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
2
Q
Treatment for capillary hemangiomas
A
Propranolol during proliferative phase
3
Q
What type of capillary malformation disappears?
A
Medial (salmon patch, angel kiss )
lightens with time
Lateral (Port wine stain) persists
4
Q
What is the natural progression of venous malformations?
A
Grows slowly in childhood, then expands rapidly during puberty
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
6
Q
What is the DDx of nasal obstruction in the newborn?
A
Rhinitis Dacrocystocele Pyriform aperture stenosis Choanal atresia Tumors
7
Q
What is the most common type of branchial cleft cyst?
A
Second branchial cleft cyst
8
Q
Where does the first branchial cleft cyst appear?
A
Angle of mandible (type 2; more common) Postauricular crease (type 1)
9
Q
Where is a second branchial cleft cyst found?
A
Externally: anterior border of SCM
Internally: tonsillar fossa
10
Q
How is microtia managed?
A
Early BAHA if bilateral
Surgery at age 5–8
Imaging if cholesteatoma suspected
11
Q
What forms the inferior turbinate?
A
Maxilloturbinal
12
Q
Symptoms of DiGeorge Syndrome
A
CATCH-22
Cardiac abnormalities (truncus arteriosus, tetralogy) Abnormal facies Thymic aplasia Cleft palate Hypoparathyroidism/Hypocalcemia 22q11 deletion
13
Q
What condition must be considered if nasal polyps are found in a child?
A
Cystic fibrosis
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
15
Q
What is the most common pattern of velopharyngeal closure?
A
Coronal
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
17
Q
Steeple sign
A
Croup
laryngotracheitis
18
Q
Treatment for croup
A
Humidification
Rac epi
Plus/minus steroids
19
Q
Thumb print sign
A
Epiglottitis
20
Q
Treatment for epiglottitis
A
Low threshold for intubation, Have trach kit ready
IV antibiotics
21
Q
What upper airway infections require possible intubation
A
Epiglottitis
Bacterial tracheitis (superinfection after croup)
Retropharyngeal abscess
22
Q
Major blood supply of the adenoids
A
Pharyngeal branch of the internal maxillary
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
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
25
What patient should be admitted overnight after the tonsillectomy?
Severe OSA
Emesis or hemorrhage
Age less than three years
Patient lives greater than one hour away
26
How does PANDAS present
Obsessive compulsive disorder symptoms after a group a strep infection
27
Complications of adenotonsillectomy
Hemorrhage
Pulmonary edema (loss of obstruction & auto-PEEP)
Hypoxemia
VPI
Nasopharyngeal stenosis
Atlantoaxial subluxation (Grisel's syndrome)
Halitosis
28
What is the most common neck mass in a child?
Inflammatory adenitis
29
What is the most common salivary gland mass in a child?
Hemangioma
30
What is the most common salivary gland neoplasm in a child?
Pleomorphic adenoma
31
What is the most common salivary gland malignancy in a child?
Mucoepidermoid carcinoma
32
What are the small blue sound malignancies in children?
Lymphoma
Sarcoma
Rhabdomyosarcoma
33
Age-related subtypes of rhabdomyosarcoma
Embryonal - infants/children
Alveolar - adolescents
Pleomorphic - adults
34
Treatment for thyroglossal duct cyst
1) ultrasound for normal thyroid
| 2) Sistrunk procedure
35
Differential for pediatric BOT mass
Lingual thyroid
Thyroglossal duct cyst
Vallecular cyst
36
What syndrome is associated with endolymphatic sac tumors?
von Hippel Lindau
37
What is seen in Pierre-Robin sequence?
Retrognathia
Glossoptosis
Cleft palate
38
What is the order of airway interventions for Pierre-Robin?
Prone positioning
Nasopharyngeal airway
Intubation
Surgery
39
What mutation causes achondroplasia?
FGFR-3 mutation
| Autosomal dominant
40
What is included in VACTERL syndrome?
```
Vertebral/vascular anomalies
Anal atresia
Cardiac anomalies
Tracheal anomalies
Esophageal anomalies
Renal/radial bone anomalies
Limb anomalies
```
41
What are the autosomal dominant ENT conditions?
```
WANT-BCS
Waardenburg
Apert
Neurofibromatosis
Treacher-Collins
Branchio-oto-renal
Crouzon
Stickler
```
42
Findings in Waardenburg syndrome
```
Pigmentation abnormalities (white forelock, heterochromia iridum)
Dystopia canthorum (wide nasal bridge)
Unilateral or bilateral SNHL
```
43
Findings in Apert and Crouzon syndromes
```
Large forehead and jaw
Exophthalmos
Parrot-beaked nose
Syndactyly and cervical fusion
May have mental retardation
```
44
Where are the mutations for neurofibromatosis?
Type I: chromosome 17
| Type II: chromosome 22
45
Features of type one neurofibromatosis
```
Cafe au lait spots
Lisch nodules
Cutaneous neurofibromas
Acoustic neuromas (5%)
Possible CNS involvement
```
46
What is the only FDA-approved indication for auditory brainstem implant?
Neurofibromatosis type II
47
Features of mandibulofacial dysostosis
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
Features of Stickler syndrome
Myopia, Retinal detachment, Cataracts
Hypermobile joints, Arthritis
SNHL or mixed HL (80%)
49
What are the autosomal recessive syndromes with ENT symptoms?
```
PUG-J
Pendred
Usher
Goldenhar
Jervell-Lange-Nielson
```
50
What is seen with Pendred syndrome?
```
SNHL - Mondini's aplasia or EVA syndrome
Euthyroid goiter (iodine metabolism defect)
```
51
What is seen with Usher syndrome?
Hearing loss
Ataxia
Retinitis pigmentosa (progressive vision loss)
52
What are the subtypes of Usher syndrome?
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
What is seen with Goldenhar syndrome?
Hemifacial microsomia
| Microtia, EAC atresia, ossicular abn
54
What is seen in Jervell-Lange-Nielsen syndrome?
Profound b/l SNHL
| Stokes-Adams attacks (arrhythmia & syncope)
55
Cause of Alport syndrome
Defect in Type IV collagen
56
What is seen in Alport syndrome?
SNHL and glomerulonephritis
57
What are the X-linked recessive syndromes associated with SNHL?
Alport
Norrie
Otopalatodigital
Wildervaank
58
What is seen in Norrie syndrome?
Blindness
Early-onset SNHL
+/- mental retardation
59
What is seen in otopalatodigital syndrome?
```
Conductive HL (malformed ossicles)
Craniofacial anomalies
Widely spaced 1st & 2nd toes
```
60
What is seen in Wildervaank syndrome?
Klippel-Feil malformation (fused c-spine)
Sensorineural or mixed HL
CNVI paralysis
61
What is seen in velocardiofacial syndrome?
```
Abnormal facies
VPI
Cleft palate
VSD
Medial ICA's
```
62
What is associated with congenital pyriform aperture stenosis?
Central megaincisor
| Cerebral malformations-get MRI
63
Choanal atresia:
Male or female?
Mixed or bony?
Unilateral or bilateral?
Female (2:1)
Mixed (70%)
Unilateral (70%)
64
When to repair choanal atresia
Unilateral: wait until one year old
Bilateral: temporary airway and correct early
65
What syndromes are associated with choanal atresia?
ACT TV
```
Apert
Crouzon/CHARGE
Treacher Collins
Edward
Velocardiofacial
```
66
What is seen with CHARGE syndrome?
```
Coloboma
Hearing defects
Atresia (choanal)
Retardation of growth
Genital defects (males)
Endocardial cushion defect
```
67
What causes cleft lip?
Failure of fusion of the maxillary swelling with the medial nasal process
68
What causes cleft palate?
Failure of bilateral Palatine shelves to fuse at midline with developing nasal septum
69
Signs of a possible submucous cleft palate
Bifid uvula
Zona pellucida
Notched hard palate
70
Cleft lip:
Males or females?
Unilateral or bilateral?
Males (2/3)
| Unilateral (80%; more commonly left)
71
When should you repair a cleft lip?
Rule of 10's:
10 weeks old
10 kg weight
Hemoglobin of 10
72
When should you repair a cleft palate?
9-12 months
73
ETT size in kids
(Age/4)+4 then subtract 1/2 size
74
What is the max pressure that a cuff can be inflated to?
20mmHg
Any higher will exceed capillary filling pressure
75
Where in relation to the spine is the larynx in an infant vs. an adult?
Infant: C2
Adult: C6
76
Where is the lesion if biphasic stridor is heard?
Subglottic
77
What are the characteristics of stridor for laryngomalacia?
Inspiratory stridor
| Worse while supine
78
Treatment for laryngomalacia
```
Medical:
-GERD control
Surgical:
-AE fold division
-Epiglottic adhesion
-Resection of arytenoid redundancy
-Trach
```
79
Branchial arch derivatives of the parathyroids
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
Which parathyroids are more constant in location?
Superior parathyroids
They are generally located more posterior and medial than the inferior parathyroids (on dorsal thyroid)
81
Types of laryngeal clefts
Type I: Supraglottic
Type II: Subglottic, partial cricoid
Type III: Total cricoid
Type IV: Laryngotracheoesophageal cleft
82
When should a child receive surgery for vocal cord paralysis?
If airway compromised - urgently
| If no airway compromise - 5-7y (may have spontaneous recovery)
83
What causes RRP?
Recurrent Respiratory Papillomatosis
| Caused by HPV 6, 11
84
What are the types of RRP?
JORRP <5y (maternal trans; more aggressive)
| AORRP ~30y (sexual trans)
85
Treatment for RRP
Surgical debulking
Laser
Interferon, Cidofovir
86
What is the danger of RRP?
Respiratory insufficiency
| Degeneration to SCCa (3-5%)
87
What is the grading system for subglottic stenosis?
Cotton-Meyer
I: <50%
II: 50-70%
III: 70-99%
IV: No lumen
88
Most common types of TEF
1) Proximal EA, distal TEF (85%)
2) Isolated EA
3) H-type
89
What type of necrosis is associated with caustic ingestions?
Acidic - coagulative necrosis (superficial)
| Alkaline - liquefactive necrosis (deep)
90
When should esophagoscopy be performed after a caustic ingestion?
12-24h
91
How are caustic ingestions graded?
Grade 1: mucosal erythema
Grade 2: non-circumferential exudates
Grade 3: Circumferential exudates
Grade 4: Circumferential exudates with perforation
92
Management of caustic ingestions
Grade 1-2: Observation, consider steroids
| Grade 3-4: Surgery, consider esophagectomy
93
Where do foreign bodies get stuck in the esophagus?
Cricopharyngeus
Aortic arch
Left mainstem bronchus
LES
94
What is the minimum workup for congenital SNHL?
EKG
Ophthalmologic evaluation
MRI or CT
95
Most common bugs seen in otitis externa
Pseudomonas, Then Staph aureus
96
Indications for acute myringotomy
AOM with:
- A toxic child
- Unresponsive to antibiotics
- Suppurative complications
- Facial paralysis
- Intracranial complication/Meningitis
97
What are the chronic indications for tympanostomy tube placement?
Recurrent AOM (3 in six months or 4 in a year)
Bilateral OME >3mo, unilateral OME >6mo
Speech/language delay
Severe retraction pocket
98
What type of auditory testing is used on Young children?
Behavioral observational audiometry: 0-6mo
Visual reinforcement audiometry: 6mo-2y
Conditioned play audiometry: 2-5y
99
What are the common single gene mutations seen in congenital SNHL?
Pendred
| Connexin 26 & 30
100
What are the criteria for cochlear implantation?
1y of age
Bilateral severe-to-profound hearing loss
Limited benefit from hearing aids
Family commitment and educational plan
101
Contraindications to cochlear implantation
Narrow IAC
Cochlear nerve aplasia
Michel aplasia
102
What are the various forms of membranous labyrinth dysplasia?
Complete membranous labyrinthine dysplasia (Siebenmann-Bing)
Limited membranous labyrinthine dysplasia:
1. Cochleosaccular dysplasia (Scheibe; most common)
2. Cochlear basal turn dysplasia (Alexander)
103
Which SCC is most commonly affected by developmental anomalies?
Lateral SCC
It develops last
104
What diameter is considered EVA?
>1.5mm
105
What do the waves on ABR represent?
```
EECOLII
I & II: Eighth nerve
III: Cochlear nucleus
IV: Olivary complex (superior)
V: Lateral lemniscus
VI & VII: Inferior colliculus
```
106
Factors predictive of needing to go to the OR for RPA
WBC > 20k
| Age < 15mo
107
What tests should all patients with Down syndrome undergo?
Polysomnography (by age 4)
Hearing tests every six months initially
~ 60% of these patients have OSA
108
What is the first step for an infant in respiratory distress?
Nasopharyngeal airway placement
109
Where is the most common site for a congenital laryngeal web?
Anterior commisure
110
What side is more common for cleft lip?
Left
111
What side is more common for microtia?
Right
112
What week does the pinna begin to develop?
6th
113
When is canal atresia repaired in microtia?
Medpor: Before or during
Rib: After
