Transcript Respiratory conditions

Respiratory conditions
Anne Aspin
2010
Embryology
 Atresia
of oesophagus with fistula
 Atresia of trachea with fistula
 Laryngo-tracheo-oesophageal clefts
 System
of folds, blocked pathway
 Adriamycin
(rat research)
 Defects
caused by improper development
of the pleuro-peritoneal cavity
 Failure
of muscularisation of the
lumbocostal and pleuro-peritoneal canal,
weak part of diaphragm.
 Pushing
of intestine through foramen of
Bochdalek of diaphragm.
 Premature
return of intestine to abdo
cavity but canal still open
 Abnormal
persistance of lung in pleuro
peritoneal cavity, preventing closure of
cavity
 Abnormal
development of early lung.
 Of
these theories failure of the pleuroperitoneal membrane to meet the
transverse septum is likely explanation for
diaphragm herniation
 Lack
of embryological evidence
 Day 13,(L) Day 14 (R), disturbed
development (rats) = 4-5/52 embryos.
Lung hypoplasia
 From
day 14 of deformation lung
hypoplasia caused by liver growing
through diaphragmatic defect into thoracic
cavity.
 Liver
grows at a faster rate than the lungs.
Head and Neck Examination
Respirations – 30 – 60 bpm
 Abnormal < 30, > 60 bpm, nasal
flaring,intercostal recesssion


Apnoea, anoxia, alkalosis
 Slow, weak, rapid signifies brain damage
 Tachypnoea, congenital heart disease, resp
disease.
 Asymmetry, phrenic nerve palsy, CDH,
atelectasis
Examination of the nose
 Broad
flat, chromosomal abnormality
 Patency, choanal atresia, tumour
 Sneezing
 Bloody discharge, syphilis
Examination of the mouth and
throat
 Excessive
saliva
 Abnormal structures, cleft lip and palate,
micrognathia, large tongue, absent or
unequal reflexes, prematurity or CNS
anomaly
 Distended neck veins indicate chest or
pneumomediastinal mass.
Oesophageal atresia
 Bubbly
secretions
 Apnoea
 Cyanosis
 Immediate vomiting on feeding
 Unable to pass ng tube
 Replogle tube, continual pharyngeal
suction
Types of oesophageal atresia
and fistula
86%
7%
4%
Types continued
1%
<
<
1
1
Lungs and Thorax
 Crackles
and rhonchi present first four
hours after birth.
 Abnormal: decreased abdominal breathing
 Thoracic and asymmetrical breathing –
phrenic nerve damage, CDH,
 Hyperresonance may indicate
pneumomediastinum, pneumothorax, CDH
Thickened epiglottis
Oedematous narrowed sub
epiglottic trachea
Tracheobronchogram
Collapse of right main bronchus
Indications for bronchoscopy
Stridor
Unexplained wheeze
Unexplained or persistent cough
Haemoptysis
Suspected foreign body
Suspected airway trauma,
chemical, or thermal injury
Suspected tracheobronchial
fistula
Suspected tracheobronchial
stenosis
Radiological abnormalities
Persistent or recurrent
consolidation or atelectasis
Recurrent or persistent infiltrates
Lung lesions of unknown
aetiology
Immunosupressed patients
Identify cause of pneumonia
Recurrence of disease
Cystic fibrosis
Identify cause of infection
Intensive care
Examine for the position, patency,
or damage related to
endotracheal or tracheostomy
tubes
Facilitation of endotracheal
intubation
Endobronchial stent placement
Bronchoscopy
 Early
dates, removal of foreign bodies
 Rigid
bronchoscope (telescope fits down),
complete control of airway, ventilation
 Flexible
bronchoscope (bundles of optical
fibres, light to the tips), children from 3yrs
Complications of bronchoscopy
 Pneumothorax
8%
 Incidence reduced if bronchoscope
avoiding right middle lobe
 Haemorrhage
following biopsy
 Pyrexia, dyspnoea
Choanal atresia
 Complete
or partial
 Bilateral or unilateral
 Dyspnoea, apnoea when feeding
 Thick mucus in nasal cavities
 Feeding difficulties
 Blockage of catheter at 3cm.
 Stents are required.
Congenital laryngeal stridor
 Laryngomalacia
 Inspiratory
stridor
 Suprasternal indrawing
 Noise increase with crying, decrease with
sleeping
 Cause:
long, curved epiglottis
 Spontaneous recovery 2-3years.
Common causes
– 60%
 Congenital subglottic stenosis
 Vocal cord palsy - unilateral, birth trauma
– temporary
 Bilateral vocal cord palsy assoc other
congenital anomalies
 Laryngomalacia
Morimoto et al (2004)
 97
patients 1991-2001
 Laryngomalacia
32%
 Vocal cord palsy and laryngeal stenosis
22%, within 2/12, severe dyspnoea
 Haemangioma or papilloma 11%
 Cystic disease 7%
cont
2
/ 31 of laryngomalacia and 2 / 22 VCP
had neuromuscular disorders
 3 of VCP complicated by laryngeal
stenosis
 33 / 97 Tracheostomy
 Sometimes
stridor is the only presenting
symptom. Past history important
Case history
 6/12
girl
 Fever, coughing
 Inspiratory stridor
 Palpable neck swelling, bulging
pharyngeal wall
 Limited movement of neck
 ? spasmodic croup, lymphadenitis coli
 Found to be retro pharyngeal abscess
Treatment
 Oral
incision
 Drainage of abscess
 Antibiotics
Unilateral vocal cord paralysis
 Stridor
 Laryngospasm
 Dyspnoea
 Cause
by abnormal innervation of nerve
branches into adductor fibers
Research
Objective
 Determine
stridor at rest after oral
Prednisolone 1mg/kg
 And
whether quick response after mild
croup
Method
 Retrospective
explicit chart review of
children over 1 year of age admitted to a
teaching hospital
 Patient demographics
 Croup scores at AE
 Duration of stridor at rest after steroids
Results
 188
cases analysed
 Median duration at rest was 6.5 hrs, range
0.5 hrs- 82 hrs
 Patients with low score at AE recovered
quicker in response to steroids, early
discharge home.
Amphotericin induced stridor
 Adverse
effects reported Amphotericin B
 Dyspnoea
 Tachypnoea
 Bronchospasm
 Haemoptysis
 hypoxia
Objective
 To
review mechanism of action and
reports of respiratory adverse effects for
Amphotericin B, the liposomal
preparations for Amphotericin B and the
differential diagnosis of stridor
search 1966 – 2002 looking for
possible mechanisms and
immunoregulatory effects of Ampho B
 Medline
Results
 Amphotericin
B shows increase in tumour
necrosis factor alpha (TNF alpha)
concentrations in macrophages.
 Induces
prostaglandin E2 synthesis,
increasing production of interleukin1 beta
in mononuclear cells
Conclusion
 Amphotericin
B induces production of TNF
alpha, interferon gamma and interleukin 1
beta which have toxic effects.
Medicines for children
dose infused over 30 mins – 100mcg
 Renal impairment
 Low serum pott, mag, phos
 Lft’s
 arrhythmias
 Pulmonary reactions if Amph and
leucocyte Tx.
 Test
Subglottic stenosis, 1-8%
 Tracheostomy
 Cystic
hygroma
 Haemangioma
Case history 1
 Girl,
3.55kg, LSCS, 37/40
 TTN, ett, ventilation
 Day 3, pyrexia, measle like
exanthema,thrombocytopenia
 Diagnosis, toxic shock syndrome. Ax.
 Day 5 yellow tracheal secretions, glottis
red, not swollen
 MRSA, Day 13 extubated, stridor.
Case history 2
 Baby
girl, 2.790kg, LSCS, 37/40.
 At 3hrs, ett,ventilated, TTN
 Day 3, pyrexia
 Day 6 yellow secretions, epiglottis red, not
swollen
 Diagnosis: laryngotracheitis, MRSA
 Tracheostomy
Tracheomalacia
 Normal
struts of cartilage which maintain
the trachea patent are either malformed
(OA,TOF) or compressed by vessels.
 Collapse
of trachea
 Apnoea,
resus (bag and mask opens
airway)
 Where
site of fistula repair in TOF:
Supporting cartilage framework not fully
formed, floppy airway
Specialised lining cells (goblet and cilia) are
replaced by squamous cells, less effective
in protecting airway.
Severe tracheomalacia
 4-6mths
age
 Excessive wheeze
 Cyanosis
 Particularly during feed
 Near death episodes
 Trachea collapses, no air can pass
through
Tests for tracheomalacia
 Radiography
 Barium
(side on)
meal
 Bronchoscopy
 Respiratory function tests
Case history 1

24/40, antenatal steroids 48hrs, wt 765g
 Ventilated 20 days, stridor
 At 100 days failure to extubate laryngotracheobronchomalacia

90% occlusion lower trachea
 70% occlusion left main bronchus
 Unsuccessful aortapexy, cpap, trache
 At 18ths no malacia
Case history 2
 25/40,
772g, male, hyaline membrane
disease, curosurf x2
 Ventilated 6/52, recurrent stridor
 Subglottic stridor, Day 160
tracheobronchogram, collapse right
bronchus
Case history 3
 34/40,
infant of diabetic mum, bw 1162g
 Moderate severe RDS, curosurf, vent 21/7
 Oxygen desats at one year, vented again.
 Tracheobronchogram at 16mths, severe
malacia of left main bronchus
 Cpap
via tracheostomy.
Compressive disorder
 Double
aortic arch, (embryiological)
 Compresses right main bronchus and
lower trachea
 This
condition is result of failure of
posterior cricoid lamina and trachea
oesophageal septum to fuse
 MRI
Pulmonary artery sling
CCAM
 Chin
and Tang (1949)
 Proliferation of cysts resembling
bronchioles
 25% of all lung lesions
Pathogenesis and pathophysiologic
features
 Focal
arrest of fetal lung development
before 7th week development
 Secondary to pulmonary insults
 4-26% associated with other congenital
anomalies
Types of CCAM
 Type
1. 2-10cm diameter, large cysts
accompanied by small cysts
 Type 2. small relatively uniform cysts
resembling bronchioles, 0.5cm-2cm size
 Type 3. Microscopic cysts, solid
 Type
2/3 assoc with pulmonary
sequestration (arterial supply)
Differential diagnosis
 Absence
of bronchial cartilage
 Absence of bronchial tubular glands
 Presence of tall columnar mucus
epithelium
 Over production of terminal bronchiolar
structures without alveoli
 Massive enlargement of the affected lobe
displacing other structures.
Cystic adenomatoid malformation
 Single
or multi cystic mass in pulmonary
tissue.
 Cysts are lined with cuboid and columnar
cells which appear as alimentary tract
origin
 Affects lower lobes
 Complete removal to avoid malignancy in
future
Mortality / morbidity
 1:
25,000-35,000 Canada
 Type 3 extensive
 56% regress when identified in utero
 Equal sexes
Congenital diaphragmatic hernia
– 5000 births
 Failure of closure of the pleuroperitoneal
at 8-10 week
 Abdominal contents in chest
 Liver develops in chest, comes down to
abdo cavity- lung hypoplasia
 1:3500
 20%
right sided
 1-4% bilateral
 80% left sided
 Medical
management
 Surgery when conventional ventilation
 Pulmonary hypoplasia
 Hypoxia, hypercarbia
 Pulmonary vasoconstriction
 Pulmonary hypertension
 Poor gas exchange, right to left shunt.
Long term outcomes
 Recurrent
chest infections
 Gastro oesophageal reflux
 Pulmonary hypertension
 Developmental delay
 Deafness
 Recurrence of hernia
Congenital lobar emphysema
 Uncommon
 Life
threatening
 Respiratory distress due to hyperinflation
of the affected lobe, resulting in total
collapse of normal lung
 Unilobar alveoli distension
Study
 1995-2002
retrospective chart review
 5 boys, 3 girls with clinical and radiological
diagnosis of CLE
 Age range 11 days- 10 years
 Five patients lobectomy, 3 medical
management
 Like
father like son
 Mothers and daughters
 Inherited
 Antenatal scan
 Decrease with ongoing pregnancy
 However, air trapping and RDS and need
lobectomy in some
 Associated with congenital heart disease
References
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of pulmonary hyperplasia (congenital cystic adenomatoid malformation).
BMJ Case Reports. doi:10.1136/bcr.01.2010.2679
Calvert J and Lakhoo K (2007). Antenatally suspected congenital cystic
adenomatoid malformation of the lung : postnatal investigation and timing of
surgery. Journal of Pediatric Surgery. Vol 42, Iss 2, p411 - 414
Congenital Cystic Adenomatoid Malformation.
http://pediatrics.uchicago.edu/chiefs/AMreport/CCAM_files/outline.htm
Mandell G (2003). Congenital Cystic Adenomatoid Malformation.
E Medicine. http://www.emedicine.com/radio/topic 186.htm
Marshall K, Blane C, Teitelbaum D, Leevuren K (2000).
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