extralobar pulmonary sequestration

Definition: Extralobar pulmonary sequestration (ELPS) are segmentsof lung tissue ("accessory lungs") with a distinct pleural investment and complete anatomic and physiologic seprartion from the normal lung. Blood supply is usually by anomalous systemic artery with venous drainage into the azygos or hemizygos system.

The abnormal tissue may be located either above or below the diaphragm, most often on the left side.

Images

 Extrapulmonary sequestration w/ perfect anomalous penetrating pleural vessel. Histology shows malpositioned large vessels.

• https://twitter.com/hematogones/status/839861696755871745

Digital cases

 HPC:413 : Cystic congenital adenomatoid malformation in extralobar pulmonary sequestration

Localization

 right extralobar pulmonary sequestration (10%)
 left extralobar pulmonary sequestration (90%)
 infradiaphragmatic extralobar pulmonary sequestration

Variants

 bronchopulmonary sequestration supplied from the renal artery (2326448)

Associations

 congenital cystic adenomatoid malformation (CCAM) (10441623)

• inside CCAM
• outside CCAM

 renal anomalies

• cystic nephroma (16773421)

 diaphragmatic hernia

 malformative syndrome

• association anomalous pulmonary venous return, pulmonary sequestration, bronchial atresia, aplastic right upper lobe, pericardial defect and intrathoracic kidney (5944198)

Case records

 Case 10460

See also

 thoracic malformations
 pulmonary malformations