Respiratory conditions

1
Respiratory conditions

• Anne Aspin
• 2005

2
Embryology

• Atresia of oesophagus with fistula
• Atresia of trachea with fistula
• Laryngo-tracheo-oesophageal clefts
• System of folds, blocked pathway
• Adriamycin (rat research)

3

• 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.

4

• 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.

5

• Of these theories failure of the
  pleuro-peritoneal 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.

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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.

7
Head and Neck Examination

• Respirations 30 60 bpm
• Abnormal lt 30, gt 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

8
Examination of the nose

• Broad flat, chromosomal abnormality
• Patency, choanal atresia, tumour
• Sneezing
• Bloody discharge, syphilis

9
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.

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Oesophageal atresia

• Bubbly secretions
• Apnoea
• Cyanosis
• Immediate vomiting on feeding
• Unable to pass ng tube
• Replogle tube, continual pharyngeal suction

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Types of oesophageal atresia and fistula
86
7
4
12
Types continued
1
lt1
lt1
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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

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Thickened epiglottis
15
Oedematous narrowed sub epiglottic trachea
16
Tracheobronchogram
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Collapse of right main bronchus
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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

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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

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Complications of bronchoscopy

• Pneumothorax 8
• Incidence reduced if bronchoscope avoiding right
  middle lobe
• Haemorrhage following biopsy
• Pyrexia, dyspnoea

21
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.

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Congenital laryngeal stridor

• Laryngomalacia
• Inspiratory stridor
• Suprasternal indrawing
• Noise increase with crying, decrease with
  sleeping
• Cause long, curved epiglottis
• Spontaneous recovery 2-3years.

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Common causes

• Laryngomalacia 60
• Congenital subglottic stenosis
• Vocal cord palsy - unilateral, birth trauma
  temporary
• Bilateral vocal cord palsy assoc other congenital
  anomalies

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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

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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

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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

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Treatment

• Oral incision
• Drainage of abscess
• Antibiotics

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Unilateral vocal cord paralysis

• Stridor
• Laryngospasm
• Dyspnoea
• Cause by abnormal innervation of nerve branches
  into adductor fibers

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Research

• Objective
• Determine stridor at rest after oral Prednisolone
  1mg/kg
• And whether quick response after mild croup

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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

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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.

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Amphotericin induced stridor

• Adverse effects reported Amphotericin B
• Dyspnoea
• Tachypnoea
• Bronchospasm
• Haemoptysis
• hypoxia

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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
• Medline search 1966 2002 looking for possible
  mechanisms and immunoregulatory effects of Ampho B

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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

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Conclusion

• Amphotericin B induces production of TNF alpha,
  interferon gamma and interleukin 1 beta which
  have toxic effects.

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Medicines for children

• Test dose infused over 30 mins 100mcg
• Renal impairment
• Low serum pott, mag, phos
• Lfts
• arrhythmias
• Pulmonary reactions if Amph and leucocyte Tx.

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Subglottic stenosis, 1-8

• Tracheostomy
• Cystic hygroma
• Haemangioma

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Case history 1

• Girl, 3.55kg, LSCS, 37/40
• TTN, ett, ventilation
• Day 3, pyrexia, measle like exanthema,thrombocytop
  enia
• Diagnosis, toxic shock syndrome. Ax.
• Day 5 yellow tracheal secretions, glottis red,
  not swollen
• MRSA, Day 13 extubated, stridor.

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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

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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)

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• 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.

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Severe tracheomalacia

• 4-6mths age
• Excessive wheeze
• Cyanosis
• Particularly during feed
• Near death episodes
• Trachea collapses, no air can pass through

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Tests for tracheomalacia

• Radiography (side on)
• Barium meal
• Bronchoscopy
• Respiratory function tests

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Case history 1

• 24/40, antenatal steroids 48hrs, wt 765g
• Ventilated 20 days, stridor
• At 100 days failure to extubate
  laryngo-tracheobronchomalacia
• 90 occlusion lower trachea
• 70 occlusion left main bronchus
• Unsuccessful aortapexy, cpap, trache
• At 18ths no malacia

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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

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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.

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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

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Pulmonary artery sling
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CCAM

• Chin and Tang (1949)
• Proliferation of cysts resembling bronchioles
• 25 of all lung lesions

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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

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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)

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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.

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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

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Mortality / morbidity

• 1 25,000-35,000 Canada
• Type 3 extensive
• 56 regress when identified in utero
• Equal sexes

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Congenital diaphragmatic hernia

• 13500 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

56

• 20 right sided
• 1-4 bilateral
• 80 left sided

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• Medical management
• Surgery when conventional ventilation
• Pulmonary hypoplasia
• Hypoxia, hypercarbia
• Pulmonary vasoconstriction
• Pulmonary hypertension
• Poor gas exchange, right to left shunt.

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Long term outcomes

• Recurrent chest infections
• Gastro oesophageal reflux
• Pulmonary hypertension
• Developmental delay
• Deafness
• Recurrence of hernia

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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

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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

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• 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