General Conditions Flashcards
1
Q
choanal atresia:
- how many occur with other associated conditions
- which condition most commonly
A
- 70% associated with other conditions
- 10-20% a/w CHARGE (CHD7)
2
Q
close clinical mimic of choanal atresia?
A
pyriform aperture stenosis - narrowing caused by bony abnormality of anterior nasal aperture
3
Q
congenital midline nasal masses: example in order of decreasing frequency
A
Dermoids (=60% of all), gliomas and encephaloceles
4
Q
airway obstruction symptoms that are worse when awake vs asleep point to what location of airway?
A
worse when sleeping: pharyngeal airway (enlarged tonsils, adenoids, tongue, midface hypoplasia)
Obstruction worse when awake + exacerbated by exercise = laryngeal, tracheal, or bronchial
5
Q
natural history of laryngomalacia
A
Symptoms usually appear within the first 2 weeks of life (usually by 6 weeks), and can increase in severity for up to 6 months – resolves by 12-18 months
6
Q
what is the second most common congenital laryngeal anomaly cause of stridor?
A
congenital subglottic stenosis
7
Q
DDx of laryngomalacia (give 6)
A
a. Subglottic stenosis
b. Vocal cord paralysis
c. Vascular ring
d. Laryngeal mass (cyst or haemangioma)
e. Subglottic haemangioma
f. Tracheomalacia
8
Q
bilat vs unilateral VC paralysis
A
bilat: VC usually midline so normal cry and can be asymptomatic early on, stridor and obstructive symptoms more common, medial position means aspiration less likely
unilat: weak cry, stridor less common, aspiration more likely
9
Q
which syndrome is most associated with congenital laryngeal webs?
A
22q11.2 deletion (Di George)
10
Q
laryngocoele vs laryngeal saccular cyst
A
laryngocele: from dilatation of laryngeal ventricle. lumen communicates with larynx, so intermittently fills with air causing usually episodic hoarseness and dyspnoea
laryngeal saccule: more common in children. mucous containing cyst, not connected to laryngeal lumen. usually constant resp distress/stridor
11
Q
Most common cause of secondary tracheomalacia?
A
aberrant innominate (or brachiocephalic) artery
12
Q
most common type of symptomatic vascular ring? most common vascular ring anomaly?
A
double aortic arch most common symptomatic one
aberrant right subclavian artery most common overall
13
Q
barium oesophagram: indentation where indicates what kind of abnormality?
A
anterior oesophageal indentation = aberrant pulmonary artery / innominate
posterior = aberrant subclavian
anterior and posterior = complete ring e.g. double aortic arch
14
Q
symptoms of tracheomalacia
A
- Chronic brassy barking or seal-like cough
- Respiratory distress
- Wheezing and/or stridor
- ‘Dying’ spells
15
Q
bronchomalacia:
- common symptom
- common presentation
- common complication
A
- Low-pitched monophonic wheeze - predominantly during expiration, over central airways
- persistent congestion despite no URTI
- PBB
16
Q
which lung agenesis has worse mortality: right or left?
A
right lung agenesis is worse
17
Q
lung agenesis/aplasia: findings on CXR? which imaging modality is diagnostic?
A
CXR: unilateral lung likely with mediastinal shift -> would need to Ix for other cause e.g. FB/mass
CT is diagnostic
18
Q
some examples of intra vs extra thoracic causes of lung hypoplasia
A
intrathoracic: CDH, masses, reduced pulmonary perfusion, CPAM
extrathoracic: oligohydramnios, skeletal dysaplsia causing narrow fetal thorax e.g. achondrogenesis / OI
19
Q
CPAM: what is it?
A
congenital pulmonary airway malformation (previously CCM) = hamartomatous lung tissue formation
20
Q
types of CPAM: incidence, origin, prognosis
A
type 0 = <3% least common, acinar dysplasia, microcystic in whole lung, poor prognosis
type 1 = 60% most common, bronchi/prox bronchioles, macrocystic usually only in one lobe, good prognosis
type 2 = 20%, terminal bronchioles, microcystic, poor prognosis - a/w other abnormalities inc renal agenesis, pulmonary sequestration and cardiac issues
type 3 = <10%, solid +/- cystic large lesions, poor prognosis
type 4 = 10%, acinar formation, can’t distinguish from type 1 radiologically
21
Q
which type of CPAM is a/w cancer?
A
type 4 a/w pleuropulmonary blastoma
22
Q
median age of diagnosis of CPAM
A
at 21 week scan
23
Q
antenatal clinical manifestations of CPAM (name 4)
A
i. Hydrops fetalis
ii. Mediastinal shift
iii. Pleural effusions
iv. Polyhydramnios
24
Q
what is pulmonary sequestration?
A
Aberrant formation of segmental lung tissue that has NO connection with the bronchial tree or pulmonary arteries.
25
classify and differentiate types of pulmonary sequestration
intralobar (~80%): is in a lobe, doesn't have its own pleura, more common in late childhood or adolescence
extralobar (20%): has its own pleura, more common in boys, almost always in left lung, more common in neonates
26
which type of pulmonary sequestration is more likely to be associated with other abnormalities?
50-60% extra-lobar a/w other things e.g. CDH, vertebral anomalies, CPAM / pulmonary hypoplasia
27
what is a bronchogenic cyst?
congenital malformation of bronchial tree (a foregut malformation)
28
most common location of a bronchogenic cyst?
middle mediastinum on right (~60-90%)
29
do bronchogenic cysts commonly communicate with the bronchial tree or not?
not commonly, therefore not usually air filled
30
congenital lobar emphysema: causes?
50% no cause identified
25% from air trapping via ball-valve:
1) intrinsic e.g. bronchial stenosis, intra-luminal e.g. mec granuloma
2) extrinsic e.g. vascular anomalies, intrathoracic masses
3) other e.g. bronchial atresia
31
most common lung site for congenital lobar emphysema?
i. Left upper lobe: most common, 40-45%
ii. Right middle lobe: 30%
iii. Right upper lobe: 20%
iv. May involve more than a single lobe: 5%
v. Much rarer in the lower lobes
32
CXR findings for congenital lobar emphysema
- area of hyperlucency in the lung with a paucity of vessels
- Mass effect with mediastinal shift and hemidiaphragmatic depression
33
congenital pulmonary lymphangectasia - 3) possible pathophysiologies
i. Pulmonary venous obstruction = produces an elevated trans vascular pressure and engorges pulmonary lymphatics
ii. Generalised lymphangiectasia = generalised disease of several organ systems e.g. in Noohna's
iii. Primary lymphangiectasia = limited to the lung
34
cervical lung hernia: causes?
20% congenital from weakness in suprapleural membrane + neck msucles
80% acquired: pulmonary disease with lots of coughing e.g. asthma, cardiothoracic surgery
35
usual presenting complaint of a cervical lung hernia
usually a neck mass noticed while straining or coughing
36
how does congenital lobar pneumonia usually present?
Present in the 1st 2 weeks of life with tachypnoea
37
bronchogenic cysts - when to treat?
Always resect due to risk of infection (primarily), also rare risk of malignancy
38
what is scimitar syndrome?
partial or complete anomalous pulmonary venous drainage of a hypoplastic lung to the inferior vena cava. The term derives from the curvilinear shadow created by the anomalous pulmonary vein on the chest radiograph.
type of pulmonary venolobar syndrome
39
2 pathophysiologies explaining emphysema
1) compensatory hyperinflation (acute/chronic) e.g. pneumonia, empyema
2) obstructive overinflation: results from partial obstruction of a bronchus or bronchiole when it becomes MORE difficult for air to leave the alveoli than to enter
40
what is bronchiolitis obliterans? most common causes in kids?
bronchiolar inflammation with FIBROSIS associated with luminal stenosis and occlusions.
common causes in kids - post-infectious (ADENO), or post transplant
41
What is BO called when it occurs in transplant patients?
BOS - high mortality. 1/3 lung transplants get it.
42
BO vs BOOP
BOOP/ Cryptogenic Organising Pneumonia (COP):
- BO more fixed obstruction with PFTs, BOOP restrictive
- BO CXR hyperinflation/normal, BOOP ground glass interstitial
- BOOP more responsive to steroids
43
which age group is BOOP more common in?
55-60yo
44
common causes of hypersensitivity pneumonia in children
i. Exposure to pet birds (feathers in bedding)
ii. Humidifiers and hot tubs for thermophilic organisms and MAC
iii. Mould from prior flooding or damp condensation
45
histopathological findings of hypersensitivity pneumonia
lung biopsy needed to confirm Dx when critical elements not there:
Poorly formed, non-caseating granulomas
46
BAL findings in HP
Lymphocytosis stimulation by the offending antigen, with low CD4:CD8 ratio (Ie. CD8 higher than normal)
47
Granulomatosis with Polyangiitis (Wegner's)
- what size vessel
- what kind of Ab is associated with it
- small vessel (formally, but also medium)
- ANCA vasculitis (cANCA in 90%)
48
most commonclinical manifestations of Wegner's
ENT - rhinosinusitis + nosebleeds, septal perforation >> saddle nose!
lungs - tracheobronchial stenosis, ILD, alveolar haemorrhage
kidneys - GN
then eyes, heart, joints, rash etc.
49
sarcoidosis in children vs adults
- Skin rash, iridocyclitis, and arthritis seen most often WITHOUT pulmonary symptoms
- pulmonary disease less progressive cf adults
- ocular disease more likely to be progressive cf adults
50
most common lab findings with sarcoidosis
high Ca - produced by macrophages!
high ACE - produced by T cells!
hypergammaglobulinaemia
anaemia of chronic disease
51
sarcoidosis:
- lung BAL/biopsy results
- CXR
- non-caseating granulomas ( or Langhan's giant cells)
- CXR: bilat hilar lymphadenopathies
52
pulmonary sarcoidosis - when to treat?
will resolve 75% without treatment
treat when stage III i.e. when reticular abnormalities predominate and hilar LN already regressing
treat with steroids
53
which of the eosinophilic lung diseases often presents seriously acutely?
AEP - acute eosinophilic pneumonia. often need mechanical ventilation for resp failure
54
what is loeffler syndrome?
transient pulmonary eosinophilia 2nd to helminthic infections. self-limiting usually <1 month
55
name the major pulmonary eosinophilias (mnemonic)
CC DHAAL:
CEP (chronic)
Churg-Strauss
Drugs - NSAIDs, phenytoin, anti-microbials - nitrofurantoin
AEP
allergic bronchopulmonary aspergillosis
hypereosinophilic syndrome
loeffler syndrome
56
AEP vs CEP
AEP idiopathic but more likely to have environmental than CEP (which is entirely idiopathic) e.g. smoke, vape. more acute +++
CEP mostly in adults
57
BAL diagnosis of AEP/CEP
>25% or more of eosinophils
58
EGPA/Churg-strauss:
- which antibody is associated
- common symptoms/systems affected / diagnostic criteria
- one of the 3 ANCA vasculitides - MPO-ANCA (but also can be ANCA neg)
- asthma (resp)
- pulmonary opacities
- mono/polyneuropathies
- >10% eosinophils peripherally
- kdineys - GN
59
ILDs - what do you see on PFT?
restrictive ventilatory deficit
60
primary spontaneous PTX: associated mutation and conditions
1. FLCN (folliculin) gene mutation: familial cases of spontaneous pneumothorax
2. Collagen synthesis defects – Ehlers-Danlos, Marfan syndrome
61
thoracocentesis - where to insert needle?
1. Second intercostal space mid clavicular line on the side of the pneumothorax
2. Insert vertically, above or below the rib
62
pneumomediastinum in kids: suggestive signs
subcut emphysema is diagnostic
mediastinal crunch (hamman sign)
dyspnoea
63
pneumomediastinum in kids: which age group and causes
peak in neonates (spontaneous) and
late infancy/early childhood due to LRTI.
adolescence (spont, thin males)
asthma also causes
64
chylothorax - which side of the lung does it usually occur at?
rarely bilat - usually right
65
what is chylothorax?
= pleural collection of fluid formed by the escape of chyle from the thoracic duct or lymphatics into the thoracic cavity
66
some causes of chylothorax (and which is most common)
a. Thoracic duct injury as a complication of cardiac surgery = most common in children
b. Chest injury
c. ECMO
d. Primary or metastatic intrathoracic malignancy – particularly lymphoma
e. Newborns – during delivery
f. LESS COMMON = lymphangiomatosis, restrictive pulmonary disease, thrombosis of the duct, superior vena cava or subclavian vein, TB or histoplasmosis, congenital anomalies of lymphatic system
67
what feeds do you choose to give in chylothorax, and why?
Enteral feeds with low fat or medium-chain TG, high protein diet, or TPN
- Dietary exclusion of long chain fatty acids – this avoids their conversion into monoglycerides and free fatty acids (FFA) which are transported as chylomicrons to the intestinal lyph ducts
- Medium chain triglycerides (MCTs) can be provided – absorbed directly into intestinal cells and transported directly to the liver via the portal vein – BYPASSING the thoracic duct
68
most common viral cause of bronch
RSV 50%
69
what mab can you give for RSV bronch, and which patient groups would you consider it in?
palivizumab - reduces risk of hospitalisation:
- Preterm infants with or without chronic lung disease of prematurity or congenital heart disease
- Infants with haemodynamically significant congenital heart disease
- Children with anatomic pulmonary abnormalities or neuromuscular disorder
- Immunocompromised children assessed on an individual basis
70
what is the evidence for HFNC in bronch?
HFNC doesn't shorted O2 therapy, but does avoid ICU admissions
71
bronchodilation and constriction is mediated by which receptors and nervous systems?
PNS: bronchoconstriction (M3 receptors)
SNS: bronchodilation (B2 receptors)
72
bronchodilator reversibility is defined as what change of FEV?
improvement of FEV1 of 12% in absolute values
73
transient hypoxemia post salbutamol: explain
-usually in first 30mins after ventolin
- Beta 2 effects also include pulmonary vasodilation, thereby countering the normal protective hypoxic pulmonary vasoconstriction that occurs, thereby leading to increased intrapulmonary shunt and hypoxemia
- need supplemental O2 for a short while
74
options for preventative asthma medication
i. Inhaled corticosteroids
ii. Montelukast
iii. Cromone
iv. Inhaled corticosteorids + LABA
75
examples of inhaled steroids for asthma (and most common)
beclomethasone, budesonide, ciclesonide, fluticasone propionate
76
what is montelukast's MOA, and when would we trial it first in place of ICS?
1. The child is unable to use inhaled therapy
2. The child also has significant allergic rhinitis
3. The parents have strong concerns about adverse effects of ICS
77
how long do we give for a trial of ICS vs montelukast?
ICS: 2-3 months
montelukast: 4 weeks
78
why are cromones (cromoglycate / nedocromil) more complex?
more frequent dosing and meticulous daily care to prevent clogging of inhaler device
79
Why are LABAs never first line in children?
LABA also results in phosphorylation and internalisation of beta 2 receptor – results in increased mortality
80
what age can symbicort be used?
formoterol + salmeterol (symbicort) >/= 6yo
LABAs have no evidence in 5 or under
81
what mabs can be used in asthma? how do they work and how often do they have to be taken?
Omalizumab (anti-IgE)
- Prevents binding of free IgE to high affinity receptors on basophils and mast cells
- Approved for moderate to severe allergic asthma in children 12 years or older
- Delivered by SC every 2-4 weeks
Mepolizumab
- An add-on maintenance therapy for severe asthma with an eosinophilic phenotype in patients age 12 years and older
i- Anti-IL-5 Mab injected SC every 4 weeks
- Decreases the production an survival of eosinophils, a major inflammatory cell involved in asthma pathogenesis
82
classify asthma by frequency of symptoms
infrequent episodic: flares >6weeks apart
frequent episodic: flares <6 weeks apart, but fine in between
persistent: daytime Sx >2x/week. nocturnal once/week.
83
what is plastic bronchitis?
recurrent episodes of airway obstruction secondary to large proteinaceous branching casts – take the shape of and obstruct the tracheobronchial tree
84
plastic bronchitis - associated conditions (4)
a. Lymphangitic disorders
b. Pulmonary infections
c. Acute chest syndrome of sickle cell disease
d. **Congenital cardiac disease – Fontan: occurs in 14%**
85
what is the most important predictor of a specific cause of cough in children
wet vs dry
86
3 most common causes of persistent cough in kids
1. Protracted bacterial bronchitis (most common 0-2 years)
2. Asthma
3. Bronchiectasis
87
chronic cough duration
>4 weeks
88
Suggest some aetiologies with the following types of cough:
i. Onset in infancy (and barking)
ii. Dry (worse at night)
iii. Dry (paroxysmal)
iv. Wet (productive)
v. Onset in older childhood (honking)
i. Onset in infancy (and barking): tracheomalacia
ii. Dry (worse at night): post-viral cough, chronic non-specific cough of childhood, asthma
iii. Dry (paroxysmal) : pertussis
iv. Wet (productive) : suppurative lung disease = chronic suppurative bronchitis, CF, immunodeficiency, primary ciliary dyskinesia, inhaled FB
v. Onset in older childhood (honking): psychogenic
89
Suggest some lung aetiologies with the following types of exam findings with cough:
- dextrocardia
- organomegaly
- digital clubbing
- pectus carinatum
- dextrocardia: PCD
- organomegaly: CF, storage disorders, malignancy, haemaglobinopathy
- digital clubbing: suppurative lung disease
- pectus carinatum: chronic airway obstruction
90
dry staccato cough in infancy =
chlamydia
91
most common causes dry cough in kids (4)
o Asthma
o Allergic rhinitis
o GORD
o TB
92
most common cause of chronic wet cough in kids
PBB
93
3 most common causative organisms of PBB
H. influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis
94
current diagnostic criteria for PBB
■ Wet cough lasting at least 4 weeks
■ Absence of other findings to identify another cause of
the cough
■ Resolution of the cough with at least 2 weeks of an
antibiotic (usually augmentin)
95
classic cxr findings of PBB
bilat peribronchial accentuation or 'cuffing' or 'doughnut' sign
96
what is the narrowest part of the trachea?
subglottis - surrounded by cricoid cartilage
97
inspiratory stridor = ?
expiratory stridor = ?
biphasic stridor = ?
Inspiratory stridor = stridor originating from obstruction in the extrathoracic region
Expiratory stridor = stridor originating from obstruction in the intrathoracic region
Biphasic stridor = fixed (rather than dynamic) central airway obstruction
98
at what age do bronchogenic cysts and laryngeal clefts usually manifest?
infancy-toddler (not neonatal)
99
vascular rings - usually insp/exp stridor?
exp - its intra-thoracic usually
100
Recurrent respiratory papillomatosis (RRP) usually caused by which HPV valents?
6 and 11 (covered by gardasil)
101
Recurrent respiratory papillomatosis (RRP) - age groups affected?
bimodal age distribution and manifests most commonly in children younger than five years (juvenile-onset) or in persons in the fourth decade of life (adult-onset)
102
what does vit d have to do with stridor?!
hypocalcaemia reported to cause laryngeal spasm (e.g. vit d def or rickets)
103
common 3 causative organisms retropharyngeal abscesses
group A Streptococcus
S. aureus
anaerobic organisms, e.g. Bacteroides, Peptostreptococcus, and Fusobacterium
occasionally hib.
103
cxr findings:
- epiglotittis
- croup
- bacterial tracheitis
- retropharyngeal abscess
- epiglotittis - thumb sign
- croup - steeple sign
- bacterial tracheitis - steeple sign (but older kid)
- retropharyngeal abscess - widening of pre-vertebral tissue (but indistinguishable from vertebral asbcess on plain xr)
104
compare bacterial tracheitis to epiglottits
-drooling uncommon in bacterial tracheitis; epiglottitis 3 D's (drooling, dysphagia, distress)
-bacterial tracheitis - poor response to steroids
-epiglottitis classically younger
105
vocal cord dysfunction - more common in which gender
females 3:1
106
some causes of wheeze
1. asthma / bronch
2. FB
3. structural e.g. vascular rings
4. tracheobronchomalacia
5. VC dysfunction
6. mucociliary clearance issue e.g. CF, bronchiectasis
107
what findings might you find in a cxr for FB
- opaque FB only in 20%
- hyperinflation
- atelectasis
- infection
- mediastinal shift
108
aspirated FB - where is it most likely to lodge?
right lung (60%): RMB (52%), middle lobe bronchus <1%, lower lobe bronchus 6%
109
aside from trauma / friable tissue, what are some other causes of epistaxis in children?
- FB, nasal polyps, bleeding diatheses, vascular malformations, and nasopharyngeal tumours
- Hereditary haemorrhagic telangiectasia, varicosities, haemangiomas, bleeding diatheses (particularly vWD), HTN, renal failure
- Juvenile nasal angiofibroma – seen in adolescent males, resulting in recurrent often severe nosebleeds
110
what is the most common childhood cause of nasal polyposis?
50% CF patients have polyps and it is RARE for non-CF children to have it
111
what meds can you give for nasal polyps?
IN steroids
doxycycline, very good for size and symptoms
112
what is the most common cause of chronic hoarseness in kids?
vocal nodules
113
what can cause vocal nodules in kids? how to distinguish between these causes?
a. Chronic vocal abuse or misuse > voice is worse in the evening
b. Laryngopharyngeal reflux > hoarseness worse in the morning
114
association between cutaneous and subglottic haemangiomas
1% of children with cutaneous haemangioma have subglottic haemangioma
50% of those with subglottic haemangioma will have cutaneous haemangioma
115
what kind of cutaneous haemangiomas are associated with airway ones?
those in beard distribution: including pre-auricular skin, chin, anterior neck and/or lower lip
116
what syndrome do you need to think about when there are large plaque-type haemangiomas?
up to 1/3 will have PHACE:
posterior fossa malformations
haemangiomas
arterial anomalies
cardiac anomalies
eye abnormalities and endocrine
sternal cleft
117
DDx for anterior mediastinal mass (mnemonic)
4Ts:
• Thymus – thymic cyst
• Teratoma – germ cell
• Thyroid
• Terrible lymphoma (T lymphoma, T ALL)
118
pulmonary haemosiderosis - how does it happen, and common presenting issues?
- recurrent episodes of DAH (diffuse alveolar haemorrhage)
- IDA, haemoptysis, cxr alveolar infiltrates
119
where in the lungs is atelectasis most common in children?
In 90% of children involves upper lobe; 63% involves right upper lobe
120
what can cause pectus excavatum?
a. Associated with CT disorder (Marfan, Ehler’s Danlos)
b. Acquired secondarily to chronic lung disease, neuromuscular disease, or trauma
121
Two conditions associated with pectus CARInatum
a. Mitral valve disease
b. Coarctation of aorta
122
what are some pregnancy effects of maternal smoking?
• Spontaneous miscarriage
• Placental abruption
• PPROM
• Placenta praevia
• Pre-term delivery
• Low birth weight
• Ectopic pregnancy
123
what are some post-natal effects of maternal smoking?
• SIDS
• Respiratory infections – bronchitis, pneumonia
• Asthma, atopy, otitis media
• Infantile colic
• Bronchiolitis
• Short stature
• Lower reading and spelling scores, reduced school performance
• Shorter attention spans, hyperactivity
• Childhood obesity
124
differentiate between severe and late onset CCHS
i. Severe CHHS = hypoventilation manifests during wakefulness sand sleep
ii. Late onset CCHS (LO-CCHS) = symptoms manifest after 1 month of age but hypoventilation is typically during sleep only
125
gene most associated with congenital hypoventilation syndromes, and:
- most common mutation in that gene
- de novo/inherited?
paired-like homeobox 2b (PHOX2B)
Polyalanine repeat expansion mutation (PARM) in 90%
most de novo, 25% inherited
126
CHS with ANSD - two most common associated conditions
a. Hirschsprung’s disease – 20%
b. Neural crest cell origin tumours
127
what is ROHHAD?
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. Starting after 1.5 years of age, and also hypothalamic dysfunction and absence of a CCHS-related PHOX2B mutation
128
nocturnal hypoventilation occurs in REM/non-REM for A) neuromuscular disorders B) CCHS
neuromuscular - in REM
CCHS - in non-REM
129
with what FVC in NMD is NIV indicated?
BIPAP / CPAP – indicated when FVC< 50% predicted
