Ch10. Peds Oto Flashcards
1
Q
Waldeyer’s Ring
A
Circle of lymphoid tissue including palatine/faucial tonsils, pharyngeal tonsils/adenoids, lingual tonsils, tubal tonsils of Gerlach (near fossa of Rosenmuller); lateral bands and posterior pharynegal wall complete the ring
2
Q
Arterial supply to palatine tonsils
A
- Lingual artery (dorsal lingual br)
- Facial artery (ascending palatine and tonsillar arteries)
- Ascending pharyngeal artery
- Maxillary artery (greater palatine and descending palatine arteries
3
Q
Venous drainage of tonsils
A
Lingual and pharyngeal veins (internal jugular vein)
4
Q
Lymphatic drainage from tonsils
A
No afferent lymphatics, drainage into superior deep cervical and jugular digastric lymph nodes
5
Q
Innervation to the tonsils
A
- Anterior pillar (CN X; palatoglossus)
- Posterior pillar (CNX; palatopharyngeus)
- Tonsillar fossa (CN IX, X; superior constrictors)
6
Q
Histology/tonsillar zones (1st)
A
- Reticular epithelium: foreign material presented to lymphatic cells via 10-30 cryps/tonsil (blind tubules of squamous epithelium) -> antigen presenting cells (M-cells) -> lymphoid germinal center -> interdigitating dendritic cells and macrophages -> helper T cells -> memory B-cells (nasopharynegal or systemic migratino) and plasma cells (crypts)
7
Q
Histology/tonsillar zones (2nd)
A
- Extrafollicular area: contains T-cells
8
Q
Histology/tonsillar zones (3rd)
A
- Lymphoid follicle: composed of the mantzle zone (mature B-cells) and the germinal center (active B-cells)
9
Q
What encapsulates the tonsils?
A
Tonsils encapsulated by special portions of pharyngobasilar fascia
10
Q
Arterial supply to the adenoids
A
Ascending pharyngeal artery from external carotid, minor branches from maxillary artery (ascending pharyngeal branch), facial artery (ascending palatine artery), thyrocervical trunk (ascending cervical), artery of the pterygoid canal
11
Q
Venous drainage from the adenoids
A
Pharyngeal veins -> facial and IJ
12
Q
Innervation to the adenoids
A
CN IX, X
13
Q
Histology of the adenoids
A
Ciliated pseudostratified columnar, stratified squamous, and transitional layers
14
Q
Organisms of acute tonsillitis
A
Most commonly Group A beta-hemolytic streptococci, Moraxella, and H. influenzae; less common organisms include Bacteroides, staphylococci, E. coli, diphtheria, syphilis, Neisseria, and viruses (EBV, adenovirus, influenza A and B)
15
Q
Phases of tonsillitis
A
Tonsillar erythema -> exudative tonsillitis -> follicular tonsillitis (yellow spots corresponding to lymphatic follicles) -> cryptic tonsillitis (chronic infection)
16
Q
Chronic adenoiditis pathophyz and SSx
A
Typically a polymicrobial infection; may be related to reflux, especially in children (difficult to distinguish from sinusitis)
SSx: persistent nasal discharge, malodorous breath, nasal obstruction (snoring), association with recurrent otitis media and sinusitis
17
Q
How to determine hyponasal speech (i.e. adenoid hyperplasia)?
A
Pinch nose does not change speech, “M” words
18
Q
What is adenoid facies?
A
Open mouthed, dark circles under eyes, flattened midface, high arched palate
19
Q
What to suspect if unilateral tonsillar hyperplasia?
A
Consider neoplasm (lymphoma, leukemia, SCC) or unusual infections (M. tuberculosis, atypical mycobacteria, actinomycosis, fungal)
20
Q
What to do in acute respiratory distress from obstructive tonsillar and adenoid hypertrophy?
A
Use nasal trumpet (rarely requires intubation) with a short course of corticosteroids, prolonged course of Abx (3-6 weeks) or nasal corticosteroid sprays for adenoid hyperplasia; tonsillectomy and adenoidectomy for definitive therapy
21
Q
PTA pathophyz( two)
A
- Spread of infection outside tonsillar capsule into the peritonsillar space
- Infection in a peritonsillar minor salivary gland (Weber gland), controversial
Typically begins at superior pole
22
Q
Uvular deviation in PTA
A
To the contralateral side
23
Q
Infections mononucleosis pathophyz
A
EBV (Ebstein-Barr virus) selectively infects B-lymphocytes (90%); CMV and other viruses less commonly involved (10%)
24
Q
Dx test for Mono
A
Heterophile antibodies in serum (Monospot test, Paul Bunnell test; rapid kits 85% sensitive, ~100% specific) will be 40% negative in first 2-3 weeks
Presence of 80-90% mononuclear and 10% atypical lymphocyets on smear
IgM firs tmonth only; IgG appears at 1 week, present for life
25
Abx in Mono
Antimicrobials for secondary infections (amp/amox may cause a severe rash from hypersensitivity)
26
Sleep physiology of newborn
<2 months
Preterm: sleep staged differently; quiet, active, and intermediate sleep; quiet is similar to NREM, active is similar to REM (~50%), intermediate is mix
Term: sleep/wake differentiation occurs by 37 weeks GA
27
Sleep physiology of infant
2mo-1yr
| Percentage of REM sleep declines
28
Sleep physiology of toddler
1-3 yrs
1 yr: 30% REM, may nap 1-2 hours/day, normal sleep time 13-15 hours
2 yr: 25% REM similar to adults, separation anxiety and night awakenings
29
Sleep physiology of school age/prepubescent
5 yr: 20% REM, naps not required, adult-like sleep cycles (90-110 minutes)
6-12 yrs: slow wave sleep is maximal, then abruptly drops off in puberty and declines throughout adulthood; circadian phase preferences develop (morning versus evening); parasomnias may develop
30
Sleep physiology of adolescent
should get 9 hrs of sleep
| at risk for behavior-induced insufficient sleep syndrome
31
Peds sleep-disordered breathing
Defined as: abnormal respiratory pattern during sleep that includes snoring, mouth breathing, and pauses in breathing
32
Spectrum of SDB
Snoring (10%)
Upper airway resistance syndrome
Obstructive hypoventilation
Obstructive sleep apnea (OSA: 1-3%)
33
Pathophyz of SDB
Upper airway osbruction from adenotonsillar hyperplasia MC
Obesity (25-40% have SDB
Carniofacial anomalities/syndromes
Prematurity
Daycare exposure and freq URI, smoking exposure
Ethnicity (Af-am MC)
Neuromuscular disorders
34
Indications for PSG in SDB (AAO-HNS CPG 2011)
1. Prior to tonsillectomy in presence of obesity, Down syndrome, craniofacial anomalies, neuromuscular disorders, sickle cell disease, mucopolysacchridoses
2. Discordance between tonsillar size and SDB symptoms
35
Peds OSA complications
DD, FTT (increased energy expenditure, feeding impairment, reflux from high negative intrathoracic pressures), cardiorespiratory complications (cor pulmonale, pectus excavatum), behavioral disorders
36
Peds PSG sleep scoring: apneic events
Drop in airflor >=90% (oronasal thermal sensor) that lasts the duration of 2 baseline breaths (does NOT req 10 seconds)
37
Peds PSG sleep scoring: hypopneic events
Nasal air pressure decreases by >= 30% lasting the duration of 2 breaths and must be associated with a >=3% oxygen desaturation or an arousal/awakening
38
Peds PSG sleep scoring: respiratory effort related arousals/RERAs
nasal air pressure flattens and decreases in amplitude but NOT >= 30% and is associated with snoring, noisy breathing, incerased work of breathing or elevation of end-tidal or transcutaneous PCO2
39
Peds PSG sleep scoring: AHI
Apnea-hypopnea index, number of obstructive apneas and hypopneas per hour of sleep
40
OSA scoring scale (AAO-HNS CPG 2012)
1. Normal AHI <= 1, O2 nadir >92%
2. Mild OSA: AHI >1, <= 5
3. Mod OSA: AHI >5, <= 10
4. Severe OSA: AHI >10, O2 nadir <80%
41
Hypoventilation syndrome
if arterial (or end-tidal) CO2 >50 mm Hg for >25% of total sleep time
42
Central sleep apnea
similar to adults but event must last >= 20 seconds or 2 missed breaths with an arousal/awakening or with a >=3% desaturation
Consider medical causes, Arnold Chiari malformation, metabolic disorders, cardiac disease, medications (opioids), high altitude
43
Primary sleep apnea of infancy
Central apnea of >=20 seconds associated with immaturity of brainstem respiratory control centers
Lead to bradycardia and hypoxemia, may require resuscitation or stimulation
More common in preemies
44
Periodic breathing
series of 3 episodes of central apneas lasting >3 seconds each occuring within 20 seconds
Frequent in Arnold-Chiari patients
45
Congenital central hypoventilation syndrome (Ondine's Curse)
PHOX2b gene mutation causing a failure of autonomic control of breathing at night
Rx: tracheotomy and long-term ventilation during sleeps/naps
46
Sudden infant death syndrome
Abrupt, unanticipated death of unknown etiology
RFs: male, preemie, prone position sleep, multiple births, maternal young age (teen), family hx
Prevent by supine position sleep ("back to sleep"), firm mattress, no bed sharing, removal of surrounding soft objects
47
Behavior insomnia of childhood: sleep-onset type
caused by reliance on an inappropriate sleep association and inability fo "self soothe" (e.g., rocking, bottle, parents' bed)
Rx: CBT, extinction ("crying it out"), gradually reduced parent intervention, daytime naps, discontinued feeding after 6 months, consider preemtive scheduled awakenings (!5 minutes before anticipated awakening)
48
Behavior insomnia of childhood: limit-setting type
characterized by parents' inability to establish appropriate sleep behaviors, "child stalling" or refusals
Rx: CBT, consistent bedtime, parent education, may provide a transitional object (e.g., stuffed animal, blanket)
49
Patient care recommendations from AAO-HNS CPG 2011
1. Preop: avoid anxiolytics or sedatives; if nec, then monitor for hypoventilation and hypoxemia
2. Intraop: intraop Decadron reduces postop nausea/vomiting; SDB children are at increased risk of airway collapse and delayed emergency; avoid or reduce intraop opioids for SDB children; local anesthesia injection should NOT be used
3. Postop: SDB children require more intensive nursing care and monitoring; strong recc against periop Abx (not shown to reduce postop hemorrhage, pain, or return to normal function significantly)
50
Pain control after tonsillectomy
Tylenol and ibuprofen
Codeine not recc; postop opiates must be used with caution (abnormal metabolism related to CYP2D6, causing overproduction of metabolites)
Ketorolac contraindicated due to increased bleeding risk
51
Monitored overnight stay after tonsillectomy
Considered in <3 years old, syndromic, comorbidities, obesity (>99th percentile BMI), live >1 hour from hospital, unreliable parents, bleeding disorder, or severe OSA (AHI >=10, O2 nadir <80%)
REM rebound may occur in severe OSA patients after 18hrs, which may cause hypoventilation and hypoxemia
52
ICU admission after tonsillectomy
Severe OSA, comorbidities that cannot be managed on a ward, significant airway obstruction and O2 desat in recovery room unresponsive to repositioning and O2
53
Tonsillectomy indications
1. Tonsillar hyperplasia resulting in SDB or OSA associated with cor pulmonale
2. Suspected malignancy
3. Tonsillitis resulting in febrile convulsions (may require Quinsy tonsillectomy)
4. Persistent or recurrent tonsillar hemorrhage
5. FTT (not attiributable to other causes)
54
What is Paradise criteria?
```
Relative indication for tonsillectomy
7 infections in 1 year
5 infections/yr x2
3 infections/yr x3
(infx defined as sore throat with 1+ more of fever >38.3, cervical adenopathy, tonsillar exudate, or pos strep test)
```
55
Most common complications after T&A?
Most common serious complication is bleeding (0.5-5%)
56
Types of bleeding T&A complications
Three.
1. Intraoperative
2. Immediate post-op
3. Delayed post-op
57
Describe intraoperative T&A bleeding complications.
Arterial injury such as aberrant carotid artery, retrained tonsillar tissue, tears in posterior pharyngeal wall
58
Describe immediate postoperative bleeding after T&A
Bleeding <24 hrs post-op. 0.2-2.2%.
| Similar to intraoperative, may be due to inadequate hemostasis during surgery typically from vessel spasm
59
Describe delayed post-op bleeding after T&A
>24hrs post-op. 0.1-3%.
| ~5-10 days post-op, due to eschar sloughing
60
Describe post-op edema after T&A
Due to sudden relief of airway obstruction from long-standing adenotonsillar hyperplasia resulting in a sudden drop in intrathoracic pressure, increased pulmonary blood volume, and increased hydrostatic pressure
May occur immediately or after a few hours
Rx: PEEP, mild diuresis
61
Describe VPI after T&A
Results from an incompetent velopharyngeal inlet; increased risk with submucosal cleft palate, history of nasal regurgitation, or preoperative hypernasality
Rx: speech therapy (typically resolves); if persistent pharyngeal flap or palatal lengthening and consider testing for 22q11
62
Describe Grisel syndrome
Atlantoaxial (C1-C2) subluxation resulting in spread of inflammation from the OP to the cervical ligaments resulting in laxity and spinal cord compression; rare, increased risk with Down syndrome
Rx: orthopedic consult, may need Abx, cervical collar
63
Ideal age for septoplasty?
After puberty (~15-16 years old)
64
Describe mandibular distraction osteogenesis
Progressive elongation of native mandible and soft tissue envelope by performing bilateral sagittal osteotomies and placement of internal and external distraction devices
65
indications for MDO
Micrognathia/retrognathia, airway obstruction, feeding difficulties, tracheostomy decannulation, severe obstructive sleep apnea, hemifacial microsomia
Commonly performed for Pierre-Robin Sequence and Treacher Collins, Nager, velocardiaofacial and Pfeiffer syndromes
66
Phases of MDO?
Four.
1. Lacenty
2. Active distraction
3. Consolidation
4. Hardware removal
67
Latency phase of MDO?
First one of four.
1-5 days; shorter in toddlers/young children, wait 5-7 days in adults; allows hematoma formation and platelet-derived growth factor production; if too short may lead to fibrous union and if too long may lead to callus
68
Active distraction phase of MDO?
1-2 mm/day until supraglottic obstruction relieved
69
Consolidation phase of MDO?
4-12 weeks
70
Complication of MDO
Infection, scarring, nerve injury (marg, inferior alveolar, facial nerve), dental injury (tooth bud injury with tooth loss, dentigerous cysts), malocclusion (anterior open bite), poor healing (malunion, nonunion, relapse), device failure, TMJ ankylosis
71
Most common H&N neoplasm in children
Infantile hemangioma
72
Origin of infantile hemangioma
Endothelial origin
73
Presentation of infantile hemangioma
Superficial type presents at birth or within 2 weeks as a white spot (Herald Path) or a small red spot; deep type usually discovered a few weeks after birth
74
Epidemiology of infantile hemangioma
```
MC female (3:1 ratio)
10% population incidence
```
75
Pathophysiology of infantile hemangioma
Unknown, possibly from disrupted placental cells or stem cells
76
Histopathology of infantile hemangioma
IHC markers GLUT1 and LeY (Lewis Y antigen)
| Biological markers: beta-fibroblast growth factor, urokinase
77
Stages of infantile hemangioma
1. Proliferative <= 12 months old, endothelial cell hyperplasia, elevated mast cells, multilaminar basement membrane
2. Involuting 50% regress by 5 years old, 70% by 7 years; fibrosis, decreased cellularity
3. Involuted: soft mass of excess skin and fibrofatty tissue, scarring, telangiectasias, atrophy
78
Types of infantile hemangioma
1. Superficial/cutaneous (strawberry or capillary hemangioma): bright strawberry-red color progresses to wind color then grey
2. Deep (cavernous hemangioma): covered by skin, appears blue
3. Compound: mixed
79
Define PHACES Syndrome
Posterior cranial fossa anomalies (req MRI)
Hemangioma (facial segmental)
Arterial/carotid anomalies
Cardiac anomaly/Coarctation of the aorta (req cardiac echo)
Eye anomaly
Sternal pit
MC females (90%); consider if large segmental hemangioma
80
Maffucci Syndrome
Multiple deep hemangiomas +/- visceral vascular lesions
Dyschondroplasia
Chondrosarcoma in 25%
81
Complications of infantile hemangioma
In 20%
Cosmetic deformity, amblyopia (eye involvement), ulceration (10%), infection, bleeding (rare, reassure that it will not "burst"), airway compromise (laryngeal), thrombocytopenia, and high output cardiac failure (rare)
82
Dx for Subglottic Hemangioma
Endoscopy (avoid biopy), CT and MRI may confirm diagnosis
| PA and lateral neck x-rays may show unilateral or posterior subglottic lesion
83
Describe cutaneous infantile hemangioma
MAy present anywhere in H&N (concerning in eyelid and orbit)
Common in parotid and oral cavity (lip), 1% of children with a cutaneous lesion will have a subglottic hemangioma
84
Describe laryngeal infantile hemangioma
Sessile lesions, present with inspiratory or biphasic stridor within the first few months of life that is worse with crying (engorgement), dysphonia, dysphagia (rare)
50% of children with a subglottic hemangioma will have a cutaneous lesion
85
Describe nasal infantile hemangioma
MC in Little's area (Kisselbach's plexus) or inferior turbinate
86
Describe ear/periauricular infantile hemangioma
May deform the ear or ear canal (CHL)
87
Describe parotid infantile hemangioma
50% of parotid hemangiomas associated with cutaneous hemangiomas
88
When should one order ultrasound in infantile hemangioma?
If >3 hemangiomas, consider abdominal ultrasound for occlult lesions (eg liver)
89
Define complications from infantile hemangioma
```
VASCO
impaired Vision or hearing
imparied Swallowing
important Cosmetic effect or with ulceration
high Output cardiac failure
```
90
Rx infantile hemangiomas without complications
Observation, propranolo, oral steroids, surgical excision (cold or pulsed dye laser)
91
Rx infantile hemangiomas with complications
Propranolol is standard of care for hemangiomas in the proliferative stage, consider trial of oral steroids if >12 months old, consider vincristine for severe cases
92
Propranolol considerations in infantile hemangiomas
Baseline vital signs, recent normal cardiovascular and pulmonary exams (ECG or glucose screening no longer req w normal exam)
Usually responds within 2-12 weeks; continue until 1 year old then taper
Contraindications include cardiogenic shock, sinus bradycardia, hypotension, greater than first-degree heart block, heart failure, bronchial asthma, and drug hypersensitivity (higher stroke risk in PHACES)
93
Two types of congenital hemangioma
Congenital hemangioma is present at birth
1. RICH: rapidly involuting, involutes by 6-14 months; typically GLUT-1 neg
2. NICH: non-involuting, does not involute; GLUT-1 negative. Rx: surgery; consider preoperative angiography with embolization for large lesions.
94
Define TA and KHE
Tufted angioma (TA) and kaposiform hemangioendothelioma (KHE) are a spectrum of vascular tumors of arteries and veins that are GLUT-1 negative
95
Describe TA to KHE
Congenital or acquired
>50% present <1 year old
Variable growth and regerssion paterns
96
Describe KHE growth pattern
Aggressive permeating muscle, soft tissue and bone
97
Describe TA/KHE histopathology
Hypercellular tufts of capillaries in the reticular dermis
May be associated with dilated lymphatic vessels
Deeper spindle cells indicate KHE
Positive for D2-40 (lymphatic marker)
98
Describe SSx of TA/KHE
Macules/patches (mottled red), plaques (red-purple, indurated), annular patterns of presentation; tenderness; hyperhidrosis, hypertrichosis (hard on palpation)
99
Describe visceral involvement of TA/KHE
Visceral involvement almost always associated with platelet trapping
100
How to diagnose congenital hemangioma?
CBC, DIC (diffuse intravascular coagulation) panel, MRI, biopsy if needed (may need in >1 location)
101
Rx for congenital hemangioma
Observation, cryotherapy, pulsed dye laser, surgical excision
102
Define Kasabach-Merritt Phenomenon
Sequestration of platelets and severe thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy in KHE or TA (not infantile hemangioma)
Suspect with rapidly enlarging lesion
Can be life-threatening
103
Rx Kasabach-Merritt Phenomenon
Drug combinations/chemotherapy (vincristine) or surgery
104
Define vascular malformations
Vascular channel malformations secondary to a defect in morphogenesis
Characterized as high flow (arteriovenous) or low flow (lymphatic, capillary, venous) malformations
105
How are vascular malformations different from hemangiomas?
VM are present at birth (although may manifest later secondary to hormonal changes, severe infections near vessels or trauma)
Grows with child (endothelium hypertrophies) and therefore does not regress
Sudden increase in size is concerning
106
Histopathology of vascular malformations
Mature endothelium with normal mitosis (versus hemangioma, which has increased mitosis in the proliferative phase
107
Associated symptoms of vascular malformations
Sturge-Weber syndrome
Klippel-Trenaunay syndrome (KTS; combination of capillary, venous, and lymphatic types of VM)
Parkes-Weber syndrome (arteriovenous and capillary malformation in association with skeletal/soft tissue hypertrophy)
Blue rubber bleb (venous malformation with associated GI lesions)
108
Sturge-Weber syndrome
(SWS; unilateral port-wine stain in CN V1 distribution with extension to the leptomeninges)
109
Klippel-Trenaunay syndrome
(KTS; combination of capillary, venous, and lymphatic types of VM)
110
Parkes-Weber syndrome
(arteriovenous and capillary malformation in association with skeletal/soft tissue hypertrophy)
111
Blue rubber bleb
(venous malformation with associated GI lesions)
112
Types of low flow vascular malformations
1. Lymphatic malformation
2. Capillary malformation
3. Venous malformation
113
Epidemiology of lymphatic malformations
90% present <3 years old (65% present at birth)
May persist in adulthood
Associated with venous malformations (lymphatics and venous system develop concurrently)
114
Histopathology of lymphatic malformation
Abnormal development or obstruction of primitive jugular lymphatics that undergo irregular growth with lymphoendothelial hyperplasia
Increase in mast cells during proliferative phase, fewer mast cells during the involutional stage (D2-40 stain)
115
Classification of lymphatic malformation
Macrocystic (>=1 cysts, >= 2mL)
Microcystic (<2 mL)
Mixed (formerly called cystic hygroma and lymphangioma)
116
What are cystic hygroma and lymphangioma now?
Mixed lymphatic malformation
117
Staging of lymphatic malformation
```
Modified de Serres
I: unilateral infrahyoid
II: unilateral suprahyoid
III: unilateral infrahyoid and suprahyoid
IV: bilateral infrahyoid and suprahyoid
V: bilateral infrahyoid and suprahyoid
VI: bilateral infrahyoid and suprahyoid
VII: retropharyngeal
M: mediastinal extension (I-III typically macrocystic and IV-VI typically microcystic)
```
118
SSx of lymphatic malformation
Soft painless compressible mass (lymphatic dilation) with skin discoloration (macrocystic); soft noncompressible masses with mucosal or skin vesicles (microcystic); both types may cause dysphagia, dyspnea; may remain dormant; may become painful with acute infection
119
Dx lymphatic malformation
Physical exam, MRI
120
Complications from lymphatic malformation
Respiratory distress from respiratory tract compression, infection (common, presents as a rapid enlargement of the malformation), disfigurement, spontaneous hemorrhage into macrocysts causing rapid enlargement
121
Rx lymphatic malformation
Sclerotherapy (doxycycline, bleomycin), sirolimus (rapamycin), early conservative excision when symptomatic (spare vital structures), low rate of recurrence if compeltely removed (only 50% if gross tumor remains); if detected prenatally and airway concerns, may require EXIT procedure
122
Define capillary malformation
Also called port-wine stain, stork bite, salmon patch (nape of the neck) or angel kiss (forehead)
123
Classification of capillary malformation
Medial (salmon patch, usually lightens and disappears with time)
Lateral (port-wine stain, always persists, usually follows CN V on face and darkens with time; involvement of lateral thigh and knee indicates Klippel-Trenaunay syndrome
124
SSx of capillary malformation
Bright red, scarlet at birth fades to pink in infants but then gradually enlarges and deepens to dark purple by adulthood
Medial type is lighter at birth
125
Rx of capillary malformation
Pulsed dye laser
126
Define venous malformation
Ectatic venous channel network which has a tendency to grow slowly in childhood, then expand rapidly with hormonal changes or trauma
Often involves the aerodigestive tract
127
SSx of venous malformation
Compressible mass, skin discoloration (none to dark blue/purple); airway obstruction, dysphagia,; symptoms often get worse with recumbent position or Valsalva
128
Dx of venous malformation
Physical exam, Doppler ultrasound, MRI
129
Rx of venous malformation
Surgery, laser, sclerotherapy (sodium tetradecyl sulfate, ethanol), anticoagulatns for symptomatic thrombosis
130
Types of high flow vascular malformations
Only arteriovenous malformation (AVM)
131
Define arteriovenous malformation
Shunting between the arterial systems via anomalous capillary beds
132
Growth of AVM
typically small and stable in childhood, then rapid growth in 2nd-3rd decade of life with puberty and/or trauma
133
AVM Staging
Schobinger staging
Stage I: quiescent
Stage II: expansion (bruit, thrill, warm throbbing)
Stage III: destruction (ulcers, bleeding, bony changes)
Stage IV: systemic (congestive heart failure, left ventricular hypertrophy)
134
SSx of AVM
Warm, pulsatile intermittently growing lesion with skin discoloration; bruit
135
Dx of AVM
Physical exam, pulsed Doppler, CTA, MRA and sometimes still digital angiography
136
Complications from AVM
Local tissue destruction and hemorrhage
137
Rx for AVM
Embolization; some require surgical excision
138
When are newborns preferential nasal breathers?
For 6-20 weeks (variable)
139
When should you suspect nasal obstruction?
Newborns with tachypnea, cyclical cyanosis (worse with feeding, improve with crying) FTT, rhinorrhea, unable to pass 6 French flexible suction catheter
140
Dx congenital nasal disorders and neonatal nasal obstruction
Assessment for dysmorphisms (telecanthus, broad nasal bridge, nasal pits), ocular discharge, periocular infection/edema, nasal endoscopy, oral exam (palate arching or clefting), CT or MRI if tumor suspicion
141
DDx of congenital nasal disorders and neonatal nasal obstruction
Rhinitis of the newborn, viral rhinitis, dacryocystocele, prifirom aperture stenosis, midnasal stenosis, choanal atresia, tumors (encephalocele, glioma, dermoid, teratoma, hemangioma), septal deviation (birth trauma, rare)
142
Rx for congenital nasal disorders and neonatal nasal obstruction
Address underlying cause, for bilateral obstruction or airway distress consider an oral cannula (McGovern nipple, oral airway) prior to intubation, tracheotomy reserved for severe cases
143
What is a McGovern nipple?
Large nipple with end cut off
144
Define idiopathic rhinitis of the newborn
Considered after negative work-up for nasal obstruction and other causes of rhinitis
145
Pathophysiology of idiopathic rhinitis of the newborn
Possibly secondary to maternal estrogen, infectious (congenital syphilis "snuffles", chlamydia), early allergic rhinitis (high prevalence of familial atopy), ciliary dyskinesia, hypothyroidism
146
SSx of idiopathic rhinitis of the newborn
Abundant rhinorrhea (mucoid), nasal mucosal edema, stertor, tachypnea, poor feeding
147
Rx for idiopathic rhinitis of the newborn
Frequent suctioning, nasal saline sprays, nasal steroid drops, avoid stenting if possible
148
Peds allergic rhinitis associated SSx
decreased energy and stamina, sleep deprivation, limitaitons in organized sports and outdoor activities, poor school performance
149
Risk factors for peds allergic rhinitis
urban living, obesity, no breast feeding, environmental tobacco smoke
150
Complications of peds allergic rhinitis
Asthma exacerbation (asthma in 48% of AR patients, but rhinitis in 80% of asthma patients, risk of asthma related to severity and duration of rhinitis), sinusitis, otitis media, sleep disorders, craniofacial abnormalities (upturned nose or "allergic salute," elongated "adenoid" facies), decreased cognitive functioning
151
When to consider immunotherapy?
Sublingual and intradermal immunotherapy may be considered >4-5 years old, may be protective against new sensitivities and development of asthma
152
Pathophysiology of choanal atresia
Persistence of the bucconasal membrane or abnormal migratio of neural crest cells into the nasal vault resulting in a complete bony (30%), mixed bony-membraneous (70%), or membranous (rare) defect of the posterior nasal cavity
Bony components are from the pterygoid plate and vomer
153
Epidemiology of choanal atresia
MC females; unilat (65%; R>L)
154
SSx of choanal atresia
Unilateral presents with rhinorrhea, nasal obstruction
Bilateral presents at birth with cycles of apnea and cyanosis followed by crying due to obligate nasal respiration in neonates
155
Dx choanal atresia
Mirror to detect condensation, attempt transnasal passage of 6 French catheter, attempt nose blowing with an occluded nostril, nasal endoscopy, CT
156
Associated syndromes with choanal atresia
CHARGE syndrome (MC concurrent syndrome in bilateral atresia, 50%), Apert, Treacher Collins, Crouzon, trisomy 21, 22q11 deletion
157
Rx for choanal atresia
Surgical repair via transnasal (usually endoscopic), transantral (creation of large cavity for recurrent cases), transseptal, or transpalatal (classic operation, may disrupt orthodontic growth, less common today) appraoch
Post-op stenting less common with endoscopic repair; unilateral atresia may be repaired electively
Bilateral atresia must be addressed during first weeks of life, early meausre includes establishing an oral airway (eg, McGoevrn nipple) intubation not usualyl required
158
DDx unilateral peds nasal mass
```
Vascular (JNA, hemangioma, AVM)
Infectious/inflammatory (polyp, rhinolith)
Neoplastic/mass (encephalocele, glioma, neurofibroma)
Drug-related
Idiopathic
Congenital (nasolacrimal duct cyst)
Autoimmune/allergic
Truamatic (foreign body)
Endocrine
```
159
Fonticulous frontalis
Embryologic space that normally fuses during teh development of the frontal bones
160
Prenasal space
Embryologic spaces between the nasal bone and nasal cartilage
161
Foramen cecum
Region between ethmoid and frontal bones, connects with prenasal space
162
Pathophysiology of neurogenic tumors
Dura projects through the foramen cecum, the fonticulus frontalis (intranasally), or the prenasal space into skin (extranasally) with failure of anterior neuropore closure
163
Dx neurogenic tumors
MRI/CT to evalute intracranial extention
| Do not biopsy
164
Pathophysiology of encephalocele
failured closure of neuropore or failed migration of neural crest cells results in ependymal-lined meninges herniation through the base of skull
Communicates with subarachnoid space (CSF filled)
165
Types of encephalocele by layers
MC lumbar-sacral region
1. Meningocele: meninges only
2. Meningoencephalocele: meninges and brain elements
3. Meningoencephalocystocele: meninges, brain, and a part of the ventricular system
166
Types of encephalocele by location
1. Occipital: most common (75%)
2. Sincipital (frontoethmoidal): defect between frontal and ethmoid bones at the foramen cecum; nasofrontal (glabellar lesion), nasoethmoidal (lateral nose lesion), and nasoorbital (medial orbital wall lesion) subtypes
167
SSx of encephalocele
Bluish, soft, pulsatile, compressible mass that changes with straining and crying, transilluminates
Intranasal encephaloceles often confused with nasal polyps
168
Dx of encephalocele
CT/MRI reveals bony defect
| Furstenburg test
169
What is Furstenburg test?
Compression of IJ cuases increases in size of the mass due to increased CSF pressure
170
Complications of encephalocele
meningitis, nasal obstruction, cosmetic deformity, hydrocephalus
171
Rx for encephalocele
surgical excision similar to glioma, must also close the dural defect to prevent CSF leak and brain herniation (neurosurgical consultation)
172
Pathophysiology of nasal glioma
Sequestered glial tissue or "pinched off encephalocele" results in unencapsulated collection of heterotopic glial cells
15% connect to dura by a fibrous stalk
173
Location of nasal glioma
Extranasal (60%) or intranasal (30%), typically not midline
174
SSx of nasal glioma
Firm, nonpulsatile mass, skin-covered, does nto change in size with straining, noncompressible, does not transilluminate, may look like intranasal polyp, broad nasal dorsum
175
Dx of nasal glioma
CT and/or MRI to evaluate for intracranial extension (15%), neg Furstenburg test
176
Complications of nasal glioma
Meningitis, nasal obstruction, cosmetic deformity
177
Rx for nasal glioma
Surgical excision (intranasal appraoch for small tumors, extranasal appraoch for larger tumors), may require craniotomy for intracranial involvement (neurosurgical consultation)
178
What is the most common midline nasal mass?
Nasal dermoid
179
Pathophysiology of nasal dermoid
defective obliteration of dural tissue in prenasal space or fonticulus frontalis forms a dermal cyst
180
Which germ layers for nasal dermoid?
May contain both mesodermal and ectodermal components including adnexal tissue (hair follicles, sweat glands, sebaceous glands)
181
Location of nasal dermoid
MC at nasal dorsum but may occur anywhere from glabella to nasal tip (extranasal, intranasa, or intracranial)
182
SSx of nasal dermoid
Presents at birth; forms a fistulous tract, pit, or cyst
Firm, noncompressibel, does not transilluminate
Tuft of hair may protrude from pit
183
Dx of nasal dermoid
CT and/or MRI to evaluate for intracranial extension (@5%), negative Furstenburg test
184
Complications of nasal dermoid
Meningitis, CSF leak, repeated local infection, cosmetic deformity
185
Rx for nasal dermoid
Meticulous excision, must excise complete tract (usually subcutaneous although may dive deep into nasal bone and intracranially)
186
Pathophysiology of Rathke's cleft cyst
Persistent craniopharyngeal canal from failure of the obliteration of Rathke's pouch (a diverticulum of ectoderm that invaginates to form the anterior pituitary and pars intermedius)
187
What is the Rathke's pouch?
A diverticulum of ectoderm that invaginates to form the anterior pituitary and pars intermedius
188
Location of Rathke's pouch
Nasopharynx
189
SSx of Rathke's pouch?
Typically asymptomatic, smooth mass in nasopharynx, may enlarge and impinge on the pituitary
190
Dx of Rathke's pouch
CT or MRI, biopsy
191
Rx for Rathke's pouch
Endoscopic removal or marsupialization if symptomatic, antibiotics if infected
192
What is another name for nasolacrimal duct cyst?
Dacryocystocele
193
Pathophysiology of dacryocystocele
Failure of opening of the distal nasolacrimal duct (valve of Hasner) with cyst formation from accumulation of secretions
194
Location of dacryocystocele
Nasolacrimal duct (anteriorly in the inferior meatus)
195
SSx of dacryocystocele
Usually asymptomatic, epiphora, nasal obstruction, may cause respiratory distress and difficulty feeding in infants with large obstructive cysts (obligate nasal breathing)
196
When do dacryocystocele resolve?
~85% spontaneously resolve by 9 months of age
197
Dx dacryocystocele
Nasal exam, nasal endsocopy, CT or MRI
198
Rx for dacryocystocele
Endoscopic marsupialization (if presence of infection, respiratory obstruction, feeding difficulties) or Abx with excision for recurrent infected lesions (consider ophtho consultation)
199
Pathophysiology of Thornwaldt's cyst
Arises from a pharyngeal notochord remnant (pharyngeal bursa or pouch of Luschka)
200
Location of Thornwaldt's cyst
Nasopharynx (midline, surrounded by adenoid tissue)
201
SSx of Thornwaldt's cyst
asymptomatic, smooth mass found in nasopharynx; rare infection, postnasal drip, referred otalgia, or otitis media with effusion
202
Dx of Thornwaldt's cyst
CT or MRI, biopsy
203
Rx for Thornwaldt's cyst
Observation, Abx with marsupialization or excision for infected lesions
204
What sinuses are affected the most in peds rhinosinusitis?
Typically involves maxillary and anterior ethmoid sinuses (sphenoid and frontal sinuses typically do not develop until 8-12 years old)
205
Where is the larynx in an infant?
At level of C2-3, descends during infancy C6-7 by adulthood
206
Where is the smallest cross-sectional area in infant and adults?
Infant is at subglottis (4-5 mm diameter in neonate), whereas in adult it is at glottis inlet (rima)
207
unit decrease in subglottic stenosis results in
1 mm of subglottis edema in the neonate can reduce airway by more than 50%
208
ETT size approximation
ETT size = (age + 16)/4
209
3 weeks dev for respiratory primordium
thickening of foregut (primitive foregut) forming ventral laryngotracheal groove
210
4 weeks dev for respiratory primordium
laryngotracheal groove evaginates to form the laryngotracheal diverticulum dividing the foregut into a dorsal (esophagus) and vental portion (larynx, trachea, lung) separated by the tracheoesophageal septum (incomplete fusion results in persistent lrayngopharyngeal or tracheoesophageal communication)
211
5 weeks dev for respiratory primordium
larynx develops fro mmesenchymal swellings from sixth branchial arch with 3 tissue swellings (1 median swelling behind the hypobranchial eminence, which forms the epiglottis, and 2 lateral swellings that form the arytenoid cartilages) surrounding the laryngeal orifice
212
6 weeks dev for respiratory primordium
Epithelial proliferation obliterates the laryngeal lumen
213
8 weeks dev for respiratory primordium
larynx evident
214
10 weeks dev for respiratory primordium
recanalization of larynx and trachea (no recanalization results in stenosis)
215
Laryngeal branchial arch derivatives from arch II
lesser horn and upper portion of hyoid bone
216
Laryngeal branchial arch derivatives from arch III
greater horn and lower portion of the hyoid bone
217
Laryngeal branchial arch derivatives from arch IV
supraglottic structures (thyroid cartilage), superior laryngeal nerve (SLN) structures (cricothyroid muscle and pharyngeal constrictors)
218
Laryngeal branchial arch derivatives from arch V/VI
glottic/subglottic structures (cricoid, cuneiform, corniculate, and arytenoid cartilages) and recurrent laryngeal nerve (RLN) structures (all intrinsic laryngeal muscles except the cricothyroid
219
Esophageal embryology
Epithelial proliferation obliterates the lumen, recanalization occurs by 8th-10th week (abnormal recanalization results in congenital stenosis)
220
Characters of stridor
Inspiratory stridor suggests supraglottic or glottic pathology, biphasic stridor suggests glottic or subglottic, expiratory stridor alone suggests distal tracheal or bronchial sourec
221
Utility of AP and lateral neck x-ray in peds airway
Unilateral subglottic lesion (eg, hemangioma), subglottic narrowing ("steeple sign"), epiglottic widening ("thumb sign")
222
Utility of CXR in peds airway
Evaluate for foreign body aspiration (insp/exp CXR or lat decubitus film may demonstrate the foreign body and/or diaphragmatic flattening and mediastinal shift), other pulmonary causes
223
Utility of CT neck/chest in peds airway
vascular compression
224
Utility of MRI neck chest in peds airway
extrinsic compression, best for lymphovascular malformation work up
225
What does L&B evaluate for in peds airway?
Evaluate for malacia (intrinsic or extrinsic), stenosis (location, length, etiology), fistulae and/or laryngeal cleft, masses or other lesions
226
Cotton-Myer grading system for subglottic stenosis
Stage I: 1-50% obstruction
Stage II: 51-70% obstruction
Stage III: 71%-99% obstruction
Stage IV: no detectable lumen
227
Indications for peds trach
Premature infants with cardiopulmonary or neurologic impairment requiring prolonged ventilator support
Upper airway obstruction, craniofacial anomalies
>2 kg
228
Position of hyoid in peds trach
hyoid overlies superior laryngeal cartilage
229
Peds trach technique
Small vertical or horizontal skin incision, stay sutures placed laterally through trachea on eitehr side of proposed vertical trachotomy incision (labeled "right" and "left"), tracheotomy incision most comonly at 2nd or 3rd tracheal ring, ICU until first trach tube change postop day 5-7
230
Strategy for downsizing or decannulating trach tube
Endoscopy to eval for subglottic stenosis or suprastomal grnuloma
231
Peds aerodigestive evaluation
```
L&B to eval for structural abnormality of aerodigestiev tract (eg larynegeal cleft, H-type tracheoesophageal fistula)
Flex bronch (bronchoalveolar lavage for cytology and microbio)
EGD with biopsy, pH impedance probe
```
232
Surgical options of salivary management
If excessive sialorrhea and/or aspiration causing respiratory illness
1. Botox: inject submandibular and parotid glands
2. Submandibular gland excision and parotid duct ligation: severe cases (surgical rerouting of submandibular ducts also an option in patients who do not aspirate
233
Etiology of congenital high airway obstruction syndrome
obstruction by congenital mass, eg teratoma, epignathus (teratoma arising from basisphnoid region and filling oral cavity; often protruding through the hard palate), lymphovascular malformation
234
What is EXIT procedure?
Ex-utero intrapartum procesdure, where airway is secured at delivery while the patient is on placental circulation
235
Most common cause of congenital stridor
laryngomalacia
236
Pathophysiology of laryngomalacia
immature, constricted laryngeal anatomy with poor structural support and/or tone
237
SSx of laryngomalacia
intermittent inspiratory stridor that may improve when prone
Exacerbated with feeding, crying, or when supine
Retractions, poor weight gain, FTT
Presents within weeks of birth
Normal voice
Usually self-limited and resolved by 18-24 months
238
MC laryngeal findings on laryngomalacia
1. Inward collapse of A-E folds, arytenoid cartilage, and epiglottis into laryngeal inlet during inspiration
2. short aryepiglottic folds
239
When is supraglottoplasty done for laryngomalacia
Release short A-E folds +/- remove excess mucosal tissue from arytenoids for severe dyspnea, failure to thrive, obstructive sleep apnea
240
Etiology of congenital bilateral vocal fold immobility
1. Neurologic
2. Iatrogenic
3. Idiopathic
241
Which cause is most common for congenital bilateral vocal fold immobility?
Idiopathic
242
What are the neurologic cuases for congenital bilateral vocal fold immobility?
Arnold-Chiari malformation and meningomyelocele
243
What are the iatrogenic causes for congenital bilateral vocal fold immobility?
Birth trauma, increased risk with complicated or forceps delivery (traction injury, may be temporary)
244
List some surgical options for vocal fold intervention
Lateralization, arytenoidopexy, arytenoidectomy, posterior cordotomy, posterior cricoid cartilage grafting, laryngeal reinnervation
245
Location of congenital webs
MC anteriorly, thin or thick
246
Pathophysiology of congenital webs
incomplete recanalization of the airway at 10th week of embryologic development
247
Ssx of congenital webs
weak cry at birth, aphonia, variable degrees of respiratory obstruction (inspiratory or biphasic stridor)
248
Dx for congenital webs
flexible nasopharyngoscopy, direct laryngoscopy
249
Rx for congenital webs
genetics evaluation (eg, 22q11 deletion); endoscopic lysis +/- brief period of intubation or endoscopic keel placement, open lysis via laryngofissure with keel placement, may require tracheotomy
250
Prognosis for congenital vs acquired stenosis
Congenital more likely to improve over time compared to acquired, and is frequently associated with syndromes (22q11 deletion, Down, CHARGE)
251
Pathophysiology of congenital subglottic stenosis
incomplete recanalization; small diameter or elliptical cricoid cartilage (<3.5 mm diameter is abnormal); first tracheal ring partially telescoped under cricoid cartilage (trapped ring)
252
Types of congenital subglottic stenosis
1. Membraneous: circumferential, thickened mucous glands or fibrous tissue
2. Cartilaginous: abnormal cricoid, trapped ring
3. Mixed
253
Type of stridor for SGS
biphasic stridor, retractions at rest
254
DDx of SGS
croup, bacterial tracheitis, subglottic hemangioma, subglottic cysts, vocal fold parlaysis, complete tracheal rings
255
When to do open surgical management for SGS?
For more severe or multilevel stenosis, endoscopic grade I-II failures, previous recon failures, hx difficult intubation, poor pulmonary function, sedation issues, medical comorbidities
256
Types of open surgical intervention for SGS
1. Expansion: anterior cricoid split, anterior cotal cartilage graft (single-stage, thyroid ala or costal cartilage), posterior cricoid split, posterior costal cartilage graft (posterior glottis stenosis, bilateral vocal fold immobility)
2. Resection: cricotracheal resection (severe stenosis, previous airway recon, stenosis >3 mm from vocal folds)
3. Slide tracheoplasty
4. tracheotomy
257
Etiology of congenital tracheal stenosis
Often due to complete tracheal rings
| Associated with congenital cardiac abnormalities (eg, pulmonary artery sling)
258
Tracheomalacia types
Intrinsic
| Extrinsic
259
Intrinsic tracheomalacia pathophysiology
Poor cartilaginous strength, low tone
260
Comorbidities of intrinsic tracheomalacia
associated with premature infants with bronchopulmonary dysplasia (prolonged intubation and positive airway pressure) and tracheoesophageal fistula)
261
SSx intrinsic tracheomalacia
failure to extubate, expiratory or biphasic stridor, barky cough, exacerbated with infection.
Usually self-limiting as cartilage stiffens with growth over years but may persist in children with tracheoesophageal fistula and a large pouch.
262
Dx intrinsic tracheomalacia
history, flexible tracheoscopy, bronchoscopy (r/o tracheoesophageal fistula, laryngeal cleft)
263
Rx intrinsic tracheomalacia
observation (typically resolves with growth), may require noninvasive ventilation, stenting (internal), splinting (external), tracheopexy or tracheotomy
264
Types of vascular compression causing extrinsic tracheomalacia
1. Double aortic arch
2. Right aortic arch
3. Anomalous innominate artery
4. Anomalous left common carotid
5. Left aortic arch with right descending thoracic aorta
6. Pulmonary artery sling
7. Retroesophageal right subclavian artery (dysphagia lusoria)
265
Describe double aortic arch
Most common vascular anomaly to cause stridor.
right 4th brnachial arch persists
right aortic arch wraps around eosphagus and trachea
266
Describe right aortic arch
right 4th branchial arch remains instead of left; association with tetralogy of Fallot in 30%
Types:
1. right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum
2. right aortic arch with mirror-image branching and retroesophageal ligamentum arteriosum
267
Describe anomalous innominate artery
left innominate originates more distally, innominate crosses over trachea from left to right
268
Describe pulmonary artery sling
left pulmonary artery originates from right pulmonary artery, slings around right mainstem bronchus, then between trachea and esophagus
Associated with complete tracheal rings, tracheomalacia, and stenosis
269
Describe retroesophageal right subclavian artery
Dysphagia lusoria. MC arch anomaly
| Right subclavian artery originates as last branch from descending aorta
270
SSx extrinsic tracheomalacia
biphasic stridor (external compression of trachea), barking cough, dyspnea with feeding, dysphagia
271
Dx extrinsic tracheomalacia
bronchoscopy, barium esophagram, CT with contrast, MRA
272
Rx extrinsic tracheomalacia
symptomatic compression requires surgical intervention
273
Describe laryngeal cleft and laryngotracheoesophageal cleft
Failure of tracheoesophagela septum to develop
274
SSx laryngeal cleft and laryngotracheoesophageal cleft
stridor, dysphagia, aspiration, recurrent pneumonia (mild clefts); respiratory distress at birth (severe clefts)
275
Associations with laryngeal cleft and laryngotracheoesophageal clefts
Opitz G/BBB syndrome
Pallister-Hall syndrome
Tracheoesophageal fistula, esophageal atresia, laryngomalacia, congenital heart defects
276
Dx laryngeal cleft
direct laryngoscopy +/- diagnostic laryngeal gel injection
| Ancillary testing: MBSS to eval for aspiration, CXR
277
Classification of laryngeal cleft
```
Benjamin-Inglis classification
I: interarytenoid defect
II. incomplete cricoid involvemnt
III: complete cricoid invovelemtn
IV: below thoracic inlet
```
278
Rx laryngeal cleft
goal is secure airway and avoid chronic aspiration
Symptomatic type I-II and certain type III clefts can be repaired endoscopically, most type III and all IV require open repair
279
Pathophysiology of congenital tracheoesophageal fisulta
Failure of recannulation of the esophagus or developmental failure of teh tracheoesophageal septum
280
Pathophysiology acquired tracheoesophageal fisulta
secondary to tracheostomy, long-term intubation, tumor, inflammation, trauma
Results in communication between lumen of the esophagus and the trachea
281
Types of tracheoesophageal fistula
A. isolated esophageal atresia without TEF: second most common (~10%), associated with polyhydramnios
B. esophageal atresia with proximal TEF
C. esophageal atrea with distal TEF
D. esophageal atresia with proximal and distal TEF
E/H. Isolated tracheoesophageal fistula (H-type): rarely remains asymptomatic until later in life
282
SSx of tracheoesophageal fisulta
immediate gagging and cyanosis after birth, gas-filled stomach
May present later in life wit cough, recurrent aspiration pneumonia, gagging
Long-term symptoms related to tracheomalacia and poor esophageal motility
283
Dx of tracheoesophageal fistula
barium esophagram, bronchoscopy, esophagoscopy
284
Rx for tracheoesophageal fistula
division (and reconstruction of the trachea/esophagus as needed)
285
Causes of acquired glottic and subglottic stenosis
blunt trauma, endotracheal tube trauma, infection, caustic ingestion, foreign body
286
SSx acquired glottic and subglottic stenosis
Inspiratory of biphasic stridor, dyspnea, cough
287
Dx acquired glottic and supraglottic stenosis
flexible laryngoscopy, bronchoscopy
288
Rx acquired glottic and supraglottic stenosis
endoscopic or open techniques depending on severity
289
Causes of acquired subglottic and tracheal stenosis
Eight.
1. endotracheal intubation
2. postoperative
3. granulomatous disease
4. infectious
5. idiopathic
6. trauma
7. systemic
8. neoplasia
290
Describe endotracheal intubation as a cause for acquired subglottic and tracheal stenosis
pressure necrosis results in ulceration and cartilage exposure, healing occurs by secondary intention causing fibrosis and stenosis
291
Describe postoperative causes for acquired subglottic and tracheal stenosis
pressure necrosis from a high tracheostomy, cricothyroidotomy, or failed previous airway surgery
292
Describe granulomatous disease as a cause for acquired subglottic and tracheal stenosis
TB (MC granulomatous disease of larynx), sarcoidosis, rhinoscleroma (klebsiella), granulomatosis with polyangiitis (Wegener's)
293
Describe infectious causes for acquired subglottic and tracheal stenosis
leprosy (epiglottic and vocal fold ulceration), syphilis, blastomycosis, coccidiomycosis, histoplasmosis
294
Describe idiopathic causes for acquired subglottic and tracheal stenosis
amyloidosis, unknown
295
Describe trauma as a cause for acquired subglottic and tracheal stenosis
foreign body, caustic ingestion, blunt trauma, hematoma, thermal injury
296
Describe systemic causes for acuqired subglottic and tracheal stenosis
connective tissue disorders, GERD (presumed), radiation effects
297
Describe neoplasia as a cause for acquired subglottic and tracheal stenosis
chondroma, fibroma, malignancy
298
SSx for acquired subglottic and tracheal stenosis
dyspnea, biphasic stridor, cough, dysphagia, cyanosis, exercise limitation
299
Dx for acquired subglottic and tracheal stenosis
Endoscopy, CT of neck, directed labs for various etiologies
300
Rx for acquired subglottic and tracheal stenosis
Surgical management similar to congenital subglotitc stenosis (which is what)
301
Risk factors for subglottic cyst
cause of stridor in a previously intubated infant (hx of prematurity and reflux)
302
Rx for subglottic cyst
consider unroofing endoscopically with caterization of base; propensity for recurrence
303
Causes for unilateral vocal fold immobility
birth trauma, intubation, PDA ligation, aortic arch anomaly repair, tracheoesophageal fistula repair
304
What is the other word for croup?
acute laryngotracheobronchitis
305
What ist he most common infectious cause of stridor in children?
acute laryngotracheobronchitis or croup
306
Where does acute laryngotracheobronchitis most commonly affect?
subglottic region
307
What viruses are implicated in acute laryngotracheobronchitis?
```
Parainfluenza 1 (most common cause), 2, and 3
Influenza A, rhinovirus, repiratory syncytial virus, adenovirus, coinfection with multple viruses
```
308
SSx of acute laryngotracheobronchitis
inspiratory of biphasic stirdor, gradual onset, long course (3-7 days), low-grade fever, relief in the recumbent position, brassy/barky cough (worse at night), hoarse, nontender larynx, no dysphagia, no drooling
309
Dx for acute laryngotracheobronchitis
H&P, plain neck x-rays (steeple sign: narrowed subglottis on PA neck x-ray), flexible nasopharyngoscopy (not required)
310
Rx for acute laryngotracheobronchitis
1. Assess airway: intubation or tracheotomy rarely required unless there is co-existing laryngeal abnormality like SGS
2. Medical management: humidified oxygen, corticosteroids, nebulized racemic epinephrine, Abx not required unless suspect bacterial superinfection
3. Endoscopy: if no resolution with conservative management
311
Other names for bacterial tracheitis
Exudative tracheitis
| Membraneous laryngotracheobronchitis
312
Pathophysiology of bacterial tracheitis
Bacterial superinfection following viral URI prodrome (Staph aureus)
313
SSx of bacterial tracheitis
thick secretions in airway, fibrinous sloughing (exudative) membrane in trachea, high fever, tender larynx and trachea
314
Dx of bacterial tracheitis
clinical suspicion, lateral neck x-ray may show shaggy appearance of trachea (membranes), flexible laryngoscopy may reveal exudative membranes in the subglottis and upper trachea, microlaryngoscopy and bronchoscopy
315
Rx for bacterial tracheitis
aggressive pulmonary hygiene, parenteral Abx, microlaryngoscopy and bronchoscopy with membrane clearing and culture, may require intubation
316
Can Diphtheria occur if vaccinated?
Yes. Milder form may present despite immunization
317
Diphthreia pathogen
Corynebacterium diphtheriae
318
SSx for Diphtheria
thick, gray-green plaques
| Memranous, friable exudate on tonsils, pharynx, and larynx
319
Rx for Diphtheria
airway management, laryngoscopy, isolation precautions, diphtheria antitoxin, antibiotics (PCN, erythromycin), humidity
320
what is the other name for epiglottitis?
Acute supraglottitis
321
Pathogen for acute supraglottitis?
classically Haemophilus influenzae b
| Older children and adults may have infection with gram-positive bacteria
322
Risks of acute supraglottitis
children >1 year old to adult (MC between 2-6 years old)
| Rare since Hib vaccine
323
Ssx of acute supraglottitis
```
sudden onset (hours) and short course
High fever, odynophagia, dysphagia, droolin, dyspnea, "sniffing position" (neck hyperextended with chin protruding), no cough, mulffled voice, tender larynx
```
324
Dx of acute supraglottitis
Clinical features, plain neck x-ray (thumbprint sign: swollen epiglotis on lateral x-ray)
325
Complications of acute supraglottitis
bacteremia/sepsis, acute airway obstruction (death)
326
Airway Rx acute supraglottitis
avoid aggravating patient and precipitating airway collapse (if high suspicion and symptomatic, establish airway in OR via intubation with preparation for trach)
DL with epiglottis cultures
For mild symptoms in stable patient with questionable diagnosis, consider plain x-ray, flexible laryngoscopy
327
Medical Rx for acute supraglottitis
Monitored bed, parenteral antibiotics and corticosteroids for 7-10 days, extubation when clinical improvement and decreased epiglottic edema
328
Locations of lesions of recurrent respiratory papillomatosis
wartlike, irregular, exophytic, between junction of ciliated and squamous epithelium (limen vestibuli, midzone epiglottis, ventricle margin, undersurface of true vocal fold)
Nasopharyngeal, tracheal, and bronchial lesions at sites of squamous metaplasia
329
Types of RRP
1. Juvenile onset <12 years, multiple sites, recurrent, may resolve spontaneously
2. Adult onset >=12 years, may involve single site, recurrence less common, identical histology
330
Complications of RRP
Rarely pulmonary involvement
Hemorrhage and abscess
Respiratory compromise
331
Rx for RRP
Endoscopic: freq microlaryngoscopy and bronchoscopy with conservative excision employing cold techniques, microdebrider, lasers (KTP or PDL for anterior commissure)
Annual biopsy to r/o dysplasia
AVOID TRACH as may cause squamous metaplasi aand site for expansion
Adjunctive: cidofovir, interferon, retinoids, acyclovir, photodynamic therapy, bevacizumab
332
Airway surgery and eosinophilic esophagitis
Implicated as a cause of airway surgery failure when uncontrolled
333
SSx of eosinophilic esophagitis
feeding intolerance, failure to thrive, vomiting and abdominal pain, food impaction, rhinitis and rhinosinusitis, voice complaints, noisy breathing, reactive airway disease, aspiration, airway edema, SGS
334
Dx of eosinophilic esophagitis
esophageal symptoms and characteristic histopathology of >= 15 eosinophils per high powered field in the absence of routine GERD or eosinophilia throughout the GI tract
335
Rx for eosinophilic esophagitis
allergic treatments include oral corticosteroids, elimination diets, and immune-modulators
336
MC non-food object in peds foreign body of esophagus
coins
337
Most common location
cricopharyngeus
338
Most common foreign body of the airway
Food including peanuts (MC)
339
Dx airway foreign body
CSR with inspiratory/expiratory phases (evaluate for air trapping if object not radioopaque, object may produce ball-valve effect causing the affected side to be hyperinflated; atelectasis may cuase affected side to be hypoinflated)
Left/right lateral decubitus phases (affected lung may not undergo normal collapse when dependent), neck x-ray
340
Caustic ingestion what is worse alkali or acidic?
Alkali agents pH >11.5, causes liquefaction necrosis (deeper penetration, more severe damage to the esophagus)
vs. coagulation necrosis (damage limited to the mucosa)
341
Stages of esophageal caustic injury
<24 hours: dusky submucosal edema +/- ulceration
2-5 days: submucosal inflammation (gray coagulum) with thrombosis
5-7 days: sloughing of superficial layer
1 week: fibrosis of deep layers and formation of scars and strictures
2-5 weeks: contraction
342
Assessment of injury after caustic ingestion
```
Direct esophagoscopy required to evaluate esophageal and gastric injury (ideal 24-48 hrs), if performed too early (<12 hrs) may miss severity of dmaage, if performed >48 hours then esophagus too damaged risking perforation
Barium swallow (esophagram): first diagnostic tool >48 hrs; also obtain for progressive dysphagia to evaluate for stricture formation and to confirm perforation
```
343
Types of esophgeal burns
First degree: superficial mucosal injury
Second degere: transmucosal injury:
Thrid degree: transmural injury
344
Composition of branchial arch
composed of a cartilaginous bar, brachiometric nerve, muscular component, and aortic arch artery
345
Composition of branchial groove or cleft
external, lined with ectoderm
346
Composition of branchial membrane
formed between branchial groove and pouch
347
Composition of branchial pouch
internal, linked with endoderm, contains a ventral and dorsal wing
348
Name of branchial arch I
Mandibular arch
349
Cartilage of branchial arch I
Mekel's cartilage
Malleus head and neck, incus body and short process, anterior malleal ligament, mandible (formed from intramembranous ossification)
350
Nerve/muscle of branchial arch I
CN V3.
| Muscles of mastication, tensor tympani, tensor veli palatini, mylohyoid, and anterior digastric muscles
351
Artery of branchial arch I
Maxillary artery
352
What are hillocks of His of branchial arch I?
1. Tragus
2. Helical crus
3. Helix
353
Name of branchial arch II
Reichert's cartilage
Manubrium of malleus, long process and lenticular process of the incus, stapes (except vestibular part of footplate), styloid procsess, stylohyoid ligament, lesser cornu and upper half of hyoid
354
Nerve/muscle of branchial arch II
CN VII.
| Muscles of facial expression, stapedius, stylohyoid, and posterior digastric muscles
355
Artery of branchial arch II
Stapedial artery (degenerates; if too aerly causes microtia/atresia)
356
What are hillocks of His of branchial arch II?
4. Antihelix crus
5. Scapha
6. Lobule
357
Cartilage of branchial arch III
greater cornu and lower half of hyoid
358
Nerve/muscle of branchial arch III
CN IX.
| Stylopharyngeus muscle, superior and middle constrictors
359
Artery of branchial arch III
Common and internal carotid
360
Cartilage of branchial arch IV
thyroid and cuneiform cartilage
361
Nerve/muscle of branchial arch IV
superior laryngeal nerve.
| Cricothyroid muscles and inferior pharyngeal constrictors.
362
Cartilage of branchial arch V/VI
cricoid, arytenoid, and corniculate cartilage
363
Nerve/muscle of branchial arch V/VI
recurrent laryngeal nerve
| Intrinsic laryngeal muscle (except cricothyroid muscle)
364
Artery of branchial arch V/VI
ductus arteriosus and pulmonary arteries
365
Branchial cleft I
EAC, outer TM (dorsal part)
366
Branchial cleft II
obliterates
367
Branchial cleft III
obliterates
368
Branchial cleft IV
obliterates
369
Branchial cleft V
obliterates
370
Branchial pouch I
eustachian tube, middle ear (mastoid air cells), inner TM
371
Branchial pouch II
supratonsillar fossa, palatine tonsils, middle ear
372
Branchial pouch III
epithelial reticulum of thymus, inferior parathyroids
373
Branchial pouch IV
parafollicular cells (C-cells) of thyroid, superior parathyroids
374
Describe thyroid embryology
Endoderm between the first and second branchial arch on the floor of pharynx (foramen cecum) invaginates around the 4th week and descends into mesencyhmal tissue along the path of the thyroid duct (anterior to the hyoid bone), forming a ventral diverticulum that differentiates at the distal end into the thyroid anlage
The proximal portion typically atrophies by the 6th week.
375
Thyroid embryonic pathology
athyreosis (rare), ectopic thyroid (may be found anywhere along the thyroid duct from the tongue as a lingual thyroid to the sternal notch), thyroglossal duct cyst
376
Describe parathyroid embryology
Third dorsal branchial pouch -> inferior parathyroids and thymus
Fourth dorsal branchial pouch -> superior parathyroids and C-cells of the thyroid
377
Parathyroid embryonic pathology
Supernumerary parathyroids, aberrant parathyroids (most common location at the anterior superior mediastinum)
378
What percent of peds head and neck masses are benign?
80-90% of pediatric H&N masses are benign
379
DDx lateral neck mass
Congenital (branchial anomaly, laryngocele)
Inflammatory (lymphadenitis)
Vascular (hemangioma, lymphatic malformation, venous malformation)
Neoplastic (thyroid malignancy, thymic cyst, lipoma, neuroblastoma, lymphoma)
Traumatic (pseudotumor of infancy)
380
DDx midline neck mass
congenital (thyroglossal duct cyst, ectopic thyroid, dermoid cyst, teratoma, thymic cyst)
Inflammatory (lymphadenitis, plunging ranula)
Neoplastic (thyroid malignancy)
381
DDx submandibular mass
Inflammatory (plunging ranula, sialadenitis, lymphadenitis)
Vascular (lymphatic malformation)
Neoplastic (salivary gland neoplasm)
382
PFAPA syndrome
Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis, idiopathic condition in <5 years old, >=3 monthly episodes lasting 5-7 days that include fever/chills/malaise, pharyngitis, aphthous stomatitis, tender cervical lymphadenopathy
383
Rx PFAPA syndrome
Corticosteroids in the acute phase and consider tonsillectomy
384
Pathophysiology of branchial cleft anomalies
Developmental alterations of the branchial apparatus result in cysts (no opening), sinuses (single opening to skin or digestive tract), fistulas (opening to skin and digestive tract), or cartilaginous remnants
385
SSx of branchial cleft anomalies
anterior neck mass (anterior to SCM, deep to platysma)
May have an associated subcutaneous palpable cord
Fistulas and sinuses may express mucoid discharge
Secondary infections (commonly after URI) cuase periodic fluctuation of size, tenderness, and purulent drainage
Cartilaginous remnants appear as small horns or firm bumps
386
Dx branchial cleft anomalies
physical exam, CT w con, MRI, laryngoscopy to visualize internal opening if there are associated symptoms of if it will change the surgical approach
387
Histopathology of brnachial cleft anomalies
Lined by squamous epithelium
388
SSx first branchial cleft cyst
usually presents as a preauricular cyst (type I) or at the mandibular angel or submandibular region (type II)
389
Pathways of Type I first branchial cleft cyst
Type I is ectodermal elements only; duplicated EAC
Typically begins periauricularly -> passes lateral (superior) to CN VII -> parallels the EAC -> ends as a blind sac near the mesotympanum
390
Pathways of Type II first branchial cleft cyst
More common; ectodermal and mesodermal elements; duplicated membranous EAC and pinna
Presents near angle of mandible -> passes lateral or medial to CN VII -> ends near or in the EAC
391
Rx for first branchial cleft cyst
Abx when secondarily infected (avoid I&D), full excision after resolution of infection (risk of facial nerve injury), may need superficial parotidectomy
392
What is the most common branchial cleft abnormality?
Second branchial cleft cyst
393
SSx second branchial cleft cyst
Cyst along anterior border of SCM
394
Fistula pathway of second branchial cleft cyst
external opening at anterior neck -> along carotid sheath -> between external and itnernal carotid arteries -> deep to CN VII and superficial to CN XII and CN IX -> internal opening at middle constrictors or in tonsillar fossa
395
Relationship between second branchial cleft cyst and second arch derivatives and third arch derivatives
the course of the second branchial cleft cyst runs deep to second arch derivatives and superficial to third arch derivatives
396
Rx second branchial arch cleft
Abx when secondarily infected (avoid I&D), full excision after resolution of infection
397
SSx third branchial cleft cyst
cyst in lower anterior neck (less common); majority on left side
Prsents as abscess neck mass (stridor, dysphagia, feeding difficulties), recurrent acute suppurative thyroiditis; often associated with thymic cysts
398
Fistula pathway of third branchial cleft cyst
External opening at lower anterior neck -> superficial to CN X and common carotid artery -> superficial to CN XII -> deep to CN IX -> pierces thyrohyoid membrane staying above superior laryngeal nerve -> itnernal opening at upper (base of piriform sinus
399
Rx for third branchial cleft cyst
Treat infection with Abx; full excision after resolution of infection, avoid I&D
Excision must include partial thyroidectomy (superior pole of affected side)
Endoscopic obliteration of the piriform opening increasingly popular
400
Tell us about fourth branchial cleft cyst
Extremely rare; Ssx are similar to third branchial cleft cyst
401
Fistula pathway of fourth branchial cleft cyst
external opening at lower anterior neck -> loop aroud CN XIII -> posterior to common carotid artery -> thoracic component with loop below the aorta (left) or around subclavian artery (right) -> posterior to thyroid gland -> tracheoesophageal groove -> pierces cricothyroid membrane beneath superior laryngeal nerve -> apex of piriform sinus; recurrent laryngeal nerve remains deep to tract
402
Rx fourth brnachial cleft cyst
similar to third branchial cleft cyst, follow the tract
403
Pathophysiology of thyroglossal duct cyst
familure of complete obliteration of thyroglossal duct (Creatd from tract of embryologic thyroid descent: from floor of embryologic pharynx [tuberculum impar], which later forms the foramen cecum, down to midline neck
404
SSx thyroglossal duct cyst
midline neck mass with cystic and solid components, elevates with tongue protrusion (attached to hyoid bone), typically inferior to hyoid bone and superior to thyroid gland; may have fibrous cord, dysphagia, globus sensation
405
Dx thyroglossal duct cyst
neck ultrasound (confirm presence of a thyroid gland; in 1% of cases, the TGDC contains the only functional thyroid tissue)
406
Histopathology of thyroglossal duct cyst
lined with respiratory and squamous epithelium, possible thyroid follicles and colloid
407
Complications of thyroglossal duct cyst
Rare malignant potential, secondary infection
408
Rx for thyroglossal duct cyst
Sistrunk procedure (excision of cyst and tract, including cuff of tongue base and mid-portion of hyoid bone, 3% recurrence; without hyoid resection 20-50% recurrence)
409
Define lingual thyroid
failure of thyroid gland to descend from the foramen cecum; presents as a posterior tongue mass, usually in females; in 75% it is the only functional thyroid tissue; potential malignant transformation
410
SSx of lingual thyroid
dysphagia, dysphonia, stertor; frequently hypofunctioning
411
DDx of lingual thyroid
thyroglossal duct cyst, lymphatic malformation, vallecular cyst, midline tumors (dermoid, teratoma)
412
Dx of lingual thyroid
CT and/or neck ultrasound (identify caudal thyroid tissue), thyroid function tests, consider I-123 or technetium scan
413
Rx of lingual thyroid
May observe if asymptomatic (watch closely for malignancy); ablative radioactive iodine considered in older adults; excision (transoral or transcervical) with possible thyroid tissue transplantation; almost all options require thyroid hormone supplementation or supplementation
414
Pathophysiology of thymic cyst
remnant of third pharyngeal pouch between angle of mandible and midline neck
415
SSx thymic cyst
unilateral (usually left) enck mass
416
Dx of thymic cyst
biopsy, serum calcium (associated parathyroid disorders, DiGeorge syndrome/22q11, CT/MRI)
417
Rx for thymic cyst
excision, may req thoracic surgery consultation
418
Pathophysiology of dermoid cyst and teratoma
Derived from pluripotent embryonal stem cells
419
Types of dermoid cyst and teratoma
1. Teratoma: composed of all 3 embryologic layers
2. Dermoid cyst: ectodermal and mesodermal elements only (MC)
3. Teratomoma: differentiated to organ structure (usually fatal)
4. Epignathi: differentiated to body parts (usually fatal)
420
SSx dermoid cyst and teratoma
Soft midline neck mass, may be associated with tufts of hair
421
Dx of dermoid cyst and teratoma
biopsy
422
Complications of dermoid cyst and teratoma
rare malignant potential
423
Rx for dermoid cyst and teratoma
excision
424
What are the other names for congenital torticollis?
fibromatosis colli, sternocleidomastoid tumor of infancy, pseudotumor of infancy
425
Pathophysiology of congenital torticollis
intrauterine or birth trauma causing muscle injury, hematoma, and resultant fibrosis (typically of the SCM)
426
SSx of congenital torticollis
Presents 2-4 weeks after birth; head and neck held to diseased side, chin toward healthy side; firm thickened mass confined to SCM (may be tender)
427
Dx of congenital torticollis
H&P (if biopsied looks malignant because of muscle cell death and regeneration with necrosis and mitotic figures)
428
Rx for congenital torticollis
physical therapy, observation
429
Lemierre's syndrome
thrombophlebitis of the internal jugular vein following pharyngitis, PTA or suppurative lymphadenitis
Systemic microemboli leading to dyspnea and cough, pleuritic chest pain, pneumonia, arthralgia, endocarditis, meningitis, sepsis
Fusobacterium necrophorum
430
Rx Lemierre's syndrome
I&D; IV Abx for 3-6 weeks
| Anticoagulation controvertial
431
SSx atypical mycobacterial infection
slowly enlarging, nontender, indurated neck mass with progressiev skin discoloration (purplish), skin fixation, and possible fistulization
Common in anterior neck triangle and parotid region; corneal ulceration is the most common H&N manifestation
Does not cause pulmonary involvement but may colonize respiratory tract
Natural history is to suppurate and drain for 3 years then "burn out"
432
Dx atypical mycobacterial infection
culture (req 2-4 weeks for growth), PCR, PPD (weak or negative)
433
Rx atypical mycobacterial infection
triple antibiotic therapy (clarithromycin, rifampin, ethambutol for 6 motnhs)
Full surgicla excision vs I&D curettage
434
What is the most common pediatric H&N malignancy?
lymphoma is most common pediatric malignancy of the H&N (excluding retinoblastoma and CNS malignancy)
435
What are previous names of langerhans cell histiocytosis? What is it?
Histiocytosis X and reticuloendotheliosis
| Noninfectious granulomatous disease
436
Pathophysiology of langerhans cell histiocytosis
Granulomatous disease of unknown etiology, manifests as a proliferation of bone marrow-derived histiocytes
437
Histopathology of langerhans cell histiocytosis
sheets of polygonal histiocytes, Birbeck granules ("zipper pattern), rod-shaped cytoplasmic organells with a central linear density and a striated appearance ("tennis-rackets")
438
What are types of langerans cell histiocytosis?
Eosinophilic granuloma
Hand-Schuller-Christian disease
Letterer-Siwe Disease
Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker syndrome)
439
Define CHARGE syndrome
coloboma, heart disease, atresia, retardation, genital hypoplasia, ear anomalies
CHD7
