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Key Points

  • Bronchopulmonary sequestration (BPS) is a mass of nonfunctioning lung tissue that does not communicate with the bronchial tree.

  • Prenatal diagnosis is possible by noting an echodensetriangulararea of tissue, often with an obvious systemic feeding vessel, such as from the descending aorta.

  • BPS can be intralobar (same pleural cover as normal lung) or extralobar (separate pleural cover), and can be intrathoracic or extrathoracic.

  • Main differential diagnosis for intrathoracic BPS includes type III CCAM, teratoma, and diaphragmatic hernia, while the differential for intra-abdominal BPS includes mesoblastic nephroma and neuroblastoma.

  • At least 75% of prenatally diagnosed cases of BPS resolve spontaneously, while those associated with hydrops, pleural effusions, or mediastinal shift have a much worse prognosis.

  • Fetal intervention by means of thoracoamniotic shunting may be an option for cases with coexisting pleural effusions prior to 30 weeks' gestation.

  • For large lesions that persist prenatally, and for those associated with hydrops, delivery should occur in a tertiary care center.

  • Elective surgical excision is recommended for most cases of asymptomatic BPS that persist postnatally.


Bronchopulmonary sequestration (BPS) is a mass of nonfunctioning pulmonary tissue that lacks an obvious communication with the tracheobronchial tree and receives all or most of its blood supply from anomalous systemic vessels (Carter, 1959). There appears to be a spectrum of sequestration with, at one extreme, an abnormal vessel supplying a nonsequestered lung and, at the other extreme, abnormal pulmonary tissue but without anomalous vascular supply. Terminology has become increasingly complicated with terms such as congenital bronchopulmonary foregut malformation (CBPFM) and malinosculation used (Gerle et al., 1968; Heithoff et al., 1976; Clements and Warner, 1987). CBPFM refers to intralobar or extralobar BPS associated with a communication with the gastrointestinal tract. The spectrum of CBPFM includes intralobar sequestration, extralobar sequestration, congenital cystic adenomatoid malformation (CCAM), bronchogenic cyst, Scimitar syndrome, and duplication cyst. Malinosculation describes the spectrum of congenital lung anomalies, in which there is abnormal connection of one or more of the four major components of the lung tissue (Clements and Warner, 1987). While much emphasis has been placed in the past in differentiating between BPS and CCAM, it is now clear that both can coexist in the same lesion, where it is referred to as a hybrid lesion. Extralobar sequestration and CCAM type II coexisting together has been reported in 25% to 50% of extralobar sequestration cases (Conran and Stocker, 1999) (see Chapter 35).

There are two forms of BPS: intralobar and extralobar. Intralobar is the more common malformation seen in infants and children, accounting for 75% of cases of BPS, and it shares the same pleural investment with the normal lung (Savic et al., 1979; Collin et al., 1987). Extralobar BPS accounts for 25% of cases in infants and children, has a separate pleura from the lung, and may be either intrathoracic ...

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