Congenital Lung Abnormalities Research Articles

Role of Multidetector Computed Tomography with Contrast in Evaluation of Congenital Lung Malformations in Pediatric Age Group at a Tertiary Care Hospital: An Observational Study

Background: Computed tomography (CT) has a larger role in the noninvasive examination of congenital lung abnormalities, thanks to the introduction of multidetector CT (MDCT). If a surgical lesion is suspected, it is often necessary to use cross-sectional imaging, such as CT, for diagnosis confirmation, further lesion definition, and preoperative evaluation. Aims and Objectives: The aim of this study was to enhance comprehension of the occurrence and varieties of congenital lung abnormalities in children, as well as to assess and compare the diagnostic precision of MDCT with that of surgical intervention. Methodology: MDCT with contrast CT was performed in children with respiratory complaints and prenatal history of suspicion in targeted imaging for fetal anomalies (TIFFA) or congenital lung malformations (CLMs) using TOSHIBA 8 slice CT; the study population consisted of 40 pediatric patients with a history of chest anomalies during pregnancy in TIFFA or children with abnormal chest X-ray suspicion of lung anomalies for 18 months. Results: In a study of 40 pediatric patients who underwent MDCT with contrast to evaluate CLMs, 6 were excluded from further evaluation with contrast due to the absence of pulmonary anomalies on plain CT, leaving a possible diagnosis of s CLM in the remaining 34 cases. Of these, cystic pulmonary airway malformations (CPAM) (41.1%) were the most common, followed by congenital lobar emphysema (17.6%), bronchogenic cyst (14.7%), lung agenesis (11.6%), and phylogenetic lung. Conclusion: To notice the characteristics and related anomalies of CLMs and aid in the presurgical evaluation of CLMs, the study concludes that MDCT with contrast is the imaging of choice.

Congenital Lung Malformations: A Pictorial Review of Imaging Findings and a Practical Guide for Diagnosis.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.

Lower respiratory tract infections in children with congenital lung abnormalities.

We aimed to determine if the incidence of lower respiratory tract infections (LRTI) among children with asymptomatic, observationally managed congenital lung abnormalities (CLA) differed from that of symptomatic patients who underwent surgery. Second, we sought to compare the pre- and post-resection incidence of LRTI in patients who underwent surgery. This retrospective cohort study included patients born between 1999 and 2021 with CLA confirmed by CT scan who were enrolled in a prospective longitudinal follow-up program. The LRTI incidence rates at 1, 2, 5, 8, and 12 years were compared between surgically and observationally managed patients using incidence rate ratios (IRR). Differences in pre- and post-resection LRTI incidence rates among patients who underwent CLA-related surgery were assessed through IRR. Among 217 included patients, 81 (37%) had undergone surgery and 136 (63%) had been observationally managed. The LRTI incidence rates did not significantly differ at any follow-up moment between the surgical and observational groups. Among the children who underwent CLA-related surgery, the pre-resection LRTI incidence rates were significantly higher than the post-resection LRTI incidence rates (IRR of 3.57, 95% confidence interval: [2.00; 6.33], p < .001). We could not demonstrate differences in LRTI incidence throughout childhood between patients with surgically and observationally managed CLA. We recommend discussing cases of LRTI in patients with CLA in a multidisciplinary setting, using additional diagnostics such as chest X-ray to screen for CLA involvement, enabling a well-considered decision on surgical resection of the lesion.

1236 A Challenging Transition Between Volume Assured Modes in a Patient with Congenital Lung Abnormalities

1236 A Challenging Transition Between Volume Assured Modes in a Patient with Congenital Lung Abnormalities

X-RAY DIAGNOSIS OF LUNG HYPOPLASIA

Pulmonary hypoplasia is a rare form of congenital disorder that leads to the underdevelopment of structural elements of the lungs. Although rare, it is associated with significant neonatal morbidity and mortality. Congenital lung abnormalities are frequently discovered in the early life following routine radiographic imaging and investigations. We report the case of a baby, at the age of one day. He was transferred to the NICU (Neonatal Intensive Care Unit, Tbilisi) with acute respiratory failure. This case report aims to aid future early detection to achieve better diagnostic outcomes.

Long-term neurodevelopment in children with resected congenital lung abnormalities.

To determine whether children who underwent resection of a congenital lung abnormality (CLA) are at higher risk for neurodevelopmental impairments than peers in the general population. The study population consisted of children born between 1999-2018 who underwent resection of a symptomatic CLA. Neurocognitive development (intelligence, memory, attention, visuospatial processing, executive functioning) and motor function of this population are monitored through our structured, prospective longitudinal follow-up program at the ages of 30months, 5, 8, and 12years. We compared study population scores with Dutch norm values using one-sample t-tests and one-sample binominal proportion tests. Forty-seven children were analyzed. The 8-year-olds showed significant impairments in sustained attention through the Dot Cancellation Test (mean z-scores -2.4; [-4.1; -0.8], p = 0.006 and -7.1; [-12.8; -1.4], p = 0.02 for execution speed and fluctuations respectively). Visuospatial memory was impaired at 8years, though only in 1 out of 3 assessment tools (Rey Complex Figure Test z-scores (-1.0; [-1.5; -0.5], p < 0.001). Further neurocognitive outcomes were unimpaired at all tested ages. Regarding motor function outcomes, mean z-scores of total motor functioning were unimpaired across assessed ages. However, at 8years, significantly more children than expected had definite motor problems (18% vs 5%, 95% CI [0.052; 0.403], p = 0.022). Conclusion: This evaluation reveals impairment in some subtests of sustained attention, visuospatial memory and motor development. However, globally, normal neurodevelopmental outcomes were found throughout childhood. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning. What is Known: • In general, surgically managed CLA cases seldom suffer from long-term surgery-related morbidity and show favorable lung function. What is New: • Long-term neurocognitive and motor function outcome appear unimpaired within surgically managed CLA cases. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning.

Congenital lung abnormalities on magnetic resonance imaging: the CLAM study

ObjectivesFollow-up of congenital lung abnormalities (CLA) is currently done with chest computer tomography (CT). Major disadvantages of CT are exposure to ionizing radiation and need for contrast enhancement to visualise vascularisation. Chest magnetic resonance imaging (MRI) could be a safe alternative to image CLA without using contrast agents. The objective of this cohort study was to develop a non-contrast MRI protocol for the follow-up of paediatric CLA patients, and to compare findings on MRI to postnatal CT in school age CLA patients.MethodsTwenty-one CLA patients, 4 after surgical resection and 17 unoperated (mean age 12.8 (range 9.4–15.9) years), underwent spirometry and chest MRI. MRI was compared to postnatal CT on appearance and size of the lesion, and lesion associated abnormalities, such as hyperinflation and atelectasis.ResultsBy comparing school-age chest MRI to postnatal CT, radiological appearance and diagnostic interpretation of the type of lesion changed in 7 (41%) of the 17 unoperated patients. In unoperated patients, the relative size of the lesion in relation to the total lung volume remained stable (0.9% (range − 6.2 to + 6.7%), p = 0.3) and the relative size of lesion-associated parenchymal abnormalities decreased (− 2.2% (range − 0.8 to + 2.8%), p = 0.005).ConclusionNon-contrast-enhanced chest MRI was able to identify all CLA-related lung abnormalities. Changes in radiological appearance between MRI and CT were related to CLA changes, patients’ growth, and differences between imaging modalities. Further validation is needed for MRI to be introduced as a safe imaging method for the follow-up of paediatric CLA patients.Key Points• Non-contrast-enhanced chest MRI is able to identify anatomical lung changes related to congenital lung abnormalities, including vascularisation.• At long-term follow-up, the average size of congenital lung abnormalities in relation to normal lung volume remains stable.• At long-term follow-up, the average size of congenital lung abnormalities associated parenchymal abnormalities such as atelectasis in relation to normal lung volume decreases.

Isolated unilateral absence of pulmonary artery- an unusual cause of hemoptysis: a case report

BackgroundIsolated unilateral absence of one pulmonary artery (IUAPA) is a rare malformation and a rare cause of hemoptysis. It occurs due to malformation of the sixth aortic arch during embryogenesis. This condition has variable presentations like dyspnoea, reduced exercise tolerance, chest pain, pleural effusion, recurrent bronchopneumonia, pulmonary hypertension, and haemoptysis can be asymptomatic.Case PresentationWe describe a case of a 34-year-old female presenting with recent onset hemoptysis associated with mild dyspnoea and cough. Chest Radiograph revealed unilateral hyperlucent lung field which was later diagnosed as Isolated unilateral absence of pulmonary artery on high resolution computed tomography of chest with angiography.ConclusionAbsent pulmonary artery should be considered as an important differential in cases presenting with hemoptysis. This case emphasises the significance of combining computed tomography with pulmonary angiography to accurately evaluate and define congenital lung abnormalities.

Preoperative visualization of congenital lung abnormalities: hybridizing artificial intelligence and virtual reality.

When surgical resection is indicated for a congenital lung abnormality (CLA), lobectomy is often preferred over segmentectomy, mostly because the latter is associated with more residual disease. Presumably, this occurs in children because sublobar surgery often does not adhere to anatomical borders (wedge resection instead of segmentectomy), thus increasing the risk of residual disease. This study investigated the feasibility of identifying eligible cases for anatomical segmentectomy by combining virtual reality (VR) and artificial intelligence (AI). Semi-automated segmentation of bronchovascular structures and lesions were visualized with VR and AI technology. Two specialists independently evaluated via a questionnaire the informative value of regular computed tomography versus three-dimensional (3D) VR images. Five asymptomatic, non-operated cases were selected. Bronchovascular segmentation, volume calculation and image visualization in the VR environment were successful in all cases. Based on the computed tomography images, assignment of the CLA lesion to specific lung segments matched between the consulted specialists in only 1 out of the cases. Based on the three 3D VR images, however, the localization matched in 3 of the 5 cases. If the patients would have been operated, adding the 3D VR tool to the preoperative workup would have resulted in changing the surgical strategy (i.e. lobectomy versus segmentectomy) in 4 cases. This study demonstrated the technical feasibility of a hybridized AI-VR visualization of segment-level lung anatomy in patients with CLA. Further exploration of the value of 3D VR in identifying eligible cases for anatomical segmentectomy is therefore warranted.

Bronchial atresia presenting with two bronchoceles

Bronchial atresia is a rare congenital lung abnormality characterized by the focal obliteration of the bronchial lumen, on a lobar, segmental or subsegmental level, usually associated with peripheral mucus impaction (bronchocele or mucocele) [1,2]. It presents with adjacent segmental or subsegmental hyperinflation, and reduction of vascularization of the involved segment or lobe.

A case of surgical treatment of lobar emfysema in a newborn

Lobar emphysema still remains a significant issue of pediatric surgery. It accounts for 14% of all congenital anomalies of the respiratory system. The development of intra-thoracic tension syndrome leads to a critical oxygen supply disorder. The main method of treatment of congenital pathology in case of severe respiratory disorders is operative. However, an unrecognized anomaly of lung development leads to incorrect surgical tactics. A clinical case of surgical treatment of a newborn with lobar emphysema of the lung complicated by intra-thoracic tension syndrome is presented. Video-assisted thoracoscopic resection of the lower lobe of the left lung was performed. Six months after the intervention, a complete restoration of the function of external respiration was detected. Timely full-fledged surgical treatment contributed to the elimination of the cause of the development of an urgent condition in congenital lung abnormality, effective restoration of organ functions and favorable outcome.

Reevaluating Congenital Heart Surgery Center Performance Using Operative Mortality

BackgroundThe Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) provides observed-to-expected (O/E) operative mortality ratios to more than 100 congenital heart centers in North America. We compared the current approach for estimating O/E ratios to approaches incorporating information on diagnosis as moderators of procedures, other unused risk factors, and additional variation in confidence interval construction to characterize center performance. MethodsBayesian additive regression trees (BART) and lasso models linked operative mortality to diagnosis-procedure categories, procedure-specific risk factors, and syndromes/abnormalities. Bootstrapping accounted for variation in the STS-CHSD (STS bootstrap) and lasso CIs. We compared O/E estimates, interquartile range of CI widths, and concordance of center performance categorizations (worse-than-, as-, or better-than-expected mortality) of the new approaches to the STS-CHSD. ResultsIn 110 surgical centers including 98,822 surgical operative encounters, there were 2818 (2.85%) operative mortalities (center range, 0.37%-10%). Compared with the STS-CHSD, BART- and lasso-estimated O/E ratios varied more and had narrower confidence intervals (interquartile range of confidence interval: STS-CHSD = 1.11, STS bootstrap = 0.98; lasso = 0.80; BART = 0.96). Concordance of performance categorization with the STS-CHSD ranged from 84% (lasso) to 91% (STS Bootstrap); more than 70% of discordant centers improved categories. Discordant centers had smaller volumes, fewer operative mortalities, and treated more patients with congenital lung abnormalities. ConclusionsRelative to the STS-CHSD, up to 16% of hospitals changed performance categories, most improving performance. Given the significance of quality reports for congenital heart centers, inclusion of additional risk factors and unaddressed variation should be considered.

Congenital Lung Abnormalities: Experience of a Tertiary Care NICU in Delhi, A Case Series

Respiratory distress is the most common cause of neonatal admission. Cystic lung lesions are rarer cause of the same. These are heterogeneous lesions with varied antenatal and postnatal manifestations. The outcomes of antenatally diagnosed lung malformations have showed more improvement than previously thought of. With advancement in imaging techniques, more definitive diagnosis and timely intervention, even in-utero interventions, can be planned. Overall, survival rates have also improved over the past 2 decades. A majority of infants are asymptomatic and do not require surgery in neonatal period, and the expectant management is usually followed in most centers, but some may have mass effect and require either in-utero or early neonatal intervention. For asymptomatic lesions, the timing of surgery remains controversial as there are propagators of both early and late intervention. A multidisciplinary team approach is required for its optimal management. We present here a set of 3 babies who were diagnosed as having cystic lung lesions antenatally but later these turned out to be completely different entities.

Quantitative CT imaging analysis to predict pathology features in patients with a congenital pulmonary airway malformation

BackgroundRisk 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. MethodsArchival 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. ResultsThirty-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. ConclusionsSmaller 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 evidenceLevel III.

VATS resection of basal pyramid segments in sequestration of the left lower lobe

Lung sequestration is a congenital malformation represented by a non-functioning pulmonary parenchyma supplied via an aberrant artery of systemic circulation. This malformation makes up 1-6% of all congenital lung abnormalities. In 1946, Pryce M.D. proposed the term «lung sequestration» for the disease first described by the Austrian pathologist C. Rokitansky in 1856. Lung sequestration becomes quire common due to development and availability of X-ray diagnostic methods. An aberrant artery can arise from the great vessels (thoracic and abdominal aorta) and smaller arteries (coronary, intercostal arteries). To date, there are 2 forms of lung sequestration (intralobar and extralobar). We report one of the options for surgical treatment of lung sequestration.

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.

1758 H-type Tracheoesophageal Fistula: A Rare Cause of Cough and Dysphagia in Adults

INTRODUCTION: Tracheoesophageal fistula (TEF) without associated esophageal atresia (H-type) is a rare congenital anomaly, accounting for ∼4% of esophageal malformations. It is defined as a connection between the posterior tracheal and anterior esophageal walls and is typically diagnosed before age 3. However, it can occasionally be seen in adults with chronic cough and respiratory infections. We present a 38-year-old female with a new diagnosis of H-type TEF. CASE DESCRIPTION/METHODS: A 38-year-old female (G16P12-0-3-12) with multiple prior admissions for hypoxia (presumed due to asthma and aspiration in the setting of achalasia) was admitted with HELLP syndrome. Prior pulmonary function tests showed severe airway obstruction and echocardiogram showed normal left and right ventricular systolic function. She reported coughing when supine and with meals, a history of congenital lung abnormality, severe infancy pneumonia, upper lobe resection, diagnosis of achalasia, and yearly admissions for pulmonary issues. After delivery, she was transferred to the pulmonary service for management of her hypoxia. A TEF without inflammation was noted on chest CT and confirmed by EGD and bronchoscopy. EGD showed a 3 mm proximal esophageal fistula. Bronchoscopy demonstrated a normal oropharynx and vocal cords (VC), erythematous larynx, inflamed arytenoids, a 4 mm proximal TEF without mucosal abnormalities, and right upper lobe (RUL) absence. A swallow study showed aspiration on the posterior side of the trachea presumed due to the TEF. Surgical repair rather than endoscopic clip or stent placement was determined to be the best treatment via cervical approach esophagoplasty, muscle flap reconstruction TEF repair, and laparoscopic Heller myotomy with Dor fundoplication. DISCUSSION: H-type TEF is rare and can mimic chronic lung disease due to aspiration, recurrent infection, and bronchiectasis. It must be considered in adults with recurrent respiratory infections, coughing with eating/drinking, and rarely, hemoptysis. A chest CT, bronchoscopy, esophagoscopy, and barium study are crucial to confirm the diagnosis. While endoscopic clip closure of fibrotic TEFs has been successful in 62%, it may obliterate surgical tissue planes. Surgery is the optimal treatment for H-type TEF and reported to be 75-94% successful. As H-type TEF disrupts myenteric plexus development with impaired esophageal peristalsis and lower sphincter dysfunction, a Heller myotomy and Dor fundoplication should be offered.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

Congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM), which was previously known as congenital cystic adenomatoid malformation, is one of the most common congenital lung abnormalities.In recent years, more and more reports about the disease have been published and its classifications also have new changes.This article introduces the concept of CPAM, classification, clinical manifestations, differential diagnosis, and treatment strategies. Key words: Congenital pulmonary airway malformation; Congenital cystic adenomatoid malformation; Congenital lung abnormalities