Congenital Lung Disease Flashcards
4 phases of intrauterine pulmonary development
- Embryonic phase (weeks 1-5)
- development of lung buds and primitive 5 lobes
- Pseudoglandular phase (weeks 5-16)
- development of bronchial tree and pulm vasculature
- Cannicular phase (weeks 16-26)
- development of bronchioles/alveolar ducts
- Terminal sac phase (weeks 26-40)
- development of primitive alveoli
- surfactant production (type II pneumocytes)
Most common anomaly of the trachea
Trachoesophageal Fistula (TEF)
Classification system for TEF
Gross Classification
- Type A (8%): EA without TEF
- Type B (1%): EA with proximal TEF
- Type C (87%): EA with distal TEF
- Type D (1%): EA with proximal and distal TEF
Excessive air within esophagus, stomach and intestinal tract; inability to pass NGT
TEF
TOC for TEF
Right extrapleural thoracotomy
Right cervical incision
Divison of fistula
Repair of esophageal defect
Interposition muscle flp (to reduce likelihood of recurrence)
Abnormal segment of lung tissue that has no communication with tracheobronchial tree
Pulmonary sequestration
Arterial supply for pulmonary sequestraton most frequently from _
Thoracic aorta
Alternate sources:
abdominal aorta
intercostal artery
multiple sources
Anatomy of arterial/venous supply to pulmonary sequestration
Arterial supply usually enters sequestration away from hilum and most often at base of lung
Venous return most commonly into pulmonary veins (can also be into systemic venous system)
Pulmonary sequestration may be associated with __
Esophageal fistula (both arise from forgut)
Frequency of extralobar/intralobar sequestration
Extralobar (25%)
Intralobar (75%)
Characteristics of Extralobar sequestration?
- distinct from remaining lung (have own pleura)
- located above the dome of diaphragm (90% located at the base of left lung)
- venous return is MC systemic (azygous, hemiazygous)
- 20% drain to pulmonary veins
- associated with other congenital anomalies
- may contain malignancies
MC location of ELS
base of left lung (90%)
ELS assocated with what other congenital anomlies
- congenital diaphragmatic hernia
- congenital cystic adenomatoid malformation (CCAM)
- pericardial cysts
- cardiac defects
- esophageal achalasia
Various locations of ELS
- pericardium
- diaphragm
- below diaphragm in retroperitoneum
Definition of ILS
cystic abnormalities within the visceral pleura of a given lung that communicate with the normal lung tissue through the pores of Kohn
MC location of ILS
posterior segment of LLL
(usually ILS located in right and left lower lobes)
MC location for venous drainage of ILS
Pulmonary veins (90% of ILS)
Sx of sequestration
Recurrent pulmonary infections and hemoptysis
Dx w/u for sequestration
- CT or MRI
- UGI series (if concern for enteric communication)
- Angiography (rare, to identify feeding vessels)
Tx of sequestration
- Lobectomy (MC)
- Sometimes segmentectomy is sufficient
- Early ID and control of arterial supply to control bleeding complications
Definition of bronchial cysts
Abnormal budding of trachea
Characteristics of bronchogenic cysts
MC mediastinal cysts (60%)
Typically located along right paratracheal area
May be attached to carina or lobar bronchus
Bronchial communication rare
Histology of bronchogenic cysts
Inner lining of ciliated pseudostratified respiratory epithelium
Morphology of bronchogenic cysts
Unilocular (2-10 cm)
May contain normal bronchial elements (smooth muscle and cartilage)
Maybe filled with blood, mucus, milky fluid, pus (if infected)