Lobar Emphysema Research Articles

Congenital Lobar Emphysema at Right Upper Lobe: Case Report

Emphysema is a disease of a lung condition due to airway and or alveolar abnormalities usually a chronic progressive lung disease caused by significant exposure to noxious particles or gasses that causes shortness of breath, but Congenital Lobar Emphysema is a rare malformation developmental of a lung, especially at right upper lobe of the lung, and it can cause severe respiratory distress in the newborn baby. Herein, we aimed to report our case of Congenital Lobar Emphysema

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

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

Neonatal Outcomes of Multiple Congenital Thoracic Lesions

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.

High comorbidity rates in congenital lobar emphysema and the effect on clinical presentation.

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 lobar emphysema: a 10-year case series review

Congenital lobar emphysema: a 10-year case series review

Surgical Management Of Congenital Thoracic Disorders: A 15-Year Center Experience.

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.

The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran

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.

Lobar Emphysema Successfully Operated in an Extreme Premature Neonate

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.

The use of bronchial occlusion test in a preterm infant with severe bronchopulmonary dysplasia complicated by severe lobar emphysema

In infants with severe bronchopulmonary dysplasia (sBPD), severe pulmonary lobar emphysema may occur as a complication, contributing to significant impairment in ventilation. Clinical management of these infants is extremely challenging and some may require lobectomy to improve ventilation. However, prior to the lobectomy, it is very difficult to assess whether the remaining lung parenchyma would be able to sustain adequate ventilation postoperatively. In addition, preoperative planning and perioperative management are also quite challenging in these patients. This paper reports the utility of selective bronchial occlusion in assessing the safety and efficacy of lobectomy in a case of sBPD complicated by severe right upper lobar emphysema. Since infants with sBPD already have poor lung development and significant lung injury, lobectomy should be viewed as a non-traditional therapy and be carried out with extreme caution. Selective bronchial occlusion test can be an effective tool in assessing the risks and benefits of lobectomy in cases with sBPD and lobar emphysema. However, given the technical difficulty, successful application of this technique requires close collaboration of an experienced interdisciplinary team.

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.

Giant Congenital Lobar Emphysema: A Rare Cause of Persistent Respiratory Distress in Neonatal Intensive Care

Congenital lobar emphysema (CLE) is a rare congenital anomaly of the lung causing excessive aeration of one or more lobes of a histologically normal lung. It manifests in the first weeks of life and rarely in the neonatal period, with respiratory distress due to compression atelectasis and often associated with mediastinal displacement. CLE poses a challenge in terms of diagnosis and positive pressure ventilation due to air trapping. We report the case of a newborn presenting with persistent respiratory distress. A thoracic CT scan was performed, confirming the diagnosis of CLE. The definitive diagnosis is provided by histology of the operative specimen, revealing emphysematous lesions. Treatment involves lobectomy after stabilization of respiratory function. Surgical abstention is possible for asymptomatic or paucisymptomatic lesions.

A Review of Management of Congenital Sublobar Hyperinflation at a Single Institution.

There has been an increased recognition of a subset of congenital lobar emphysema (CLE), termed congenital sublobar hyperinflation (CSLH), which may affect only a segment of lung as opposed to an entire lobe. This is an uncommon variant for which there is a paucity of information in published literature. The majority of CLE are managed surgically. Current literature suggests non-operative management for CSLH. However, there has been slow adoption of non-operative management and there is not a well-established observation pathway. A retrospective review of all pediatric patients diagnosed with CSLH at a single institution was performed from 2017 to 2023 to determine if this variant may be safely managed with observation. A total of 10 patients were identified. Of these, three patients had consolidation on cross-sectional imaging; therefore, operative intervention was undertaken given diagnostic uncertainty. All patients managed observationally remained asymptomatic. This case series validates non-operative management for patients with asymptomatic CSLH.

Congenital lobar emphysema mis-interpreted as tension pneumothorax: a case report and literature review

Introduction and importance: Congenital lobar emphysema (CLE) is a rare developmental lung anomaly that occasionally remains asymptomatic until adulthood. The right diagnosis is very crucial in its management. Case presentation: The authors report the case of a 29-year-old otherwise healthy, nonsmoker male. Referred after management of a presumptuous diagnosis of tension pneumothorax. His symptoms at initial presentation were a chronic cough and progressive exertional dyspnea. His chest radiography revealed absent lung markings in the right lung field with a trachea deviation to the left, interpreted as a tension pneumothorax. However, chest tube insertion worsened his dyspnea. A computed tomography scan, though initially interpreted as pneumothorax, showed findings consistent with CLE. Discussion: Adult CLE is a very rare presentation. A computed tomography scan is the imaging of choice. Even though watchful waiting is reasonable in selected patients, lobectomy remains the standard of care, preferably via video-assisted thoracic surgery. Conclusion: Failure to recognize CLE or other cystic lung lesions can lead to harmful procedures.

Congenital Lobar Emphysema in Children: Case Series.

Recurrent wheezing is very common in infants. When these symptoms appear early without a free interval, a pulmonary malformation should be investigated. Congenital lobar emphysema is a rare abnormality of the lower respiratory tract. Here, we report a case series of six cases of congenital lobar emphysema between 2015 and 2023. Clinical and radiological data were collected according to an operating sheet previously established in our pediatric pneumo-allergology unit. They all had recurrent wheezing and dyspnea. Chest radiography and chest CT were consistent with the diagnosis of congenital lobar emphysema.All patients had lobectomywithout complications.

Pediatric Thoracoscopy: Ten Years Experience in Tertiary Hospital

Purpose: A minimally invasive Thoracoscopic Surgery (TS) offers several options in diagnosis and surgical treatment in pediatric surgery. This study evaluates experience and outcome of thoracoscopic surgery in pediatrics during 10 years period in tertiary hospital. Patients and methods: This retrospective study conducted at King Faisal Hospital and Research Center, Jeddah, Saudi Arabia from 2012 to 2022. Data of 43 patients who underwent TS were enrolled. Data include patient’s demographic characteristics, co-morbidity, diagnosis, type of thoracoscopic procedures, time of intervention, length of hospital stay, post-operative complications. Results: Mean age of patients was 4.99 years. Females were more than males (55.8 vs. 44.2%). Sixteen (37.2%) patients had comorbidities. Thoracoscopic proce- dures were feasible in 43 children and adolescents with chest diseases including lung empyema (20.9%), congenital lobar emphysema (14.0%), interstitial lung disease (14.0%), congenital pulmonary airway malformation (11.6%), mediastinal mass (9.3%), lung metastasis (11.6%), lymphoma (4.7%), chronic inflamma- tions (7.0%), hemothorax (2.3%), bronchogenic cyst (2.3%), pleural effusion (2.3%) and pulmonary sequestration and right diaphragmatic hernia (2.3%). Proce- dures were made mostly for lung biopsy (25.0%), lobectomy (22.7%), decortication (20.9%), mediastinum mass resection (6.8%), lung metastasectomy (6.8%), lymph node biopsy (4.5%), diagnostic (4.6%), cyst excision (4.6%), diaphragmatic hernia repair (2.3%) and thymectomy (2.3%). Duration of hospital stay ranged from 2-180 days. All of the cases had chest drain (100.0%) and some of them required post-operative intubations (30.2%). Conversion rate to open was (4.7%). Complications were pneumothorax (7.0%), bleeding (2.3%) and air leak (2.3%). Conclusions: Thoracoscopic approach is a challenging method for diagnosis and treatment in pediatrics. However, complications were reported as pneumo- thorax, bleeding and air leak. In order to improve results, conversion rates, and the capacity to handle thoracoscopic complications, proper training is crucial.

Congenital Lobar Emphysema

A 29-year-old male had complained of right-sided chest pain for the past 2 years. On evaluation, breath sounds were diminished in the right mammary, axillary, infra-axillary, and infrascapular areas on auscultation, and hyperresonance was noted on percussion. Computed tomography chest plain done showed right lower lobe superior segment emphysema with obliterated segmental tertiary bronchus suggestive of congenital segmental emphysema. Bronchoscopy done showed narrowed right lower lobe lateral segment and Bronchoalveolar lavage (BAL) analysis done was normal. He became symptomatically better and hence managed conservatively. Congenital lobar emphysema is a rare developmental malformation of the lungs due to deficient development of bronchial cartilages, leading to overinflation of the affected lobe. With a wide range of presentations, it poses a diagnostic and therapeutic dilemma. Hence, knowledge of the diagnosis and treatment is essential.

Quantifying emphysema in lung screening computed tomography with robust automated lobe segmentation.

Anatomy-based quantification of emphysema in a lung screening cohort has the potential to improve lung cancer risk stratification and risk communication. Segmenting lung lobes is an essential step in this analysis, but leading lobe segmentation algorithms have not been validated for lung screening computed tomography (CT). In this work, we develop an automated approach to lobar emphysema quantification and study its association with lung cancer incidence. We combine self-supervised training with level set regularization and finetuning with radiologist annotations on three datasets to develop a lobe segmentation algorithm that is robust for lung screening CT. Using this algorithm, we extract quantitative CT measures for a cohort () from the National Lung Screening Trial and analyze the multivariate association with lung cancer incidence. Our lobe segmentation approach achieved an external validation Dice of 0.93, significantly outperforming a leading algorithm at 0.90 (). The percentage of low attenuation volume in the right upper lobe was associated with increased lung cancer incidence (odds ratio: 1.97; 95% CI: [1.06, 3.66]) independent of risk factors and diagnosis of whole lung emphysema. Quantitative lobar emphysema improved the goodness-of-fit to lung cancer incidence (, ). We are the first to develop and validate an automated lobe segmentation algorithm that is robust to smoking-related pathology. We discover a quantitative risk factor, lending further evidence that regional emphysema is independently associated with increased lung cancer incidence. The algorithm is provided at https://github.com/MASILab/EmphysemaSeg.

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.

The long-term outcomes of symptomatic congenital lobar emphysema patients.

Surgical (OP) management for symptomatic congenital lobar emphysema (CLE) is the standard of care with nonoperative (NOP) approach applied for asymptomatic cases. The aim of this study is to report the outcomes for NOP approach to the care of symptomatic CLE infants. A retrospective study of CLE patients treated 2000-2021 at a single institution. Patients with CLE and respiratory symptoms were included. Overall, 23 children had symptomatic CLE, and 12 had NOP management. The median age at diagnosis was 38.5 days (50) in the NOP group versus 25 days (20) in the OP group (p = 0.31). There was no significant difference in the location of the involved lobe, term birth, postnatal diagnosis and gender, and both groups required noninvasive support in 33% of the cases. There was a trend towards higher frequency of oxygen support in the OP group preoperatively (89% vs. 42%, p = 0.07). The median length of stay was 14 days in the NOP group compared to a median postsurgery stay of 7.5 days in the OP group. In follow-up, there was no significant difference in respiratory readmission in first year of life, growth delay, treatment with asthma medication or body mass index in the NOP versus OP group. None of the children in the NOP group required surgery during follow-up. A NOP approach for symptomatic CLE infants can have favorable long-term outcomes. Further studies will be required to identify markers to aid in clinical decision-making.

Thoracotomy in Children: Review from a Low-Income Country.

Thoracotomy is indicated for several congenital and acquired disorders in children. It is among the surgical procedures which require a well-trained and dedicated surgical, anesthesia and critical care team which can be difficult to assemble in a low-income country setup. As the pattern and outcome of thoracotomy in children remained unreported from such setting, this study aims to shed light on this matter. A descriptive cross-sectional review was conducted. Children who have undergone thoracotomy for non-cardiac pathologies were included in the study. Demographic and clinical data were collected by chart review. Frequencies and percentages were used to describe categorical variables while mean, median, standard deviation and interquartile range were calculated for continuous variables. A total of 68 patients were operated on in the study period, out of which 44 (64.7%) were males. The mean ages of the children at the time of diagnosis and procedure were 4.05 ± 3.9 years and 4.14 ± 4.03 years, respectively. The most common indication for thoracotomy was pulmonary hydatid cyst (17; 25%) followed by congenital lobar emphysema (11; 16.2%). Muscle sparing posterolateral thoracotomy was the most common approach in 66 (97.1%) patients. The analgesic medications that were used in the post-operative period were paracetamol, diclofenac, ibuprofen, tramadol and morphine. Combined analgesics were administered in two-thirds of the patients while a single analgesic was used in the rest of the children. No regional blocks were administered post operatively as pediatric size catheters were not available. The morbidity and mortality rates were found to be 11.8% and 8.8%, respectively. The most common indication for thoracotomy in this study was pulmonary hydatid cyst. The provision of post-thoracotomy analgesia in our institution is suboptimal as evidenced by no use of regional blocks and poor practice of administering multimodal analgesia. Thoracotomy was associated with fairly high morbidity and mortality.