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SEQUESTRATION
By – HAPPY MALIK (3rd course)
• INTRODUCTION
• PATHOGENESIS
• TYPES
• PATHOLOGY
• CLINICAL PRESENTATION
• ASSOCIATED ANOMALIES
• EVALUATION
• DIFFERENTIAL DIAGNOSIS
• TREATMENT
INTRODUCTION
• Bronchopulmonary sequestration (BPS) or sequestration is a
rare congenital abnormality of the lower respiratory tract.
• It consists of a non-functioning mass of lung tissue that lacks
normal communication with tracheobronchial tree and
receives its arterial blood supply from systemic circulation.
• Bronchopulmonary sequestrations constitute approximately
0.15-6.4 % of all congenital pulmonary malformations.
• Anatomically classified as
- Intra lobar Sequestration (ILS)
- Extra lobar Sequestration (ELS)
• INTRALOBAR SEQUESTRATION (ILS) : - 75 %
- Located within the normal lobe and does not have its
own visceral pleura.
• EXTRALOBAR SEQUESTRATION (ELS) : - 25 %
- Located outside the normal lung and has its own visceral
pleura.
RARE TYPES
- Hybrid BPS/CPAM lesions :- BPS occurs in
combination with CPAM. These hybrid lesions have
histological features of CPAM and blood supply from a
systemic artery.
- Bronchopulmonary-foregut malformation (BPFM)A
Rare variant of sequestration where sequestrated lung tissue is
connected to the gastrointestinal tract.
PATHOGENESIS
• The most frequently supported theory of sequestration
involves formation of accessory lung bud that develops from
the ventral aspect of the primitive foregut.
• The pleuripotent tissue from this additional lung bud
migrates in a caudal direction with the normally developing
lung.
• The accessory lung bud receives its blood supply from
vessels that connect to the aorta and cover the primitive
foregut.
• Early embryologic development of the accessory lung bud
in pseudoglandular stage (5-17weeks of gestation) results in
formation of the sequestration within normal lung tissue. This
sequestration is encased within the same pleural covering of
normal lung resulting in the Intrapulmonary OR Intralobar
sequestration
• Later development of the accessory lung bud results in the
Extralobar OR Extrapulmonary type that may give rise to
communication with the GI tract.
INTRALOBAR SEQUESTRATION
• ILS are located within a normal lobe and lack their own
visceral pleura.
• Accounts for 75% of BPS.
• Males and females are equally affected.
• Most occur in lower lobes, 60% located in posterior basal
segment of left lower lobe.
• The right lower lobe may be affected in approximately
onethird of cases
• Generally do not have communication with tracheobronchial
tree.
• Intralobar sequestration usually present in late childhood or
in young adults.
• Arterial blood supply is by lower THORACIC AORTA or
upper Abdominal aorta and Venous drainage is usually via
the pulmonary veins
• Infrequently, in 10% - 15% bronchopulmonary foregut
malformations and skeletal anomalies
(like scoliosis and rib anomalies) may be associated.
• Within visceral pleura of normal lobe
EXTRALOBAR SEQUESTRATION