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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