2Embryology Atresia of oesophagus with fistula Atresia of trachea with fistulaLaryngo-tracheo-oesophageal cleftsSystem of folds, blocked pathwayAdriamycin (rat research)
3Defects 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.
4Premature return of intestine to abdo cavity but canal still open Abnormal persistance of lung in pleuro peritoneal cavity, preventing closure of cavityAbnormal development of early lung.
5Of these theories failure of the pleuro-peritoneal membrane to meet the transverse septum is likely explanation for diaphragm herniationLack of embryological evidenceDay 13,(L) Day 14 (R), disturbed development (rats) = 4-5/52 embryos.
6Lung hypoplasiaFrom 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.
8Examination of the nose Broad flat, chromosomal abnormalityPatency, choanal atresia, tumourSneezingBloody discharge, syphilis
9Examination of the mouth and throat Excessive salivaAbnormal structures, cleft lip and palate, micrognathia, large tongue, absent or unequal reflexes, prematurity or CNS anomalyDistended neck veins indicate chest or pneumomediastinal mass.
10Oesophageal atresia Bubbly secretions Apnoea Cyanosis Immediate vomiting on feedingUnable to pass ng tubeReplogle tube, continual pharyngeal suction
11Types of oesophageal atresia and fistula 86%7%4%
13Lungs and ThoraxCrackles and rhonchi present first four hours after birth.Abnormal: decreased abdominal breathingThoracic and asymmetrical breathing – phrenic nerve damage, CDH,Hyperresonance may indicate pneumomediastinum, pneumothorax, CDH
18Indications for bronchoscopy StridorUnexplained wheezeUnexplained or persistent cough HaemoptysisSuspected foreign bodySuspected airway trauma, chemical, or thermal injurySuspected tracheobronchial fistulaSuspected tracheobronchial stenosisRadiological abnormalitiesPersistent or recurrent consolidation or atelectasisRecurrent or persistent infiltratesLung lesions of unknown aetiologyImmunosupressed patientsIdentify cause of pneumoniaRecurrence of diseaseCystic fibrosisIdentify cause of infectionIntensive careExamine for the position, patency,or damage related to endotracheal or tracheostomy tubesFacilitation of endotracheal intubationEndobronchial stent placement
19Bronchoscopy Early dates, removal of foreign bodies Rigid bronchoscope (telescope fits down), complete control of airway, ventilationFlexible bronchoscope (bundles of optical fibres, light to the tips), children from 3yrs
20Complications of bronchoscopy Pneumothorax 8%Incidence reduced if bronchoscope avoiding right middle lobeHaemorrhage following biopsyPyrexia, dyspnoea
21Choanal atresia Complete or partial Bilateral or unilateral Dyspnoea, apnoea when feedingThick mucus in nasal cavitiesFeeding difficultiesBlockage of catheter at 3cm.Stents are required.
22Congenital laryngeal stridor LaryngomalaciaInspiratory stridorSuprasternal indrawingNoise increase with crying, decrease with sleepingCause: long, curved epiglottisSpontaneous recovery 2-3years.
24Morimoto et al (2004) 97 patients 1991-2001 Laryngomalacia 32% Vocal cord palsy and laryngeal stenosis 22%, within 2/12, severe dyspnoeaHaemangioma or papilloma 11%Cystic disease 7%
25cont2 / 31 of laryngomalacia and 2 / 22 VCP had neuromuscular disorders3 of VCP complicated by laryngeal stenosis33 / 97 TracheostomySometimes stridor is the only presenting symptom. Past history important
26Case history 6/12 girl Fever, coughing Inspiratory stridor Palpable neck swelling, bulging pharyngeal wallLimited movement of neck? spasmodic croup, lymphadenitis coliFound to be retro pharyngeal abscess
27TreatmentOral incisionDrainage of abscessAntibiotics
28Unilateral vocal cord paralysis StridorLaryngospasmDyspnoeaCause by abnormal innervation of nerve branches into adductor fibers
29ResearchObjectiveDetermine stridor at rest after oral Prednisolone 1mg/kgAnd whether quick response after mild croup
30MethodRetrospective explicit chart review of children over 1 year of age admitted to a teaching hospitalPatient demographicsCroup scores at AEDuration of stridor at rest after steroids
31Results 188 cases analysed Median duration at rest was 6.5 hrs, range 0.5 hrs- 82 hrsPatients with low score at AE recovered quicker in response to steroids, early discharge home.
33ObjectiveTo review mechanism of action and reports of respiratory adverse effects for Amphotericin B, the liposomal preparations for Amphotericin B and the differential diagnosis of stridorMedline search 1966 – 2002 looking for possible mechanisms and immunoregulatory effects of Ampho B
34ResultsAmphotericin 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
35ConclusionAmphotericin B induces production of TNF alpha, interferon gamma and interleukin 1 beta which have toxic effects.
36Medicines for children Test dose infused over 30 mins – 100mcgRenal impairmentLow serum pott, mag, phosLft’sarrhythmiasPulmonary reactions if Amph and leucocyte Tx.
38Case history 1 Girl, 3.55kg, LSCS, 37/40 TTN, ett, ventilation Day 3, pyrexia, measle like exanthema,thrombocytopeniaDiagnosis, toxic shock syndrome. Ax.Day 5 yellow tracheal secretions, glottis red, not swollenMRSA, Day 13 extubated, stridor.
39Case history 2 Baby girl, 2.790kg, LSCS, 37/40. At 3hrs, ett,ventilated, TTNDay 3, pyrexiaDay 6 yellow secretions, epiglottis red, not swollenDiagnosis: laryngotracheitis, MRSATracheostomy
40TracheomalaciaNormal struts of cartilage which maintain the trachea patent are either malformed (OA,TOF) or compressed by vessels.Collapse of tracheaApnoea, resus (bag and mask opens airway)
41Where site of fistula repair in TOF: Supporting cartilage framework not fully formed, floppy airwaySpecialised lining cells (goblet and cilia) are replaced by squamous cells, less effective in protecting airway.
42Severe tracheomalacia 4-6mths ageExcessive wheezeCyanosisParticularly during feedNear death episodesTrachea collapses, no air can pass through
43Tests for tracheomalacia Radiography (side on)Barium mealBronchoscopyRespiratory function tests
44Case history 1 24/40, antenatal steroids 48hrs, wt 765g Ventilated 20 days, stridorAt 100 days failure to extubate laryngo-tracheobronchomalacia90% occlusion lower trachea70% occlusion left main bronchusUnsuccessful aortapexy, cpap, tracheAt 18ths no malacia
45Case history 225/40, 772g, male, hyaline membrane disease, curosurf x2Ventilated 6/52, recurrent stridorSubglottic stridor, Day 160 tracheobronchogram, collapse right bronchus
46Case history 3 34/40, infant of diabetic mum, bw 1162g Moderate severe RDS, curosurf, vent 21/7Oxygen desats at one year, vented again.Tracheobronchogram at 16mths, severe malacia of left main bronchusCpap via tracheostomy.
47Compressive disorder Double aortic arch, (embryiological) Compresses right main bronchus and lower tracheaThis condition is result of failure of posterior cricoid lamina and trachea oesophageal septum to fuseMRI
49CCAMChin and Tang (1949)Proliferation of cysts resembling bronchioles25% of all lung lesions
50Pathogenesis and pathophysiologic features Focal arrest of fetal lung development before 7th week developmentSecondary to pulmonary insults4-26% associated with other congenital anomalies
51Types of CCAMType cm diameter, large cysts accompanied by small cystsType 2. small relatively uniform cysts resembling bronchioles, 0.5cm-2cm sizeType 3. Microscopic cysts, solidType 2/3 assoc with pulmonary sequestration (arterial supply)
52Differential diagnosis Absence of bronchial cartilageAbsence of bronchial tubular glandsPresence of tall columnar mucus epitheliumOver production of terminal bronchiolar structures without alveoliMassive enlargement of the affected lobe displacing other structures.
53Cystic adenomatoid malformation Single or multi cystic mass in pulmonary tissue.Cysts are lined with cuboid and columnar cells which appear as alimentary tract originAffects lower lobesComplete removal to avoid malignancy in future
54Mortality / morbidity 1: 25,000-35,000 Canada Type 3 extensive 56% regress when identified in uteroEqual sexes
55Congenital diaphragmatic hernia 1:3500 – 5000 birthsFailure of closure of the pleuroperitoneal at 8-10 weekAbdominal contents in chestLiver develops in chest, comes down to abdo cavity- lung hypoplasia
57Medical managementSurgery when conventional ventilationPulmonary hypoplasiaHypoxia, hypercarbiaPulmonary vasoconstrictionPulmonary hypertensionPoor gas exchange, right to left shunt.
58Long term outcomes Recurrent chest infections Gastro oesophageal refluxPulmonary hypertensionDevelopmental delayDeafnessRecurrence of hernia
59Congenital lobar emphysema UncommonLife threateningRespiratory distress due to hyperinflationof the affected lobe, resulting in total collapse of normal lungUnilobar alveoli distension
60Study 1995-2002 retrospective chart review 5 boys, 3 girls with clinical and radiological diagnosis of CLEAge range 11 days- 10 yearsFive patients lobectomy, 3 medical management
61Like father like sonMothers and daughtersInheritedAntenatal scanDecrease with ongoing pregnancyHowever, air trapping and RDS and need lobectomy in someAssociated with congenital heart disease
62ReferencesAnkers D, Sajjad N, Green P, McPartland J (2010). Antenatal management of pulmonary hyperplasia (congenital cystic adenomatoid malformation). BMJ Case Reports. doi: /bcrCalvert 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, pCongenital Cystic Adenomatoid Malformation.Mandell G (2003). Congenital Cystic Adenomatoid Malformation.E Medicine htmMarshall K, Blane C, Teitelbaum D, Leevuren K (2000).Congenital Cystic Adenomatoid Malformation. Impact of PrenatalDiagnosis and Changing Strategies in the Treatment of the AsymptomaticPatient. 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 :