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bronchopulmonary-dysplasia-bpd-prevention - SUMMARY AND RECOMMENDATIONS
●Definition– Clinically, BPD is defined as an ongoing need for supplemental oxygen and/or respiratory support at either 28 days postnatal age or 36 weeks postmenstrual age (calculator 1) in a preterm neonate with radiographic evidence of parenchymal lung disease (image 1). Various criteria are used to define BPD (tab...
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bronchopulmonary-dysplasia-bpd-prevention - INTRODUCTION
Bronchopulmonary dysplasia (BPD; also known as neonatal chronic lung disease [CLD]) is a major cause of respiratory illness in preterm infants. It is an important contributing factor in the increased risk of mortality and morbidity in the preterm population. This topic will provide an overview of strategies used to pre...
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bronchopulmonary-dysplasia-bpd-prevention - TERMINOLOGY
●Prematurity– Different degrees of prematurity are defined by gestational age (GA), which is calculated from the first day of the mother's last period, or birth weight (BW), as summarized in the table (table 1) and discussed in detail separately. (See"Preterm birth: Definitions of prematurity, epidemiology, and risk f...
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bronchopulmonary-dysplasia-bpd-prevention - OUR APPROACH
The following is a summary of the strategies that we use to reduce the incidence of BPD in infants who are at risk for developing BPD. The combination of interventions addresses the multiple risk factors implicated in the pathogenesis of BPD (algorithm 1). (See"Bronchopulmonary dysplasia (BPD): Clinical features and d...
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bronchopulmonary-dysplasia-bpd-prevention - OUR APPROACH - Initial general measures
General measures are provided to all infants who are at risk for BPD (extremely preterm [EPT] infant, gestational age <28 weeks). General measures are provided to all infants who are at risk for BPD (extremely preterm [EPT] infant, gestational age <28 weeks). ●Antenatal steroids – Antenatal glucocorticoids are appropri...
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bronchopulmonary-dysplasia-bpd-prevention - OUR APPROACH - Respiratory support
The goal for respiratory support for infants at risk for BPD is to maintain adequate oxygenation and ventilation while minimizing respiratory intervention that may lead to lung injury. Our approach is briefly summarized here. These interventions are discussed in greater detail separately. (See The goal for respiratory ...
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bronchopulmonary-dysplasia-bpd-prevention - OUR APPROACH - Postnatal glucocorticoids
We do We do not routinely administer postnatal systemic or inhaled glucocorticoids to prevent BPD. Systemic glucocorticoids are reserved for EPT infants who remain ventilator-dependent and/or require oxygen supplementation >50 percent at two to four weeks postnatal age. This is discussed in detail separately. (See "Pos...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Overview
●Measures that are routinely used– The following interventions are generally used in combination to improve outcomes (including a reduction in the risk of BPD) in at-risk preterm infants, especially extremely preterm infants (EPT; gestational age [GA] <28 weeks) (algorithm 1): •Antenatal glucocorticoid therapy (see"An...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Glucocorticoids - -Antenatal glucocorticoids
Antenatal glucocorticoid therapy is an effective intervention for prevention of respiratory distress syndrome (RDS) resulting in less need for mechanical ventilation and oxygen supplementation (risk factors for BPD). Antenatal glucocorticoids are appropriate for pregnant individuals from 23 to 34 weeks of gestation who...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Glucocorticoids - -Postnatal glucocorticoids
We do We do not routinely administer postnatal systemic or inhaled glucocorticoids to prevent BPD. Systemic glucocorticoids are reserved for EPT infants who remain ventilator-dependent and/or require oxygen supplementation >50 percent at a postnatal age of two to four weeks. This is discussed in detail separately. (See...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Surfactant
Exogenous surfactant therapy given within the first 30 to 60 minutes after birth is effective in the prevention and treatment of RDS and reduces the need for mechanical ventilation and oxygen supplementation (risk factors for BPD). The use of early surfactant to prevent and treat RDS is discussed separately. (See Exoge...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Fluid management
The goal of fluid management is to maintain neutral or slightly negative fluid balance. Our usual practice is to restrict total fluid intake to 130 to 140 mL/kg per day after the first week of life. However, the fluid status of the patient must be monitored frequently to avoid dehydration or overhydration as fluid need...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Ventilation strategies to minimize lung injury
Mechanical ventilation (MV) has been a lifesaving intervention in the care of preterm infants at risk for RDS due to premature lung development. However, mechanical ventilation causes tissue injury and inflammation due to volutrauma that contributes to BPD. As a result, MV strategies aim to minimize lung injury while a...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Caffeine
For most ELBW infants (BW <1000 g), we suggest prophylactic For most ELBW infants (BW <1000 g), we suggest prophylactic caffeine starting on the first day of life. The available clinical trial data suggest this intervention is safe and effective for reducing BPD and perhaps other long-term complications. This is discus...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Vitamin A
EPT infants may have EPT infants may have vitamin A deficiency, which may promote the development of BPD [ 2 ]. However, data are conflicting as to whether vitamin A supplementation reduces the incidence of BPD. If there is a benefit, it appears to be modest. Since the incidence of BPD varies among neonatal intensive c...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Breast milk
Mother's own milk is the preferred form of nutrition for preterm infants as it offers several advantages over formula, including prevention of BPD. (See Mother's own milk is the preferred form of nutrition for preterm infants as it offers several advantages over formula, including prevention of BPD. (See "Human milk fe...
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bronchopulmonary-dysplasia-bpd-prevention - INTERVENTIONS - Unproven interventions
Interventions that are ineffective in preventing BPD include sustained inflation in the delivery room for infants requiring respiratory support, Interventions that are ineffective in preventing BPD include sustained inflation in the delivery room for infants requiring respiratory support, inhaled nitric oxide alone or ...
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bronchopulmonary-dysplasia-bpd-prevention - SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See"Society guideline links: Bronchopulmonary dysplasia".)
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bronchopulmonary-dysplasia-bpd-prevention - INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5thto 6thgrade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for...
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bronchopulmonary-dysplasia-bpd-prevention - SUMMARY AND RECOMMENDATIONS
●Definition– Clinically, BPD is defined as an ongoing need for supplemental oxygen and/or respiratory support at either 28 days postnatal age or 36 weeks postmenstrual age (calculator 1) in a preterm neonate with radiographic evidence of parenchymal lung disease (image 1). Various criteria are used to define BPD (tab...
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bronchopulmonary-dysplasia-bpd-prevention - ACKNOWLEDGMENT
The editorial staff at UpToDate acknowledge James Adams, Jr., MD, who contributed to an earlier version of this topic review.
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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...
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bronchopulmonary-sequestration - INTRODUCTION
Bronchopulmonary sequestration (BPS), sometimes referred to simply as pulmonary sequestration, is a rare congenital abnormality of the lower airway. It consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and that receives its arterial blood supply from the sys...
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bronchopulmonary-sequestration - DEFINITIONS
BPS is a nonfunctioning mass of lung tissue, with airway and alveolar elements, that lacks normal communication with the tracheobronchial tree and receives its arterial blood supply from the systemic circulation. The subtypes are classified anatomically, as follows: ●Intralobar sequestration (ILS)– An ILS (also known a...
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bronchopulmonary-sequestration - EPIDEMIOLOGY
Congenital abnormalities of the lower respiratory tract are rare, found in approximately 1 in 10,000 to 35,000 live births [4]. Among these, the most common is congenital pulmonary airway malformation (CPAM), while BPS represents only 0.15 to 6.40 percent [6]. In several reports, even tertiary care referral centers dia...
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bronchopulmonary-sequestration - PATHOGENESIS
The embryologic basis for the development of BPS and other congenital abnormalities of the lower airway are not fully understood [5,14]. The most widely accepted embryologic theory is that BPS originates early in the pseudoglandular stage of lung development (5 to 17 weeks of gestation), prior to separation of the aort...
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bronchopulmonary-sequestration - ANATOMIC CHARACTERISTICS
Sequestrations are characterized by their location, connection to pulmonary or other structures, vascular supply, and association with other abnormalities. By definition, their arterial blood supply is from the systemic circulation.
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bronchopulmonary-sequestration - ANATOMIC CHARACTERISTICS - Intralobar sequestration
Intralobar sequestrations (ILS) are located within a normal lobe and lack their own visceral pleura. Most ILS occur in the lower lobes, but they can occur anywhere within the thorax [ Intralobar sequestrations (ILS) are located within a normal lobe and lack their own visceral pleura. Most ILS occur in the lower lobes, ...
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bronchopulmonary-sequestration - ANATOMIC CHARACTERISTICS - Extralobar sequestration
Extralobar sequestrations (ELS) are located outside the normal lung and have their own visceral pleura, with a pedicle that contains the vascular connections. They vary in size but usually are relatively small compared with the normal lobes. The vast majority are in the left hemithorax, and the most common location is ...
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bronchopulmonary-sequestration - ANATOMIC CHARACTERISTICS - Hybrid BPS/CPAM lesions
Hybrid lesions, with features of BPS and CPAM, occur in a substantial proportion of BPS [ Hybrid lesions, with features of BPS and CPAM, occur in a substantial proportion of BPS [ 3,30,31 ] and comprise 15 to 40 percent of all cystic lung lesions [ 32 ]. These lesions have blood supply from a systemic artery consistent...
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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...
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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...
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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...
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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...
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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 ...
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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...
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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...
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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 ...
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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...
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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...
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bronchopulmonary-sequestration - MANAGEMENT
The approach to treatment depends upon whether the patient has symptoms (respiratory distress or recurrent infections) or is asymptomatic.
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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...
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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...
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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...
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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...
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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). ...
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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...
52
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'...
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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...
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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...
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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. This has led to increased survival of extremely low birth weight (ELBW) infants and the evolution of a new type o...
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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...
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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...
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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...
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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...
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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...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA
BPD may include parenchymal lung disease, pulmonary hypertension (PH), and large airway disease. These disorders are often not found in isolation, particularly with severe disease. A study found that nearly three-quarters of infants with severe BPD had two or more of these manifestations [81].
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Asthma-like symptoms
Recurrent wheezing episodes are very common in children with BPD, beginning in the preschool years and continuing into adulthood [ Recurrent wheezing episodes are very common in children with BPD, beginning in the preschool years and continuing into adulthood [ 4,16,82-84 ]. Adolescents also experience reduced exercise...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Pulmonary hypertension
PH, a condition characterized by elevated pulmonary artery pressure, develops in 20 to 40 percent of infants with BPD [ PH, a condition characterized by elevated pulmonary artery pressure, develops in 20 to 40 percent of infants with BPD [ 105-107 ]. The risk for PH is highest in very premature and VLBW infants, as wel...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Central airway disease
The central airways span the glottis to the lobar or segmental bronchi. Acquired damage to the airways in infancy may persist into early childhood and beyond. Infants and young children with BPD are at increased risk of developing central airway collapse or obstruction, which can lead to "BPD spells" or cyanotic or lif...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Central airway disease - -Acquired tracheobronchomalacia
This complication of BPD was more common in infants and children with classic BPD who were treated with prolonged positive pressure ventilation (PPV). Tracheobronchomalacia is characterized by abnormally compliant, collapsible central airways and may be a consequence of barotrauma, chronic or recurrent infection, chron...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Central airway disease - -Glottic and subglottic damage
Injury to the glottis and surrounding structures has been reported after endotracheal intubation in newborns [ Injury to the glottis and surrounding structures has been reported after endotracheal intubation in newborns [ 128-137 ]. This can occur more commonly in ELBW infants who require prolong PPV with an endotrache...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Central airway disease - -Tracheal and bronchial stenosis and granuloma formation
Acquired tracheal or bronchial stenosis or granuloma formation has been reported in a subset of infants with BPD as old as 17 months [ Acquired tracheal or bronchial stenosis or granuloma formation has been reported in a subset of infants with BPD as old as 17 months [ 119,120,126,138-142 ]. Stenosis and granulation fo...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Sleep-disordered breathing
Patients with a history of BPD are at increased risk for sleep-disordered breathing. Accordingly, polysomnography is recommended for infants, children, and adolescents with a history of BPD and symptoms of sleep-disordered breathing, including persistent snoring, failure to thrive, or persistent need for supplemental o...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Sleep hypoxemia
Infants with a history of BPD are more likely to experience hypoventilation and hypoxemic episodes during sleep [ Infants with a history of BPD are more likely to experience hypoventilation and hypoxemic episodes during sleep [ 154-158 ], and these episodes may be clinically silent [ 155 ]. Many infants and children wi...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Respiratory infection
Up to 50 percent of children with BPD require rehospitalization during the first two years of life due to a respiratory illness [ Up to 50 percent of children with BPD require rehospitalization during the first two years of life due to a respiratory illness [ 170,171 ]. These infections, which are usually due to viruse...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - RESPIRATORY DISORDERS ASSOCIATED WITH BRONCHOPULMONARY DYSPLASIA - Obstructive lung disease in adulthood
Several studies have reported that adults with a history of BPD are more likely to exhibit airflow obstruction compared with controls [ Several studies have reported that adults with a history of BPD are more likely to exhibit airflow obstruction compared with controls [ 56,183 ]. As an example, a cohort of adults who ...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE
Children, adolescents, and young adults with BPD may be followed up in a number of outpatient clinic settings. These include a pediatrician's office or a hospital-based follow-up clinic. In either case, the patient should be referred to a pulmonologist familiar with the care of these often medically fragile patients. T...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - General measures
The following measures are appropriate for all infants and children with a history of BPD to reduce their risk for respiratory disease: The following measures are appropriate for all infants and children with a history of BPD to reduce their risk for respiratory disease: ●Close adherence to immunization schedules recom...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues
Specific needs that are relevant to subgroups of infants are discussed below: Specific needs that are relevant to subgroups of infants are discussed below:
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Oxygen therapy
Hypoxic episodes in infants and children can be linked to and worsen already impaired lung mechanics, elevated airway resistance, and obstruction [ Hypoxic episodes in infants and children can be linked to and worsen already impaired lung mechanics, elevated airway resistance, and obstruction [ 155,161,162 ]. Oxygen su...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Tracheostomy
●Management in the home setting– Infants with obstructive central and upper airway disease or who require long-term chronic ventilation may require a tracheostomy for months or even years after hospital discharge [197]. Such infants require coordinated care involving their primary clinician, pediatric otolaryngologist,...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Home ventilators
Children with BPD on home ventilation have substantial mortality risks at home, as high as 18.6 percent in one single-center study [ Children with BPD on home ventilation have substantial mortality risks at home, as high as 18.6 percent in one single-center study [ 207 ]. In another study of 102 children with respirato...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Cardiorespiratory monitoring
Routine use of cardiorespiratory monitors is generally not indicated for children with BPD after hospital discharge. However, cardiorespiratory monitors may be appropriate for the following groups of infants: Routine use of cardiorespiratory monitors is generally not indicated for children with BPD after hospital disch...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Pulmonary hypertension
Infants who continue to have a need for supplemental oxygen should be screened for PH before hospital discharge, as described in a separate topic review. Infants diagnosed with PH require close follow-up with a PH specialist. Follow-up is particularly important during the first few months after discharge and during res...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Asthma and asthma-like symptoms
Wheezing and airway hyperresponsiveness are common among children and adolescents with a history of BPD, as noted above (see Wheezing and airway hyperresponsiveness are common among children and adolescents with a history of BPD, as noted above (see 'Asthma-like symptoms' above). Steps to minimize risk include avoidanc...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - MANAGEMENT AFTER NEONATAL INTENSIVE CARE UNIT DISCHARGE - Management of specific issues - -Obstructive sleep apnea
Children and young adults with a history of prematurity are at increased risk for obstructive sleep apnea and should be evaluated if they have suggestive signs or symptoms, such as apneic pauses with sleep, loud snoring, restless sleep, daytime irritability, morning headaches, etc. Polysomnography is also indicated for...
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complications-and-long-term-pulmonary-outcomes-of-bronchopulmonary-dysplasia - SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See"Society guideline links: Pulmonary hypertension in children"and"Society guideline links: Bronchopulmonary dysplasia".)
83
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'...
85
congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - SUMMARY AND RECOMMENDATIONS
●Pathophysiology– CDH is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the chest, thereby compressing the lung and interfering with normal fetal lung development. With increased compression of the developing lung by the herniated abdominal contents, there are corresponding decre...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - INTRODUCTION
Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that allows abdominal viscera to herniate into the chest. Affected neonates usually present in the first few minutes to hours after birth with respiratory distress that can range from mild to life-threatening. With improvements in antenata...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - PREVALENCE
Reported prevalence rates of CDH range from 2 to 3 cases per 10,000 live births [1,2]. It is more common in males than females, with a male-to-female ratio of 1.4 to 1 [2]. Approximately 5 to 10 percent of cases are associated with a chromosomal abnormality or genetic syndrome [2]. (See'Associated conditions'below.)
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - PATHOGENESIS AND EMBRYOLOGY
CDH results from failure of normal closure of the pleuroperitoneal folds during the fourth to tenth weeks postfertilization, which allows herniation of viscera into the thoracic cavity. This interferes with normal lung development and has other adverse consequences. The reason for failure of normal diaphragmatic closur...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - IMPACT ON CARDIOPULMONARY DEVELOPMENT
Because herniation occurs during a critical period of lung development, clinical manifestations of CDH result from the pathologic effects of the herniated viscera on lung development. With rising severity of lung compression, there are corresponding decreases in bronchial and pulmonary arterial branching, resulting in ...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - CLINICAL MANIFESTATIONS - Prenatal presentation
Many patients with CDH are identified through routine prenatal ultrasound. Prenatal ultrasound can also identify other associated anomalies (eg, cardiac abnormalities). Prenatal presentation and diagnosis are discussed in greater detail separately. (See Many patients with CDH are identified through routine prenatal ult...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - CLINICAL MANIFESTATIONS - Postnatal findings - -Presentation
Postnatally, infants with CDH most often present with respiratory distress in the first few minutes after birth. Less commonly, a small subset of patients with CDH have minimal or no symptoms in the newborn period and present later in life. (See Postnatally, infants with CDH most often present with respiratory distress...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - CLINICAL MANIFESTATIONS - Postnatal findings - -Physical findings
Physical findings in the newborn include a barrel-shaped chest, a scaphoid-appearing abdomen (because of loss of the abdominal contents into the chest), and absence of breath sounds on the ipsilateral side. In patients with a left-sided CDH, the heartbeat is displaced to the right because of a shift in the mediastinum....
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - CLINICAL MANIFESTATIONS - Postnatal findings - -Laterality
In most cases of CDH, herniation occurs on the left. Right-sided CDH occurs in approximately 15 percent of cases and bilateral herniation in 1 to 2 percent [ In most cases of CDH, herniation occurs on the left. Right-sided CDH occurs in approximately 15 percent of cases and bilateral herniation in 1 to 2 percent [ 8-10...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - CLINICAL MANIFESTATIONS - Associated conditions
Associated congenital abnormalities are seen in approximately 50 percent of newborns with CDH. In some cases, the associated abnormalities are identified prenatally. However, in many cases, the full extent of associated abnormalities is not appreciated until after delivery. (See Associated congenital abnormalities are ...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIAGNOSIS - Prenatal
Many cases of CDH are diagnosed prenatally by routine antenatal ultrasound screening. This is discussed separately. (See Many cases of CDH are diagnosed prenatally by routine antenatal ultrasound screening. This is discussed separately. (See "Congenital diaphragmatic hernia: Prenatal issues", section on 'Prenatal diagn...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIAGNOSIS - Postnatal
While most infants with CDH are diagnosed prenatally, a small subset present postnatally with respiratory distress. In these newborns, stabilization efforts in the delivery room take precedence over diagnostic testing. Once the newborn is stabilized, chest imaging and echocardiography are performed. While most infants ...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIAGNOSIS - Postnatal - -Initial stabilization
Postnatally, newborns with CDH often present with severe respiratory distress within the first few minutes after birth. Stabilization in the delivery room includes intubation, placement of a nasogastric tube, and other measures to support the newborn’s respiratory and hemodynamic status. These interventions are discuss...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIAGNOSIS - Postnatal - -Chest imaging
Chest radiograph findings include: Chest radiograph findings include: ●Herniation of abdominal contents (usually air- or fluid-containing bowel) into the hemithorax with little or no visible aerated lung on the affected side (image 1) ●Displacement of mediastinal structures (eg, heart) towards the contralateral lung ●...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIAGNOSIS - Postnatal - -Echocardiography
All neonates with CDH should undergo echocardiography early in the postnatal course to detect any associated cardiac anomalies, evaluate ventricular function, assess the size of the patent ductus arteriosus, assess the degree and direction of ductal and intracardiac shunting, and to assess for pulmonary hypertension (P...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - LATE PRESENTATION
Infrequently, mild CDH defects present after the neonatal period. In a case series of 15 children who presented late with CDH, the mean age at presentation was 18 months (range 38 days to 10 years) [24]. The main presenting symptoms were respiratory complaints in 40 percent of patients, gastrointestinal (GI) symptoms i...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - DIFFERENTIAL DIAGNOSIS
The differential diagnosis of neonatal CDH includes other causes of neonatal respiratory distress, including infections (sepsis, pneumonia) and noninfectious etiologies (table 1). CDH is differentiated from these conditions by the characteristic chest radiograph finding of herniated abdominal contents into the thorax ...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See"Society guideline links: Pulmonary hypertension in children".)
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5thto 6thgrade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-clinical-features-and-diagnosis - SUMMARY AND RECOMMENDATIONS
●Pathophysiology– CDH is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the chest, thereby compressing the lung and interfering with normal fetal lung development. With increased compression of the developing lung by the herniated abdominal contents, there are corresponding decre...
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congenital-diaphragmatic-hernia-cdh-in-the-neonate-management-and-outcome - SUMMARY AND RECOMMENDATIONS
●Initial medical management– Initial management of the neonate with congenital diaphragmatic hernia (CDH) includes the following measures (see'Initial interventions'above): •Intubation– All newborns with CDH are immediately intubated in the delivery room (or upon diagnosis if the neonate is diagnosed postnatally) to pr...
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