L4 CONGENITAL ANOMALIES Flashcards
INTRODUCTION
Developmental defects of the lung include the following[1]:
1 • Agenesis or hypoplasia of both lungs, one lung, or single lobes
2 • Tracheal and bronchial anomalies (atresia, stenosis, tracheoesophageal fistula)
3 • Vascular anomalies
4 • Congenital lobar overinflation (emphysema)
5 • Foregut cysts
6 • Congenital pulmonary airway malformation
7• Pulmonary sequestrations
PULMONARY HYPOPLASIA
—Is the defective development of both lungs (one may be more affected than the other).
—This may result in decreased weight, volume, and acini disproportional to the body weight and gestational age.
It is caused by a variety of abnormalities that compress the lung(s) or impede normal lung expansion in utero such as [congenital diaphragmatic hernia and oligohydramnios.]
FOREGUT CYSTS
—This arises from an abnormal detachment of primitive foregut.
—The common site include: [the hilum or middle mediastinum.]
Depending on the wall structure, these cysts are classified as:
1.Bronchogenic (most common)
2.Esophageal
3.Enteric.
BRONCHOGENIC CYSTS
—Is rarely connected to the tracheobronchial tree.
—Microscopically: A cyst lined by ciliated pseudostratified columnar epithelium with squamous metaplasia occurring in areas of inflammation.
—Surgical resection is curative.
PULMONARY SEQUESTRATION
DEFINITION:
—the presence of discrete mass of lung tissue without normal connection to the airway system.
—Blood supply to the sequestered area arises not from the pulmonary arteries but from the aorta or its branches.
The classifications are:
1.Extralobar sequestration.
2,Intralobar sequestration.
EXTRALOBAR SEQUESTRATION
—They are seen external to the lung.
—The common sites include: thorax or mediastinum.
— They most commonly occur in infants as abnormal mass lesions.
—They may be associated with other congenital anomalies.
INTRALOBAR SEQUESTRATION
—occur within the lung substance in older children
—often associated with Bronchiectasis or Recurrent localized infections
