Bronchopulmonary Sequestration (BPS)

Bronchopulmonary sequestration is an uncommon type of congenital lung lesion.  

Known also as pulmonary sequestration, bronchopulmonary sequestration (BPS) is a rare birth defect in which a portion of the baby’s lung tissue develops without being attached to the airways. This nonfunctioning tissue mass receives its blood supply from the aorta (the body’s main artery) instead of the arteries that feed the lungs.

There are two main types of BPS:

  • Intralobular BPS – Comprising about 75% of total cases, intralobular BPS occurs when the nonfunctioning tissue develops inside a lung lobe.
  • Extralobular BPS – This type of BPS is characterized by nonfunctioning tissue that develops with its own pleural cover outside a lung lobe.

BPS Causes

The exact cause of bronchopulmonary sequestration isn’t clear, but male babies have a slightly higher chance of developing this condition than females. There is currently no known familial predisposition or genetic link for BPS.

BPS Symptoms

Bronchopulmonary sequestration usually does not cause serious problems for babies in utero. However, in rare cases, the tissue mass may push on the baby’s diaphragm or dislodge the heart from its original position, leading to life-threatening fetal complications such as heart failure.

After birth, a baby with BPS may display:

  • Difficulty breathing
  • Wheezing
  • Shortness of breath
  • Difficulty feeding
  • Frequent lung infections
  • Trouble gaining weight

The frequency and severity of these symptoms will vary depending on the size and location of the tissue mass. Some babies may not show any signs of BPS.

BPS Diagnosis

Bronchopulmonary sequestration often appears as a bright, white spot around the baby’s lungs during a routine pregnancy ultrasound. The ultrasound will also show a blood vessel supplying the tissue mass, which is a strong indicator of BPS.

BPS Treatments

Tampa General Hospital’s Women’s Institute provides a full spectrum of specialized, high-risk obstetrics care to mothers and babies with prenatal complications such as bronchopulmonary sequestration. Many cases of BPS—around 75%—shrink on their own and may not require treatment until after birth, which typically includes surgery to remove the tissue mass. In more severe cases, fetal surgery may be necessary for babies with BPS prior to 30 weeks. Some mothers may opt to deliver early if their baby is past 30 weeks and is at risk of experiencing BPS complications.