1. Respiratory conditions
Anne Aspin
2010

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.

6. 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 < 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

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.

10. Oesophageal atresia Bubbly secretions Apnoea Cyanosis
Immediate vomiting on feeding
Unable to pass ng tube
Replogle tube, continual pharyngeal suction

11. Types of oesophageal atresia and fistula
86% 7% 4%

12. Types continued 1%
<1
<1

13. 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

14. Thickened epiglottis

15. Oedematous narrowed sub epiglottic trachea

16. Tracheobronchogram

17. Collapse of right main bronchus

18. 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

19. 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

20. 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.

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

23. Common causes Laryngomalacia – 60% Congenital subglottic stenosis
Vocal cord palsy - unilateral, birth trauma – temporary
Bilateral vocal cord palsy assoc other congenital anomalies

24. 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%

25. 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

26. 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

27. Treatment
Oral incision
Drainage of abscess
Antibiotics

28. Unilateral vocal cord paralysis
Stridor
Laryngospasm
Dyspnoea
Cause by abnormal innervation of nerve branches into adductor fibers

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

30. 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

31. 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.

32. Amphotericin induced stridor
Adverse effects reported Amphotericin B
Dyspnoea
Tachypnoea
Bronchospasm
Haemoptysis
hypoxia

33. 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

34. 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

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

36. Medicines for children
Test dose infused over 30 mins – 100mcg
Renal impairment
Low serum pott, mag, phos
Lft’s
arrhythmias
Pulmonary reactions if Amph and leucocyte Tx.

37. Subglottic stenosis, 1-8%
Tracheostomy
Cystic hygroma
Haemangioma

38. 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.

39. 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

40. 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)

41. 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.

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

43. Tests for tracheomalacia
Radiography (side on)
Barium meal
Bronchoscopy
Respiratory function tests

44. 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

45. 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

46. 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.

47. 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

48. Pulmonary artery sling

49. CCAM
Chin and Tang (1949)
Proliferation of cysts resembling bronchioles
25% of all lung lesions

50. 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

51. Types of CCAM
Type cm 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)

52. 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.

53. 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

54. Mortality / morbidity 1: 25,000-35,000 Canada Type 3 extensive
56% regress when identified in utero
Equal sexes

55. Congenital diaphragmatic hernia
1:3500 – 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

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

58. Long term outcomes Recurrent chest infections
Gastro oesophageal reflux
Pulmonary hypertension
Developmental delay
Deafness
Recurrence of hernia

59. 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

60. 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

61. 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

62. References
Ankers D, Sajjad N, Green P, McPartland J (2010). Antenatal management of pulmonary hyperplasia (congenital cystic adenomatoid malformation). BMJ Case Reports. doi: /bcr
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, p
Congenital Cystic Adenomatoid Malformation.
Mandell G (2003). Congenital Cystic Adenomatoid Malformation.
E Medicine htm
Marshall K, Blane C, Teitelbaum D, Leevuren K (2000).
Congenital Cystic Adenomatoid Malformation. Impact of Prenatal
Diagnosis and Changing Strategies in the Treatment of the Asymptomatic
Patient. American Journal of Roentgenology. 175:
Samuel M, Burge D (1999). Management of Ante-natally Diagnosed Pulmonary Sequestration Associated with Congenital Cystic Adenomatoid Malformation. Thorax. 54:
West D, Nicholson A, Colquhoun I, Pollock J (2006). Bronchioloaveolar carcinoma in congenital cystic adenomatoid malformation of lung. Annals of Thoracic Surgery. 83 :