flashpath - lung - pulmonary sequestration
TRANSCRIPT
FLASHPATHH A Z E M A L I
PULMONARY SEQUESTRATI
ONH A Z E M A L I
CLINICAL• Congenital malformation
• Nonfunctioning lung lobe or segment
• Not connected to the airway system
• Not supplied by pulmonary arteries– Receives blood supply from systemic (aortic) branches
• Either Extra-lobar (outside the lung) or Intra-lobar (within the lung)
CLINICALExtra-lobar Intra-lobar
Incidence Less common More commonAge of presentation Infants
(below 1 year)Older children and
even AdultsGender Male > Females Equal
Foregut communication and associated anomalies
Very Commone.g. diaphragmatic
hernia
Very rare
Chronic inflammation, fibrosis and Honeycomb changes
Very rare Very Common
GROSSExtra-lobar Intra-lobar
Mass Solid, Spongy mass
Solid, Fibrotic mass has multiple,
variable-sized cystic areas
Location Outside the lunganywhere above, within, or below the diaphragm
Within the lung
Side Mainly left side Mainly left lung(lower lobe)
Pleura Its own “separate” pleural covering
The same pleural covering of the lung
Venous drainage Systemic (azygous) Pulmonary
GROSS
Intra-lobarsequestration
MICROSCOPY• Intra-lobar:
– Marked chronic inflammation, fibrosis, cystic changes• May be because it can be undiagnosed for long time, it shows a higher risk for recurrent infections and fibrosis
– Not associated with CPAM type 2 (Congenital pulmonary airway malformation)
• Extra-lobar:– No significant inflammation or fibrosis
• May be because it is often discovered early in life, there is no time to develop infections or fibrosis
– Associated with (CPAM) type 2 in 50% of cases• Areas of irregular, markedly dilated bronchioles within parenchyma
DIFFERENTIAL DIAGNOSISO t h e r c y s t i c l u n g d i s e a s e s :• Congenital:
– Bronchogenic cysts– Congenital pulmonary cysts– Congenital pulmonary airway malformation– Congenital lobar emphysema
• Acquired:– Emphysema– Healed abscess– Honeycombing
• Mixed:– Cystic fibrosis
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