Congenital Lobar Overinflation Research Articles

Prenatal ultrasound, magnetic resonance imaging and therapeutic options for fetal thoracic anomalies: a pictorial essay.

Congenital thoracic anomalies are uncommon malformations that require a precise diagnosis to guide parental counseling and possible prenatal treatment. Prenatal ultrasound (US) is the gold standard imaging modality to first detect and characterize these abnormalities and the best modality for follow-up. Fetal magnetic resonance imaging (MRI) is a complementary tool that provides multiplanar assessment and tissue characterization and can help estimate prognosis. Prenatal treatment is increasingly being used in fetuses with signs of distress and to potentially decrease morbidity and mortality. In this essay, the authors illustrate side-by-side US, MRI and therapeutic options for congenital thoracic anomalies in cases that presented to a tertiary pediatric hospital during the 7-year period 2014-2021. Entities included are congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, hybrid lesions, foregut duplications cysts and congenital lobar overinflation. Treatment options include maternal steroids, thoraco-amniotic shunt and fetal endotracheal occlusion. Recognition of typical findings in congenital thoracic anomalies is helpful to establish diagnosis, predict prognosis and plan perinatal treatment.

Congenital lobar emphysema: A rare cause of respiratory failure in neonates.

Congenital lobar overinflation, also known as congenital lobar emphysema (CLE), is an uncommon (1/20,000-30,000 live births) abnormality characterized by hyperinflation of one or more pulmonary lobes, usually with contralateral displacement of the mediastinum. While the etiology of most cases of CLE is poorly understood and labeled idiopathic, some cases are thought to be due to an intrinsic or extrinsic bronchial wall abnormality causing a ball valve mechanism with resultant hyperinflation of the affected lobe. CLE tends to have a predilection for males presenting insidiously in the first 6 months of life and have respiratory distress and progressive failure, with 50% of cases being asymptomatic at birth. Acquired forms of lobar emphysema are similar but are secondary to prolonged exposure to oxygen and positive pressure ventilation in premature infants. Clinical presentation is variable, ranging from wheezing, increased respiratory effort, cyanosis, feeding difficulties, and reflux or respiratory failure. Chest radiography (CXR) is the initial imaging obtained for any neonate with respiratory distress, which can aid in diagnosis showing overinflation, while computerized tomography (CT) remains the gold standard for confirmatory diagnosis. Treatment is variable and based on clinical severity. Some cases can be managed conservatively, while more severe cases require surgical intervention with lobectomy. Here, we present a case of a neonate in respiratory distress soon after birth, had initial improvement with supportive care, and was found to be secondary to CLE.

Fetal imaging of congenital lung lesions with postnatal correlation.

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.

A single institution's experience with the management of peripheral bronchial atresia.

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. 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. 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). 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 IV.

Is surgical intervention routinely required for congenital lobar overinflation? A case series from a tertiary hospital in Riyadh, Saudi Arabia

IntroductionCongenital lobar overinflation (CLO) is a congenital overinflation of a pulmonary lobe. The treatment choice depends on the severity of its symptoms. Surgical intervention is indicated for patients with significant symptomatology, while a conservative approach is used to treat incidental and mildly symptomatic lesions. However, the conservative approach for children with mild symptoms is not very common among pulmonologist. Therefore, we evaluated this approach to treating mildly symptomatic children. MethodsThis retrospective study examined mildly symptomatic patients (n = 14) with a radiological diagnosis of CLO between June 2005 and August 2018 who were treated conservatively at KFSHRC in Riyadh. The participants’ ages ranged between two days and four years, with follow-up period ranged from four months to 10 years. ResultsFourteen patients with CLO—who were 2 days to four years old and comprised 10 boys (71.4%) and four girls (28.6%)—were treated conservatively. All patients were symptomatic upon presentation, and their main clinical findings were tachypnea (85.7%) and dyspnea (78.6%). A single lobe was affected for ten patients (71.4%). Congenital cardiac anomalies founded in six patients (42.9%). Radiological image showed overinflation of all patients’ affected lobes. Significant mediastinal displacement was observed among two patients (14.3%). During their follow-up periods, nine patients (64.3%) became asymptomatic, three (21.4%) showed improvement, and two (14.3%) remained symptomatic and underwent lobectomy. ConclusionsThe good outcomes for mildly symptomatic children with CLO in our series indicate that the conservative approach can be considered to treat these children at any age, along with close follow-up.

Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.

To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM. This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center-Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth. Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM. CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Early neonatal lobectomy for ILE; is it feasible?

IntroductionCongenital lobar emphysema (CLE) also called congenital lobar overinflation and infantile lobar emphysema is a rare congenital anomaly of lung often presents in the neonatal period, with hyperinflation of one or more pulmonary lobes.ImportanceAuthor is reporting here an uneventfully done earliest ever neonatal lobectomy for ILE.

Congenital Lobar Overinflation (CLO) presenting as Severe Pneumonia

Congenital Lobar Overinflation (CLO) presenting as Severe Pneumonia

Congenital lung abnormality quantification by computed tomography: The CLAQ method.

IntroductionTo 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.MethodsVolumetric 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.ResultsCT‐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.ConclusionCT‐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.

Associated Anomalies in Congenital Lung Abnormalities: A 20-Year Experience

Introduction: A congenital lung abnormality (CLA) is often found in conjunction with other abnormalities but screening guidelines for newborns with CLA have not yet been reported. We aimed to assess the incidence of associated anomalies in CLA patients born or followed up at our centre and the need for additional screening of newborns with a CLA. Methods: From a retrospective chart review of all patients born with a CLA between January 1999 and January 2019, we identified patients diagnosed with a congenital pulmonary airway malformation, bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst, or lung agenesis. Associated anomalies were noted and categorized according to the affected organ system. Results: Twenty-eight (14%) of 196 CLA patients had a major associated anomaly. This was most frequent in conjunction with a lung agenesis (100%) or bronchogenic cyst (29%). Congenital heart defects (32%) and gastrointestinal defects (18%) were the most frequently associated anomalies. Examination of newborns with a CLA should focus on the cardiovascular and gastrointestinal tract, and a chest and abdominal radiograph may be useful to assess signs of major associated anomalies, regardless of the clinical course.

Three-dimensional Ultrashort Echotime Magnetic Resonance Imaging for Combined Morphologic and Ventilation Imaging in Pediatric Patients With Pulmonary Disease

Ultrashort echotime (UTE) sequences aim to improve the signal yield in pulmonary magnetic resonance imaging (MRI). We demonstrate the initial results of spiral 3-dimensional (3D) UTE-MRI for combined morphologic and functional imaging in pediatric patients. Seven pediatric patients with pulmonary abnormalities were included in this observational, prospective, single-center study, with the patients having the following conditions: cystic fibrosis (CF) with middle lobe atelectasis, CF with allergic bronchopulmonary aspergillosis, primary ciliary dyskinesia, air trapping, congenital lobar overinflation, congenital pulmonary airway malformation, and pulmonary hamartoma.Patients were scanned during breath-hold in 5 breathing states on a 3-Tesla system using a prototypical 3D stack-of-spirals UTE sequence. Ventilation maps and signal intensity maps were calculated. Morphologic images, ventilation-weighted maps, and signal intensity maps of the lungs of each patient were assessed intraindividually and compared with reference examinations. With a scan time of ∼15 seconds per breathing state, 3D UTE-MRI allowed for sufficient imaging of both "plus" pathologies (atelectasis, inflammatory consolidation, and pulmonary hamartoma) and "minus" pathologies (congenital lobar overinflation, congenital pulmonary airway malformation, and air trapping). Color-coded maps of normalized signal intensity and ventilation increased diagnostic confidence, particularly with regard to "minus" pathologies. UTE-MRI detected new atelectasis in an asymptomatic CF patient, allowing for rapid and successful therapy initiation, and it was able to reproduce atelectasis and hamartoma known from multidetector computed tomography and to monitor a patient with allergic bronchopulmonary aspergillosis. 3D UTE-MRI using a stack-of-spirals trajectory enables combined morphologic and functional imaging of the lungs within ~115 second acquisition time and might be suitable for monitoring a wide spectrum of pulmonary diseases.

Clinical and surgical aspects of congenital lobar over-inflation: a single center retrospective study

BackgroundCongenital lobar overinflation (CLOI) is one of the most important causes of infantile respiratory distress (RD). We aim to evaluate our experience in CLOI management emphasizing on clinical features, diagnostic modalities, surgery and outcomes.MethodsThis is a retrospective study for all CLOI cases undergoing surgical management at Qena University Hospital. Demographic data, clinical data, radiographic findings, surgery and postoperative follow-up were reviewed.ResultsA total of 37 neonates and infants with CLOI were presented to our center between January 2015 and January 2019; their mean age was 111.43 ± 65.19 days and 22 were males. All cases presented with RD; and cyanosis in 19 cases. 15 cases presented with recurrent pneumonia and fever. Diminished breath sounds on the affected side and wheezes were the main clinical findings in 30 and 22 cases respectively. On CXR, emphysema was detected in all cases. A confirmatory CT chest was done for all cases. Left upper lobe was affected in 23 cases, right middle lobe in 7 and right upper lobe in 7 cases.Lobectomy was done in thirty-one cases; their mean age at surgery was 147.58 ± 81.49 days and 19 were males. Postoperative complications were noted in 5 cases and postoperative ventilation was required for 2 of them. No morbidity or mortality was reported. The follow-up duration ranged from 3 months to 1 year and all patients were doing well except one case that lost follow up after 3 months.ConclusionCLOI is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile RD. CT chest is the most useful diagnostic modality. Early management of CLOI improves outcome and avoid life-threatening complications. Surgical management is the treatment of choice in our center without recorded mortality.

Congenital Lobar Overinflation: A Rare Enigmatic Lung Lesion on Prenatal Ultrasound and Magnetic Resonance Imaging.

To report the ultrasound (US) features in prenatal cases of suspected congenital pulmonary airway malformation or unspecified lung lesions with a final surgical pathologic diagnosis of congenital lobar overinflation (CLO). Institutional Review Board-approved radiology and clinical database searches from 2001 to 2017 were performed for prenatally diagnosed lung lesions with a final diagnosis of CLO. All patients had detailed US examinations in addition to magnetic resonance imaging (MRI). Size, echotexture, and vascularity were assessed with US, and the signal and vascularity were assessed with MRI. Follow-up prenatal US scans, postnatal imaging, and postnatal outcomes were reviewed. The study population consisted of 12 patients. The median gestational age was 23.3 weeks. The median congenital pulmonary airway malformation volume-to-head circumference ratio was 0.66. Lesion locations were 6 in the lower lobes (4 right and 2 left), 5 in the upper lobes (3 left and 2 right), and 1 in the right middle lobe. The texture was homogeneously echogenic relative to the normal lung in 100% with no visualized macrocysts. Hypervascularity by color Doppler US was observed in 5 cases (41.7%). A T2 hyperintense lung lesion was identified by MRI in 12 of 12 cases (100%), with elongated vessels identified in 11 of 12 cases (91.7%). All 12 cases had pathologically proven CLO. Congenital lobar overinflation should be considered in cases of prenatal echogenic lung lesions without macrocysts or classic findings of bronchial atresia. Hypervascularity may be an important imaging feature of a subset of CLO. Most cases become less conspicuous, decrease in size without overt hydrops, and are asymptomatic postnatally.

Imaging of congenital lung malformations

Congenital lung malformations are a heterogeneous group of anomalies that involve the lungs and tracheobronchial tree (congenital airway pulmonary malformation, bronchial atresia, bronchogenic cyst, congenital lobar overinflation, pulmonary cyst, hamartoma, pulmonary isomerism and azygous lobe), vascular abnormalities (arteriovenous malformations, anomalous pulmonary venous return, pulmonary artery sling, interrupted pulmonary artery, pulmonary varix, pulmonary vein stenosis and pulmonary lymphangiectasia), or frequently both entities (pulmonary sequestration, pulmonary maldevelopment and scimitar syndrome). Advances in diagnostic imaging (including sonography, multi-detector computer tomography, magnetic resonance imaging and angiography) have increased their detection during both antenatal and postnatal periods, and radiological characterisation, which in turn influence patient counselling and management stratification. An educational illustration of the clinical application in characterisation of these malformations is presented.

P23 The incidence of congenital thoracic malformations: an increasing trend from 1994 to 2011 in a defined population

IntroductionCongenital thoracic malformations (CTMs) are an uncommon heterogenous group of disorders including congenital cystic adenomatous malformation (CCAM), pulmonary sequestration (PS), congenital lobar overinflation (CLO), hybrid lesions and bronchogenic cysts. They...

Role of multi slice computed tomography in the evaluation of congenital anomalies of tracheobronchial tree and lungs

ObjectiveTo evaluate the role of MSCT in the evaluation of congenital anomalies of tracheobronchial tree and lung Patients and methodsTwenty nine patients with congenital anomalies of tracheobronchial tree and lung were examined using four and six MDCT. ResultsSeven patients (24%) had congenital cystic adenomatoid malformation where successful differentiation of the type was possible. Three patients (10.3%) with bronchopulmonary sequestration were categorized as two (7%) intralobar and one (3.5%) extralobar sequestrations. Four patients (14%) had congenital lobar overinflation. Four patients had Scimitar syndrome with right pulmonary artery hypoplasia, right sided lung hypoplasia, anomalous arterial supply and anomalous pulmonary venous drainage together with pulmonary hypertension. Abnormal bronchial anatomy was revealed in two patients with pulmonary isomerism and one patient with situs inversus. Bronchogenic cyst, tracheal bronchus, Kertagner's syndrome, pulmonary agenesis and horse shoe lung were seen in one patient each. Dynamic MSCT shows the lunate configuration of the trachea during forced expiration in one patient with tracheomalacia. In one patient with tracheo-esophageal fistula, the extent of fistulous tract and the resulting bronchial aspiration were demonstrated. ConclusionMSCT proved to be indispensable as a preoperative planning modality and an essential investigation for congenital anomalies of the lung and tracheobronchial tree.

Congenital Lung Abnormalities: Embryologic Features, Prenatal Diagnosis, and Postnatal Radiologic-Pathologic Correlation

Congenital lung abnormalities are being detected more frequently at routine high-resolution prenatal ultrasonography. The most commonly encountered anomalies include lung agenesis-hypoplasia complex (pulmonary underdevelopment), congenital pulmonary airway malformations, congenital lobar overinflation, bronchial atresia, bronchogenic cysts, congenital high airway obstruction syndrome, scimitar syndrome, and bronchopulmonary sequestration. Recognizing the antenatal and postnatal imaging features of these abnormalities is necessary for optimal prenatal counseling and appropriate peri- and postnatal management. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.306105508/-/DC1.

Interdisciplinary Teams and the Road to Discovery

HomeRadiologyVol. 254, No. 1 PreviousNext REVIEWS AND COMMENTARYPERSPECTIVESInterdisciplinary Teams and the Road to DiscoveryJonathan S. Lewin Jonathan S. Lewin Author Affiliations1From the Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 601 N Caroline St, Suite 4210, Baltimore, MD 21287.Address correspondence to the author (e-mail: [email protected]).Jonathan S. Lewin Published Online:Dec 14 2009https://doi.org/10.1148/radiol.09091395MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractThe development and efforts of interdisciplinary research teams hold great promise for the future of our field, and with some forethought and insight, these teams may represent the most powerful pathway toward the next generation of advances in imaging and imaging-guided intervention.© RSNA, 2010References1 Surowiecki J. The wisdom of crowds. 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Issues in the mentor-mentee relationship in academic medicine: a qualitative study. Acad Med 2009;84(1):135–139. Crossref, Medline, Google Scholar9 Lee JM, Anzai Y, Langlotz CP. Mentoring the mentors: aligning mentor and mentee expectations. Acad Radiol 2006;13(5):556–561. Crossref, Medline, Google Scholar10 Berk RA, Berg J, Mortimer R, Walton-Moss B, Yeo TP. Measuring the effectiveness of faculty mentoring relationships. Acad Med 2005;80(1):66–71. Crossref, Medline, Google Scholar11 Zerzan JT, Hess R, Schur E, Phillips RS, Rigotti N. Making the most of mentors: a guide for mentees. Acad Med 2009;84(1):140–144. Crossref, Medline, Google ScholarArticle HistoryReceived July 29, 2009; Revision requested July 30; revision received August 23; final version accepted August 24.Published online: Dec 14 2009Published in print: Jan 2010 FiguresReferencesRelatedDetailsCited ByContinuing to Thrive in Academic Radiology Despite Decreasing ReimbursementSunilJeph, KathleenGundry, JonathonMaffie, Jonathan G.Martin, Gloria J. 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Congenital masses of the lung: prenatal and postnatal imaging evaluation.

Congenital masses of the lung are a spectrum of interrelated abnormalities that includes congenital lobar overinflation, bronchogenic cyst, congenital cystic adenomatoid malformation (CCAM) and sequestration. The prenatal and postnatal imaging features of these lesions are reviewed, emphasizing the importance of serial prenatal sonograms and postnatal imaging studies, including radiography and computed tomography. Masses that become inconspicuous, or disappear on serial prenatal sonograms are discussed, as well as the importance of postnatal imaging studies in the evaluation of these lesions. Finally, the management of congenital masses of the lung is reviewed, emphasizing the importance of imaging studies in the preoperative evaluation.

Congenital Cystic Disease of the Tracheobronchial Tree in Infants and Children

Cystic anomalies of the tracheobronchial tree in infants and children are usually of congenital origin and consist of pulmonary sequestration (PS), cystic adenomatoid malformation (CAM), congenital lobar overinflation (CLO), and bronchogenic cyst (BC). These four anomalies present a different clinical picture, are often difficult to diagnose, and require surgical management. From December 1974 to April 1993, 44 patients were operated on for congenital cystic disease of the tracheobronchial tree. There were 25 male patients (57%) and 19 female patients (43%), ranging in age from 1 day to 18 years. Thirty-three children (75%) were younger than 1 year and 31 (70%) were younger than 6 months at the time of surgery. There were 17 CAMs (38%), nine intralobar (20%) and six extralobar (13%) PSs, seven CLOs (16%), and six BCs (13%). All patients with CAM, CLO, and intralobar PS were symptomatic and presented with either progressive respiratory distress or recurrent pulmonary infections with cystic changes noted on chest roentgenograms. Computed tomography was the most definitive diagnostic study performed, aided by selective use of ultrasonography and arteriography. All patients with CAM, CLO, and intralobar PS were treated with lobectomy; no segmentectomies were performed. Extralobar PSs and BCs were managed with resection of the cyst alone. There was one death (2%) in this series in a 1-day-old infant with a CAM who died of bilateral pulmonary hypoplasia. The remaining 43 patients (98%) are alive and well to date, with follow-up ranging from 2 months to 19 years. None of the patients who underwent a lobectomy have suffered any physical limitations during subsequent growth and development. Congenital cystic disease of the tracheobronchial tree can be diagnosed with computed tomography and selective use of ultrasonography and arteriography. Lobectomy is the appropriate treatment for CAM, CLO, and intralobar PS and is well tolerated without any subsequent long-term limitation in physical activity.