Dr. Ahmed Fathalla Ibrahim LOWER RESPIRATORY ORGANS • • • • LARYNX TRACHEA BRONCHI LUNGS PHARYNGEAL ARCHES RESPIRATORY PRIMORDIUM RESPIRATORY PRIMORDIUM RESPIRATORY PRIMORDIUM 6 RESPIRATORY PRIMORDIUM • Begins to form in the middle of 4th week • Laryngotracheal groove: a median outgrowth from the caudal end of the floor (ventral wall) of primordial pharynx, caudal to 4th pharyngeal pouch • Respiratory diverticulum: produced by deepening (evagination) of the groove, located ventral to caudal part of foregut RESPIRATORY PRIMORDIUM • Tracheal bud: it is the enlarged distal end of diverticulum • Longitudinal tracheoesophageal folds develop in diverticulum, approach each other & fuse to form tracheoesophageal septum dividing cranial part of foregut into: 1. Ventral part: laryngotracheal tube: primordium of larynx, trachea, bronchi & lungs 2. Dorsal part: pharynx, esophagus • Both parts maintain communication through primordial laryngeal inlet EMBRYOLOGICAL ORIGIN • Endoderm lining laryngotracheal tube: epithelium & glands of all respiratory organs • Fourth & sixth pharyngeal arch cartilages: all laryngeal cartilages EXCEPT epiglottis • Sixth pharyngeal arch muscles: intrinsic muscles of larynx • Mesenchyme of hypopharyngeal eminence: epiglottis • Splanchnic mesoderm surrounding foregut: connective tissue, cartilage, smooth muscles, blood & lymphatic vessels of trachea, bronchi & lungs DEVELOPMENT OF BRONCHI & LUNGS DEVELOPMENT OF BRONCHI & LUNGS • During 5th week, tracheal bud divides into 2 primary bronchial buds • Main bronchus: formed by primary bronchial bud together with its surrounding splanchnic mesoderm, divided into: secondary (lobar), tertiary (segmental) & intrasegmental branches • By 24 weeks, 17 orders of branches have formed & respiratory bronchioles have developed • After birth, 24 orders of branches are present DEVELOPMENT OF BRONCHI & LUNGS • As the lungs expand: 1.They invaginate pleura & acquire a visceral layer of pleura (derived from splanchnic mesoderm) 2.They grow caudally into body wall & lie close to heart. The thoracic body wall becomes lined by a parietal layer of pleura (derived from somatic mesoderm) MATURATION OF LUNGS 1. 2. 3. 4. Pseudoglandular period Canalicular period Terminal saccular period Alveolar period MATURATION OF LUNGS PSEUDOGLANDULAR PERIOD (6-12 WEEKS) • Lung resembles an exocrine gland • Only bronchial & primordial terminal bronchioles are formed • Respiration is not possible CANALICULAR PERIOD (16-26 WEEKS) • Lumina of bronchi & terminal bronchioles enlarge • Respiratory bronchioles & alveolar ducts develop • Lung tissue becomes highly vascular • Respiration is possible • Fetus born at this period often dies because of immaturity of systems TERMINAL SACCULAR PERIOD (26 – BIRTH) • Alveolar ducts give rise to terminal saccules (primordial alveoli) • Terminal saccules: cuboidal epithelium begins to change into squamous • Capillaries begin to bulge into alveoli to establish blood-air barrier that permits adequate gas exchange • Terminal saccules are lined with: 1. Type I pneumocytes (across which gas exchange occurs): main type of cells 2. Type II pneumocytes (secrete surfactant) TERMINAL SACCULAR PERIOD (26 – BIRTH) Surfactant: • Role: counteracts surface tension forces & facilitates expansion of alveoli • Production: begins by 20 weeks & increases during last 2 weeks of pregnancy • By 26-28 weeks: surfactant is produced in sufficient amount for survival of prematurely born fetus ALVEOLAR PERIOD (32 WEEKS – 8 YEARS) • Number of respiratory bronchioles and terminal saccules (primordial alveoli) increases • Type I pneumocytes of terminal saccules becomes extremely thin squamous so that adjacent capillaries bulge into it • Alveolocapillary membrane is sufficiently thin to allow gas exchange ALVEOLAR PERIOD (32 WEEKS – 8 YEARS) • Lungs begin to work after birth • Transition from dependence on placenta for gas exchange to lungs requires: 1.Production of sufficient amount of surfactant 2.Development of alveolocapillary membrane that is sufficiently thin ALVEOLAR PERIOD (32 WEEKS – 8 YEARS) • Maturation of alveoli (about 95%) occurs after birth • From birth to third year: Increase in size of lungs is mostly due to increase in number of respiratory bronchioles & primordial alveoli • Immature alveoli have the potential for forming additional primordial alveoli • Immature alveoli increases in size & become mature TRACHEOESOPHAGEAL FISTULA TRACHEOESOPHAGEAL FISTULA • Most common anomaly • Associated with esophageal atresia in more than 85% of cases • Often associated with polyhydramnios • Cause: incomplete fusion of treacheoesophageal folds resulting in a defective tracheoesophageal septum & faulty partitioning of foregut into trachea & esophagus TRACHEOESOPHAGEAL FISTULA Clinical manifesttaions: • Infants cough & choke when swallowing (due to accumulation of saliva in upper respiratory tract) • Infants regurgitate when swallowing milk (milk fills esophageal pouch & is regurgitated) • Pneumonitis RESPIRATORY DISTRESS SYNDROME • Also known as hyaline membrane disease • Affects about 2% of newborn (especially premature newborn) • Cause: deficiency of surfactant • Manifestation: lungs are underinflated, alveoli contain a fluid with a high protein content that resembles a hyaline membrane RESPIRATORY DISTRESS SYNDROME • Predisposing factors: 1.Prolonged intrauterine asphyxia 2.Deficiency of thyroxine • Treatment: glucocorticoid OTHER ANOMALIES • Stenosis (narrowing) or atresia (obstruction) of trachea: due to unequal partitioning of foregut into esophagus & trachea • Congenital lung cysts: disturbance in bronchial development leads to dilation of terminal bronchi & formation of cysts filled with fluid or air • Agenesis of lung: due to failure of bronchial bud to develop