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Respiratory conditions Anne Aspin 2010. Embryology  Atresia of oesophagus with fistula  Atresia of trachea with fistula  Laryngo-tracheo-oesophageal.

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Presentation on theme: "Respiratory conditions Anne Aspin 2010. Embryology  Atresia of oesophagus with fistula  Atresia of trachea with fistula  Laryngo-tracheo-oesophageal."— Presentation transcript:

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 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 <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  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 7 th 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 of the affected lobe, resulting in total collapse of normal lung  Unilobar alveoli distension

60 Study  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 E Medicine htmhttp://www.emedicine.com/radio/topic 186.htmhttp://www.emedicine.com/radio/topic 186.htm  Marshall K, Blane C, Teitelbaum D, Leevuren K (2000). Congenital Cystic Adenomatoid Malformation. Impact of Prenatal Congenital Cystic Adenomatoid Malformation. Impact of Prenatal Diagnosis and Changing Strategies in the Treatment of the Asymptomatic Diagnosis and Changing Strategies in the Treatment of the Asymptomatic Patient. American Journal of Roentgenology. 175: 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 :


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