >25%
A: Alanto-axial joint laxity due to adenoidectomy A: End tidal CO2 >50mmHg >8% of total sleep time (or >53 at
A: Vertebral body decalcification and laxity of anterior transverse any point), or >45mmHg >60% of total sleep time
ligament between axis and atlas due to inflammation/infection in
the nasopharynx 188. Q: Indications for PSG postsurgery for pediatric OSAS
A: Spontaneous subluxation occurs 1 week post op – Pain and A: Persistent snoring
Torticollis A: Preoperative Severe OSA
A: Preoperative OSA complications
181. Q: Management of Peritonsillar Abscess (infection of a
A: Age <1 year
peritonsillar salivary/Weber gland)
A: Hydration 189. Q: Hunter’s syndrome
A: Analgesia A: X-linked, type II mucopolysaccharidosis
A: Incision & drainage or needle aspiration (75% effective), A: Deficiency of beta-galactosidase
culture A: Macrocephaly, broad face, low nasal bridge, death usually
A: Antibiotics, parenteral, enteral, or combined occurs from infiltrative CMO & valvular disease leading to CHF
A: If unsuccessful – CT scan, reincision & drainage, Quinsy
190. Q: Hurler’s syndrome
tonsillectomy
A: AR, Type 1 mucopolysaccharidosis
182. Q: Six Complications of Peritonsillar Abscess A: Deficiency of α-1-iduronidase which breaks down heparan-,
A: Airway obstruction dermatan- and keratan-sulfates
A: Dehydration A: Visceromegaly, macroglossia, macrocephaly, progressive
A: Spread to other spaces neurologic dysfunction, death in 1st decade
A: Carotid artery erosion
191. Q: Differences between the pediatric & adult larynx
A: IJV thrombophlebitis (Lemierre’s syndrome)
A: Pediatric larynx higher in neck (C3/4 vs C5/6)
A: Sepsis
A: Epiglottis curved/omega shaped, in contact with soft palate
183. Q: Causes of Unilateral Tonsillar hyperplasia A: Thyroid cartilage oblique, no defining angle, overlapping with
A: Neoplastic – Lymphoma hyoid & cricoid
A: Infectious – Mycobacteria (tuberculosis, atypical), A: Arytenoids are relatively large
Actinomycosis, Fungal A: Infant vocal cords 4-4.5mm long at birth, adults 14-23mm
A: Infant true vocal cord 50% composed of vocal process of
184. Q: Ddx of a Congenital Tonsillar mass (3)
arytenoid, in adults 25-33%
A: Teratoma
A: Funnel shape, Infant subglottis narrowest portion of airway,
A: Hemangioma
4.5-5 mm at full term
A: Lymphatic malformation
A: Infant subglottis is loose, with a lot of submucosal glands
185. Q: High risk groups for Pediatric OSAS
192. Q: Age when cricoid is no longer narrowest segment of
A: Abnormal airway anatomy – Down’s, Pierre-Robin,
airway
Achondroplasia, Craniosynostoses, Treacher-Collins,
A: 8 years
Macroglossia, Klippel-Feil, Pyriform aperture stenosis,
Laryngomalacia, Masses 193. Q: Embryology of the tracheoesophageal system
A: Neuromuscular disease – CP, Down, Hypothyroidism, A: During the fourth week (26 days)
A: Laryngotracheal groove appears as median outgrowth from the
caudal end of the ventral wall of the primitive pharynx
A: Laryngotracheal diverticulum forms by 28 days
A: Tracheoesophageal folds fuse into a septum that separates the
laryngotracheal tube
A: Arytenoid swellings from neural crest cell-derived mesenchyme
of the 4th and 6th arches
A: Laryngeal epithelium occludes lumen at 8th week, and
recanalization occurs by 10th week.
194. Prenatal sonographic findings of CHAOS?
A: Increased in lung size and echogenicity
A: Fluid-filled & dilated trachea
A: Fetal hydrops
A: Polyhydramnios
195. Q: Location of stridor by its pattern
A: Inspiratory = dynamic supraglottis and glottis
A: Biphasic = subglottis and cervical trachea
A: Expiratory = fixed intrathoracic trachea
196. Q: Stridor history mnemonic “SPEC SPEAR”
A: S = Severity, parents’ Subjective impression
A: P = Progression of obstruction over time
A: E = Eating/feeding difficulties
A: C = Cyanotic spells
A: S = Sleep disordered breathing
A: P = history of Prematurity
A: E = history of Endotracheal intubation
A: A = possibility of foreign body Aspiration
A: R = Radiographics that detect a specific abnormality
197. Q: 10 Signs of airway obstruction
A: Stridor
A: Dyspnea, tachypnea
A: Tachycardia
A: Diaphoresis, circumoral pallor, anxiety/restlessness
A: Retractions – tracheal tug, suprasternal, intercostal, substernal
A: Flaring of nasal alae,
A: Use of accessory respiratory muscles
A: Cyanosis, in extreme cases
A: Respiratory arrest
198. Q: The 5 “A” of stridor
A: Age
A: Acuteness
A: Appearance (toxic or non-toxic)
A: Acoustics (volume, pitch, phase)
A: Associated symptoms (dysphonia, cough, drooling, posturing,
dysphagia)
199. Q: Airway imaging modalities
A: Plain soft tissue films of the neck, AP (croup) + lateral
(epiglottittis & RPA)
A: CXR AP + lateral (FB & tracheal stenosis)
A: Inspiratory & expiratory chest films (FB)
A: Airway Fluoroscopy (dynamic, awake & sleep, best for OSA)
A: Barium swallow (vascular compression)
A: Electron beam CT, or spiral CT scan with apnea
A: MRI of the airway (intrathoracic vascular anomalies & masses)
A: Bronchogram (after MRI, if difficult tracheobronchial stenosis)
A: Laryngeal U/S (linear 7.0, 10 MHz, B-mode real-time)
200. Q: Indications for endoscopic examination in pediatric
stridor (PAUSe)
A: Progressive stridor
A: parental Anxiety
A: Unusual features – Cyanotic attacks, apneic attacks, dysphagia,
aspiration, recurrent pneumonias, failure to thrive, radiologic
abnormality
A: Severe stridor
201. Q: What 4 pediatric airway abnormalities are improved in
the prone position?
A: Laryngomalacia
A: Pierre-Robin sequence
A: Vascular compression
A: Mediastinal mass
202. Q: Clinical characteristics of Laryngomalacia
A: Variable inspiratory stridor – Begins in first few days/weeks
after birth, worse with crying, feeding, or supine position with
H&N flexed, better when prone or with H&N extended
A: Signs of intermittent upper airway obstruction
A: Normal cry
A: Normal general health and development
203. Q: Factors influencing development of laryngomalacia
A: Shortened aryepiglottic folds
A: Anterior collapse of cuneiform cartilage
A: Immature neuromuscular control
A: Reflux
204. Q: How is LPR different from classic GERD?
A: Patients have head & neck symptoms but heartburn is
uncommon
A: Predominantly upright (day-time) reflux
A: Normal esophageal motility
A: Most do not have esophagitis, as in GERD
A: Laryngopharyngeal epithelium is more susceptible to reflux
related injury than esophageal epithelium
205. Q: Six Evaluations for Laryngopharyngeal Reflux (LPR)
A: Gastric emptying scan (milk scan) technetium 99 m
A: 24 hour double-probe pH monitoring
A: Barium swallow
A: Broncho-alveolar lavage for lipid laden macrophages (LLM,
70% needed)
A: Esophagogastroduodenoscopy with Biopsy (EGD, suspected
eosinophilic esophagitis)
A: Diagnostic markers pepsin & carbonic anhydrase isoenzyme III
(CA-III)
206. Q: Non-surgical management of laryngomalacia
A: Observation (90%)
A: Anti-reflux
A: Position
A: Thickened feeds
A: Frequent smaller meals
A: CPAP
207. Q: Five Indications for surgeries for severe
laryngomalacia (10%)
A: FTT, weight loss, feeding difficulty
A: Life-threatening episode, cyanotic attacks, respiratory distress,
documented desaturation
A: Cor pulmonale, pulmonary hypertension
A: Obstructive sleep apnea
A: Severe chest deformity
208. Q: Interventions for severe laryngomalacia
A: Supraglottoplasty/epiglottoplasty – Unilateral or bilateral,
division of aryepiglottic fold, partial epiglottis amputation, removal
of redundant supra-arytenoid mucosa and lateral borders of
epiglottis, removal of cuneiform & corniculate cartilages
A: Epiglottopexy with glossoepiglottic adhesion
A: Tracheostomy (rare)
209. Q: Six Complications of Supraglottoplasty
A: Bleeding
A: Temporary dysphagia
A: Aspiration
A: Temporary worsening of airway
A: Supraglottic Stenosis (< 5% if bilateral, preserve islands of
mucosa, esp. interarytenoid)
A: Re-operation (15% if unilateral)
210. Q: Methods of Voice Assessment
A: Audiotape & videotape Recording (speech therapist)
A: Parent & child Questionnaire
A: Fiberoptic laryngoscopy with video-stroboscopy
A: Spectral voice Analysis = Multi-Dimensional Voice Program
(MDVP)
211. Q: Ddx of pediatric vocal cord paralysis (NATIVE)
A: Neurologic (brainstem tumor, CP, hydrocephalus,
meningomyelocele, hypoxic encephalopathy, hypotonia)
A: Arnold-Chiari malformation (ALWAYS consider in neonate)
A: Birth Trauma (C-section, nuchal cord, Recovery >9 mos)
A: Iatrogenic injury (CVS, PDA, TEF)
A: Idiopathic (47%, especially bilateral)
A: Infectious – Syphilis
A: Vascular anomalies
A: Everything else – Mobius, Charcot-Marie Tooth
3: >50% Bilateral
212. Q: 4 types of Chiari malformations
A: I – Protrusion of cerebellar Tonsils
A: II – Protrusion of cerebellar Vermis, lower Pons and Medulla
(Arnold-Chiari malformation)
A: III – Herniation of Cerebellum (high cervical meningocele)
A: IV – Cerebellar Hypoplasia (Dandy-Walker syndrome)
213. Q: ENT manifestations of Arnold-Chiari malformation
A: CN IX-XII difficulties
A: Bilateral vocal cord paralysis, respiratory distress
A: Poor Feeding
A: Aspiration
214. Q: Management of Bilateral VCP
A: +/- urgent intubation
A: +/- ACLS management
A: Supportive measures (upright positioning, thickening of
formula, observation, management)
A: Tracheotomy (> 2 years to allow for spontaneous recovery,
50%)
A: Lateralization (arytenoidectomy +/- CO2 laser cordotomy,
arytenoidopexy, cordotomy)
A: Open procedures / Reanimation / Electrical pacers
3: Decannulation rates > 60%
215. Q: Benjamin & Inglis classification of Laryngeal Clefts
A: I – Interarytenoid
A: II – Partial cricoid (below VCs)
A: III – Through entire cricoid into cervical trachea
A: IV – Distal (thoracic) trachea
216. Q: Laryngeal cleft associated syndrome?
A: Pallister-Hall Sx
217. Q: Best imaging study for Laryngeal Cleft
A: Barium swallow (gastrograffin bad for lungs)
218. Q: Management of Laryngeal Clefts by grade
A: I – Observation, anti-reflux & thickened feeds, vs. Endoscopic
repair (2 layer closure)
A: II or III – Laryngofissure for precise multilayered anatomic
closure
A: IV – Laryngofissure & Sternotomy vs. Lateral pharyngotomy &
Thoracotomy (postop ECMO, no ETT)
219. Q: Anomalies associated with Laryngeal Clefts
A: Esophageal atresia +/- TEF (#1, 6% of TEFs have clefts,
continued aspiration)
A: Cleft lip/palate
A: CHD, GI anomalies
A: G syndrome (Opitz-Friass) – Hypertelorism, Hypospadias, Cleft
lip/palate
A: Pallister-Hall syndrome – Bifid epiglottis, Hypothalamic
hamartoblastoma, Hypopituitarism, Imperforate anus, Postaxial
polydactyly
220. Q: Relative frequency of Laryngeal Webs by location
A: Glottic = 75%
A: Subglottic = 7%
A: Supraglottic = 2%
221. Q: Investigations for Laryngeal Web
A: Flexible laryngoscopy
A: +/- Rigid laryngoscopy + bronchoscopy (site, thickness,
horizontal and vertical extent)
A: +/- X-ray (Sail sign = Persistent tissue between VCs +
subglottis, R/O SGS)
A: +/- FISH for anterior glottic web (22q11.2 deletion)
223. Cohen’s classification of congenital glottic webs?
A: Type I: It involves less than 35% of the glottis, no subglottic
extension.
A: Type II: The web involves 35-50% of the glottis and can be thin
or thick, these may be associated with some subglottic extension of
stenosis.
A: Type III: This type involves 50-75% of the glottis. The web is
usually very thick anteriorly and may thin out as it extends
posteriorly; these almost always have a subglottic component to
them. Patients have marked vocal dysfunction and have moderate-
to-severe airway symptoms.
A: Type IV: The web involves 75-90% or more of the glottis and is
uniformly thick both anteriorly and posteriorly. The patient is
usually aphonic. Severe airway obstruction is usually present and
almost always requires an emergency tracheotomy.
224. Q: Treatment of Glottic Webs by type
A: Observation, if possible delay surgery to >3 yo
A: Endoscopic repair – Lysis via laser/cold knife, can be staged
(one side then other), serial Dilations, +/- Stent, Suturing of free
edges, Local flaps, Mitomycin C
A: Type I – Divide with laser/cold steel at age 3-4
A: Type II – Incise along one cord then serial dilations or incise
along other cord 2 weeks later; If keel required, trach needed
A: Type III/IV – Trach, corrective procedure at age 3-4,
Laryngofissure with Stent/Keel or LTR
225. Q: Laryngeal atresia types
A: Complete absence of laryngeal lumen
A: I – Supraglottic + Infraglottic
A: II – Infraglottic
A: III – Glottic
226. Q: Cri-du-Chat syndrome
A: Deletion chromosome 5p (1/50 000 births)
A: CNS – Microcephaly, 1% profound MR, hypotonia, CVD?
A: ENT – High pitched stridor (cat cry), Hypertelorism, Broad
nasal root, Cleft lip/palate
A: Laryngoscopy – Elongated/narrowed diamond-shaped
endolarynx, interarytenoid muscle paralysis, Cleft?
227. Q: Beckwith-Wiedemann syndrome
A: Sporadic occurrence
A: “Overall growth syndrome”, Macroglossia, Omphalocele,
Visceromegaly, Cytomegaly of adrenal cortex
A: Macroglossia may cause airway obstruction or chronic alveolar
hypoventilation
228. Q: Ddx of small blue cell malignancies of childhood (5)
A: Rhabdomyosarcoma
A: Undifferentiated soft-tissue sarcomas
A: Lymphoma
A: Primitive NeuroEndocrine Tumor (PNET)/Ewing’s sarcoma
A: Neuroblastoma
A: Others: Melanoma/Merckel cell Ca/SNUC/plasmacytoma in
adults
229. Q: Ddx of aural polyp in pediatrics
A: Cholesteatoma
A: Eosinophilic granuloma (Type 1 LCH)
A: Rhabdomyosarcoma
230. Q: Most common benign neoplasm of larynx in children
A: Recurrent respiratory papillomatosis (RRP)
3: Second most common cause of hoarseness in children
231. Q: Etiology and types of RRP
A: Etiology – HPV infection (6 & 11)
A: Juvenile onset (<12 years) – More common & more aggressive,
typically diagnosed by 2-4 years, vertical transmission from
mother during parturition (risk 1:80-1:500)
A: Adult onset (>12 years) – Peaks between 20-40 years
232. Q: Triad of symptoms for RRP
A: Progressive hoarseness (most common symptom)
A: Stridor
A: Respiratory distress
233. Q: Eight Common sites of lesion for RRP
A: Limen Vestibuli (junction of the nasal vestibule and nasal cavity
proper)
A: Nasopharyngeal surface of Soft Palate
A: Midline of laryngeal surface of Epiglottis
A: Upper & lower Ventricle margins
A: Undersurface of the Vocal Cords
A: Carina
A: Bronchial Spurs
A: Tracheostomy site (iatrogenic junction)
3: Preferentially junction of squamous epithelium with respiratory
epithelium
234. Q: Treatment modalities for RRP
A: Tracheostomy – Increased risk of distal spread, avoid unless
absolutely necessary
A: Surgical debulking – Standard of care, remove as much disease
as possible, preserve normal structures
A: Adjuvant therapy – 10% of patients will require some form
235. Q: Possible surgical modalities for RRP
A: Phono-microsurgery
A: Microlaryngoscopy with Cup forceps
A: Microlaryngoscopy with Microdebrider
A: Microlaryngoscopy with Laser (KTP, Nd:YAG, CO2, flash
lamp pulsed dye)
A: Cryosurgery
236. Q: 10 adjuvant therapies for RRP
A: a-Interferon therapy (most common, exact mechanism
unknown)
A: Indole-3-carbinol diet supplementation (found in cruciferous
vegetables)
A: Retinoic acid
A: Methotrexate
A: Antivirals (Ribavirin, Acyclovir, Cidofovir)
A: HspE7 (in phase 2 trials currently) = Heat shock protein to E7
A: Photodynamic therapy
A: Mumps vaccine
237. Q: Criteria for use of adjuvant therapies for RRP
A: >4 surgeries/year
A: Rapid regrowth with airway compromise
A: Distal multisite spread of disease
238. Q: Triad of juvenile onset RRP risk factors
A: First born (75% of cases)
A: Vaginal delivery
A: Teenage mother
A: other include prolonged 2nd stage of labor
239. Q: Adult onset RRP risk factors
A: Frequent oral sex >6
A: Multiple sex partners >26
240. Q: RRP prophylactic Cesarean section recommendations
A: Not routinely recommended
A: Should be strongly considered in Young, Primiparous mothers
with recent HPV infection and genital warts
A: ~1:400 risk of vertical transmission of HPV in mother with
condyloma accuminata
241. Q: Three Risk factors for increased spread of RRP
A: Juvenile
A: Young age A: Bilateral end expiratory wheezing with rales
A: HPV type 11 A: Radiographic findings (increased pulmonary markings & fluid
overload)
242. Q: Percentage of extralaryngeal spread of adult and
juvenile onset RRP 250. Q: Management of post-obstructive pulmonary edema
A: 30% Children A: Fluid restriction
A: 15% Adult A: Diuretics
A: CPAP
243. Q: Natural history of RRP
A: Spontaneous regression 251. Q: 5 TEF types in descending order of frequency
A: Not associated with puberty (CAEBD)
A: C:Esophageal atresia (EA) & distal TEF (85%)
244. Q: Seven most common GER related laryngeal disorders
A: A: Isolated EA (10%)
in pediatrics (CHARLES)
A: E: Isolated true TEF (H-type, 4%)
A: Chronic Cough
A: B: EA with proximal TEF (0.5-1%)
A: Hoarseness
A: D: EA with double TEF (0.5)
A: Aspiration
A: Recurrent Croup
A: Laryngomalacia
A: Episodic Laryngospasm
A: Subglottic Stenosis
245. Q: Four anatomic structures are seen on Barium swallow *TEF types
causing compression
252. Q: Presentation, 4 investigations, and 2 interventions for
A: Cricopharyngeus (C6)
TEF
A: Aortic arch (T4)
A: Present with immediate feeding problems and aspiration
A: Left mainstem bronchus (T6)
A: Investigations – Inability to pass a feeding tube, X-rays (curled
A: Lower esophageal sphincter
tube in the pharynx & air bubble in the stomach), Fluoroscopy,
3: Common sites of injury in caustic ingestion
Endoscopy (mainstay)
246. Q: Seven most common congenital Vascular anomalies A: Management – Surgical correction, Dilation of strictures
that can cause Tracheomalacia?
253. Q: Describe the components of VACTERL syndrome?
A: Innominate artery compression (most common, arises more
A: V – Vertebral/Vascular anomalies
medially than normal, anterior compression)
A: A – Anal atresia
A: Double Aortic arch (most common true vascular ring):
A: C – Cardiac anomalies (PDA, valve problems)
compresses both trachea and esophagus
A: TE – Tracheoesophageal fistula
A: Right Aortic arch with left ligamentum arteriosum
A: R – Renal/Radial bone anomalies
A: Anomalous left Carotid artery
A: L – Limb anomalies (extra digits, shortened limbs)
A: Anomalous right Subclavian artery (Dysphagia lusoria: arises
from left descending aorta, passes behind E (85%) or between 254. Q: Eight tracheal causes of stridor
T&E (15%), associated with a non-recurrent right RLN) A: Tracheomalacia
A: Pulmonary artery sling (left pulmonary artery arises off the right A: Hemangioma
pulmonary artery, passes between T&E; associated with complete A: Vascular compression
tracheal rings) A: TE fistula
A: Pulmonary artery dilation A: Tracheal stenosis
3: 2 aortic, 2 pulmonary, 1 carotid, 1 innominate, and 1 subclavian A: Tracheal cyst
A: Tracheal agenesis (with a TEF)
247. Q: Innominate artery compression pattern and specific
A: Tracheal bronchus
sign to look for
A: Compresses anterior tracheal wall (R more than L) 255. Q: Nine Surgical options of Tracheal Stenosis
A: Pulsatile, should affect the R arm pulses??? A: Endoscopic (Balloon or serial dilatations, Cold knife, Laser,
Stents)
248. Q: 3 Absolute and 4 relative indications for surgical
A: Augmentative (Tracheoplasty with Rib cartilage, Pericardium)
treatment for compression due to vascular anomalies
A: Resection and Anastomosis (Wedge resection, Segmental
(AFP RESP)
resection <50% tracheal length, Slide tracheoplasty)
A: Absolute – Reflex Apnea (from vagal stimulation)
A: Cadaveric Homograft with Dumon stent
A: Absolute – Failure of medical management of Severe
respiratory distress after 48 hours 256. Q: 8 methods of reducing tension across the Tracheal
A: Absolute – Prolonged intubation Anastomosis suture line
A: Relative – Repeated URTI episodes A: Suprahyoid release
A: Relative – Exercise intolerance A: Infrahyoid release
A: Relative – Significant FTT & dysphagia A: Peritracheal mobilization
A: Relative – Coexisting Pathology (SGS, asthma, CF, or previous A: Intercartilaginous incisions
TEF repair) A: Perihilar release
A: Dissection of the pulmonary vasculature
249. Q: Clinical signs of post-obstructive pulmonary edema
A: Transplantation of the left mainstem bronchus
A: Pink frothy secretions
A: Neck flexion with chin-to-chest suturing(Grillo/Guardian stitch)
A: Hypoxemia
A: How much tension is too much? Apparently 1700 gm. How do
you measure? No clue. Any clinical significance? I don’t think so.
257. Q: Five complications of tracheal stenosis repair
A: RLN damage
A: Granulation tissue
A: Infection
A: Dehiscence
A: Fistulization
258. Q: Agents responsible for caustic ingestion and their
damage pattern
A: Corrosives/Acids – Coagulative necrosis, superficial coagulum
prevents deeper damage
A: Caustics/Bases – Liquefactive necrosis, early disintegration of
tissues with deep penetration, significant damage at pH >12
A: Bleaches – pH ~7, esophageal irritants
259. Q: Three most important questions on history about
Caustic agents
A: pH
A: Volume ingested
A: Consistency
260. Q: Staging of Esophageal Burn injury
A: Grade 1 – mucosal erythema (superficial)
A: Grade 2 – mucosal erythema with noncircumferential exudate
(transmucosal)
A: Grade 3 – circumferential exudates (transmucosal)
A: Grade 4 – circumferential exudates with perforation
(transmural)
261. Q: Three degrees of Endoscopic Appearance for
esophageal burn injury
A: First Degree – Nonulcerative esophagitis, mild erythema, edema
of mucosa
A: Second Degree – Whitish exudate, erythema, underlying
ulceration that may extend into the muscularis
A: Third Degree – Dusky or blackened transmural tissue, deep
ulcerations that may extend into periesophageal tissue, lumen may
be obliterated
262. Q: Two instances when esophagoscopy should be aborted
in esophageal burn injury
A: Cannot see the lumen
A: Severe, Third Degree burn
263. Q: Five areas of extrinsic esophageal compression likely to
be burned in Caustic ingestion
A: UES – Cricopharyngeus
A: Aortic arch
A: Left mainstem bronchus
A: LES
A: Gastroesophageal junction
264. Q: Initial management strategy for Caustic Ingestions
A: ABCs, ABG, NPO, IVF
A: Judicious dilution with water or milk up to 15 ml/kg (do not
induce vomiting or give neutralizing agents)
A: CXR, Abdominal series
A: Esophagoscopy – Timing controversial, between 24-48 hours;
insertion of NG tube at time of esophagoscopy; identifies amount
of damage, circumferential or not
A: Gastrograffin swallow – Initial evaluation if >48 hours
postingestion, to identify perforation, repeat at 6 weeks to identify
stricture formation
A: Steroids most useful for grade 2 injuries (1-2 mg/Kg/day, max
60 mg/day, for 21 days)
A: Antibiotics controversial usage empirically (14 days)
A: Antireflux medications
A: Lathyrogens – Reduce collagen cross binding, to decrease
esophageal stricture, unproven benefit (penicillamine,
ßaminoproprionitrile, acetylcysteine)
A: Sucralfate – Protects esophageal mucosa against gastric acid,
and reduces granulation
3: Presence of oral injury cannot accurately predict presence or
absence of more distal involvement
265. Q: Definitive management for grade 3/4 Caustic Injuries
A: Stenting with NGT if risk of stricture (serial esophageal
dilatation for the treatment of strictures)
A: Thoracotomy for mural exam if difficult to determine
transmural injury
A: Early esophagectomy (blunt vs. thoracotomy) with
reconstruction
A: Esophagectomy/gastrectomy with exploratory laparotomy to
remove necrotic tissue
266. Q: 10 complications of caustic ingestion
A: Stricture formation (most common)
A: Tracheoesophageal fistula
A: Pneumonia
A: Esophageal perforation
A: Mediastinitis
A: Gastric perforation
A: Peritonitis
A: Sepsis
A: Death
A: Schatzki’s ring (late)
A: Complete stenosis (late)
A: Barrett’s esophagus (late)
A: Esophageal carcinoma (late)
267. Q: Discuss Acute Laryngotracheobronchitis (Croup)
A: Definition – Most common cause of stridor in children
A: Ddx – URTI, supraglottitis, bacterial tracheitis, retropharyngeal
abscess
A: Diagnosis – Clinical and radiographic
A: Causes – Parainfluenza type 1 (most common), 2 & 3, Influenza
A, RSV, Rhinovirus, Measles, Adenovirus
A: Clinical – URI prodrome, slow onset, affects 6 months – 3
years, variable/minimal fever, hoarse with barking cough, can
develop respiratory difficulty with inspiratory stridor, better in
supine position
A: Complications – Obstruction, pulmonary edema, pneumonia,
bacterial tracheitis
A: Tests – Croup series, “steeple sign” on AP views
A: Treatment – Expectant, humidification, racemic or
levoepinephrine (0.5 ml of 2.25% solution in 3cc NS), steroids
controversial (decadron 0.6-1 mg/Kg), intubation if medical
therapy fails, use ETT 0.5 mm smaller than estimated, extubate
when air leak detected
268. Q: Discuss Bacterial tracheitis
A: Definition – Complication of laryngotracheobronchitis
A: Ddx – of URTI, croup, supraglottits, retropharyngeal abscess
A: Diagnosis – Clinical +/- bronchoscopic
A: Causes – S. aureus, also S. pyogenes, H. influenzae, M.
catarrhalis
A: Clinical – URI prodrome, rapid onset in children 6 months – 8
years; high fever, hoarseness with cough, dysphagia, toxic
symptoms, no drooling
A: Tests – CBC, ABG, croup series, irregularity of airway on CXR
A: Treatment – OR intubation, bronchoscopic suction and cultures
of airway exudates, extubation when normothermic, decreased
secretions, air leak present
269. Q: Discuss Supraglottitis (Epiglottitis)
A: Definition – Acute inflammation of the supraglottis
A: Ddx – URTI, croup, bacterial tracheitis, retropharyngeal abscess
A: Diagnosis – History, clinical presentation; radiographics only if
diagnosis is in question
A: Causes – HiB (rare now), #1 Strept pneumo, GABHS, Staph,
Klebsiella, H. parainfluenzae
A: Clinical – Mild URI prodrome, rapid onset of high fever, toxic
symptoms, drooling, dysphagia; affects children from 1 year to
adulthood, peak is 2-6 years
A: Complications – Airway obstruction, death
A: Tests – Lateral soft tissue neck x-ray, “thumbprint sign”, can
culture epiglottis once airway is secure
A: Treatment – Do not agitate child, OR intubation, rigid/flexible
bronchoscopy, IV antibiotics (ceftriaxone, cefotaxime,
ampicillin/sulbactam), extubation usually within 48 hours once
swelling down and air leak present
270. Q: Name all the important Nodes in the head and neck
A: Node of Star (Submandibular)
A: Rouvier (retropharyngeal)
A: Delphian (Pre-tracheal)
A: Virchow (Supraclav)
271. Q: Discuss Retropharyngeal abscess
A: Definition – Purulent collection originating from the necrotic
degeneration of a retropharyngeal node of Rouvier
A: Ddx – URTI, croup, bacterial tracheitis, supraglottitis,
parapharyngeal abscess, PTA
A: Diagnosis – Clinical & radiographic
A: Causes – Mixed bacteriae (Streptococci, S. aureus, H.
influenzae, Bacteroides, Peptostreptococci, Fusobacteria)
A: Clinical – URI prodrome in children usually <6 years, fever,
sore throat, progressive dysphagia, drooling
A: Complications – Airway compromise, mediastinitis, rupture &
aspiration pneumonia, sepsis, arterial rupture, arterial septic
emboli, jugular vein thrombosis, vertebral osteomyelitis, death
A: Tests – Lateral soft tissue neck x-ray = Subcutaneous gas,
widening of prevertebral tissues (>2x diameter of C2 body 90%
sensitive, or >6 mm at C2 and/or >2 cm at C6, >7 mm peds/adults
at C2, or >14 mm peds and >22 mm adults C6), CT scan
A: Treatment – Secure airway, IV antibiotics, possible OR
drainage (transoral vs. transcervical)
272. Q: Pediatric otologic development
A: Pinna – Near adult size at 4-5 years, full size by 9 years
A: Tympanic membrane – Adult sized at birth, horizontal because
of incomplete ossification of EAC, vertical position reached by 2
years
A: External auditory canal – Incomplete ossification at birth leads
to increased compliance on impedance audiometry until age?
A: Eustachian tube – 50% adult length at birth, 10 degrees, enters
nasopharynx @ hard palate level; by 5-7 years lateral portion rises,
tube lengthens & widens, 45 degrees, enters nasopharynx at
inferior turbinate level
A: Ossicles & petrous temporal bone – Adult sized at birth
A: Mastoid antrum – Present at birth, increases in size during 1st
year, pneumatization continues into childhood, fully developed
mastoid & styloid by 3 years
273. Q: Relevant eustachian tube anatomy
A: Length = ~36 mm in adults, ~18 mm long in infants
A: 1/3 bony, 2/3 cartilaginous
A: Wide at both ends, narrow in midportion at the isthmus (1 x 2
mm)
A: Lateral end 4 mm above floor of epitympanum, relatively
horizontal, meets cartilaginous portion at a 160 degree angle,
cartilaginous portion then descends at a 40-45 degree angle, 45
degrees off sagittal, into the nasopharynx
A: Torus Tubarius – Formed by soft tissue overlying the medial
cartilaginous end of the tube
A: Rosenmuller’s fossa – Nasopharyngeal mucosal fold found
posterior to torus
274. Q: Four muscles related to the eustachian tube
A: Tensor veli palatini/dilator tubae
A: Levator veli palatini
A: Salpingopharyngeus (originates off torus tubarius, interdigitates
with Palatopharyngeus)
A: Tensor tympani
275. Q: Tensor veli palatini anatomy
A: Lateral head origin from scaphoid fossa & greater sphenoid
wing, swings anterior, lateral & inferior, tendon around hamulus,
inserts onto posterior hard palate and palatine aponeurosis
276. Q: 3 functions of the Eustachian tube
A: Pressure regulation/ventilation
A: Protection from nasopharyngeal reflux
A: Drainage of middle ear secretion
277. Q: JCIH Position Statement 1994 for neonatal hearing
screening (FAT MOH BA)
A: Family history of hereditary childhood SNHL
A: Craniofacial Abnormalities and/or external ear malformations
A: Findings associated with syndrome involving HL
A: TORCHS infections
A: Bacterial Meningitis
A: Ototoxic medications
A: Hyperbilirubinemia requiring exchange transfusion
A: Birth weight under 1500g
A: APGAR 0-4 at 1 minute or 0-6 at 5 minutes (Anoxia/Admission
to ICU/Afterward…)
A: Mechanical ventilation >5 days
278. Q: JCIH Position Statement 1994 for infant (29d-2y)
hearing screening (SPIT MORE)
A: Stigmata or other findings associated with a syndrome known to
include SNHL
A: Parent/caregiver concern regarding hearing, speech, language,
or developmental delay
A: Infections associated with SNHL
A: Head Trauma with associated LOC or skull fracture
A: Bacterial Meningitis
A: Ototoxic medications
A: Recurrent or persistent OME for at least 3 months
279. Q: The new JCIH 2007 Position Statement was released in
early October 2007.18 The following is a list of some of the
significant changes from the previous statement:
A: The definition of targeted hearing loss was expanded to include
neural hearing loss (eg. auditory neuropathy/dyssynchrony).
A: Separate protocols are recommended for neonatal intensive care
units (NICUs) and well-baby nurseries. Auditory brainstem
response screenings are recommended for all NICU babies, as well
as babies admitted for greater than 5 days, so that neural hearing
loss will not be missed.
A: Referrals should be made directly to an audiologist for
comprehensive testing to include diagnostic auditory brainstem
response (ABR) for all infants who do not pass ABR screening in
the NICU.
A: Rescreening of all infants should include re-evaluation of both
ears, even if the infant only failed one ear in the initial screening.
A: Audiologists with expertise in evaluating newborns should
conduct diagnostic evaluations.
A: Children identified with hearing loss should be fit with
amplification within 1 month of diagnosis.
A: A genetics consultation should be offered to families of infants
diagnosed with hearing loss.
A: All children identified with hearing loss should undergo an
evaluation by an otolaryngologist and have at least one
examination to assess visual acuity with a pediatric
ophthalmologist.
A: All children with any degree of bilateral or unilateral hearing
loss should be considered eligible for early intervention services.
A: Families should be made aware of all communication options
and available hearing technologies (presented in an unbiased
manner).
A: Early intervention services should be provided by professionals
with expertise in hearing loss.
280. Q: OAE’s for hearing screens
A: Sensitivity ~95%, specificity ~85%
A: TEOAE used most often, DPOAE being used more
A: Estimates hearing in 1-6 kHz range, can go higher?
281. Q: Five Factors influencing success of OAE’s for hearing
screens
A: Noise level in test environment
A: Vernix/debris in the EAC
A: EAC collapse
A: Middle ear fluid/mesenchyme/dysfunction
A: Decreased responses in low birth weight infants & preemies
282. Q: Two Factors affecting impedance tympanography in
neonates
A: Incomplete ossification of the EAC causing greater compliance
A: Persistence of Middle ear fluid or Mesenchyme
283. Q: ABR for hearing screens
A: Sensitivity 98%, specificity 96%
A: Click stimuli from 30-40 dB nHL
A: Estimates hearing in 1-4 kHz range
A: Requires natural sleep or conscious sedation
A: Newborn ABR composed of waves I, III, V
A: ABR reaches adult levels by 18 months to 3 years
284. Q: 3 types of behavioral auditory testing
A: Behavioral observation audiometry (BOA)
A: Visual reinforcement audiometry (VRA)
A: Conditioned play audiometry (CPA)
3: For infants/children from 0 to 5 years in age
285. Q: Behavioral observation audiometry (BOA)
A: For infants from 0 to 6 months
A: Based on reflex responses & state changes
A: Unconditioned responses, variable & imprecise, habituate
rapidly
286. Q: Visual reinforcement audiometry (VRA)
A: For toddlers 6 months – 2 years
A: Operant conditioning – Look for auditory stimuli (lights,
motion)
287. Q: Conditioned play audiometry (CPA)
A: For children 2-5 years
A: Child performs a task in response to an auditory stimulus
A: Play activity changes to keep the child’s interest
288. Q: Indications for bone conducting hearing aids
A: Atresia/microtia
A: Abnormally small EACs
A: Chronic otorrhea
A: Single sided deafness
A: large meatoplasty/CWD mastoids that cannot fit a HA
3: For situations where air conducting hearing aids do not fulfill
the amplification needs for CHL
289. Q: Four indications for imaging in pediatric hearing loss
A: Evaluation for cochlear implantation
A: Recurrent meningitis
A: Sudden or progressive SNHL (especially with head trauma)
A: Impact on counseling (atresia?)
290. Q: Describe the classification for congenital inner ear
malformations
A: Membranous inner ear malformations (~80%)
A: Bony & Membranous malformations (~20%)
A: Labyrinthine anomalies (40% of radiologically abnormal
Cochleas will have SCC abnormality)
A: Aqueductal anomalies
A: IAC abnormalities
291. Q: Describe the pathology for Membranous inner ear
malformations (3)
A: Bing-Siebenmann – Complete membranous labyrinthine
dysplasia
A: Scheibe – Most common histopathologic aplasia, limited
membranous cochleosaccular dysplasia (pars inferioris)
A: Alexander – Partial aplasia of cochlear duct at basal turn;
patients have high frequency HL
292. Q: Describe the pathology for Bony & Membranous
malformations (3)
A: Michel aplasia – Complete labyrinthine aplasia (arrest at end of
3rd week prior to Otic Capsule formation)
A: Cochlear anomalies – Common cavity(Cock deformity) (4th
week), Cochlear Aplasia (5th week), Cochlear Hypoplasia (6th
week),
A: Mondini –Incomplete bony and membranous labyrinth, cochlea
may be present as a curved tube (7th week)
293. Q: What is Michel Aplasia
A: Complete labyrinthine aplasia (3rd week arrest), rare, no
hearing
294. Q: What is Common Cavity?
A: cochlea and vestibule are confluent without internal
architecture, 20%, 4th week or (abberant later week)
295. Q: What is Cochlear Aplasia?
A: absent cochlea, rare, zero hearing, arrest in cochlear bud
Formation
A: still possible to implant these kids into the vestibule if an
audiological response is reliably demonstrated before surgery
296. Q: Cochlear Hypoplasia?
A: 6th week arrest, cochlea has single turn or less, 15% of cochlear
deformities, variable hearing depending on membranous
involvement.
297. Q: Mondini?
A: 7th week arrest, 1.5 basal turns. Most common (50%), lack of
interscalar septum, variable hearing (normal to profound
deafness), organ of corti development and neural development is
variable.

298. Q: Order cochlear disorders, most to least common

A: Mondini (~50-55%)
A: Common Cavity (20-25%)
A: Cochlear Hypoplasia (15%)
A: Cochlear aplasia (3%)
A: Michel’s aplasia (1%)
299. Q: Describe the pathology for labyrinthine anomalies
A: 40% of radiologically abnormal (osseous) Cochleas will have
SCC abnormality  
A: SCC Dysplasia 4x as common as SCC Aplasia
A: Lateral canal affected most often = Last to develop 306. Q: Altmann’s classification of atresia (1955)

300. Q: Describe the pathology for Aqueductal anomalies

A: VA normally 0.4-1 mm in diameter when measured halfway
between common crus and external aperture
A: Enlarged VA >1.5 mm, usually bilateral, typically born with
normal to mild SNHL and ~40% eventually develop Profound
SNHL; prone to sudden SNHL with head trauma
A: Enlarged CA to be diagnosed radiographically, the intraosseous
portion coursing toward the vestibule must be enlarged beyond 1
mm; external aperture measures 3-4 mm and even beyond 6 mm
301. Q: Describe the pathology for IAC abnormalities
A: Narrow IAC <3 mm, if facial function present then CN VIII
likely Absent
A: Widened IAC >10 mm, associated with stapes Gusher 307. Q: Four criteria important in the De la Cruz classification
of congenital aural atresia
302. Q: Mechanism of hearing loss in EVAS? A: Mastoid pneumatization
A: Hydrostatic Forces are transmitted either 1) from the A: Oval window footplate
endolymphatic sac through an enlarged endolymphatic duct or 2) A: Footplate/CN VII relationship
from the subarachnoid space through cochlear modiolar defects to A: Inner ear
hair cells within the cochlea damaging them 3: Normal in Minor, abnormal in Major
A: 2nd theory is that the endolymphatic sac contains fluid with 308. Q: Describe Jahrsdoerfer’s grading system for congenital
abnormal osmolarity and that reflux of this fluid into cochlea by aural atresia
way of a patent endolymphatic duct damages the neuroepithelium A: Presence of the stapes (2 points)
303. Q: Classification of Microtia A: Oval window status
A: Class I – Mild deformity, auricle decreased in size A: Round window status
A: Class II – Curving vertical ridge of tissue, all major structures A: Facial nerve course through the middle ear
present but with absolute deficiency of tissue A: Malleus/incus complex
A: Class III – Rudimentary soft tissue structure, no recognizable A: Incus-stapes connection
auricle or canal (peanut ear) A: Middle ear space
A: Class IV - anotia A: Mastoid pneumatization
A: External ear appearance
304. Q: Classification of EAC/ME Deformity (Ombredanne) 3: 10 point system based on high resolution CT findings of the
A: Minor – EAC may be mildly stenotic, ME space & TM normal temporal bone; ≤5 = not surgical candidates, ≥8 = 80% success
or slightly small, conductive hearing loss secondary to ossicle
absence or fixation 309. Q: Two audiometric tests useful for bilateral aural atresia
A: Major – EAC & TM usually absent, decreased or absent middle A: Bone conduction ABR – Wave I generated at distal CN VIII,
ear space, ossicles rudimentary or absent, fused or fixed to atretic little crossover
plate, CN VII displaced A: ECOG – For minor atresia, with transtympanic, TM, or EAC
electrode
305. Q: Schuknecht classification of aural atresia (1989)? 310. Q: Six Syndromes associated with Microtia/Aural Atresia
A: CHARGE
A: Goldenhar’s/Hemifacial macrosomia
A: Treacher-Collins
A: Crouzon’s disease
A: Pierre-Robin sequence
A: Branchio-oto-renal
311. Q: General management approach for Aural Atresia
A: BC ABR/ECoG – For Unilateral to confirm other ear is normal, A: Race (First Nations)
and for Bilateral to evaluate cochlear function A: Genetic predisposition
A: High resolution CT of the temporal bone at age 5-6 A: Ciliary dyskinesia
A: Uniateral microtia & aural atresia – Do not reconstruct canal, as A: Adenoids (reservoir of infection & mechanical ET obstruction)
speech & learning will develop normally A: ET dysfunction (short, horizontal, compliant)
A: Bilateral aural atresia – Begin with the Better developed ear as A: Cleft palate, Craniofacial abnormality, Down’s
child approaches school age, and depending on the results, may A: Immune deficiency
proceed with second ear within next few years A: Atopy (disputed)
A: Defer surgery to 6-8 years, allows growth of contralateral ear A: +ve family Hx
(template) and rib cage (cartilage graft source)
320. Q: Ten Environmental risk factors for AOM
A: Bone conduction hearing aids for Bilateral cases
A: Daycare attendance (2.6x)
312. Q: Four criteria for surgery in Aural Atresia A: Season (Fall/Winter)
A: Presence of Cholesteatoma – Emergent indication, and reason A: URTIs
every aural atresia requires CT at some point A: Older siblings
A: Normal Sensorineural function (just CHL) A: Parental history of OM
A: Ossicular mass present A: Passive smoking
A: Middle ear space at least 50% normal size A: Low S/E status (overcrowding, poor sanitation)
A: Lack of breastfeeding
313. Q: Two absolute and 1 relative contraindications to
A: Night-time bottle (horizontal position)
surgical correction of Aural Atresia
A: Pacifier use
A: Abnormal inner ear Morphology demonstrated on CT scan
A: Abnormal Cochlear function demonstrated by audiologic 321. Q: Virology of AOM (4 – RRIP!)
testing A: RSV (#1) - paramyxovirus
A: Jahrsdoerfer score ≤5/10 (relative) A: Rhinovirus
A: Influenza
314. Q: Two approaches to Aural Atresia repair
A: Parainfluenza
A: Transmastoid approach
A: Anterior approach 322. Q: Bacteriology of AOM
A: Streptococcus pneumonia
315. Q: Four facial nerve findings in middle ear Atresia
A: Heamophilus influenza
A: Tympanic segment dehiscence
A: Moraxella catarrhalis
A: Inferior/Lateral displacement of the tympanic segment
A: Streptococcus pyogenes
A: Anterior/Lateral displacement of the mastoid segment
3: Far less common are S. Aureus and GNBs
A: Therefore, more acute angle taken at 2nd genu (60 degrees vs.
90-120 normally) 323. Q: Ten Intratemporal complications of AOM
A: Chronic suppurative OM
316. Q: Five complications in Atresia surgery
A: Adhesive otitis
A: EAC stenosis
A: Tympanic membrane perforation
A: Recurrent/persistent CHL
A: Cholesteatoma
A: SNHL
A: Tympanosclerosis
A: Facial nerve injury
A: Fixation and Discontinuity of ossicular chain
A: Chronic infection
A: Mastoiditis with or without Abscess (Postauricular, Bezold’s,
3: Risks minized by near normal CN VII course, middle ear and
Zygomatic, Parapharyngeal, Retropharyngeal)
mastoid at least 2/3 normal size
A: Petrositis
317. Q: Audiometric results of Aural atresia repair A: Labyrinthitis – Serous or Suppurative
A: HL <30 dB in 50-75% A: Facial palsy
A: HL <20 dB in 15-50% A: Labyrinthine fistula (Pasha)
A: CHL or SNHL
318. Q: Classification of Otitis
A: Acute otitis media (AOM) – 1) Acute Onset of signs & 324. Q: Bacteriology of Acute Mastoiditis not due to
symptoms, 2) Presence of MEE, 3) Signs & symptoms of middle cholesteatoma
ear Inflammation (include bulging or fullness of the tympanic A: Streptococcus pneumonia
membrane (TM), erythema of the TM, and acute perforation of the A: Heamophilus influenza
TM with otorrhea) (Symptoms include otalgia, irritability, and A: Streptococcus pyogenes
fever) A: Coag negative Staph
A: Recurrent Acute otitis media (R-AOM) – ≥3x/6 mo or ≥4x/1 yr; A: Staphylococcus aureus (#3?)
recurrences in <1 mo = Same pathogen, in >1 mo = Different strain 3: Pseudomonas #2 in Bailey (in cholesteatoma?)
A: Otitis media with effusion (OME) – MEE without signs and
325. Q: Bacteriology Chronic Otorrhea with or without
symptoms of acute inflammation as found in AOM.
cholesteatoma
A: Chronic Suppurative otitis media: CSOM is defined as chronic
A: Mixed flora including Streptococcus pneumonia and Anaerobes
otorrhea (ie, lasting >6-12 wk) through a perforated tympanic
(including Bacteroides)
membrane
A: Pseudomonas (most common aerobe)
319. Q: Ten host risk factors for AOM A: Staphylococcus aureus and epidermidis
A: Age (<2 yo, first onset <6 mo) A: Gram negatives – Klebsiella, E. coli, Proteus
A: Gender (Male)
326. Q: 6 Intracranial complications of AOM 336. Q: Five Indications for Tympanocentesis/Myringotomy
A: Meningitis (#1, HiB > Pneumococcus, Mondini) A: AOM in a seriously-ill, toxic, newborn or immune deficient
A: Epidural/subdural/cerebral abscesses patient
A: Focal encephalitis A: Severe pain
A: Lateral/sigmoid sinus thrombosis (Tobey-Ayer/Quesckenstedt’s A: Suppurative complications (facial paralysis, meningitis, etc.)
Test; Griesinger’s sign)) A: Unsatisfactory response to antibiotics
A: Otitic hydrocephalus A: Suspected unusual pathogen (newborns, immunodeficiency)
A: Blindness with optic neuropathy
337. Q: Nine complications of Tympanostomy tubes
327. Q: Bacteriology of bacterial Meningitis A: Otorrhea: ~3.5% rate of persistent drainage
A: Heamophilus influenza (non-typable) A: Granulation tissue formation
A: Streptococcus pneumonia A: Myringosclerosis
A: Nisseria meningitidis A: TM perforation
A: TM atrophy, retraction, atelectasis
328. Q: Bacteriology of intracranial Abscess
A: Cholesteatoma
A: Streptococcus sp. (pneumonia, pyogenes)
A: Loss of tube in the middle ear
A: Staphylococcus sp.
A: Early extrusion
A: Proteus sp.
A: Plugged tube
A: Anaerobes (Peptococcus, Peptostreptococcus, B. fragilis)
338. Q: Etiologies of pediatric hearing loss and percentage of
329. Q: Five early Signs of Impending Complication
cases?
A: Persistence of acute infection for 2 weeks
A: Genetic = 50% (30% part of an identifiable Syndrome, AR
A: Recurrence of symptoms within 2 weeks
~75%, AD ~20%, XL ~3-4%, Mitochondrial <1%)
A: Fetid discharge during treatment
A: Environmental = 20-25% (pre-, peri-, postnatal)
A: Acute exacerbation of chronic infection, especially if fetid
A: Unknown etiology = 25-30%
A: HiB or Anaerobes
3: “Two Week Fetid Exacerbation with Type B Anaerobes” 339. Q: Percentage of Genetic hearing loss that is Syndromic
A: ~30%
330. Q: Criteria for 72 hour observation for AOM
A: Appropriate F/U 340. Q: Discuss connexin 26 (CX26)
A: Otherwise healthy children (see notes) A: Gene GJB2 product
A: Abx started if SSx persist/worsen A: DFNB1 mutation Most common association with Genetic
3: Healthy = no immune deficiency/craniofacial anomalies, Nonsyndromic hearing loss (30% of Sporadic HL, >50% of cases
no treatment failures/relapse within 30 days, no co-existing acute with an affected Sibling)
sinusitis/streptococcal pharyngitis, no underlying chronic OME, A: Six connexins form a connexon
no complicated AOM
341. Q: Mondini malformation is associated with which 6
331. Q: Pathogen causing bilateral, dull TM, non-mobile, T > syndromes?
37°C A: Mobius syndrome
A: Heamophilus influenzae A: Branchiootorenal
A: Waardenburg
332. Q: Pathogen causing unilateral, bulging TM, T >100.6
A: Pendred
(38.1)°
A: Treacher-Collins
A: Streptococcus pneumoniae
A: Klippel Feil (Wildervanck)
333. Q: Pathogen causing bullous myringitis 3: Wild Wars Teach Brothers Patience
A: Streptococcus pneumoniae
342. Q: Scheibe malformation is associated with which 4
3: Others include Mycoplasma, H. flu, Beta-hemolytic strep, M.
syndromes?
catarrhalis, Parainfluenza & influenza virus???
A: Jervell-Lange Nielsen
334. Q: Pathogen causing spontaneous perforation of TM A: Refsum (RP,
 ataxia,
 SNHL)
A: Streptococcus pyogenes A: Usher
335. Q: Eight Indications for PETs A: Waardenburg
A: Recurrent AOM (>3/6 mo, >4/1 yr, with failed medical 3: “JR Ushers Warden”
management) 343. Q: Name some Autosomal Dominant syndromes
A: OME (bilateral >3 mo or unilateral >6 mo; earlier when >25db associated with SNHL
CHL, speech/language delay, severe retraction pocket, A: Branchiootorenal syndrome (Melnick-Fraser)
disequilibrium/vertigo, or tinnitus present) A: Stickler syndrome
A: Eustachian tube dysfunction with autophony, disequilibrium or A: Waardenburg syndrome
vertigo, tinnitus, or Atelectatic TM/severe retraction pocket, A: Osteogenesis imperfecta (van der Hoeve syndrome)
unrelieved by medical management A: Otosclerosis
A: Patulous eustachian tube A: Neurofibromatosis
A: Barotitis media/Hyperbaric oxygen therapy A: Treacher-Collins syndrome
A: Suspected unusual pathogen 3: WB TOONS Dominate
A: Suppurative complication, present or suspected
A: Unsatisfactory response to antibiotics 344. Q: Describe Waardenburg’s syndrome and the 4 types
A: Definition – Autosomal dominant except type IV = AR
A: Clinical – Pigment abnormalities (white forelock, premature deafness
graying, vitiligo, heterochromia iridis), craniofacial abnormalities A: Diagnosis – Eye changes detected on Electroretinography even
(dystopia canthorum, broad nasal root, synophrys), unilateral or before funduscopic changes
bilateral SNHL, +/- vestibular Sxs A: Clinical – Retinitis pigmentosa (progressive visual loss),
A: Type I – Presence of Dystopia Canthorum, SNHL occurs in atrophy of organ of Corti/Scheibe aplasia (congenital deafness),
20%, PAX3 gene on chromosome 2 Ataxia and vestibular dysfunction common
Type II – Absence of dystopia canthorum, SNHL occurs in 50%, A: Complications – Deafness, blindness
MITF (microphthalmia transcription factor) on chromosome 3 A: Type I – Profound congenital deafness, RP onset by age 10, no
Type III – Klein-Waardenburg, features of WS1 plus Blue eyes, vestibular response, 90% of cases
Hearing impairment, upper limb Skeletal dysplasias, muscular Type II – Moderate/severe congenital deafness, onset of RP in
hypotonia, unilateral ptosis? (Pasha) teens/twenties, normal or decreased vestibular response, 10% of
Type IV – Waardenburg-Shah, AR, phenotype similar to WS2 plus cases
Hirschprung megacolon Type III – Progressive HL, RP begins in puberty, <1% of cases
Type IV – X-linked inheritance, similar to type II
345. Q: Describe Neurofibromatosis Type I (von
A: Tests – Electroretinography (measures potentials of retinas from
Recklinghausen’s disease)
light & visual stimuli), Opthalmology consult essential
A: Mutation of nerve growth factor Neurofibromin on
A: Treatment – Amplification
chromosome 17
A: CNS involvement may lead to MR, blindness, SNHL, acoustic 352. Q: Describe Jervell Lange-Nielsen disease
neuromas present in only 5% A: Definition – Autosomal recessive
A: Causes – Linked to a Potassium channel gene (KVLQT 1) on
346. Q: Diagnosis of NF Type I by 2 of the following 7 criteria
11p15.5
A: Neurofibromas (2 or more cutaneous, or 1 plexiform)
A: Clinical – Profound bilateral congenital SNHL (high
A: Café au lait spots (6 or more, >15 mm in adults, >5-10 mm in
frequencies worse), Heart disease (prolonged QT, large T waves)
children)
A: Complications – Stokes-Adams attacks, recurrent syncope,
A: Axillary or inguinal freckling
usually terminates fatally with sudden death
A: Lisch nodules (2 or more iris hamartomas)
A: Tests – ECG, Audiometry
A: Optic glioma
A: Treatment – ß-blockade, Amplification
A: A distinctive osseous lesion– pseudoarthrosis, scoliosis, or
thinning of the tibia; sphenoid wing dysplasia 353. Q: Describe Pendred syndrome
A: Family history or 1st degree relative with confirmed NF1 A: Definition – Autosomal recessive
A: Causes – Tyrosine Iodination defect leading to Euthyroid goiter
347. Q: Describe Neufibromatosis type II
A: Clinical – Goiter, Mondini or Enlarged VA’s
A: Mutation of tumor suppressor gene Merlin on chromosome 22q
A: Tests – Perchlorate discharge test
A: Autosomal dominant, but 50% of cases due to spontaneous
A: Treatment – Exogenous T4
mutations
A: 95% incidence of Bilateral Acoustic Neuromas often before age
21
A: Central Meningiomas, Gliomas, Schwannomas, early lens
opacifications (cataracts)
A: Fewer café au lait spots & cutaneous nodules than NF I
348. Q: Neufibromatosis type II subtypes (2)
A: Wishart – Early onset, rapid growth, other tumors than acoustic
neuromas
A: Gardner – Slower rate of growth & onset, usually only bilateral
acoustic neuromas
349. Q: Diagnosis of NF Type II by 1 of the following 3 criteria
A: Bilateral CN VIII masses on MRI (seen with & without Gado,
on Axial and Coronal cuts)
A: Family history or 1st degree relative with confirmed NF2, and 1
CN VIII mass (as described above)
A: Family history or 1st degree relative with confirmed NF2, and
at least 2 of 5: Neurofibroma, Meningioma, Schwannoma, Glioma, 354. Q: Name 4 X-linked Recessive syndromes associated with
Juvenile Cataracts (Posterior Sub-Capsular) SNHL
350. Q: Name 3 autosomal recessive syndromes associated with A: Wildervaank syndrome
SNHL (PUJ) A: Otopalatodigital syndrome
A: Pendred’s syndrome A: Alport syndrome
A: Usher syndrome A: Norrie syndrome
A: Jervell-Lange Nielsen syndrome A: Deafness Dystonic
3: PUJ goes to recess 3: WANDO loves SEX
351. Q: Describe Usher syndrome and the 4 types 355. Q: Describe Wildervank syndrome
A: Definition – Most common cause of congenital visual deafness; A: Definition – XR, Klippel-Feil malformation, Brevicollis, mode
Autosomal recessive except type IV = XR, 10% of hereditary of inheritance unclear
A: Clinical – SNHL or mixed loss, due to bony inner ear
(Mondini) and middle ear malformation, CN VI paralysis with eye
retraction on lateral gaze (Duane retraction syndrome), short
neck/fused cervical vertebrae, Assimilation of the Atlas (Basilar
impression), Spina bifida
356. Q: Discuss Otopalatodigital syndrome
A: Definition – XR
A: Causes – Mutation localized to Xq28
A: Clinical – CHL due to ossicular malformation, Cleft palate,
Stubby/clubbed digits, Craniofacial deformities (hypertelorism,
supraorbital deformity, flat midface, small nose), short stature,
wide space between 1st & 2nd toe
357. Q: Discuss Alport syndrome
A: Definition – AR or XR, abnormal Type IV collagen in the
glomerular basement membrane
A: Diagnosis – Renal failure and SNHL
A: Causes – Degeneration of organ of Corti and Stria vascularis
A: Clinical – Progressive SNHL (usually 2nd decade), varying
degrees of Renal disease (mild renal Dysplasia to renal Agenesis
identifiable on ultrasound), gross Hematuria associated with UTI
A: Complications – Renal failure, Death in males by age 30
A: Types – 6 subtypes, 3 AR, 3 X-linked
A: Tests – BUN, Creatinine, Urinalysis
A: Treatment – Dialysis/renal transplant important therapies (more
so in males)
358. Q: Discuss Norrie syndrome
A: Definition – XR
A: Causes – Mutation of NDP gene on Xp11.4, produces norrin
protein, structurally similar to TGF-ß
A: Clinical – Progressive SNHL (2nd/3rd decade), occasional
progressive mental deterioration, congenital/rapidly progressive
Blindness due to pseudoglioma, exudative vitreoretinopathy,
opacification, ocular degeneration
359. Q: 11 lab tests and there association with childhood SNHL
A: CBC + Diff – Leukemia/lymphoma; Platelets – Fechner
syndrome (Very rare, HF SNHL, Ocular disease, Proteinuria,
Macrothrombocytopenia)
A: BUN, CR, Urinanalysis – Alports syndrome (persistent
microscopic hematuria)
A: Glucose – Alstron syndrome (Obesity, Impaired glucose
tolerance with insulin resistance, Retinal degeneration,
Neurosensory deafness, Acanthosis nigricans, Hepatic dysfunction,
and other Endocrine abnormalities)
A: RPR, VDRL,FTA-ABS – Syphilis
A: EKG – Jervell and Lange-Nielson syndrome
A: GJB2 gene – Responsible for 50% of autosomal recessive
nonsyndromic hearing loss, found to be positive in 35% of
moderate to profound SNHL.
A: CT scan – Michel aplasia, Mondini malformation, Enlarged VA
A: MRI – Child with NF II, useful if progressive hearing loss, or
vestibular symptoms, focal neurological symptoms
A: ANA, ESR, RF – SLE, RA
A: Thryoid function tests – Cretinism and Pendred syndrome
(Pendred may have normal thyroid function tests)
360. Q: Which 4 tests should be routine when no other etiology
of childhood SNHL is found?
A: EKG
A: RPR
A: CT
A: GJB2
3: In addition to CBC, SMA7 (may also detect leukemia, Fechner,
Alstron, and Alport)
361. Q: Most common cause of chronic benign pediatric
lymphadenopathy, diagnosis, and treatment
A: Cat Scratch Disease (Bartonella henselae)
A: Serologic testing
A: Intracellular gram-negative bacillus on Warthin-Starry stain
A: Azithromycin (or self limiting)
3: Avoid I+D (draining tract)
362. Q: Condition in immunocompromised patients with B.
henselae infection
A: Bacillary angiomatosis
363. Q: Typical presentation of atypical mycobacterium
lymphadenitis, most common organism, and treatment
A: Submandibular lymphadenopathy, most common in under 12,
draining sinus if untreated, later calcifies
A: M. avium intracellulare
A: Surgical excision, can try Antibiotic triple therapy
(Clarithromycin, Rifampin, & Ethambutol) if disseminated disease
364. Q: Electron microscopy finding of Histiocytosis X (LCH)
A: Bierbeck granules
3: “Tennis-racket” or rod shaped cytoplasmic organelles with a
central linear density and a striated appearance
365. Q: Define Epipericardial ridge, 4 muscles and 2 nerves
which are derived from it
A: An elevation of mesoderm separating the developing
pharyngeal region from the embryonic pericardium
A: Forms SCM, Trapezius, Tongue and Infrahyoid musculature
A: Nerves present include XI and XII
366. Q: Proper surgical management of Type II First Branchial
arch abnormality
A: Superficial parotidectomy
A: CN VII identification
A: Trace track back to origin (typically the bony/cartilaginous
junction of EAC)
A: Cartilage resection
3: Relation to CN VII variable
367. Q: 3 and 4th Branchial abnormalities can manifest as
what condition in children
A: Acute suppurative thyroiditis
368. Q: What is CHAOS and two example of lesions that can
cause conditions
A: Congenital High Airway Obstruction Syndrome
A: Lymphangiomas and Teratomas located in the anterior
compartment
369. Q: CHAOS – how diagnosed and findings
A: Ultrasound findings in utero for diagnosis
A: Dilated airways below the level of the upper airway obstruction,
large echogenic lungs, flattened diaphragms, fetal hydrops, dilated
tracheobronchial tree
370. Q: Define EXIT procedure
A: Ex utero intrapartum treatment procedure for babies with
airway compression, partially delivered through a C-section but
remain attached by their umbilical cord to the placenta, while a
pediatric or neonatal general surgeon establishes an airway so the
fetus can breathe, the umbilical cord is cut and clamped, and the
infant is fully delivered. Then the remainder of the C-section
proceeds
371. Q: Most common infectious organism in pediatric 383. Q: Ddx of chronic drooling
bacterial cervical lymphadenitis A: Medications
A: Staphylococcus aureus A: Neurologic
A: Streptococcus pyogenes A: Indirect causes – Nasal obstruction, head position,
malocclusion, tongue size, sitting position, and emotional state
372. Q: Bacterial cause of Lemierre’s Syndrome
A: Fusobacterium necroforum 384. Q: Treatment options for Drooling
A: Speech therapy
373. Q: Non-tuberculous vs tuberculous lymphadenopathy in
A: Behavior therapy
Pediatrics
A: Dental appliance
A: Systemic symptoms – Single large node with fevers and malaise
A: Drugs (glycopyorolate, trihexyphenidyl, scopolamine, Botox)
in TB; Rare in NTB
A: Radiation
A: Lung disease – Often in TB; Rare in NTB
A: Surgery – SMG excision or Duct re-routing
A: Violaceous skin change in NTB
Parotid duct ligation,
A: PPD – Strongly positive in TB; weak or negative in NTB
Tympanic neurectomy,
A: Treatment – Medical in TB; NTB often resistant to medical,
Intraductal laser photocoagulation
therefore often require Surgical excision
385. Q: AAO-HNS indications for coagulation studies
374. Q: Most Sensitive tests for Mononucleosis
A: Only if indicated by history or if genetic information is
A: IgM(current and recent disease) and IgG(current and past
unavailable
disease) for EBV (VCA and EBNA)
3: Monospot/Heterophile Ab test is negative in early disease (for 2- 386. Q: Location and type of mutation associated with VCF
3 weeks) A: Chromosome 22q11.2
A: Hemizygous Microdeletion
375. Q: Percentage of pediatric NHL that are high grade
A: 90% 387. Q: Normal parents, with child with SNHL, chance of
further children with SNHL (all comers)
376. Q: Bifid epiglottis – what other test required
A: 14%
A: Thyroid function (dysgenesis in Bamforth syndrome)
3: Also associated with Pallister-Hall syndrome 388. Q: Black child for T and A – what need to consider first,
simple test and preoperative management
377. Q: Classification of laryngeal (saccular) cysts
A: Consider Sickle Cell anemia; 3 forms vaso-occlusive (painful)
A: Superior (extending into ventricle)
crises, aplastic crises, and splenic sequestration crises
A: Posterior (extending into the AE folds and false cord)
A: Simple test – CBC (anemia), Sickledex solubility test, then Hb
378. Q: Ddx of congenital supraglottic abnormalities (8) electrophoresis
A: Laryngomalacia A: Preop – Consult hematology, Evidence of renal, pulmonary or
A: Hemangioma cerebrovascular complications should be assessed pre-operatively.
A: Lymphatic malformation Check for signs of vaso-occlusion, fever, infection and
A: Laryngocele dehydration. transfusion to achieve a ratio of 70:30 for normal
A: Saccular cyst blood to HbS to help prevent Acute chest syndrome post op and
A: Anomalous cuneiform cartilage avoid hypoxia and precipitate a sickle cell crisis
A: Bifid epiglottis
389. Q: Diagnosis of Macroglossia – any of 3 following criteria
A: Supraglottic web
A: Extravasation of lingual apex or lingual border onto or outside
379. Q: Six techniques for improving airway in bilateral vocal the dentition
cord paralysis A: Impression of ≥ 1 teeth on lingual border visualized when
A: CO2 laser cordotomy mouth is open
A: Laser arytenoidectomy and cordectomy A: Following Sx for correction, a relapse of increased interdental
A: Open arytenoidectomy space, open bite deformity, and/or jaw deformation with
A: Arytenoidopexy malocclusion occurs
A: Arytenoid separation with cartilage grafting
390. Q: Macroglossia triad
A: Laryngeal reanimation techniques (phrenic to RLN, phrenic to
A: Open bite deformity
PCA, or omohyoid muscle pedicle)
A: Mandibular prognathism
380. Q: Define Pallister-Hall syndrome A: Malalignment
A: Laryngeal (clefts or bifid epiglottis)
391. Q: Classification of Local causes of Macroglossia (CITeN)
A: Hypothalamus abnormalities (hypothalamic hamartoblastoma,
A: Congenital – Hemangioma, Lymphangioma, Lingual thyroid
hypopituitarism)
A: Inflammatory/Infectious – Angioedema, TB, Actinomycosis,
A: Postaxial polydactyly
Dental infection, Syphilitic gumma, Riga disease, Ranula
A: Imperforate anus
A: Traumatic – Dental irritation, Hematoma, Postoperative edema,
381. Q: Age 1 and recurrent croup – what investigation Sublingual calculus
A: Endoscopy – R/O subglottic stenosis A: Neoplastic, Benign – Granular cell tumour, Neurofibroma,
Leiomyoma, Lipoma; Malignant – Carcinoma, Sarcoma
382. Q: Two conditions with congenitally shallow orbits
A: Crouzons 392. Q: Classification of Generalized causes of Macroglossia
A: Aperts (CITEn)
A: Pfeiffer A: Congenital – Primary idiopathic macroglossia, Beckwith-
Wiedemann syndrome, Down syndrome, Trisomy 4P syndrome,
Triploid syndrome, Gangliosidosis syndrome,
Mucopolysaccharidoses, Robinow syndrome
A: Inflammatory – Chronic glossitis
A: Toxic – Amyloidosis, Lipoid proteinosis, Chronic steroid
therapy
A: Endocrine – Acromegaly, Myxedema, Cretinism
393. Q: Three causes of Pseudo-Macroglossia
A: Habitual tongue posturing
A: Retrognathia/Micrognathia
A: Hypotonia of tongue
394. Q: Four treatment options for macroglossia
A: Observation
A: Orofacial therapy (for hypotonia, or lingual mal-position)
A: Surgery
A: Submucosal minimally invasive lingual excision (SMILE)
395. Q: Five indications for Macroglossia Surgery
A: Airway obstruction – BOT macroglossia
A: Speech impediment - particularly consonants requiring tip
contact w/ alveolar ridge, palate
A: Dysphagia – failure to thrive
A: Maxillofacial abnormalities – anterior open bite, prognathism,
increased ramus-body angle
A: Cosmesis
396. Q: Surgical options for Macroglossia
A: Anterior wedge
A: Tip amputation
A: Dorsum and tip
A: Central reduction
A: Block excision central & tip or Keyhole
A: Tip preservation – Kruchinsky
A: Horizontal filleting
A: Dorsal flap
397. Q: Two indications for surgery in Ankyloglossia
A: Poor feeding/sucking – First few months
A: Impaired speech – 2-3 years