Bronchopulmonary sequestration is a rare congenital abnormality of the lower respiratory tract. It leads to a non-functioning mass of lung tissue in the unborn baby that lacks a connection to the body’s normal airways. In severe cases, BPC may become life-threatening, making early detection extremely important.
- What is Bronchopulmonary Sequestration?
Bronchopulmonary sequestration occurs when a mass of abnormal lung tissue forms in the fetal lungs. In some cases, the mass may may not cause any problems for the unborn baby and can be removed after birth. The mass may even shrink on its own.
Other times, however, the mass may cause extra fluid to build up inside the baby’s chest and abdomen. Under these circumstances, bronchopulmonary sequestration may become life-threatening, which is why early detection is so important.
A BPS mass usually appears next to the lung (extralobar) or within one part of the lung (intralobar). The mass is supplied with blood by a “feeding” vessel, such as the pulmonary artery, which helps it grow. As it increases in size, the mass may cause amniotic fluid to accumulate in the chest or abdomen of the baby, making intervention necessary.
Early detection and diagnosis of BPS is critical to promote the best outcome possible. The maternal-fetal medicine specialists of Fetal Care Center Dallas are highly skilled in treatment and care for BPS. We will evaluate the severity of your baby’s condition and make treatment recommendations using the most advanced resources.
- How We Treat BPS
When a lung/chest mass is identified, a detailed ultrasound is needed to evaluate your baby for other potential conditions. An amniocentesis may be recommended to test for a genetic/chromosomal abnormality.
When a bronchopulmonary sequestration is detected, the high blood flow through the tumor can lead to excess fluid accumulation in the chest cavity (hydrops) and even heart failure.
In some cases of BPS, other abnormalities can be seen in and around the chest and upper abdomen, including compression of lung tissue or pushing of the heart into an abnormal position. Depending on the size of the mass, there may also be incomplete development or underdevelopment of the baby’s lungs, which could significantly impact the ability to breathe after delivery.
Small- or moderate-sized pulmonary sequestrations that don’t show much change during the pregnancy are generally managed after birth, usually with surgery, to remove the abnormal lung tissue. These babies typically do not have any difficulty during pregnancy or after birth.
- Fetal Interventions
There are times when treatment before birth may be necessary to promote enough lung growth so your baby is capable of surviving and thriving after delivery. Our multidisciplinary team will work with your family to put together a care plan that is tailored specifically to you and your baby.
For Healthcare Providers
- BPS: Introduction
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.
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 or subdiaphragmatic in location (Savic et al., 1979; Collin et al., 1987).
The most widely accepted theory about the embryogenesis of BPS is that a supernumerary lung bud arises caudal to the normal lung bud and migrates caudally with the esophagus. If this lung bud arises prior to the development of the pleura, the bud is invested with adjacent lung and becomes an intralobar BPS. If supernumerary development occurs subsequent to pleura formation, the bud will grow separately and become invested with its own pleura, forming an extralobar BPS (Carter, 1959).
- Incidence of BPS
BPS is seen in 0.8% to 1.4% of all pulmonary resections (Carter, 1959). There is no familial predisposition. There is a slight male predominance, which is more obvious in extralobar BPS (male to female ratio of 3:1) compared with intralobar BPS (male to female ratio of 1.5:1) (Warner et al., 1958; Carter, 1959; Williams and Enumah, 1968). Extralobar BPS is much more common in the fetus and neonate than intralobar BPS (Buntain et al., 1977; Moulik et al., 1987; Panicek et al., 1987; Felker and Tonkin, 1990; Sauerbrei, 1992).
Intralobar BPS is located within the lower lobe in 98% of cases. Extralobar BPS is usually located in the posterior lower chest and 90% of extralobar BPS is located on the left side. Up to 15% of extralobar BPS can be found either within or below the diaphragm (Berrocal et al., 2004).
- Sonographic Findings
BPS is a solid, highly echogenic mass (Figure 34-1) with a clearly defined systemic feeding vessel (Figure 34-2). There have now been many reports of the prenatal sonographic diagnosis of BPS (Newman, 1970; Jaffe et al., 1982; Romero et al., 1982; Jouppila et al., 1983; Kritstoffersen and Ipsen, 1984; Mariona et al., 1986; Thomas et al., 1987; Adzick et al., 1998; Baumann et al., 1988; Davies et al., 1989a; Morin et al., 1989; Siffling et al., 1989; Weinbaum et al., 1989; Slotnick et al., 1990; Stern et al., 1990; Boiskin et al., 1991; Heranz-Schulman et al., 1991; Dolkhart et al., 1992; Eisenberg et al., 1992; Sauerbrei, 1992; Luetic et al., 1995). The sonographic hyperechogenicity of BPS is thought to result from the interfaces created by numerous dilated bronchioles (Jaffe et al., 1982). The demonstration of the systemic blood supply to the mass by color Doppler sonography usually confirms the diagnosis (Figures 34-2 to 34-4). Occasionally, these vessels cannot be demonstrated sonographically, making it difficult to distinguish BPS from type III congenital cystic adenomatoid malformation (CCAM) of the lung. Even if an anomalous systemic blood supply to a thoracic mass can be demonstrated, which confirms the diagnosis of BPS, intralobar and extralobar BPS usually cannot be distinguished by prenatal ultrasound. Several cases of intra-abdominal extralobar BPS have been reported prenatally because of the finding of echogenic suprarenal abdominal masses (Mariona et al., 1986; Baumann et al., 1988; Davies et al., 1989b; Dolkhart et al., 1992).
Additional sonographic findings seen in association with BPS include pleural effusion, mediastinal shift, hydrops, and polyhydramnios (Morin et al., 1989, 1994a, 1994b; Gross et al., 1992). Extralobar BPS may undergo torsion of its vascular pedicle, causing venous and lymphatic obstruction, leading to pleural effusion and hydrops due to systemic venous obstruction (Vode and Kramer, 1989; Morin et al., 1994a). Fetal hydrops may result from a compressive effect of the sequestration on the inferior vena cava with venous obstruction and compromised cardiac output. Polyhydramnios may be seen in association with BPS due to esophageal obstruction or decreased swallowing. BPS associated with hydrops uniformly results in fetal or neonatal death if untreated. There have been anecdotal reports of cases treated successfully in utero by thoracoamniotic shunting of the associated pleural effusion. To date, open fetal surgery has not been reported for a BPS in contrast to hybrid CCAM.
In postnatal series of BPS, there is a high incidence of associated anomalies, especially in extralobar BPS (60% of cases) (Warner et al., 1958; Carter, 1959; Gerle et al., 1968; Sade et al., 1974; Buntain et al., 1977; Savic et al., 1979; Collin et al., 1987). The most commonly associated anomalies include congenital diaphragmatic hernia (CDH), pectus excavatum, tracheoesophageal fistula, esophageal duplication, and congenital heart disease (Warner et al., 1958; Carter, 1959; Gerle et al., 1968; Buntain et al., 1977). The intralobar type has a lower incidence of associated anomalies (10% of cases) (Carter, 1959; Gerle et al., 1968; Sade et al., 1974; Buntain et al., 1977).
- Differential Diagnosis
The differential diagnosis of intrathoracic BPS includes type III CCAM, mediastinal or thoracic teratoma, and CDH (Moulik et al., 1987; Morin et al., 1994a) (Table 34-2). Type I or II CCAMs have a characteristic cystic appearance that clearly distinguishes them sonographically from BPS, while type III CCAMs have a dense hyperechoic appearance that may be indistinguishable from BPS. Mediastinal teratomas usually have a higher density, causing acoustic shadowing behind the mass (Golladay and Mollitt, 1984). Prenatal diagnosis of BPS may be quite difficult. In a review of cases diagnosed antenatally, only 29% were diagnosed correctly (Dolkhart et al., 1992). The other cases of BPS were documented variously as tumor, diaphragmatic hernia, CCAM, neuroblastoma, collapsed lung, and abdominal mass (Siffling et al., 1989; Dolkhart et al., 1992). The distinction between CCAM and BPS when a systemic feeding vessel is not demonstrated usually comes down to the echotexture of the mass. The presence of cysts suggests CCAM, whereas solid triangular lesions are more consistent with BPS, especially in the lower thoracic region. In lesions that are without cysts, it may not be possible to distinguish type III hybrid CCAM from BPS.
The main considerations in the differential diagnosis of intra-abdominal extralobar BPS are mesoblastic nephroma and neuroblastoma (see Chapters 112 and 113) (Mariona et al., 1986; Baumann et al., 1988; Davies et al., 1989b; Ohnichi et al., 1989; Weinbaum et al., 1989; Sauerbrei, 1992). Intra-abdominal extralobar BPS can occur on as a discrete suprarenal echogenic mass with a systemic blood supply. This mass can be mistaken for neuroblastoma or mesoblastic nephroma (Ohnichi et al., 1989; Oh et al., 1993). However, mesoblastic nephroma can usually be seen arising from the kidney (Ohnichi et al., 1989). Neuroblastomas arising from the adrenal glands are most commonly cystic lesions, which distinguish them from BPS (Oh et al., 1993). There has been one case of gastric duplication in association with BPS that gave a cystic appearance to the intra-abdominal BPS (Thilenius et al., 1983).
- Antenatal Natural History
The natural history of BPS depends on whether it is an intralobar or an extralobar BPS, whether it has a thoracic or abdominal location, and the presence or absence of hydrops and other associated anomalies (Adzick et al., 1993). Older reports suggested survival among cases of prenatally diagnosed intrathoracic BPS of only 36% (van Nayenberg, 1914; Williams and Enumah, 1968; Newman, 1970; Buntain et al., 1977; Rodgers et al., 1986; Panicek et al., 1987; Felker and Tonkin, 1990; Stern et al., 1990). However, a more recent experience reported a survival of 95% (Adzick et al., 1998). This difference likely reflects the fact that today’s more advanced ultrasound techniques are picking up milder cases that would never have been apparent prenatally in the past, as well as the fact that older case reports described successful prenatal diagnosis in only the most severe cases. BPS associated with hydrops is uniformly fatal if untreated (Weiner et al., 1986). The survival rate for BPS associated with pleural effusions was 22%, but the only survivors were those in whom the pleural effusions were decompressed in utero by thoracoamniotic shunts (Kritstoffersen and Ipsen, 1984; Boiskin et al., 1991; Dolkhart et al., 1992). The survival rate for BPS associated with polyhydramnios was only 30%.
Our understanding of the natural history of this lesion when diagnosed prenatally is still evolving. It was once thought that in fetuses with BPS, hydrops invariably developed and the fetus died in utero or during the neonatal period (Warner et al., 1958; Williams and Enumah, 1968). However, MacGillivray et al. (1993) subsequently reported six cases of BPS associated with contralateral mediastinal shift that dramatically decreased in size over the course of the pregnancy and spontaneously resolved, leading to a good neonatal outcome. Postnatally, the lesions could be detected only by computed tomographic (CT) scan or magnetic resonance imaging (MRI). There were no signs of hydrops in any of these cases. Adzick et al. (1994, 1998) subsequently reported that 75% of cases of BPS diagnosed prenatally resolve spontaneously. The mechanism by which these lesions shrink is unknown. Adzick (1993) speculated that the lesions may decompress themselves into the normal tracheobronchial tree or that the lesions outgrow their vascular supply and partially involute (Rodgers et al., 1986). Another possibility is that the “disappearing” malformations are actually extralobar BPSs that undergo complete torsion about their vascular pedicle and infarct (Morin et al., 1994a).
Intra-abdominal extralobar BPS has an outcome that is somewhat better than that for intrathoracic lesions and is rarely associated with hydrops. However, polyhydramnios may still develop secondary to esophageal or gastric compression.
The BPS associated with hydrothorax is usually of the extralobar type. It is thought that torsion of the extralobar BPS about its vascular pedicle may result in venous and lymphatic obstruction, causing fluid accumulation in the ipsilateral hemithorax (Golladay and Mollitt, 1984, Coleman et al 2016). Tension hydrothorax in the fetus with BPS is fatal unless there is intervention. An alternative explanation for nonimmune hydrops, especially in intralobar BPS, is the “left-to-left” shunting that may occur due to anomalous systemic artery and venous drainage via the pulmonary veins (Thilenius et al., 1983). This “left-to-left” shunt, which occurs in no other clinical entity, results in high-output failure and nonimmune hydrops (White et al., 1974).
- Management of Pregnancy
The fetus with an echodense chest mass should be evaluated to exclude CDH, mediastinal teratoma, and CCAM. In most cases of BPS, the vascular supply from the aorta can be defined with color Doppler studies (see Figure 34-2). The adrenal glands and kidneys should be delineated to distinguish an abdominal extralobar BPS from mesoblastic nephroma and neuroblastoma. It is noteworthy that most cases of BPS diagnosed prenatally have been isolated sequestrations without associated anomalies (Felker and Tonkin, 1990). Despite this observation, it is recommended that a detailed sonographic survey for possible associated anomalies be performed in every case.
Ultrafast fetal MRI may be very useful in sorting out the differential diagnosis, demonstrating the feeding vessels and excluding other potential associated anomalies. Normal fetal lungs are homogenous and, on MRI, have a relatively high T2 signal intensity because they are filled with amniotic fluid. A sequestration cyst typically appears as a well-defined mass in the chest with a T2 signal intensity that is higher than that of the normal lung (Hubbard et al., 1999). The efficacy of MRI for detecting systemic feeding vessels is no better than that of Doppler ultrasonography. However, MRI has some advantages over ultrasound in differentiating complex cases associated with other anomalies (Table 1).
A fetal karyotype should be obtained to exclude associated chromosomal anomalies if they might influence the decision to continue the pregnancy or for cases in which fetal treatment is contemplated (see Table 34-2). Because of the reported association of BPS with congenital heart disease, fetal echocardiography should be performed (White et al., 1974). The family may choose not to continue the pregnancy if the diagnosis of BPS is made prior to 24 weeks and there are other life-threatening anomalies.
Arrangements should be made for delivery at a center with appropriate neonatal and pediatric surgical expertise. The fetus with isolated BPS, of either the intrathoracic or intraabdominal type, has a good chance of survival in the absence of hydrops, polyhydramnios, or pleural effusion and when there is a planned delivery at an appropriately staffed facility with immediate resuscitation and surgery available. BPS may regress in size in 75% of cases, even when it is associated with mediastinal shift (MacGillivray et al., 1993; Adzick et al., 1995).
- Fetal Intervention
The management of intrathoracic BPS in the fetus with hydrops depends on the gestational age (Figure 34-5). Fetuses at 30 weeks’ gestation or more should be considered for early delivery and resection ex utero. The fetus with hydrops and intrathoracic BPS diagnosed prior to 30 weeks’ gestation may however be a candidate for fetal intervention. Hydrops may be seen in cases of BPS with tension hydrothorax, causing mediastinal shift, compromised venous return to the heart, and cardiac output. Coleman et al, reported a case of intermittent torsion of a BPS associated with tension hydrothorax in a twin pregnancy diagnosed by fetal MRI (Coleman et al 2016). This case was successfully treated by interstitial laser photocoagulation of the systemic feeding vessel and aspiration of the hydrothorax. The hydrous resolved, the hydrothorax did not recur and the babies survived.
The development of a large pleural effusion in association with BPS is a fairly common scenario thought to be due to partial or intermittent torsion of the extralobar BPS about its vascular pedicle. Pleural effusion does not occur in association with intralobar BPS. In the setting of tension hydrothorax and hydrops BPS can be fatal. The usual approach to treatment of this condition has been the ultrasound guided placement of a thoracoamniotic shunt. While this does not address the underlying cause of the pleural effusion it does relieve the intra-thoracic pressure caused by the pleural effusion and is effective in keeping the chest decompressed.
A more recent advance in the treatment of BPS associated with tension hydrothorax is the use of interstitial laser photocoagulation of the systemic feeding vessel as first described by Witlox et al (Witlox et al 2007). Under ultrasound guidance an 18 gauge needle is advanced into the BPS in direct proximity to the feeding vessel. The tip of the needle must be within the mass of the BPS to prevent heat generated by the laser from being dissipated and perforation of the vessel with bleeding into the pleural cavity. After successful laser photocoagulation of the systemic feeding vessel, the laser fiber is withdrawn and the needle is advanced into the pleural effusion to drain it relieving the intra-thoracic pressure allowing the hydrops to resolve. Because the systemic feeding vessel is occluded there is no longer any blood flow to the BPS and the pleural effusion does not recur. In most cases the BPS infarcts and involutes but in some cases a small residual bit of tissue may remain which can be surgically removed at an elective postnatal procedure.
In a review of reported cases of BPS associated with tension hydrothorax we found a total of 18 cases and an additional 3 cases of our own treated with ultrasound guided interstitial laser (Witlox et al 2007, Ruano et al 2012, Coleman et al 2016). Of the 21 cases, 20 (95%) were successful in photocoagulating the systemic feeding vessel, resolution of hydrops and postnatal survival.
Thoracoamniotic shunting in these cases may correct the pleural effusion, mediastinal shift, polyhydramnios, and hydrops. Shunting is not an option for the fetus with intrathoracic BPS associated with mediastinal shift and hydrops from a large thoracic mass without pleural effusion. Because BPS associated with hydrops is uniformly fatal, fetal surgery should be considered in these cases.
The prognosis for isolated intra-abdominal BPS is better than that for intrathoracic BPS because the intra-abdominal location does not result in pulmonary hypoplasia. Polyhydramnios may be observed due to compression of the esophagus or stomach, which may cause preterm labor and delivery. The role of reduction amniocentesis remains undefined but may be considered in such cases.
- Treatment of The Newborn
Ideally, a fetus diagnosed with a large BPS should be delivered in a setting where vigorous resuscitation and appropriate therapy for a newborn with pulmonary hypoplasia can be initiated immediately. In contrast, small lesions do not require a change in delivery plans, and delivery in a community setting may be appropriate. The infant should be examined carefully to confirm the presence or absence of associated anomalies. There is a wide range of severity with BPS; the degree of pulmonary hypoplasia is the primary determinant of outcome. The newborn with intraabdominal BPS usually has no respiratory compromise and can undergo elective resection. The treatment of the newborn with an intrathoracic BPS is determined by the severity of pulmonary hypoplasia. Therapeutic needs may vary from minimal (not requiring ventilatory support) to severe [requiring ventilatory and vasopressor support, high-frequency oscillatory ventilation, and/or extracorporeal membrane oxygenation (ECMO)]. Large pleural effusions should be treated immediately by tube thoracostomy. In the infant with pulmonary hypoplasia secondary to BPS, thoracotomy should be deferred until it is clear the infant has stabilized and the pulmonary hypertension has improved as in the management of CDH (Hazebrock et al., 1989; Langer et al., 1989, Deeney et al 2018). It is not uncommon for the infant’s condition to transiently deteriorates after surgery because of changes in chest wall compliance, pulmonary vascular resistance, and pulmonary hypertension superimposed on pulmonary hypoplasia.
- Surgical Treatment
The surgical approach to BPS is straightforward, with the exception of the management of anomalous blood supply. These vessels are often huge, thin-walled, and elastic, rather than muscular, arteries. In 20% of cases, these vessels are subdiaphragmatic in origin; in 15%, more than one vessel is present (Carter, 1959). Subdiaphragmatic origin of anomalous vessels is more common with right-sided lesions (Gottrup and Lund, 1978). These vessels can retract into the mediastinum or diaphragm and continue to bleed. Intraoperative death due to hemorrhage from unrecognized anomalous vessels has been reported (Harris and Lewis, 1940). Of note, one series reported that 60% of right-sided intralobar sequestrations had anomalous venous return compatible with the Scimitar syndrome (Collin et al., 1987). The importance of preoperative assessment of venous drainage, as well as arterial supply, is underscored by the reports of postoperative fatalities due to ligation of anomalous veins that constituted the sole or major venous drainage of the entire ipsilateral lung (O’Mara et al., 1978; Thilenius et al., 1983).
In the rare case of the prenatally diagnosed BPS that appears to regress, postnatal imaging studies should be obtained. If the lesion is evident on plain chest radiography, surgical resection should be planned. If chest radiography does not demonstrate the malformation, a CT or MRI scan should be performed. Even though these lesions are asymptomatic, postnatal resection should be considered because of the risks of infection, hemorrhage, and malignant transformation (Elias and Aufses, 1960; Juettner et al., 1987). When cardiac decompensation is the result of BPS, embolization of the feeding vessels may be considered. This technique can be used either as definitive treatment or in combination with resection. Complications reported after embolization include pain and pyrexia, pleural effusion, transient ischemia of the lower limb, recanalization of the artery, and persistent chest radiography changes (Corbett et al., 2004).
The resection of BPS can be performed using minimally invasive techniques, either muscle sparing thoracotomy or thoracoscopy with minimal morbidity and no mortality. Even bilateral BPS can be resected by a single operation utilizing traction the systemic artery once divided from the aorta eliminating the need for bilateral procedures (Deeney et al 2016).
- Long-Term Outcome
The resection of the intra-abdominal extralobar BPS has no affect on the pulmonary parenchyma, and the operative risks and long-term complications are the same as those for laparotomy in the newborn. The long-term outcome of intrathoracic BPS is determined by the extent of pulmonary hypoplasia. In extralobar BPS, resection results in no loss of pulmonary parenchyma. Resection of intralobar BPS requires at least segmentectomy or lobectomy, and the loss of pulmonary tissue may compound the underlying pulmonary hypoplasia in the short term. In the long term, removal of the BPS will provide room for compensatory lung growth in the remaining pulmonary tissue. It has been suggested that infants treated for BPS are at increased risk for gastroesophageal reflux, pneumonia, and pectus exconation (Corbett et al., 2004).
- Genetics and Recurrence Risk
There is no known genetic predisposition to the development of BPS. Although BPS is not known to have a familial pattern of recurrence and occurs as a sporadic anomaly, there has been a case reported of BPS recurring in male siblings (Abuhamed et al., 1996).
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Figure 34-1. Ultrasound of the fetal chest at 23 weeks’ gestation, demonstrating a homogeneous echodense triangular wedge-shaped thoracic mass, consistent with either type III cystic adenomatoid malformation or bronchopulmonary sequestration.
Figure 34-2. Color flow Doppler examination of the same fetus as in Figure 34-1, demonstrating systemic blood supply to the mass arising from the descending aorta, confirming the diagnosis of bronchopulmonary sequestration. (Reprinted, with permission, from Morin L, Crombleholme TM, Louis F, et al. Bronchopulmonary sequestration: prenatal diagnosis with chinisopathologic correlation. Curr Opin Obstet Gynecol. 1994b;6:479-481.)
Figure 34-3. Autopsy confirmation of the systemic blood supply to an intralobar bronchopulmonary sequestration. The vessel is demonstrated by the probe.
Figure 34-4. Photograph taken at autopsy, demonstrating systemic blood supply arising from the descending thoracic aorta to supply an extralobar BPS.
Figure 34-5. Algorithm for the management of fetal chest masses.