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33_0 | bronchopulmonary-sequestration - CLINICAL PRESENTATION | The clinical presentation of BPS is variable and depends upon the type, size, and location of the lesion. Many cases are initially detected by routine prenatal ultrasound examination. Most affected newborns are asymptomatic. If symptomatic, BPS usually presents with respiratory distress in the neonatal period. Intralob... |
34_0 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Prenatal | On prenatal ultrasound, BPS appears as a homogenous echogenic thoracic mass, usually solid-appearing, triangular, and often located in the lower hemithorax adjacent to the diaphragm. The size of the lesion varies considerably, ranging from very small to one that occupies most of the hemithorax, causing mediastinal shif... |
34_1 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Prenatal | ly solid-appearing, triangular, and often located in the lower hemithorax adjacent to the diaphragm. The size of the lesion varies considerably, ranging from very small to one that occupies most of the hemithorax, causing mediastinal shift [ 34-38 ]. BPS may be difficult or impossible to distinguish from microcystic co... |
34_2 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Prenatal | renatal diagnosis' .) In the majority of cases, the lesion regresses during the course of gestation. Occasionally, hydrops develops, likely because of vascular compression [34,39,40]. There are no reliable criteria for determining which lesions will grow and develop hydrops versus those that will stabilize or regress [... |
35_0 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Neonatal period | Infants with BPS or other congenital abnormalities of the lower airway may be either asymptomatic or symptomatic at birth: Infants with BPS or other congenital abnormalities of the lower airway may be either asymptomatic or symptomatic at birth: ●Asymptomatic – Most infants with BPS are asymptomatic at birth. The lesio... |
35_1 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Neonatal period | ultrasound or identified incidentally during a postnatal evaluation for other congenital anomalies. ●Symptomatic – Some infants with prenatally diagnosed lung lesions present with respiratory distress at birth or shortly thereafter; this may occur with either ILS or ELS and is more likely if the lesion is large. The sy... |
36_0 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Postneonatal | Among infants who are asymptomatic at birth, some will become symptomatic later in infancy, childhood, or even adulthood, but the natural history and magnitude of risk is poorly delineated. (See Among infants who are asymptomatic at birth, some will become symptomatic later in infancy, childhood, or even adulthood, but... |
36_1 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Postneonatal | fe but come to attention when the lesion is detected as an incidental finding on a chest radiograph. Unfortunately, the percentage of those who will become symptomatic is not known, because the natural history of infants who are asymptomatic at birth is not well described. As a result, the optimal management of an asym... |
36_2 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Postneonatal | typically presenting with fever and cough and sometimes hemoptysis or chest pain (image 3) [33,42]. This is most likely for those with ILS, which comprise approximately 75 percent of BPS. Rare complications of either ELS or ILS include heart failure due to excessive flow through the aberrant artery [11,43], massive bl... |
36_3 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Postneonatal | to develop infection. Those that do present with infection tend to be hybrid lesions with CPAM [3]. It is clear that some patients with ELS will remain asymptomatic throughout life, but the likelihood of this outcome is unknown. |
37_0 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Associated anomalies | Congenital anomalies may be associated with BPS, occurring more frequently in patients with ELS than ILS. In a series of 28 cases in children and adults, associated malformations occurred in 43 percent of ELS and 17 percent of ILS [ Congenital anomalies may be associated with BPS, occurring more frequently in patients ... |
37_1 | bronchopulmonary-sequestration - CLINICAL PRESENTATION - Associated anomalies | S and 17 percent of ILS [ 6 ]. Similar proportions were seen in an older series of 540 cases [ 50 ]. Associated anomalies include congenital diaphragmatic hernia, vertebral anomalies, congenital heart disease, and colonic duplication [ 5,14 ]. Infants with a large BPS may have pulmonary hypoplasia due to mass effects i... |
39_0 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging | All cases of BPS or other congenital abnormalities of the lower airway should be further evaluated with imaging. This includes cases that regressed or appeared to resolve in utero because few lesions resolve completely and advanced imaging is more sensitive than prenatal ultrasound for detecting small lesions. All case... |
39_1 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging | BPS or other congenital abnormalities of the lower airway should be further evaluated with imaging. This includes cases that regressed or appeared to resolve in utero because few lesions resolve completely and advanced imaging is more sensitive than prenatal ultrasound for detecting small lesions. |
40_0 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Suggested protocol | After birth, the first step is a plain chest radiograph. The second step is advanced imaging, the timing of which depends on the patient's characteristics, as outlined in the algorithm ( After birth, the first step is a plain chest radiograph. The second step is advanced imaging, the timing of which depends on the pat... |
40_1 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Suggested protocol | aphy (CT) or magnetic resonance imaging (MRI) for infants with any of the following characteristics: •Any symptoms (eg, respiratory distress) •Large BPS (occupying >20 percent of the hemithorax on ultrasonography or plain radiographs) •Risk factors for pleuropulmonary blastoma (bilateral or multifocal cysts, pneumothor... |
40_2 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Suggested protocol | h surgical planning [51]. Doppler ultrasonography also may be helpful to define the aberrant artery. These techniques have replaced the need for angiography to identify the vascular supply (image 1B). ●Low risk– Infants who are asymptomatic and do not have the high-risk characteristics outlined above should have advan... |
41_0 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | ●Chest radiograph– On a chest radiograph, sequestrations typically appear as a uniformly dense mass within the thoracic cavity or pulmonary parenchyma (image 4) [52]. Recurrent infection can lead to the development of cystic areas within the mass [52,53]. Air-fluid levels due to bronchial communication are seen in 26 ... |
41_1 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | ar sequestration (ELS) sometimes occurs in a subdiaphragmatic location or as a retroperitoneal mass. The left hemithorax is almost always involved in ELS, and it is usually involved in ILS. (See'Anatomic characteristics'above.) ●CT– The parenchymal abnormalities associated with BPS are best visualized using CT, althoug... |
41_2 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | l, a collection of many small cystic lesions containing air or fluid, or a well-defined cystic mass. Emphysematous changes at the margin of the lesion are characteristic, although they may not be visible on the chest radiograph. Conventional CT does not consistently demonstrate the aberrant systemic artery, with visual... |
41_3 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | ch also enables evaluation of abnormalities in the lung parenchyma or airways (image 6) [54,56,57]. Advances in the use of multidetector CT angiography have improved our ability to simultaneously visualize the arterial supply, venous drainage, and parenchymal involvement of pulmonary sequestrations and may make this t... |
41_4 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | However, CT allows sharper delineation of thin-walled cysts and emphysematous changes than MRI [54]. Advanced imaging with either CT and MRI are adequate to evaluate most infants, and the choice between these techniques depends primarily on institutional or clinician preference. ●Ultrasonography– Ultrasonography is not... |
41_5 | bronchopulmonary-sequestration - EVALUATION - Postnatal imaging - -Radiographic appearance | characteristic aberrant systemic artery that arises from the aorta and to delineate venous drainage. In addition, ultrasonography can be used to guide biopsy of a subdiaphragmatic mass [54,55,61,62]. The typical sonographic appearance of BPS is an echogenic homogeneous mass that may be well defined or irregular [54]. H... |
42_0 | bronchopulmonary-sequestration - DIAGNOSIS | Extralobar sequestration (ELS) may be first suspected based on prenatal ultrasonography. Intralobar sequestration (ILS) is more often suspected in an older infant or child who presents with recurrent pulmonary infection. Both types of BPS may be identified as an incidental finding on a plain radiograph in an asymptomat... |
42_1 | bronchopulmonary-sequestration - DIAGNOSIS | magnetic resonance imaging [MRI]), if an aberrant systemic artery can be identified with confidence. Advanced imaging may help to distinguish among ILS, ELS, and hybrid lesions but is not completely reliable in making these distinctions. The final definitive diagnosis is only made by pathologic examination after surgic... |
43_0 | bronchopulmonary-sequestration - DIFFERENTIAL DIAGNOSIS | The differential diagnosis of BPS includes other cystic lung lesions, such as congenital pulmonary airway malformations (CPAM). In contrast with BPS, CPAMs are connected to the tracheobronchial tree and are supplied from the pulmonary circulation. Hybrid lesions, with features of CPAM and BPS, occur in a substantial pr... |
43_1 | bronchopulmonary-sequestration - DIFFERENTIAL DIAGNOSIS | se other lesions. (See'Hybrid BPS/CPAM lesions'above and"Congenital pulmonary airway malformation".) In addition to CPAM, lesions that may coexist with BPS or mimic BPS on prenatal ultrasound or postnatal plain radiographs include other space-occupying chest lesions, including: ●Congenital diaphragmatic hernia (see"Con... |
43_2 | bronchopulmonary-sequestration - DIFFERENTIAL DIAGNOSIS | omalies of the intrathoracic airways and tracheoesophageal fistula", section on 'Bronchogenic cyst') ●Mediastinal tumors such as a teratoma or neuroblastoma (see"Epidemiology, pathogenesis, and pathology of neuroblastoma") The radiographic appearances of other congenital abnormalities of the lung are discussed in detai... |
44_0 | bronchopulmonary-sequestration - MANAGEMENT | The approach to treatment depends upon whether the patient has symptoms (respiratory distress or recurrent infections) or is asymptomatic. |
45_0 | bronchopulmonary-sequestration - MANAGEMENT - Symptomatic patients | All patients with BPS who are symptomatic should undergo surgical excision, which is curative and is associated with minimal morbidity [ All patients with BPS who are symptomatic should undergo surgical excision, which is curative and is associated with minimal morbidity [ 31,41,63,64 ]. Surgery is performed urgently i... |
45_1 | bronchopulmonary-sequestration - MANAGEMENT - Symptomatic patients | t with recurrent infection, which is usually due to intralobar sequestration (ILS) ( algorithm 1 ). Advanced thoracic imaging (computed tomography [CT] or magnetic resonance imaging [MRI]) should be performed prior to surgery to confirm the diagnosis and assist in surgical planning. (See 'Postnatal imaging' above.) Co... |
45_2 | bronchopulmonary-sequestration - MANAGEMENT - Symptomatic patients | investment. With both types, all vascular connections to the lesion must be identified and ligated. The arterial supply of these lesions may arise from the subdiaphragmatic aorta, and careful identification of the feeding vessel is crucial. Thoracoscopic lobectomy is an alternative to thoracotomy in infants and older c... |
45_3 | bronchopulmonary-sequestration - MANAGEMENT - Symptomatic patients | initial therapy. Case series describe treatment using a variety of embolization techniques [68-73]. Lesions with multiple feeding arteries may require repeated procedures, making embolization less appealing. |
46_0 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients | Infants with suspected BPS who are asymptomatic at birth should be closely observed for the first few days of life as some may develop symptoms in the immediate postnatal period [ Infants with suspected BPS who are asymptomatic at birth should be closely observed for the first few days of life as some may develop sympt... |
46_1 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients | y asymptomatic, the decision between surgical management and observation is controversial [ 75,76 ]. Management decisions for these patients are similar to those for asymptomatic patients with other congenital abnormalities of the lower airways, the most common of which are congenital pulmonary airway malformations (CP... |
47_0 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -High risk | We suggest early surgical resection for patients with any of the following characteristics, which suggest increased risk for developing complications: We suggest early surgical resection for patients with any of the following characteristics, which suggest increased risk for developing complications: ●Large lesion (occ... |
48_0 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | For asymptomatic patients with none of the above characteristics on advanced imaging, either elective surgical resection or observation is a reasonable option ( For asymptomatic patients with none of the above characteristics on advanced imaging, either elective surgical resection or observation is a reasonable option... |
48_1 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | mily's preferences after a detailed discussion of the potential benefits and risks of each approach. ●Elective surgical resection– In our practice, we generally perform surgery for asymptomatic infants with BPS, even those considered at low risk for developing complications of BPS. We generally perform this elective su... |
48_2 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | old: First, surgery is curative and not generally associated with significant complications [75,77]. Second, individuals with BPS appear to have a moderate risk for developing infection sometime later in life, particularly if the lesion is an ILS [4,17,74]. When infection or respiratory symptoms do develop, surgery bec... |
48_3 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | ctive surgery performed in asymptomatic patients (17 versus 5 percent in one of these reports [78]). Third, imaging is not always able to distinguish between BPS and CPAM or hybrid lesions, and CPAM or hybrid lesions are associated with a risk of developing complications (infection or malignant degeneration) if they ar... |
48_4 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | later surgery. (See"Congenital pulmonary airway malformation", section on 'Asymptomatic patients'.) ●Observation– Some authors recommend observation rather than surgery for asymptomatic patients, particularly if the lesion is small, noncystic, and appears to be consistent with ELS [76,79]. If observation is chosen, bot... |
48_5 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | itoring of these patients, though there is no consensus on the optimal strategy for imaging [80,81]. Thus, the optimal management for asymptomatic low-risk infants remains unclear because of limited information about the natural history of BPS and difficulty establishing a definitive diagnosis by imaging. Indeed, most ... |
48_6 | bronchopulmonary-sequestration - MANAGEMENT - Asymptomatic patients - -Low risk | nce of complications varies widely in different reports, ranging from 3 to 86 percent [76,78,82,83]. The complications are primarily infection or respiratory symptoms, typically occur between 7 and 24 months of age, and require surgery. These analyses do not report specifically on the risks for complications associated... |
49_0 | bronchopulmonary-sequestration - OUTCOME | In the absence of other significant congenital anomalies, the prognosis for children with BPS is generally very good [6,77,80,81,84,85]. Most case series do not distinguish outcomes for BPS from those for other congenital abnormalities of the lower airway, particularly congenital pulmonary airway malformations (CPAM). ... |
49_1 | bronchopulmonary-sequestration - OUTCOME | ons (such as infection) varies widely in different reports, as discussed above (see'Low risk'above). Furthermore, most of the available data come from series in which most infants had CPAM rather than BPS. For symptomatic infants who undergo emergency surgery, at least 20 percent have postoperative complications, which... |
49_2 | bronchopulmonary-sequestration - OUTCOME | he lung resection. The remaining lung parenchyma undergoes compensatory growth and development [85]. These outcomes vary across different reports, probably reflecting differences in patient selection (eg, asymptomatic lesions identified on prenatal ultrasound and managed conservatively or with elective surgery versus s... |
50_0 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | ●Definition and types– Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the lower respiratory tract. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation. The connec... |
50_1 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | An intralobar sequestration (ILS) is located within a normal lobe and lacks its own visceral pleura. This type often has aberrant connections to bronchi, lung parenchyma, or the gastrointestinal tract and often presents with recurrent infections. •An extralobar sequestration (ELS) is located outside the normal lung and... |
50_2 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | n ELS with connections to the gastrointestinal tract or intrapulmonary structures, which is unusual. ●Associated anomalies– Congenital abnormalities that are sometimes associated with BPS include congenital diaphragmatic hernia, vertebral anomalies, congenital heart disease, pulmonary hypoplasia, colonic duplication, a... |
50_3 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | clinical presentation of BPS is variable and depends on the type, size, and location of the lesion. Many cases are initially detected by prenatal ultrasound; most of these regress during gestation, while others progress and hydrops may develop. The affected newborn is usually asymptomatic but sometimes presents with re... |
50_4 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | her congenital abnormalities of the lower airway should be further evaluated with postnatal imaging. This includes cases that regressed or appeared to resolve in utero because few lesions resolve completely and advanced imaging is more sensitive than prenatal ultrasound for detecting small lesions. (See'Postnatal imagi... |
50_5 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | areas within the mass, and there may be air-fluid levels if the lesion communicates with a bronchus. •The second step is advanced thoracic imaging, the timing of which depends on the patient's characteristics, as outlined in the algorithm (algorithm 1). This is to confirm the diagnosis, including identifying the aberr... |
50_6 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | al morbidity. The procedure is performed urgently in newborns with significant respiratory distress. Surgical resection is typically performed electively in older children who present with infection. (See'Symptomatic patients'above.) •Asymptomatic,high risk– For asymptomatic patients of any age with characteristics tha... |
50_7 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | (table 1)), we suggest surgical resection rather than observation (Grade 2C). (See'High risk'above.) •Asymptomatic, low risk– For asymptomatic patients without these high-risk characteristics, either elective surgical resection or conservative management with observation are reasonable options and practice varies (alg... |
50_8 | bronchopulmonary-sequestration - SUMMARY AND RECOMMENDATIONS | nd characteristics. The surgery is elective and is usually performed between 6 and 12 months of age. Our preference for surgery is based on the good outcomes after surgery and on the risk of developing complications (primarily infection) if surgery is not performed. The likelihood and risk factors for developing compli... |
52_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | ●Overview– Early lung injury in infancy due to bronchopulmonary dysplasia (BPD) can have lifelong consequences, manifested by altered pulmonary function, upper and lower airway disease, and pulmonary hypertension (PH). Infants with extremely low birth weight (ELBW; birth weight <1000 g) are especially vulnerable. (See'... |
52_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | especially vulnerable. (See'Respiratory disorders associated with bronchopulmonary dysplasia'above.) ●Pathogenesis of BPD– In the modern era, the most important feature of BPD is impaired alveolar development, with decreased septation and alveolar hypoplasia leading to fewer and larger alveoli and dysmorphic pulmonary ... |
52_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | that many affected individuals develop nearly normal lung function and pulmonary reserve with time. In infants who were born before the advent of modern neonatal intensive care unit management techniques, BPD is typically characterized by airway injury with inflammation and alveolar septal fibrosis, known as "classic" ... |
52_3 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | ee'New versus classic bronchopulmonary dysplasia'above.) ●Issues arising during long-term management •General care– To optimize lung function, it is important to minimize lung injury by avoiding recurrent respiratory infections, minimize feeding-related aspiration, and optimize nutrition, particularly during the first ... |
52_4 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | suggests obstructive lung disease, a trial of standard asthma management techniques is appropriate. Bronchodilators are effective in approximately one-half of these patients. Similarly, children with BPD may respond to inhaled corticosteroids, but the effect is less consistent than in children with asthma. In general, ... |
52_5 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | ciated PH– All infants with moderate or severe BPD should be screened for PH using echocardiography. For most infants, the initial echocardiogram should be performed at the time the formal diagnosis of BPD is made. Earlier screening should be performed for selected infants with severe respiratory symptoms or risk facto... |
52_6 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | a. (See"Pulmonary hypertension associated with bronchopulmonary dysplasia", section on 'Screening'.) •Central airway disease– Infants with BPD, and especially those with "classic" BPD, are at risk for central airway collapse due to tracheobronchomalacia, which can exacerbate underlying thoracic airway disease. Clinical... |
52_7 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SUMMARY AND RECOMMENDATIONS | g-term dependence on mechanical ventilation and/or tracheostomy. (See'Central airway disease'above.) ACKNOWLEDGMENT— The UpToDate editorial staff acknowledges Leslie L Harris, MD, and James M Adams, Jr, MD, who contributed to an earlier version of this topic review. |
53_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - INTRODUCTION | Despite important advances in perinatal care and a steady decline in mortality rates among very low birth weight (VLBW) infants (<1500 g) during the past two decades, bronchopulmonary dysplasia (BPD) remains a major complication of premature birth and is a significant cause of long-term morbidity. Prematurity and low b... |
53_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - INTRODUCTION | ing dysfunction and poor nutrition, which are common comorbidities in infants born very prematurely. Because of its chronic nature and multifactorial causes, many infants and children with BPD will require multifaceted and multidisciplinary management well beyond the first year of life. Limited guidelines for the manag... |
53_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - INTRODUCTION | ave been published by both the European Respiratory Society and the American Thoracic Society [2,3]. The long-term consequences of BPD on the respiratory health of older children and adults are not fully described, especially because new developments in the care of premature infants have resulted in important changes i... |
53_3 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - INTRODUCTION | ects of BPD are discussed in separate topic reviews: Diagnosis and management of BPD during infancy: ●(See"Bronchopulmonary dysplasia (BPD): Clinical features and diagnosis".) ●(See"Bronchopulmonary dysplasia (BPD): Prevention".) ●(See"Bronchopulmonary dysplasia (BPD): Management and outcome".) ●(See"Pulmonary hyperten... |
54_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION | A commonly used definition of BPD is the need for supplemental oxygen or positive pressure support for more than 28 days, with severity defined by further criteria depending on gestational age [1]. More recently, a National Institutes of Child Health and Human Development working group revisited this definition to incl... |
54_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION | n) that were not in common use at the time when the earlier definition was published (table 1) [6]. (See"Bronchopulmonary dysplasia (BPD): Clinical features and diagnosis", section on 'Definitions and severity of BPD'.) |
55_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | The clinical and histologic features of BPD have changed with the advent of new technologies and approaches to care, including surfactant administration, permissive hypercapnia, and noninvasive ventilation. |
55_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | s to care, including surfactant administration, permissive hypercapnia, and noninvasive ventilation. This has led to increased survival of extremely low birth weight (ELBW) infants and the evolution of a new type of BPD, with different pathogenesis and clinical features as compared with the classic form of BPD (see The... |
55_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | s to care, including surfactant administration, permissive hypercapnia, and noninvasive ventilation. This has led to increased survival of extremely low birth weight (ELBW) infants and the evolution of a new type of BPD, with different pathogenesis and clinical features as compared with the classic form of BPD (see "Br... |
55_3 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | nts became more possible after the advent of antepartum glucocorticoid use and surfactant treatment. Interestingly, some infants born very prematurely have little or no respiratory distress at birth but develop a need for supplemental oxygen and/or positive pressure support by 36 weeks postmenstrual age. BPD in these i... |
55_4 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | ]. The lungs are characterized by fewer and larger alveoli and dysmorphic pulmonary vasculature [8]. Dysregulation of signaling pathways, which are integral to lung development, likely contribute to the pathologic changes that are seen in the BPD lung [9]. The incidence of new BPD has not changed over the past two deca... |
55_5 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | such as less invasive surfactant administration, may lead to further improvements in outcomes [12]. ●Classic BPD– Before the advent of surfactant and more modern management techniques, the most prominent features of BPD were airway injury, inflammation, and alveolar septal fibrosis. These changes were usually associate... |
55_6 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | till occasionally seen, particularly in infants with BPD requiring long-term mechanical ventilation. Infants with classic BPD and who required prolonged mechanical ventilation were prone to develop severe tracheobronchomalacia, which often caused profound cyanotic episodes [13]. When these individuals reach adulthood, ... |
55_7 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - DEFINITION - New versus classic bronchopulmonary dysplasia | e interstitial and emphysematous changes detected by chest radiography and computed tomography [14]. It is likely that some of these adults will have a predisposition to accelerated loss of lung function or early-onset chronic obstructive pulmonary disease as they enter middle age; therefore, they should be followed cl... |
56_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION | Abnormalities in pulmonary function tests (PFTs) are commonly found in children and adults with BPD [4,16-18]. PFTs frequently show decreased forced expiratory volume in one second (FEV1) and decreased ratios of FEV1to forced vital capacity (FEV1/FVC), consistent with airflow limitation and small airway obstruction [16... |
56_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION | vital capacity (FEV1/FVC), consistent with airflow limitation and small airway obstruction [16,17]. The airflow limitation may be a consequence of dysanaptic growth, in which length and diameter of the airways grow less rapidly than the lung parenchyma, which in turn can cause fixed airflow obstruction [19-22].However,... |
56_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION | en shown to be abnormal in the child with BPD, and these changes can persist into adulthood [24,25]. Infants with BPD may manifest an inability to increase their ventilatory response to hypoxia. The mechanisms are multifactorial and may include abnormalities in the peripheral and central chemoreceptor response to hypox... |
57_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Infancy | PFTs during infancy are not routinely performed in most centers but can be useful when available in tracking changes in pulmonary function, response to bronchodilators and diuretics, and overall severity of disease, particularly in infants not responding well to supportive treatment [ PFTs during infancy are not routin... |
57_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Infancy | all severity of disease, particularly in infants not responding well to supportive treatment [ 28 ]. In one study of preterm infants approximately six months of age with severe BPD, all had abnormalities on PFTs, with three distinct phenotypes (51 percent obstructive, 40 percent mixed, and 9 percent restrictive) [29]. ... |
57_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Infancy | 12 months) with BPD have demonstrated evidence of small airway disease and impaired alveolar growth. Gas trapping and small airway disease was suggested by decreased maximum expiratory flow at functional residual capacity (VmaxFRC)and low functional to total lung volume ratios (FRCHe/FRCpleth); impaired alveolar growth... |
58_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Early childhood | For young children (three to six years of age) who are not able to perform conventional spirometry, impulse oscillometry is an emerging modality for measuring lung function. Testing does not require sedation, may show increases in respiratory impedance and small airway resistance in individuals with airflow obstruction... |
58_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Early childhood | m conventional spirometry, impulse oscillometry is an emerging modality for measuring lung function. Testing does not require sedation, may show increases in respiratory impedance and small airway resistance in individuals with airflow obstruction, and may help direct therapy [ 33 ]. It is unknown whether observed chan... |
59_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | PFTs in older children, adolescents, and adults are widely available and are useful in following longitudinal changes in pulmonary function. Spirometry may be the most useful PFT in detecting lung abnormalities in extremely premature children, including those with BPD [ PFTs in older children, adolescents, and adults a... |
59_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | FT in detecting lung abnormalities in extremely premature children, including those with BPD [ 35 ]. In some centers, spirometry can be performed in children as young as three years of age [ 36 ]; however, most PFT laboratories begin testing children at six years of age. Both pre- and post-administration of inhaled bro... |
59_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | ildren who have abnormal lung function. (See "Overview of pulmonary function testing in children" .) Although clinical symptoms in individuals with BPD often improve during childhood, PFTs often remain abnormal, particularly in those with more severe disease [37-39]. One study compared preschool children with healthy c... |
59_3 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | ng disease [40]. These findings are consistent with persistent airflow obstruction and gas trapping. While lung function is markedly abnormal in infancy for most infants with BPD [19], it can improve during childhood, such that many patients born preterm may have normal lung function in early adult life [41]. Nonethele... |
59_4 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | 44], and patients with moderate to severe BPD are more likely to have ongoing impairment [20,45-47]. The deteriorating lung function is usually obstructive in nature but may have a restrictive component in some cases. Improvements in PFTs over time have been shown in several studies. In one study, sequential measuremen... |
59_5 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | flow at FRC (VmaxFRC) [46]. Approximately one-third of these patients responded to bronchodilators. Another study reported improvements in lung compliance (from 50 percent of normal at one month of age to 80 percent of normal at 36 months) and specific pulmonary conductance (from 50 percent of normal at one month to 85... |
59_6 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | ity itself is a dominant risk factor for lung injury and long-term impairment of pulmonary function. Whether or not BPD exists, the risk is compounded by postnatal lung injury [48,49]. One series reported lung function in 48 children who were born at very low birth weight (VLBW) and managed at a tertiary center after t... |
59_7 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | r FVC, FEV1, and carbon monoxide diffusion capacity (DLCO) as compared with 46 age-matched controls. No differences were found between the VLBW children with or without BPD except for a higher RV/TLC ratio in the BPD subgroup (mean difference 7 percent; 95% CI 0.4-13 percent; p = 0.03). In a similar report, 53 extremel... |
59_8 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Childhood | 1), with limited reversibility by bronchodilators. Likewise, RV/TLC and DLCO differed significantly. These measurements did not differ in ELBW subgroups with or without BPD. In a separate longitudinal cohort of extremely preterm infants studied at six years of age, FVC and FEV1values were significantly lower, and frequ... |
60_0 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | Long-term abnormalities in pulmonary function and respiratory symptoms have been reported in individuals with the classic form of BPD [ Long-term abnormalities in pulmonary function and respiratory symptoms have been reported in individuals with the classic form of BPD [ 23,52-56 ]. In a study of 147 adolescents born w... |
60_1 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | and deterioration in pulmonary function since the previous evaluation at eight years of age [ 16 ]. Similarly, long-term studies in children ages 6 to 15 years with BPD found persistent airway obstruction, airway hyperreactivity, and hyperinflation of the lungs [ 23,52-54,57-75 ]. Both vital capacity and FEV 1 were red... |
60_2 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | tory morbidity during early childhood [ 76 ] and possibly the long-term pulmonary outcome [ 77,78 ]. These studies reflect in part the management of premature infants 20 years ago; much less is known regarding the long-term outcome of infants managed with modern neonatal intensive care unit strategies. Only a few studi... |
60_3 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | unit in Norway [79]. The first group was born between 1982 and 1985, prior to the use of surfactant. The second group was born between 1991 and 1992 when surfactant was used as rescue therapy for respiratory distress syndrome. As adolescents, the mean FEV1was similar between the two groups of children with BPD (81.9 an... |
60_4 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | percent predicted) and was lower than the control groups born at term during the same time periods. In a separate systematic review and meta-analysis, long-term outcomes (measured by FEV1percent predicted) for children born preterm who required supplemental oxygen at 28 days of life were substantially better for childr... |
60_5 | complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - PULMONARY FUNCTION - Adults with classic bronchopulmonary dysplasia | study measured long-term pulmonary function outcomes in individuals between 10 and 35 years of age. Although pulmonary function deficits were found in those born extremely preterm compared with those born term, these pulmonary deficits decreased with each decade of birth from 1980 to 2000 [44], suggesting that ongoing ... |
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