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ENT > Pediatrics > Flashcards

Pediatrics Flashcards

1
Q

What are the features of PHACES Syndrome?

A 
Posterior cranial fossa anomalies
Hemangioma (facial segmental)
Arterial/carotid anomalies
Cardiac anomalies/Coarctation
Eye anomalies
Sternal pit
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2
Q

Treatment for capillary hemangiomas

A

Propranolol during proliferative phase

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3
Q

What type of capillary malformation disappears?

A

Medial (salmon patch, angel kiss )
lightens with time

Lateral (Port wine stain) persists

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4
Q

What is the natural progression of venous malformations?

A

Grows slowly in childhood, then expands rapidly during puberty

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5
Q

What is cyclical cyanosis in a newborn indicative of?

How can this be confirmed?

A

Nasal obstruction

Confirm with imaging or try to pass six French catheter

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6
Q

What is the DDx of nasal obstruction in the newborn?

A 
Rhinitis
Dacrocystocele
Pyriform aperture stenosis
Choanal atresia
Tumors
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7
Q

What is the most common type of branchial cleft cyst?

A

Second branchial cleft cyst

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8
Q

Where does the first branchial cleft cyst appear?

A 
Angle of mandible (type 2; more common)
Postauricular crease (type 1)
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9
Q

Where is a second branchial cleft cyst found?

A

Externally: anterior border of SCM
Internally: tonsillar fossa

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10
Q

How is microtia managed?

A

Early BAHA if bilateral
Surgery at age 5–8
Imaging if cholesteatoma suspected

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11
Q

What forms the inferior turbinate?

A

Maxilloturbinal

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12
Q

Symptoms of DiGeorge Syndrome

A

CATCH-22

Cardiac abnormalities (truncus arteriosus, tetralogy)
Abnormal facies
Thymic aplasia
Cleft palate
Hypoparathyroidism/Hypocalcemia
22q11 deletion
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13
Q

What condition must be considered if nasal polyps are found in a child?

A

Cystic fibrosis

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14
Q

What are the Chandler classifications Of orbital cellulitis?

A 
I: Periorbital cellulitis (preseptal)
II: Orbital cellulitis
III: Superiosteal abscess
IV: Orbital abscess
V: Cavernous sinus thrombosis
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15
Q

What is the most common pattern of velopharyngeal closure?

A

Coronal

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16
Q

In VPI, when is pharyngoplasty versus pharyngeal flaps useful?

A

Pharyngoplasty: good AP motion, poor lateral motion

Pharyngeal flaps: good lateral motion, poor AP motion

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17
Q

Steeple sign

A

Croup

laryngotracheitis

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18
Q

Treatment for croup

A

Humidification
Rac epi
Plus/minus steroids

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19
Q

Thumb print sign

A

Epiglottitis

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20
Q

Treatment for epiglottitis

A

Low threshold for intubation, Have trach kit ready

IV antibiotics

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21
Q

What upper airway infections require possible intubation

A

Epiglottitis
Bacterial tracheitis (superinfection after croup)
Retropharyngeal abscess

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22
Q

Major blood supply of the adenoids

A

Pharyngeal branch of the internal maxillary

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23
Q

What are the minor blood supplies to the adenoids?

A

Ascending Palatine branch of the facial artery
Ascending cervical branch of the thyrocervical trunk
Ascending pharyngeal artery

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24
Q

What is the blood supply to the tonsils?

A

Facial artery (tonsillar branch, ascending Palatine branch)
Dorsal lingual branch of lingual artery
Maxillary (descending Palatine, Greater Palatine)
Ascending pharyngeal artery

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25
Q

What patient should be admitted overnight after the tonsillectomy?

A

Severe OSA
Emesis or hemorrhage
Age less than three years
Patient lives greater than one hour away

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26
Q

How does PANDAS present

A

Obsessive compulsive disorder symptoms after a group a strep infection

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27
Q

Complications of adenotonsillectomy

A

Hemorrhage
Pulmonary edema (loss of obstruction & auto-PEEP)
Hypoxemia
VPI
Nasopharyngeal stenosis
Atlantoaxial subluxation (Grisel’s syndrome)
Halitosis

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28
Q

What is the most common neck mass in a child?

A

Inflammatory adenitis

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29
Q

What is the most common salivary gland mass in a child?

A

Hemangioma

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30
Q

What is the most common salivary gland neoplasm in a child?

A

Pleomorphic adenoma

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31
Q

What is the most common salivary gland malignancy in a child?

A

Mucoepidermoid carcinoma

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32
Q

What are the small blue sound malignancies in children?

A

Lymphoma
Sarcoma
Rhabdomyosarcoma

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33
Q

Age-related subtypes of rhabdomyosarcoma

A

Embryonal - infants/children
Alveolar - adolescents
Pleomorphic - adults

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34
Q

Treatment for thyroglossal duct cyst

A

1) ultrasound for normal thyroid

2) Sistrunk procedure

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35
Q

Differential for pediatric BOT mass

A

Lingual thyroid
Thyroglossal duct cyst
Vallecular cyst

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36
Q

What syndrome is associated with endolymphatic sac tumors?

A

von Hippel Lindau

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37
Q

What is seen in Pierre-Robin sequence?

A

Retrognathia
Glossoptosis
Cleft palate

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38
Q

What is the order of airway interventions for Pierre-Robin?

A

Prone positioning
Nasopharyngeal airway
Intubation
Surgery

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39
Q

What mutation causes achondroplasia?

A

FGFR-3 mutation

Autosomal dominant

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40
Q

What is included in VACTERL syndrome?

A 
Vertebral/vascular anomalies
Anal atresia
Cardiac anomalies
Tracheal anomalies
Esophageal anomalies
Renal/radial bone anomalies
Limb anomalies
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41
Q

What are the autosomal dominant ENT conditions?

A 
WANT-BCS
Waardenburg
Apert
Neurofibromatosis
Treacher-Collins
Branchio-oto-renal
Crouzon
Stickler
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42
Q

Findings in Waardenburg syndrome

A 
Pigmentation abnormalities (white forelock, heterochromia iridum)
Dystopia canthorum (wide nasal bridge)
Unilateral or bilateral SNHL
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43
Q

Findings in Apert and Crouzon syndromes

A 
Large forehead and jaw
Exophthalmos
Parrot-beaked nose
Syndactyly and cervical fusion
May have mental retardation
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44
Q

Where are the mutations for neurofibromatosis?

A

Type I: chromosome 17

Type II: chromosome 22

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45
Q

Features of type one neurofibromatosis

A 
Cafe au lait spots
Lisch nodules
Cutaneous neurofibromas
Acoustic neuromas (5%)
Possible CNS involvement
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46
Q

What is the only FDA-approved indication for auditory brainstem implant?

A

Neurofibromatosis type II

47
Q

Features of mandibulofacial dysostosis

A

aka Treacher-Collins
Malformation of first and second branchial arches
Malformed ossicles; CHL in 30%
Auricular deformity, aural atresia, preauricular pits
Mandibular and malar hypoplasia
+/- cleft lip and palate

48
Q

Features of Stickler syndrome

A

Myopia, Retinal detachment, Cataracts
Hypermobile joints, Arthritis
SNHL or mixed HL (80%)

49
Q

What are the autosomal recessive syndromes with ENT symptoms?

A 
PUG-J
Pendred
Usher
Goldenhar
Jervell-Lange-Nielson
50
Q

What is seen with Pendred syndrome?

A 
SNHL - Mondini's aplasia or EVA syndrome
Euthyroid goiter (iodine metabolism defect)
51
Q

What is seen with Usher syndrome?

A

Hearing loss
Ataxia
Retinitis pigmentosa (progressive vision loss)

52
Q

What are the subtypes of Usher syndrome?

A

Type I: (90%) profound deafness, RP by 10y, no vestibular response
Type II & III are less and less severe
Type IV is X-linked

53
Q

What is seen with Goldenhar syndrome?

A

Hemifacial microsomia

Microtia, EAC atresia, ossicular abn

54
Q

What is seen in Jervell-Lange-Nielsen syndrome?

A

Profound b/l SNHL

Stokes-Adams attacks (arrhythmia & syncope)

55
Q

Cause of Alport syndrome

A

Defect in Type IV collagen

56
Q

What is seen in Alport syndrome?

A

SNHL and glomerulonephritis

57
Q

What are the X-linked recessive syndromes associated with SNHL?

A

Alport
Norrie
Otopalatodigital
Wildervaank

58
Q

What is seen in Norrie syndrome?

A

Blindness
Early-onset SNHL
+/- mental retardation

59
Q

What is seen in otopalatodigital syndrome?

A 
Conductive HL (malformed ossicles)
Craniofacial anomalies
Widely spaced 1st & 2nd toes
60
Q

What is seen in Wildervaank syndrome?

A

Klippel-Feil malformation (fused c-spine)
Sensorineural or mixed HL
CNVI paralysis

61
Q

What is seen in velocardiofacial syndrome?

A 
Abnormal facies
VPI
Cleft palate
VSD
Medial ICA's
62
Q

What is associated with congenital pyriform aperture stenosis?

A

Central megaincisor

Cerebral malformations-get MRI

63
Q

Choanal atresia:
Male or female?
Mixed or bony?
Unilateral or bilateral?

A

Female (2:1)
Mixed (70%)
Unilateral (70%)

64
Q

When to repair choanal atresia

A

Unilateral: wait until one year old
Bilateral: temporary airway and correct early

65
Q

What syndromes are associated with choanal atresia?

A

ACT TV

Apert
Crouzon/CHARGE
Treacher Collins
Edward
Velocardiofacial
66
Q

What is seen with CHARGE syndrome?

A 
Coloboma
Hearing defects
Atresia (choanal)
Retardation of growth
Genital defects (males)
Endocardial cushion defect
67
Q

What causes cleft lip?

A

Failure of fusion of the maxillary swelling with the medial nasal process

68
Q

What causes cleft palate?

A

Failure of bilateral Palatine shelves to fuse at midline with developing nasal septum

69
Q

Signs of a possible submucous cleft palate

A

Bifid uvula
Zona pellucida
Notched hard palate

70
Q

Cleft lip:
Males or females?
Unilateral or bilateral?

A

Males (2/3)

Unilateral (80%; more commonly left)

71
Q

When should you repair a cleft lip?

A

Rule of 10’s:
10 weeks old
10 kg weight
Hemoglobin of 10

72
Q

When should you repair a cleft palate?

A

9-12 months

73
Q

ETT size in kids

A

(Age/4)+4 then subtract 1/2 size

74
Q

What is the max pressure that a cuff can be inflated to?

A

20mmHg

Any higher will exceed capillary filling pressure

75
Q

Where in relation to the spine is the larynx in an infant vs. an adult?

A

Infant: C2
Adult: C6

76
Q

Where is the lesion if biphasic stridor is heard?

A

Subglottic

77
Q

What are the characteristics of stridor for laryngomalacia?

A

Inspiratory stridor

Worse while supine

78
Q

Treatment for laryngomalacia

A 
Medical:
-GERD control
Surgical:
-AE fold division
-Epiglottic adhesion
-Resection of arytenoid redundancy
-Trach
79
Q

Branchial arch derivatives of the parathyroids

A

Superior: 4th
Inferior: 3rd

Inferior migrate upward, so may be in thymus
Superior migrate down, so may be in TE groove or carotid sheath

80
Q

Which parathyroids are more constant in location?

A

Superior parathyroids

They are generally located more posterior and medial than the inferior parathyroids (on dorsal thyroid)

81
Q

Types of laryngeal clefts

A

Type I: Supraglottic
Type II: Subglottic, partial cricoid
Type III: Total cricoid
Type IV: Laryngotracheoesophageal cleft

82
Q

When should a child receive surgery for vocal cord paralysis?

A

If airway compromised - urgently

If no airway compromise - 5-7y (may have spontaneous recovery)

83
Q

What causes RRP?

A

Recurrent Respiratory Papillomatosis

Caused by HPV 6, 11

84
Q

What are the types of RRP?

A

JORRP <5y (maternal trans; more aggressive)

AORRP ~30y (sexual trans)

85
Q

Treatment for RRP

A

Surgical debulking
Laser
Interferon, Cidofovir

86
Q

What is the danger of RRP?

A

Respiratory insufficiency

Degeneration to SCCa (3-5%)

87
Q

What is the grading system for subglottic stenosis?

A

Cotton-Meyer

I: <50%
II: 50-70%
III: 70-99%
IV: No lumen

88
Q

Most common types of TEF

A

1) Proximal EA, distal TEF (85%)
2) Isolated EA
3) H-type

89
Q

What type of necrosis is associated with caustic ingestions?

A

Acidic - coagulative necrosis (superficial)

Alkaline - liquefactive necrosis (deep)

90
Q

When should esophagoscopy be performed after a caustic ingestion?

91
Q

How are caustic ingestions graded?

A

Grade 1: mucosal erythema
Grade 2: non-circumferential exudates
Grade 3: Circumferential exudates
Grade 4: Circumferential exudates with perforation

92
Q

Management of caustic ingestions

A

Grade 1-2: Observation, consider steroids

Grade 3-4: Surgery, consider esophagectomy

93
Q

Where do foreign bodies get stuck in the esophagus?

A

Cricopharyngeus
Aortic arch
Left mainstem bronchus
LES

94
Q

What is the minimum workup for congenital SNHL?

A

EKG
Ophthalmologic evaluation
MRI or CT

95
Q

Most common bugs seen in otitis externa

A

Pseudomonas, Then Staph aureus

96
Q

Indications for acute myringotomy

A

AOM with:

  • A toxic child
  • Unresponsive to antibiotics
  • Suppurative complications
  • Facial paralysis
  • Intracranial complication/Meningitis
97
Q

What are the chronic indications for tympanostomy tube placement?

A

Recurrent AOM (3 in six months or 4 in a year)
Bilateral OME >3mo, unilateral OME >6mo
Speech/language delay
Severe retraction pocket

98
Q

What type of auditory testing is used on Young children?

A

Behavioral observational audiometry: 0-6mo
Visual reinforcement audiometry: 6mo-2y
Conditioned play audiometry: 2-5y

99
Q

What are the common single gene mutations seen in congenital SNHL?

A

Pendred

Connexin 26 & 30

100
Q

What are the criteria for cochlear implantation?

A

1y of age
Bilateral severe-to-profound hearing loss
Limited benefit from hearing aids
Family commitment and educational plan

101
Q

Contraindications to cochlear implantation

A

Narrow IAC
Cochlear nerve aplasia
Michel aplasia

102
Q

What are the various forms of membranous labyrinth dysplasia?

A

Complete membranous labyrinthine dysplasia (Siebenmann-Bing)

Limited membranous labyrinthine dysplasia:

  1. Cochleosaccular dysplasia (Scheibe; most common)
  2. Cochlear basal turn dysplasia (Alexander)
103
Q

Which SCC is most commonly affected by developmental anomalies?

A

Lateral SCC

It develops last

104
Q

What diameter is considered EVA?

105
Q

What do the waves on ABR represent?

A 
EECOLII
I &amp; II: Eighth nerve
III: Cochlear nucleus
IV: Olivary complex (superior)
V: Lateral lemniscus
VI &amp; VII: Inferior colliculus
106
Q

Factors predictive of needing to go to the OR for RPA

A

WBC > 20k

Age < 15mo

107
Q

What tests should all patients with Down syndrome undergo?

A

Polysomnography (by age 4)
Hearing tests every six months initially

~ 60% of these patients have OSA

108
Q

What is the first step for an infant in respiratory distress?

A

Nasopharyngeal airway placement

109
Q

Where is the most common site for a congenital laryngeal web?

A

Anterior commisure

110
Q

What side is more common for cleft lip?

111
Q

What side is more common for microtia?

112
Q

What week does the pinna begin to develop?

113
Q

When is canal atresia repaired in microtia?

A

Medpor: Before or during
Rib: After

ENT (10 decks)

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