Presentation on theme: "Respiratory conditions Anne Aspin 2010. Embryology Atresia of oesophagus with fistula Atresia of trachea with fistula Laryngo-tracheo-oesophageal."— Presentation transcript:
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 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.
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.
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%
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.
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
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
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 Test dose infused over 30 mins – 100mcg Renal impairment Low serum pott, mag, phos Lft’s arrhythmias Pulmonary reactions if Amph and leucocyte Tx.
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 (side on) Barium 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 laryngo- tracheobronchomalacia 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
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 7 th 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 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
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 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 Ankers D, Sajjad N, Green P, McPartland J (2010). Antenatal management 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 http://pediatrics.uchicago.edu/chiefs/AMreport/CCAM_files/outline.htmhttp://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 E Medicine. http://www.emedicine.com/radio/topic 186.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. 175:1551-1554 Patient. American Journal of Roentgenology. 175:1551-1554 Samuel M, Burge D (1999). Management of Ante-natally Diagnosed Pulmonary Sequestration Associated with Congenital Cystic Adenomatoid Malformation. Thorax. 54:701-706 West D, Nicholson A, Colquhoun I, Pollock J (2006). Bronchioloaveolar carcinoma in congenital cystic adenomatoid malformation of lung. Annals of Thoracic Surgery. 83 : 687 - 689