ABSTRACT. The article presents a comprehensive analysis of contemporary approaches to the diagnosis of congenital lung malformations and anomalies (CLMAs), which account for 5-18 % of all thoracic organ anomalies. Epidemiological aspects, classification, and morphological features of the most common forms are discussed, including congenital pulmonary airway malformation, pulmonary sequestration, congenital lobar emphysema, bronchogenic cysts, as well as rare anatomical variants such as tracheal bronchus or accessory lung. Attention is drawn to the phenomenon of hybrid lesions that combine characteristics of multiple nosologies. The significance of prenatal diagnostics, particularly ultrasound and MRI, is highlighted as essential for timely detection of pathologies and planning the management of newborns. Considerable attention is given to the genetic factors and pathogenesis of CLMAs, including the role of mutations in the FGF10 and TBX4 genes, as well as associations with primary ciliary dyskinesia. It is shown that malformation development has a polyetiological nature, combining genetic, embryological, and exogenous factors. Clinical manifestations range from asymptomatic cases to severe respiratory failure, and pathognomonic features of individual forms are identified. It is summarized that diagnosis of congenital lung malformations relies on a combination of clinical data, the results of instrumental methods (CT, MRI, bronchoscopy), histological studies, and genetic testing. The findings are crucial for timely selection of optimal therapeutic strategies, prevention of infectious complications, and reduction of the risk of malignant transformation.

Giant lobar emphysema (GLE), also known as congenital lobar overinflation (CLO), is a rare pulmonary anomaly in infants and young children characterized by hyperinflation of one or more pulmonary lobes. It may present acutely or be discovered incidentally. We report a case of a 2-year-old child who presented with acute dyspnea and was incidentally found to have giant emphysema of the left upper lobe on imaging. Surgical lobectomy was performed with favorable outcome. Although rare, GLE should be considered in the differential diagnosis of dyspnea in young children. Early recognition and appropriate management, including surgery, can lead to excellent outcomes.

Multiple congenital pulmonary malformations in a black-capped Bolivian squirrel monkey (Saimiri boliviensis boliviensis).

The Lobe Less Traveled: An Adult's Unexpected Journey With Congenital Lobar Emphysema

Congenital Lobar Emphysema Presenting as Acute Chest Pain in Adulthood

Bronchoscopic Lung Volume Reduction With Endobronchial Valves for Congenital Lobar Emphysema

Focal Pulmonary Interstitial Glycogenosis in a Newborn With Symptomatic Congenital Lobar Emphysema

In pediatric surgery, the operation of the lung parenchyma tissue is generally performed with limited essential evaluations both before and after the operation. Decisions to be taken are formed around some basic examination methods. For this reason, this review aims to determine the routine and more specific methods in the literature, which are evaluated in pre-and postoperative processes related to lung parenchyma surgeries, and to examine how these methods are applied to which diseases. In the study, pre-and postoperative examination methods of the lung were primarily examined. Later, these evaluations were classified into two separate groups: congenital and acquired diseases. Rare surgeries were excluded from the study. Among the congenital diseases, congenital pulmonary airway malformation, congenital lobar emphysema, and primary ciliary dyskinesia are discussed. Among acquired diseases, infections and cancer are discussed. In general, it has been determined that there is no regular follow-up and no standard practice. The reason for this was that the evaluation processes were not based on a particular algorithm; personal approaches came to the fore, and it was determined that the individual characteristics of the patients were very variable. The literature regarding pre- and postoperative evaluations of patients undergoing lung parenchymal surgery is impoverished. There is an information gap where multicenter systematization studies can be made of the decision-making mechanisms initiated on an institutional basis regarding those who have these surgeries.

361 A rare co-presentation of necrotizing enterocolitis and congenital lobar emphysema in a term infant

In this single-center retrospective analysis, we present case data and insights gathered over the past eight years. Additionally, we computed postnatal, pre-therapy lesion-to-lung ratios of Congenital Pulmonary Airway Malformations (CPAMs) to retrospectively assess potential outcome prediction using lesion-to-lung ratios. Data were collected between 2015 and 2022. Information such as chosen therapy, surgical duration, postoperative hospital stay, and follow-up was obtained from electronic case records. Pre-therapy pulmonary lesion volumes [mm3], lesion-to-ipsilateral-lung ratio, and lesion-to-both-lungs ratio of CPAMs were retrospectively calculated from computed tomography images using specialized software. Of the 40 identified cases, 27 had CPAM, 7 had pulmonary sequestration, 4 had bronchogenic cysts, and 2 had congenital lobar emphysema. Histological examinations of resected specimens revealed no malignancy. For CPAMs, the median surgery age was 7 months (interquartile range (IQR): 0.45-11), averaging 9.54 ± 15.01 months. The CPAM surgery lasted on average 126 ± 53 min (median 124 min (IQR 108-172)). The mean length of hospital stay was 6 ± 1.41 days for thoracoscopic surgery and 17 ± 18.23 days for open surgery. No clear link was found between the lesion ratio and management choice or surgical length. Notably, larger lesions tended to result in longer postoperative stays. CPAMs with a lesion-to-ipsilateral-lung ratio of ≤0.11 were asymptomatic. If patients present no symptoms, mild symptoms, or smaller CPAM lesions, "wait and watch" and a CT scan of the thorax up to approximately six months of age remain a reasonable approach. The true risk of malignancy remains ambiguous, especially since there was no evidence of malignancy in our biopsies. On the other hand, prophylactic surgery before symptoms arose led to earlier discharge and overall low intraoperative complications compared to symptomatic counterparts. Ultimately, the adopted therapy pathway remains a parental choice. For CPAMs, an increased lesion-to-lung ratio correlated with extended hospital stay and symptomatic presentation. However, there was no cut-off value for conservative or surgical treatment.

Bronchopulmonary malformations (BPM) are lower respiratory tract anomalies that include congenital malformations of the pulmonary airways (CMPA), bronchogenic cysts (BC), bronchopulmonary sequestrations (BPS), and congenital lobar emphysema (CLE). Prenatal detection in low- and middle-income countries is less common than in high-income ones. This study aims to show the experience in the surgical approach to BPM in a Brazilian center, with emphasis on clinical evolution and surgical results, according to the time of diagnosis (prenatal versus postnatal). We retrospectively analyzed medical records of patients under the age of 18 who underwent surgery for BPM at a referral center in a middle-income country between 2000 and 2021. Based on the time of BPM diagnosis, we divided the children into two groups: prenatal and postnatal. These groups were evaluated in terms of age at surgery, history of pneumonia before the operation, surgical outcomes (perioperative and postoperative complications, duration of mechanical ventilation, duration of chest tube, length of hospital stay), and histological type of BPM. In the cohort of 66 patients, 43 (65.1%) had a prenatal diagnosis of BPM, while 23 (34.8%) were identified after birth. Compared with patients diagnosed prenatally, those diagnosed after birth underwent surgery at a higher age (mean of 978 days ± 1341.0 versus mean of 200 days ± 360.9; p<0.01), and had a higher incidence of pneumonia before surgery (65% vs. 12%, p < 0.01). There was no association between the time of BPM diagnosis and postoperative outcomes. All patients with BPS were in the prenatal group, and all patients with CLE were in the postnatal one. There was a higher prevalence of CMPA in the prenatal group compared with the postnatal one (72% vs. 39%, p < 0.01). In a Brazilian center, approximately 2/3 of the patients had an intrauterine diagnosis of bronchopulmonary malformations and were treated early at a neonatology center. Patients diagnosed with BPM only after birth were more likely to have pneumonia and undergo surgery at an older age than patients with an intrauterine diagnosis. Prospective, multicenter studies, including asymptomatic patients treated conservatively, without surgical interventions, and patients operated by video-assisted thoracoscopy, would be well indicated to evaluate the future evolution of children with BPM and to establish protocols appropriate to the Brazilian reality.

Congenital lobar emphysema: a 10-year case series review

Patients with congenital lung malformation (CLM) may present pulmonary inflammatory changes. However, little is known about the factors influencing local inflammation. The aim of this study was to evaluate the factors that may affect inflammatory changes in CLM. Patients with CLM operated upon between 2005 and 2021 were included. The grade of inflammation was defined with a purpose-made inflammatory score (IS) ranging from 0 to 5. The association of type of CLM and age at surgery with IS was analyzed. Results are means (standard deviation). Data from 105 patients with CLM were collected, 56 had congenital pulmonary airways malformation (CPAM), 24 bronchopulmonary sequestration (BPS), and 25 congenital lobar emphysema (CLE). 91 patients (87%) had inflammatory changes. IS was 2.1 (1.5), 1.2 (1.0), and 1.3 (1.5) in CPAM, BPS, and CLE respectively (One-way ANOVA p = 0.0101). CPAM showed a significantly higher IS as compared with BPS (p = 0.0242) and CLE (p = 0.0495). Age at operation significantly correlated to IS (r2 = 0.14; p < 0.0001). Patients aged below 6months at operation had lower IS [1.4 (1.2)] as compared to those over 6months [2.0 (1.6)] (p = 0.018). Age at operation significantly correlated with the IS in CPAM (r2 = 0.17; p = 0.0016) and CLE (r2 = 0.47; p < 0.0001) patients. Patients with CLMs often present inflammatory changes in their lungs. Grade of inflammation significantly correlates with age at surgery and type of anomaly, with CPAMs having the highest grade. These findings support early resection in patients with CLM, especially in case of CPAM.

Recurrent dyspnea caused by congenital lobar emphysema in a premature child.

Introduction: Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort. Methods: Retrospective chart review of patients born between September 1, 2013, and March 31, 2023, who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics. Results: Among 701 CTL patients, 74 (10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8 (12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n = 33, 44%). Bilateral CTL were found in 9 (12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n = 36, 49%). Genetic syndromes were confirmed in 3 (4%) and additional congenital anomalies in 9 (12.9%) patients, 5 of those had multiple congenital anomalies. Of 49 (65%) patients with multilobar CTL, 25 (51%) underwent bilobectomy and 24 (49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%. Conclusion: We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.

The presence of comorbidities (CM) in congenital lobar emphysema (CLE) has been previously described with varying rates. However, the clinical implication of CM on the clinical presentation and patient outcome in CLE is unclear. This was a retrospective cohort study between 2000 and 2022 in a single institution. The study included pediatric patients diagnosed with CLE. During the study period, 36 patients were identified with CLE. The presence of respiratory symptoms at diagnosis was documented in 69% (24/35) of the patients with 48% admitted to intensive care units. The presence of CM was documented in 14/36 (39%) of the patients, with cardiovascular anomalies as the most common (22%), followed by genitourinary anomalies (11%) and large airway anomalies (8%). When comparing the CM group (n = 14) vs the non-CM group (N = 22), there was no significant difference regarding gender, term birth, age of diagnosis, and the location of the lesion. The CM group had significantly higher rates of intensive care unit (ICU) admissions (75% vs 33%, p = 0.02), longer duration of admission (median 46days vs 9, p = 0.02), and need for pre-operative non-invasive respiratory support (50% vs 5%, p = 0.002).Conclusion: The presence of CM in CLE was associated with a more severe presentation as reflected with significantly higher rates of ICU admissions, prolonged admissions, and higher need for non-invasive respiratory support. Screening for CM, mostly for cardiac anomalies, is recommended at time of CLE diagnosis. What is Known: • Congenital lobar emphysema (CLE) is a developmental lung anomaly generally associated with other comorbidities; however, their clinical implication on presentation is unclear • The prevalence of comorbidities in CLE was previously reported around 15-20%, with cardiovascular anomalies as the most common What is New: • Comorbidities are very common (39%) in CLE, and their presence is related to a more severe clinical presentation and need for respiratory support • Screening for comorbidities is recommended in CLE, specifically for cardiovascular and genitourinary anomalies.

Congenital thoracic disorders represent a spectrum of fetal lung bud development abnormalities, which may affect breathing capacity and quality of life. We aim to evaluate the impact of surgery in the treatment of 4 major congenital conditions. We performed a retrospective cohort analysis of patients who underwent surgical treatment in our tertiary center, from 2007 to 2022. Over the 15-year period, we treated 33 patients, with a male predominance of 55%. 22 patients (67%) were asymptomatic. When symptomatic, the recurrence of respiratory infections was the most common clinical presentation (18%). In 13 patients (39%), diagnosis was achieved through fetal ultrasonography. This study encompassed 13 patients with pulmonary sequestration (39%), 11 patients with bronchogenic cysts (33%), 7 patients with congenital pulmonary airway malformation (21%) and 2 patients with congenital lobar emphysema (6%). Considering solely lung malformation conditions, we accounted 22 patients with a median age of 3 [1-67] years-old. Surgery comprised bilobectomy (9%), lobectomy (77%), lobectomy with wedge resection (5%), segmentectomy (5%) and wedge resection (5%). Concerning bronchogenic cysts, we treated 11 patients with a median age of 19 [14-66] years-old. We identified 1 hilar, 1 intrapulmonary and 9 mediastinal lesions, of which 4 were paraesophageal, 4 were subcarinal and 1 was miscellaneous. Overall, surgery was conducted by thoracotomy in 61% of patients, VATS in 33% and RATS in 6%. The median drainage time was 3 [1-40] days and median hospital stay was 4 [1-41] days. There were no cases of mortality. Ensuing, 94% of patients experienced clinical improvement after surgery. Early diagnosis of congenital thoracic malformations increased considerably with the improvement in imaging technology and prenatal screening. Treatment may include expectant conservative treatment. However, in selected cases, surgery may play an important role in symptomatic control and prevention of disease progression.

IntroductionCongenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology.MethodThis retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran.ResultThe average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients’ type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04).ConclusionSome CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.

Background: Lobar emphysema in a newborn may be due to a rare congenital developmental anomaly of the lung, termed congenital lobar emphysema (CLE) or may develop secondary to lung damage, especially with positive airway pressures in premature newborns, known as acquired lobar emphysema (LE). They may have overlapping clinical and radiological features. However, surgical lobectomy is required whenever there is significant respiratory compromise. Reports of surgery in LE in extreme preterms are sparse. Clinical Description: A female newborn with gestational age of 28 weeks, weight of 1.180 kg, presented with respiratory distress after birth, initially treated as hyaline membrane disease. With worsening respiratory distress on positive airway pressures, repeat chest X-ray and computed tomography (CT) chest on day 15 of life, suggested possible LE on the left side with the tracheo-mediastinal shift to the right. Management and Outcome: Left thoracotomy and left upper lobectomy was done, following which, there was rapid improvement in respiratory distress, the newborn being extubated after 6 hours, and weaned off oxygen over the next 3 days. The baby was discharged after 8 weeks and at the last follow-up of 3 years of age, the child was growing well with no complications. Conclusion: Preterm neonates with initial radiological features of hyaline membrane disease, requiring prolonged respiratory support, may be harboring other underlying lung anomalies, which are identified by CT chest. Lobar emphysema with tracheo-mediastinal shift warrants prompt surgery, which is feasible and successful, even in a low birth-weight premature infant.

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.