1. Prepared by: Richard Ningombam
Group: 22-21
Submitted to: Kerimov R.A.
DEPARTMENT OF PATHOLOGY AND
FORENSIC MEDICINE
TOPIC: MALFORMATIONS OF THE
RESPIRATORY SYSTEM.(WEEK-2)
SOUTH KAZAKHSTAN MEDICAL ACADEMY
3. Introduction
Malformations of the respiratory system are congenital abnormalities
that affect the structure and function of the respiratory organs. Some
common malformations include A common malformation of the
respiratory system is a congenital diaphragmatic hernia (CDH). In CDH,
there is a defect in the diaphragm, the muscle that separates the chest
cavity from the abdominal cavity. This opening allows abdominal
organs to move into the chest cavity, which can lead to pulmonary
hypoplasia (underdevelopment of the lungs) and respiratory
insufficiency.
4. Tracheoesophageal Fistula (TEF):
- Type: Abnormal connection
between the trachea and
esophagus.
- Causes: Often a result of
improper separation during
embryonic development.
- Mechanism: Failure of the
tracheoesophageal septum to
form properly.
5. Congenital Diaphragmatic Hernia (CDH):
- Type: Defect in the diaphragm allowing abdominal organs to enter
the chest cavity.
- Causes: Malformation during embryonic development.
- Mechanism: Failure of the pleuroperitoneal folds to fuse, leading to
diaphragmatic opening.
6.
7. Pulmonary Sequestration:
- Type: Lung tissue separated from the
normal bronchial tree and blood supply.
- Causes: Developmental anomaly
during lung bud formation.
- Mechanism: Abnormal budding of
the foregut leading to isolated lung
tissue.
Pulmonary sequestration is a
congenital anomaly of the primitive
foregut. The most plausible theory is
the formation of an accessory
supernumerary lung bud below the
normal lung bud
8. Mechanism
Pulmonary sequestration is thought to account for less than 6 percent of
congenital lung malformations. In this condition, there is a nonfunctional segment
or lobe of dysplastic lung tissue that does not communicate with the rest of the
tracheobronchial tree and receives an anomalous vascular supply.Anatomically it is
classified into intralobar and extralobar sequestration; intralobar sequestration is
more common and accounts for 0.15% to 1.7% of all congenital lung
abnormalities.
9.
10. Congenital Lobar Emphysema:
- Overinflation of a lung lobe due to obstruction.Congenital lobar
emphysema is a rare developmental malformation of lungs. The main
etiology is the deficient development of bronchial cartilages leading to
overinflation of the affected lobe. CLE usually presents with respiratory
distress, wheezing, rhonchi, cyanosis, and difficulty in feeding.
11. Symptoms
Congenital lobar emphysema is a
rare developmental malformation of
lungs. The main etiology is the
deficient development of bronchial
cartilages leading to overinflation of
the affected lobe. CLE usually
presents with respiratory distress,
wheezing, rhonchi, cyanosis, and
difficulty in feeding.
12. Impact:
This can lead to respiratory distress and
compromised lung function.Congenital
lobar emphysema is a rare developmental
malformation of lungs. The main etiology
is the deficient development of bronchial
cartilages leading to overinflation of the
affected lobe. CLE usually presents with
respiratory distress, wheezing, rhonchi,
cyanosis, and difficulty in feeding.
13. Cystic Fibrosis:
- A genetic disorder affecting the
respiratory and digestive systems. is a
disorder that damages your lungs,
digestive tract and other organs. It's
an inherited disease caused by a
defective gene that can be passed
from generation to generation. Cystic
fibrosis affects the cells that produce
mucus, sweat and digestive juices. - Impact: Results in thick and sticky mucus,
leading to recurrent respiratory infections
and impaired lung function.
14. Causes:
- Genetic Factors: Inherited genetic mutations can predispose individuals to
respiratory malformations.Hereditary lung diseases can affect lungs, liver and/or
skin. Common disorders are alpha-1-antitrypsin (AAT), cystic fibrosis (CF), interstitial
lung disease, pulmonary alveolar microlithiasis and pulmonary arterial
hypertension (PAH). AAT deficiency can result in destruction of sensitive lung tissue.
15. - :
Exposure to certain teratogens during
pregnancy may contribute to
abnormalities.Air pollution and cigarette
smoke are the greatest threats. Some
lung diseases are caused by bacterial,
viral, or fungal infections. Studies linking
air quality to health effects are numerous
and important. Pollution can be found
outdoors, from vehicle exhaust, industrial
emissions, or wildfires, but also indoors.
Environmental Factors
17. Conclusion
The malformation of the respiratory system poses challenges that
necessitate a comprehensive and personalized approach to diagnosis and
treatment. Advances in medical research, technology, and surgical
techniques continue to improve outcomes for individuals affected by
these conditions, emphasizing the importance of ongoing efforts to
enhance our understanding and management of respiratory
malformations.The malformation of the respiratory system can have
significant and varied consequences on an individual's health and well-
being. These abnormalities may affect any part of the respiratory system,
including the nasal passages, trachea, bronchi, and lungs.
18. REFERENCES
Nelson Textbook of Pediatrics" by Robert M. Kliegman, Bonita M.D. Stanton,
Joseph St. Geme, and Nina F. Schor. Pg.145
"Robbins Basic Pathology" by Vinay Kumar, Abul K. Abbas, and Jon C. Aster.
Pg.234
Medical journals such as The New England Journal of Medicine, The Lancet, or
the American Journal of Respiratory and Critical Care Medicine.
