RESUMEN
BACKGROUND: Congenital lung malformations (CLM) are rare developmental anomalies of the fetal lung with a minority of patients exhibiting symptoms around the time of birth. Although ultrasound remains the gold standard, fetal MRI has recently been incorporated as an adjunct imaging modality in the workup and prenatal counseling of patients with CLM as it is thought to more accurately delineate lesion boundaries and diagnose lesion type. We evaluate what prenatal variables correlate with postnatal respiratory symptoms. METHODS: We performed a retrospective review of patients with prenatal diagnosis of CLM treated at our institution between 2006-2020. Fetal ultrasound and magnetic resonance imaging (MRI) parameters including maximal congenital pulmonary airway malformation volume ratio (CVR), absolute cyst volume, and observed to expected normal fetal lung volume (O/E NFLV) were correlated with outcomes including postnatal respiratory symptoms, need for supplementary oxygen or mechanical ventilation, delay in tolerating full feeds, resection in the neonatal period. RESULTS: Our study included 111 patients, all of whom underwent fetal ultrasound with 64 patients additionally undergoing fetal MRI. Postnatal respiratory symptoms were noted in 22.5% of patients, 19.8% required supplemental oxygen, 2.7% mechanical ventilation and two patients requiring urgent resection. Ultrasound parameters including absolute cyst volume and maximal CVR correlated with need for mechanical ventilation (p=0.034 and p=0.024, respectively) and for urgent resection (p=0.018 and p=0.023, respectively) and had a marginal association with postnatal respiratory symptoms (p=0.050 and p=0.052). Absolute cyst volume became associated with postnatal respiratory symptoms (p=0.017) after multivariable analysis controlling for maternal steroid administration and gestational age. O/E NFLV did not correlate with perinatal outcomes. CONCLUSION: We have found that ultrasound-based measurements correlate with postnatal respiratory symptoms, while MRI derived O/E NFLV does not. Further studies are needed to elucidate the role of MRI in the prenatal workup of congenital lung malformations. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level I.
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Enfermedades Pulmonares , Anomalías del Sistema Respiratorio , Embarazo , Recién Nacido , Femenino , Humanos , Pulmón/anomalías , Enfermedades Pulmonares/congénito , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/cirugía , Ultrasonografía Prenatal/métodos , Imagen por Resonancia Magnética/métodos , Estudios RetrospectivosRESUMEN
Congenital lung lesions are a rare group of developmental pulmonary abnormalities that are often first identified prenatally on routine second-trimester US. Congenital pulmonary airway malformation (CPAM) is the most common anomaly while others include bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and bronchial atresia. Clinical presentation is highly variable, ranging from apparent in utero resolution to severe mass effect with resultant hydrops fetalis and fetal demise. Differentiation among these lesions can be challenging because overlapping imaging features are often present. The roles of the radiologist are to identify key imaging findings that help in diagnosing congenital lung lesions and to recognize any ominous features that might require prenatal or perinatal intervention. High-resolution US and complementary rapid-acquisition fetal MRI provide valuable information necessary for lesion characterization. Postnatal US and CT angiography are helpful for lesion evaluation and for possible surgical planning. This article reviews the embryology of the lungs, the normal prenatal imaging appearance of the thorax and its contents, and the prenatal and neonatal imaging characteristics, prognosis and management of various congenital lung lesions.
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Secuestro Broncopulmonar , Malformación Adenomatoide Quística Congénita del Pulmón , Neumonía , Anomalías del Sistema Respiratorio , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/patología , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Recién Nacido , Pulmón/diagnóstico por imagen , Pulmón/patología , Embarazo , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/patología , Ultrasonografía Prenatal/métodosRESUMEN
PURPOSE: Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. METHODS: All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. RESULTS: Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). CONCLUSIONS: Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. LEVEL OF EVIDENCE: Level IV.
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Enfermedades Bronquiales , Enfermedades Pulmonares , Enfisema Pulmonar , Anomalías del Sistema Respiratorio , Enfermedades Bronquiales/diagnóstico , Niño , Femenino , Humanos , Pulmón , Embarazo , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/cirugíaRESUMEN
PURPOSE: Congenital lung malformation (CLM) volume ratio (CVR) of ≥1.1 has been shown to be highly predictive of the need for urgent, perinatal surgical intervention. The purpose of this study was to utilize this information to propose a delivery planning and clinical management algorithm based on this threshold. METHODS: A retrospective cohort study was performed for all fetuses evaluated at our fetal center between 5/2015 and 11/2020. Demographics, ultrasound findings, late gestation CVR (≥27 weeks gestational age), prenatal and postnatal treatment, and outcomes were analyzed with nonparametric univariate analysis based on late gestation CVR of 1.1. Receiver operating characteristic curve analysis was performed to evaluate association between late gestation CVR, hydrops, need for fetal intervention, and need for urgent perinatal surgery. RESULTS: Of the 90 CLMs referred to our fetal center, 65 had late gestation CVR with a majority <1.1 (47/65, 72%). All patients with late gestation CVR ≥ 1.1 were managed with resection (18/18) with most resections requiring fetal intervention or urgent neonatal resection (13/18). Late gestation CVR < 1.1 were managed with elective resection (36/47, 77%) or non-operative observation (11/47, 23%). Late gestation CVR ≥ 1.1 had 100% sensitivity and NPV for hydrops, need for fetal intervention, and need for urgent perinatal surgery. CONCLUSION: CLM with CVR ≥ 1.1 were associated with urgent perinatal surgical intervention and expectant mothers should plan for delivery at centers equipped to manage neonatal resuscitation and potential urgent neonatal resection. Conversely, CLM with CVR < 1.1 may be safe to deliver at patient hospital of choice.
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Enfermedades Pulmonares , Anomalías del Sistema Respiratorio , Algoritmos , Niño , Edema , Femenino , Edad Gestacional , Humanos , Recién Nacido , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares/congénito , Atención Perinatal , Embarazo , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/cirugía , Resucitación , Estudios Retrospectivos , Ultrasonografía PrenatalRESUMEN
Congenital lung malformations are a spectrum of developmental anomalies comprised of malformations of the lung parenchyma, airways, and vasculature. Imaging assessment plays a pivotal role in the initial diagnosis, management, and follow-up evaluation of congenital lung malformations in the pediatric population. However, there is currently a lack of practical imaging guidelines and recommendations for the diagnostic imaging assessment of congenital lung malformations in infants and children. This article reviews the current evidence regarding the imaging evaluation of congenital lung malformations and provides up-to-date imaging recommendations for pediatric congenital lung malformations.
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Diagnóstico por Imagen/métodos , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Malformaciones Vasculares/diagnóstico por imagen , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , MasculinoRESUMEN
BACKGROUND: Risk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method. METHODS: Archival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Lung disease was quantified using the newly-developed congenital lung abnormality quantification(CLAQ) scoring method and obtained percentages were used to predict histopathological signs of inflammation and presence of mucinous proliferation (MP). Because MP is presumed a precursor for mucinous adenocarcinoma in situ (AIS) this method was also used to compare CT-scans of patients with AIS to those with only CPAM. RESULTS: Thirty-three CPAM patients were included of which 13(39%) had histological signs of inflammation and 8(24%) had a MP. Patients with inflammation had a significantly smaller lesion (14% vs 38%) while those with MP had more extensive disease (54%vs17%). Patients with AIS had a significantly smaller lesion compared to CPAM patients (5%vs29%). Significant predictors for inflammation were smaller lesion size and percentage hypodensity within lesions while a larger lesion size and percentage parenchymal hyperdensity (solid lung tissue components) were predictors for MP as well as AIS. CONCLUSIONS: Smaller CPAM lesions may be more susceptible to inflammation while larger lesions may be associated with the presence of MP. Parenchymal hyperdensity is found as a predictor for MP as well as AIS and should therefore elicit more extensive gross sampling. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón , Anomalías del Sistema Respiratorio , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Humanos , Inflamación , Pulmón/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Congenital lung malformations represent a spectrum of abnormalities that can overlap in imaging appearance and frequently coexist in the same child. Imaging diagnosis in the neonatal period can be challenging; however, the recognition of several archetypal radiographic patterns can aid in narrowing the differential diagnosis. Major radiographic archetypes include (1) hyperlucent lung, (2) pulmonary cysts, (3) focal opacity and (4) normal radiograph. Here we review the multimodality imaging appearances of the most commonly seen congenital lung malformations, categorized by their primary imaging archetypes. Along with the congenital lung malformations, we present several important imaging mimickers.
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Malformación Adenomatoide Quística Congénita del Pulmón , Quistes , Enfermedades Pulmonares , Anomalías del Sistema Respiratorio , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Humanos , Recién Nacido , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares/congénito , Enfermedades Pulmonares/diagnóstico por imagen , Imagen Multimodal , Anomalías del Sistema Respiratorio/diagnóstico por imagenRESUMEN
Las malformaciones pulmonares congénitas (MPC) conforman un grupo de entidades originadas por alteraciones en la embriogénesis del pulmón y de las vías respiratorias que se producen de acuerdo al nivel del árbol traqueobronquial donde se dé el insulto o el momento de la edad gestacional. Las entidades que en la actualidad forman parte de las MPC son: malformación congénita de la vía aérea, secuestro pulmonar, quiste broncogénico, enfisema lobar congénito, atresia bronquial. Su diagnóstico puede realizarse desde la etapa prenatal, al momento del nacimiento, en la edad pediátrica o adulta por la aparición de síntomas o incidentalmente en pruebas radiológicas. El manejo de estas lesiones depende del tipo de malformación y de la severidad de los síntomas, por lo que se debe individualizar la conducta a seguir en cada caso. Aunque la mayoría de los autores recomienda la resección de la lesión, no existe en la actualidad un consenso sobre la indicación de cirugía, sobre todo en pacientes asintomáticos. Nuestro objetivo es describir los hallazgos clínicos, radiológicos y en algunos casos anatomopatológicos así como el tratamiento empleado de cuatro casos clínicos, atendidos en un hospital de tercer nivel que ejemplifican las presentaciones más frecuentes de las MPC.
Congenital pulmonary malformations (CPM) make up a group of entities caused by alterations in the embryogenesis of the lung and the respiratory tract that occur according to the level of the tracheobronchial tree where the insult occurs or the moment of gestational age. The entities that are currently part of the CPM are: congenital malformation of the airway, pulmonary sequestration, bronchogenic cyst, congenital lobar emphysema, bronchial atresia. Its diagnosis can be made from the prenatal stage, at birth or in pediatric or adult age due to the appearance of symptoms or incidentally in radiological tests. The management of these injuries depends on the type of malformation and the severity of the symptoms, so the conduct to be followed in each case must be individualized. Although most authors recommend resection of the lesion, there is currently no consensus on the indication for surgery, especially in asymptomatic patients. Our objective is to describe the clinical, radiological and in some cases histopathological findings, as well as the treatment used in four clinical cases, treated in a tertiary level hospital that exemplify the most frequent presentations of MPC.
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Humanos , Masculino , Femenino , Embarazo , Recién Nacido , Adulto , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares/diagnóstico por imagen , Diagnóstico Prenatal , Recien Nacido Prematuro , Enfermedades Pulmonares/congénitoRESUMEN
El bronquio traqueal o bronquio porcino es una rara anomalía congénita que se define como la salida del bronquio del lóbulo superior derecho directamente a la tráquea, próximo a la carina principal. En principio se considera una variante anatómica de la normalidad, pero hay que tener especial cuidado en ciertas situaciones como la intubación orotraqueal (AU)
The tracheal bronchus or porcine bronchus is a rare congenital anomaly that is defined as the exit of the bronchus from the right upper lobe directly to the trachea, close to the main carina. In principle, it is considered an anatomical variant of normality, but special care must be taken in certain situations such as orotracheal intubation (AU)
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Humanos , Femenino , Persona de Mediana Edad , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Bronquios/anomalías , Bronquios/diagnóstico por imagenRESUMEN
PURPOSE: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children. MATERIALS AND METHODS: All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics. RESULTS: Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). CONCLUSION: The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.
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Enfermedades Bronquiales , Malformación Adenomatoide Quística Congénita del Pulmón , Anomalías del Sistema Respiratorio , Adolescente , Niño , Femenino , Humanos , Lactante , Pulmón/diagnóstico por imagen , Masculino , Tomografía Computarizada Multidetector , Anomalías del Sistema Respiratorio/diagnóstico por imagenRESUMEN
BACKGROUND: Microtia is a congenital malformation of the external ear often with one or more associated congenital anomalies. The purpose of this study was to identify the characteristics and prevalence of respiratory anomalies in patients with microtia, and clarify the importance of this association in the perioperative period of patients' external ear reconstruction surgery. METHODS: Data were collected from 923 microtia patients between August 2017 and December 2020 in the Department of Auricular Reconstruction at the Plastic Surgery Hospital of Peking Union Medical College. Co-occurring respiratory anomalies were detected using chest computed tomography plus three-dimensional reconstruction and Chest X-ray. Physical examination was performed to assess the severity and type of microtia by trained clinicians. Fisher's exact test was used to analyze the relation between laterality of pulmonary underdevelopment and microtia type. RESULTS: Among the 923 participants enrolled in the study, we identified 21 cases (2.3%) having respiratory system anomalies, consisting of 6 cases with pulmonary underdevelopment (28.6% of all anomalies of respiratory system detected), 2 cases with tracheal bronchus (9.5%), 1 case with tracheal diverticula (4.8%), 11 cases with lung bullae(52.4%), and 1 case with pulmonary azygos lobe (4.8%). The laterality of pulmonary underdevelopment was related to the type of microtia (difference between types, p < 0.05), as patients with concha-type remnant ear had pulmonary underdevelopment ipsilaterally. CONCLUSIONS: This study represents the first detailed and thematic study of a association featured by microtia and respiratory anomalies. Characteristics and prevalence of respiratory anomalies was observed in a Chinese clinical microtia population. Early diagnosis of associated respiratory malformations had practical clinical significance for microtia patients, plastic surgeons and anesthesiologists. Future studies are required to improve understanding of this association and its cause.
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Microtia Congénita , Anomalías del Sistema Respiratorio , Cirugía Plástica , China/epidemiología , Microtia Congénita/epidemiología , Oído Externo , Humanos , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/epidemiologíaRESUMEN
BACKGROUND: Obesity is associated with restrictive ventilatory defects and a faster rate of decline in FVC. This association is not exclusively mediated by mechanical factors and may reflect direct pulmonary injury by adipose-derived mediators. RESEARCH QUESTION: Is adipose tissue involved in the pathogenesis of interstitial lung disease (ILD)? STUDY DESIGN AND METHODS: We evaluated the association of CT measures of pericardial, abdominal visceral, and abdominal subcutaneous adipose tissue with high-attenuation areas (HAAs) and interstitial lung abnormalities (ILAs) in a large multicenter cohort study of community-dwelling adults, using multivariable-adjusted models. We secondarily evaluated the association of adipose depot size with FVC and biomarkers of obesity and inflammation. RESULTS: In fully adjusted models, every doubling in pericardial adipose tissue volume was associated with a 63.4-unit increase in HAA (95% CI, 55.5-71.3), 20% increased odds of ILA (95% CI, -2% to 50%), and a 5.5% decrease in percent predicted FVC (95% CI, -6.8% to -4.3%). IL-6 levels accounted for 8% of the association between pericardial adipose tissue and HAA. Every doubling in visceral adipose tissue area was associated with a 41.5-unit increase in HAA (95% CI, 28.3-54.7), 30% increased odds of ILA (95% CI, -10% to 80%), and a 5.4% decrease in percent predicted FVC (95% CI, -6.6% to -4.3%). IL-6 and leptin accounted for 17% and 18%, respectively, of the association between visceral adipose tissue and HAA. INTERPRETATION: Greater amounts of pericardial and abdominal visceral adipose tissue were associated with CT measures of early lung injury and lower FVC in a cohort of community-dwelling adults. Adipose tissue may represent a modifiable risk factor for ILD.
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Adiposidad , Vida Independiente , Anomalías del Sistema Respiratorio/epidemiología , Anciano , Biomarcadores/análisis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Estudios Prospectivos , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
INTRODUCTION: Congenital bronchial atresia (CBA) is a rare airway malformation. No management guidelines exist because of limited evidence: treatment, surgical or conservative, is based on consensus and opinion. OBJECTIVE: To review the experience of a pediatric tertiary center and provide additional data about nonsurgical management of CBA and its outcomes after a structured follow-up, and to help formulate appropriate evidence-based guidelines. METHODS: A retrospective analysis of clinical, radiological, surgical, and pathological data of all pediatric patients with suspicion of CBA referred to the surgical department of the Bambino Gesù children's hospital of Rome between December 2013 and 2019, along with a review of the literature. RESULTS: Among the 18 children initially included in the study, 2 were lost to follow-up after radiological diagnosis, 4 underwent surgery for radiological suspicion of other pulmonary malformations. The final population is composed of 12 conservatively managed patients. At the end of the follow-up (median: 29 months, range 3-61), 1 patient (8%) was symptomatic. CONCLUSION: Conservative management for CBA appears to be safe. Surgery should be reserved for patients with symptomatic or complicated cases.
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Enfermedades Bronquiales , Anomalías del Sistema Respiratorio , Niño , Tratamiento Conservador , Hospitales Pediátricos , Humanos , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/cirugía , Estudios RetrospectivosRESUMEN
OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.
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Procedimientos Quirúrgicos Cardíacos/mortalidad , Cardiopatías Congénitas/cirugía , Anomalías del Sistema Respiratorio/cirugía , Procedimientos Quirúrgicos Torácicos/mortalidad , Tráquea/cirugía , Adolescente , Factores de Edad , Canadá , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Preescolar , Bases de Datos Factuales , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/mortalidad , Humanos , Lactante , Masculino , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/mortalidad , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Procedimientos Quirúrgicos Torácicos/efectos adversos , Factores de Tiempo , Tráquea/anomalías , Tráquea/diagnóstico por imagen , Resultado del Tratamiento , Estados UnidosRESUMEN
OBJECTIVES: To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans. MATERIALS AND METHODS: A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon. RESULTS: Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies. Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue. CONCLUSIONS: CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting.
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Malformación Adenomatoide Quística Congénita del Pulmón , Atelectasia Pulmonar , Anomalías del Sistema Respiratorio , Niño , Humanos , Pulmón/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: Hyperechoic lung lesions are largely detected prenatally but their underlying etiology is still poorly defined. The aim of the study was to determine the concordance between pre and postnatal diagnosis of hyperechoic lung lesions. METHODS: Prenatal ultrasound (US) evaluation was performed by a fetal medicine specialist. Postnatal diagnosis was based on CT-scan. Pre- and postnatal features were retrieved from medical charts. RESULTS: Seventy five patients were included from January 2009 to December 2018. Main prenatal diagnoses were bronchopulmonary sequestrations (BPS) (n = 24%-32%), pulmonary cystic malformations (PCM) (n = 19%-25%), congenital lobar emphysemas (CLE) (n = 15%-20%). Mediastinal shift was observed in 18 cases (24%). The prenatal detection of a systemic arterial supply had a diagnostic accuracy of 90%, while the prenatal detection of a cystic component had a diagnostic accuracy of 76.5%. All 16 neonates with prenatal isolated mediastinal shift were asymptomatic at birth. Seven neonates showed respiratory distress that was not predicted prenatally. CONCLUSIONS: Hyperechoic lung malformations reflect a heterogeneous group of lesions with a good concordance for bronchopulmonary sequestration, but not a satisfying prediction for cystic lesions.
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Pulmón/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico , Ultrasonografía Prenatal/normas , Adulto , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Pulmón/anomalías , Pulmón/fisiopatología , Masculino , Embarazo , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Anomalías del Sistema Respiratorio/epidemiología , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Tomografía Computarizada por Rayos X/estadística & datos numéricos , Ultrasonografía Prenatal/métodos , Ultrasonografía Prenatal/estadística & datos numéricosRESUMEN
Congenital pulmonary airway malformations (CPAM) are rare and occur due to a failure in lung embryological development. They are classified according to their pathological characteristics and their anatomical origin. They can occur from the antenatal period to adulthood, can be associated with hydrops fetalis, respiratory distress, recurrent infections, or in an otherwise asymptomatic patient. In this article we carry out a scope review of the literature to answer frequent questions of the clinical teams in charge of patients with CPAM, such as the antenatal approach, the different types of surgeries and the management of asymptomatic patients. Although the indication for surgery is clear in symptomatic patients, little is known about its natural history of this condition, including the possibility of spontaneous resolution and the development of complications or neoplasm. So, the treatment of asymptomatic patients continues to be controversial. Therapeutic decisions must be made by multidisciplinary teams with the informed participation of parents and patients. In our opinion, considering the excellent results of minimally invasive surgery, its low incidence of complications, and practically zero mortality when performed by experienced groups, it seems reasonable to consider elective resection of a MCVAP in asymptomatic patients.
Las malformaciones congénitas de la vía aérea pulmonar (MCVAP) son infrecuentes y ocurren debido a una falla en el desarrollo embriológico pulmonar. Se clasifican de acuerdo con sus características patológicas y a su origen anatómico. Se pueden presentar desde el periodo antenatal hasta la adultez, asociarse a cuadros de hidrops fetal, distrés respiratorio, infecciones recurrentes, o como un hallazgo en pacientes asintomáticos. En este artículo realizamos una revisión bibliográfica exploratoria para responder dudas frecuentes de los equipos clínicos a cargo de pacientes con MCVAP, como el enfrentamiento antenatal, los distintos tipos de cirugía y su abordaje, y el manejo de pacientes asintomáticos. Si bien la indicación de cirugía es clara en pacientes sintomáticos, poco se conoce acerca de su historia natural, incluyendo la posibilidad de resolverse de forma espontánea, de complicarse o de evolucionar hacia el desarrollo de una neoplasia, por lo que el tratamiento de pacientes asintomáticos continúa siendo controversial. Las decisiones terapéuticas deben ser tomadas por equipos multidisciplinarios con la participación informada de los padres y de los pacientes. En nuestra opinión, considerando los excelentes resultados de la cirugía mínimamente invasiva, su baja incidencia de complicaciones y prácticamente nula mortalidad, al ser realizada por grupos con experiencia, nos parece razonable plantear la resección electiva de una MCVAP en un paciente asintomático.
Asunto(s)
Humanos , Niño , Anomalías del Sistema Respiratorio/cirugía , Neumonectomía , Atención Prenatal , Imagen por Resonancia Magnética , Toracotomía , Radiografía Torácica , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Anomalías del Sistema Respiratorio/clasificación , Anomalías del Sistema Respiratorio/embriología , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Riesgo , Pulmón/anomalíasRESUMEN
INTRODUCTION: To date, no consensus has been reached on the optimal management of congenital lung abnormalities, and factors predicting postnatal outcome have not been identified. We developed an objective quantitative computed tomography (CT) scoring method, and assessed its value for clinical decision-making. METHODS: Volumetric CT-scans of all patients born with a congenital lung abnormality between January 1999 and 2018 were assessed. Lung disease was quantified using the newly-developed congenital lung abnormality quantification (CLAQ) scoring method. In 20 equidistant axial slices, cells of a square grid were scored according to the abnormality within. The scored CT parameters were used to predict development of symptoms, and SD scores for spirometry and exercise tolerance (Bruce treadmill test) at 8 years of age. RESULTS: CT-scans of 124 patients with a median age of 5 months were scored. Clinical diagnoses included congenital pulmonary airway malformation (49%), bronchopulmonary sequestration (27%), congenital lobar overinflation (22%), and bronchogenic cyst (1%). Forty-four patients (35%) developed symptoms requiring surgery of whom 28 (22%) patients became symptomatic before a CT-scan was scheduled. Lesional hyperdensity was found as an important predictor of symptom development and decreased exercise tolerance. Using receiver operating characteristic analysis, an optimal cut-off value for developing symptoms was found at 18% total disease. CONCLUSION: CT-quantification of congenital lung abnormalities using the CLAQ method is an objective and reproducible system to describe congenital lung abnormalities on chest CT. The risk for developing symptoms may increase when more than a single lung lobe is affected.
Asunto(s)
Pulmón/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Pulmón/anomalías , Masculino , Tomografía Computarizada por Rayos XRESUMEN
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Asunto(s)
Humanos , Femenino , Niño , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/cirugía , Pulmón/diagnóstico por imagen , Pulmón/anomalías , Tomografía Computarizada por Rayos X , Diagnóstico TardíoRESUMEN
There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate both well-recognized and more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. In Part 2 the authors review abnormalities in development, origin and course of the central branch pulmonary arteries as well as abnormal pulmonary artery caliber.