History

Definition

Pulmonary sequestration is a mass of nonfunctional, most often cystic tissue that does not communicate with the normal tracheobronchial tree. It has a systemic blood supply. It accounts for about 6% of all congenital lung malformations. >90% are in the lower lobes. ILS is more common and accounts for 75-85% of sequestrations -  ELS accounts for 15-25%.

2 types:

1. Extralobar (25%) - Has its own pleural covering. It may lie in a subdiaphragmatic position in as many as 10% of patients.
2. Intralobar (75%)

Epidemiology

Etiology

Possibly an accessory lung bud develops from the ventral aspect of the primitive foregut and migrates caudally with normal lung. The blood supply is directly from the aorta (systemic). Multiple supply arteries are found in 15% of sequestrations; 73% have blood supply from the abdominal aorta, and 18% from the thoracic aorta. Venous drainage also occurs most commonly via the systemic veins (75%), rather than pulmonary veins (25%). 

If these abnormality developmental events occur before the normal lung has developed (early) - intrapulmonary sequestration. If late, an extrapulmonary sequestration is formed. Some feel that ILS is congenital and ELS is acquired.

Intrapulmonary sequestration does not  communicate with the tracheobronchial tree or foregut. The most common site is posterior basal. Two-thirds of intrapulmonary sequestrations appear in the left lung.  

Extrapulmonary sequestration does not  communicate with the tracheobronchial tree, but foregut communication is more common. Almost all extrapulmonary sequestrations are in the left lung. 

Associations

Associated anomalies are uncommon (10%) in ILS, but CDH, CCAMs and lesions are seen in about 50% of cases of ELS.

Differential Diagnosis

CDH, CCAM, lung abscess, neoplasia, round atelectasis,  and pulmonary infarction may mimic pulmonary sequestration.

Genetics

Miscellaneous

Fetal hydrops is a complication of ELS  associated with a high perinatal mortality rate and severe respiratory distress at birth.

Diagnosis

ILS is usually identified in later childhood or adulthood. Neonates and infants are usually asymptomatic. However, most ELS are diagnosed in patients younger than 1 year, perhaps as a result of other anomalies such as CDH, cardiac malformations, and GI malformations.

ILS usually presents with infection - resolution of infection is hampered by the inadequate bronchial drainage. Mass effects from over distention can occur as a result of aeration through the pores of Kohn. Rarely, a systolic bruit or continuous murmur is heard over the affected area, from flow through the sequestration from the systemic arterial supply.

Chest radiography usually reveals a dense opacity in the posterior basal segment of the lower lobe, but a cystic appearance may also be seen. Bronchography, bronchoscopy, and arteriography are not usually indicated. CT angiography with 3-D reconstruction or MRI is helpful.

Treatment

ILS usually requires lobectomy (open or thoracoscopic) since the margins of the lesion are indistinct. Complete thoracoscopic resection of pulmonary lobes in infants and children has been described with low mortality and morbidity. ELS can be excised. The anomalous blood supply in both types must be identified early and controlled to prevent intraoperative hemorrhage.

Table

References

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