Bronchopulmonary sequestration (BPS) consists of nonfunctional lung tissue that has its own blood supply. In BPS, the abnormal lung tissue does not communicate with the tracheobronchial tree.
What is bronchopulmonary sequestration (BPS)?
Bronchopulmonary sequestration (BPS) consists of nonfunctional lung tissue that has its own blood supply. In BPS, the abnormal lung tissue does not communicate with the tracheobronchial tree. The sequestered lung may lie within the fetal chest, or in some instances, in the abdominal cavity. A characteristic feature of BPS is the identification of a systemic blood supply, usually from the aorta. Not all cases of BPS result in compromise of the pregnancy. However, in some cases, the growth of the tissue within the fetal chest hinders the normal development of the normal lung. In such cases, arrest of normal lung development, also called pulmonary hypoplasia, may result, with an increased risk for neonatal death. Pulmonary hypertension may also result from the lung compression. In some cases, the growth of the BPS tissue may result in obstruction of the lymphatic return to the chest, causing non-immune hydrops.
How is BPS diagnosed?
BPS is diagnosed antenatally with ultrasound by showing a hyperechogenic (white) lung tissue, associated with a blood vessel usually from the aorta. The normal lung tissue may be compressed, and the heart position may also be shifted. Features of nonimmune hydrops, including ascites, skin edema, pleural effusion, and others, may also be present.
Are there different degrees of BPS?
BPS patients can be classified as having a good or a poor prognosis. Patients with a poor prognosis are those in which expectant management is more likely to result in loss of the pregnancy. Poor prognosis findings include:
How can BPS be treated?
Depending on the gestational age at which BPS is diagnosed, delivery may be an option. If the diagnosis is made far from viability, different treatment options have been proposed, including:
Our group specializes in the assessment, counseling and management of patients with high-risk pregnancies in Florida.