Lobar Emphysema Research Articles

Cystic and Cavitary Lung Lesions in Children: Radiologic Findings with Pathologic Correlation

A number of diseases produce focal or multiple thin-walled or thick-walled air- or fluid-containing cysts or cavitary lung lesions in both infants and children. In infants and children, there is a spectrum of focal or multifocal cystic and cavitary lung lesions including congenital lobar emphysema, congenital cystic adenomatoid malformation, pleuropulmonary blastoma, bronchogenic cyst, pulmonary sequestration, Langerhans cell histiocytosis, airway diseases, infectious diseases (bacterial infection, fungal infection, etc.), hydatid cysts, destroid lung, and traumatic pseudocyst. For the evaluation of cystic or cavitary lung lesion in infants and children, imaging plays an important role in accurate early diagnosis and optimal patient management. Therefore, a practical imaging approach based on the most sensitive and least invasive imaging modality in an efficient and cost-effective manner is paramount. We reviewed the conventional radiographs and computed tomography findings of the most common cystic and cavitary lung lesions in infants and children.

Ventricular Septal Defect Complicated by Infantile Lobar Emphysema

An infant with ventricular septal defect, atrial septal defect, and Down syndrome (trisomy 21) was referred to our institution. The patient gradually developed infantile lobar emphysema of the right upper lobe and became dependent on mechanical ventilator support. When the patient was five months of age, we successfully performed a total correction of cardiac anomalies combined with a right upper lobectomy. The resection of the right upper lobe was performed under cardiopulmonary bypass through a median sternotomy. There was no major airway bleeding or mediastinitis. The patient was extubated on postoperative day 12 and discharged on postoperative day 29.

Case of acquired lobar emphysema mimicking pneumothorax in a neonate

Despite the improvements in prevention of acute respiratory disease in preterm infants, the incidence of bronchopulmonary dysplasia (BPD) remains largely unchanged. Acquired lobar emphysema (ALE) is an increasingly recognized complication of advanced BPD. Barotrauma, oxygen toxicity and lung immaturity are presumed to play an important role in the development of ALE in children with BPD and most cases present overinflation [1,2]. We report on an infant with BPD who developed ALE mimicking pneumothorax.

Unilateral pulmonary agenesis: A report of four cases, two diagnosed antenatally and literature review

Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.

Hiperinsuflación lobar congénita: manejo conservador como alternativa terapéutica

Congenital lobar emphysema used to be treated surgically. Congenital lobar hyperinflation is the currently recommended term, as it involves pathologically healthy lung tissue, which is why conservative management may be an option. Four cases of diagnosed congenital lobar hyperinflation are presented in which conservative treatment was chosen due to their clinical stability. Their outcome has been satisfactory with progressively normal radiology.

Congenital lobar emphysema presenting during pregnancy

Congenital lobar emphysema presenting during pregnancy

A Rare Cause of Respiratory Distress in the Newborn: Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is a rare malformation of lung development caused by the hyperinflation of the lung lobe with compression of the normal lung parenchyma and contralateral displacement of the mediastinum. It may represent immediately at birth with respiratory insufficiency or have a slower onset at 6 months of life. The treatment of choice may be conservative in mild cases or surgical removal of the affected lobe may be needed in severe cases that have persistent or progressive respiratory distress. A baby diagnosed as having CLE and whose affected lobe was surgically removed at the newborn period is presented and discussed.

Pulmonary Arterial Remodeling in Chronic Obstructive Pulmonary Disease is Lobe Dependent

Abstract Pulmonary arterial remodeling has been demonstrated in patients with severe chronic obstructive pulmonary disease (COPD), but it is not known whether lobar heterogeneity of remodeling occurs. Furthermore, the relationship between pulmonary hypertension (PH) and pulmonary arterial remodeling in COPD has not been established. Muscular pulmonary arterial remodeling in arteries 0.10-0.25 mm in diameter was assessed in COPD-explanted lungs and autopsy controls. Remodeling was quantified as the percentage wall thickness to vessel diameter (%WT) using digital image analysis. Repeat measures mixed-effects remodeling for %WT was performed according to lobar origin (upper and lower), muscular pulmonary arterial size (small, medium, and large), and echocardiography-based pulmonary arterial pressure (no PH, mild PH, and moderate-to-severe PH). Lobar perfusion and emphysema indices were determined from ventilation-perfusion and computed tomography scans, respectively. Overall, %WT was greater in 42 subjects with COPD than in 5 control subjects ([Formula: see text]). Within the COPD group, %WT was greater in the upper lobes ([Formula: see text]) and in the small muscular pulmonary arteries ([Formula: see text]). Lobar differences were most pronounced in medium and large arteries. Lobar emphysema index was not associated with arterial remodeling. However, there was a significant positive relationship between the lobar perfusion index and pulmonary arterial remodeling ([Formula: see text]). The presence of PH on echocardiography showed only a trend to a small effect on lower lobe remodeling. The pattern of pulmonary arterial remodeling in COPD is complicated and lobe dependent. Differences in regional blood flow partially account for the lobar heterogeneity of pulmonary arterial remodeling in COPD.

Rudhe syndrome: reversible shunt related lobar emphysema – how far to investigate? A report of cases and systematic review of literature

week. Providers were randomly selected from professional society lists stratified by specialty to yield completed surveys from 100 Allergists/Immunologists, 100 Otolaryngologists (ENTs), 75 Family Medicine, 75 Pediatric and 50 Ophthalmology/Optometry physicians along with 50 Nurse Practitioners (NP) and 50 Physician Assistants (PA). Results: Respondents Providers saw average of 16 ARC patients (<18yrs) per week. Nearly all allergists (95%) reported confirming ARC diagnosis with skin prick or blood test, exceeding that reported by ENTs (62%), family medicine (37%), pediatric (35%), NP/PA’s (28%) and ophthalmologists/optometrists (4%). Allergists reported recommending SIT to 39% of patients <18 years old with 28% of all patients opting to use SIT. ENTs recommend SIT to 25% of pediatric patients and report 23% use SIT. Remaining specialties recommended SIT to ≤16% of pediatric patients and reported ≤13% use SIT. Two percent of providers reported that all patients completed 3 years of SIT while majority (60%) reported most or some patients; 18% reported few, 15% reported none and 4% did not know. Conclusion: Variation in approach to diagnosis and treatment of ARC patients was reported across provider specialty. Providers consistently reported pediatric patients using SIT and completing the minimum course of therapy.

Congenital lobar emphysema: 30-year case series in two university hospitals

OBJECTIVE: To review the cases of patients with congenital lobar emphysema (CLE) submitted to surgical treatment at two university hospitals over a 30-year period. METHODS: We reviewed the medical records of children with CLE undergoing surgical treatment between 1979 and 2009 at the Botucatu School of Medicine Hospital das Clínicas or the Mogi das Cruzes University Hospital. We analyzed data regarding symptoms, physical examination, radiographic findings, diagnosis, surgical treatment, and postoperative follow-up. RESULTS: During the period studied, 20 children with CLE underwent surgery. The mean age at the time of surgery was 6.9 months (range, 9 days to 4 years). All of the cases presented with symptoms at birth or during the first months of life. In all cases, chest X-rays were useful in defining the diagnosis. In cases of moderate respiratory distress, chest CT facilitated the diagnosis. One patient with severe respiratory distress was misdiagnosed with hypertensive pneumothorax and underwent chest tube drainage. Only patients with moderate respiratory distress were submitted to bronchoscopy, which revealed no tracheobronchial abnormalities. The surgical approach was lateral muscle-sparing thoracotomy. The left upper and middle lobes were the most often affected, followed by the right upper lobe. Lobectomy was performed in 18 cases, whereas bilobectomy was performed in 2 (together with bronchogenic cyst resection in 1 of those). No postoperative complications were observed. Postoperative follow-up time was at least 24 months (mean, 60 months), and no late complications were observed. CONCLUSIONS: Although CLE is an uncommon, still neglected disease of uncertain etiology, the radiological diagnosis is easily made and surgical treatment is effective.

CONGENITAL LOBAR EMPHYSEMA

The experience of diagnosis andtreatment of congenital lobar emphysema (CLE) wassummarized in 5 children aged from 3 weeks old to10 months. Male patients was 3, female - 2. In fourobservations noted lesion of the upper lobe of the leftlung, and in one - the middle lobe of the right lung. CLEdiagnosed in all children after birth. The indicationfor surgery was a progressive increase of the affectedlobe. In all cases lobectomy was performed. Therewere no complications. The possibility of prenataldetection of EDF and differential diagnosis with othercongenital anomalies of the lungs was discussed.

Congenital Lobar Emphysema : a case Report

This case is a bout a neonate with respiratory distress and primary diagnosis of Pneumothorax who had Congenital Lobar Emphysema. This case was the basis to review the topic of Congenital Lobar Emphysema (CLE) and the corresponding literature, considering that this is an uncommon disease and although congenital lobar emphysema is rare, clinical awareness of this condition is important for early diagnosis and effective surgical treatment. Due to the severity of respiratory symptoms, congenital lobar emphysema often requires surgical treatment in the early stages of life.

Mucoid Impaction and Lobar Emphysema Due to Bronchial Atresia Associated With a Bronchogenic Cyst

The respiratory system begins to develop at 3 to 4 weeks of gestation and continues during the first 2 years of life. Much of the elaboration of the tracheobronchial tree occurs between the fifth and 17th weeks. Aberrations in development during this period lead to congenital anomalies, and the details of many of these abnormalities are unclear. One point of contention relates to the embryonic stage when bronchial atresia occurs. We present a patient who demonstrated 2 congenital pulmonary anomalies, supporting the hypothesis that bronchial atresia occurs around the fifth week of gestation. This case report discusses the clinical and radiographic findings in bronchial atresia. The case also highlights the similarities and differences between bronchial atresia and bronchogenic cysts.

Congenital lobar emphysema surgical versus conservative management

Back ground: Congenital lobar Emphysema (CLE) is a rare yet serious condition which requires early detection and management, and usually present during infancy.&#x0D; Patients and methods: This retrospective study aimed to review and evaluate methods adopted for the management of congenital lobar emphysema cases and it aimed to report and to document any observation which might need further research. The study retrospectively reviewed medical records of 53 cases whom were admitted to the department of thoracic surgery-medical city teaching complex over a period of 4 years (from June 2005 to June 2010). All of them had been diagnosed and managed as cases of congenital lobar emphysema based on their clinical picture and radiologic examination.Objectives:&#x0D; Results: The commonest age group affected were infants in the second month of life(40 patients) (75.47%), 10 patients (18.86%) within the next 3 months, 2 cases (03.77%)were over 6months old and only one case (01.88%) was over 1y. The number of male patients in this study was higher than the female patients (38 versus 15) with a male to female ratio was 2.53:1. The affected lobes were left upper lobe in 39 patients (73.60%), right middle lobe in 9 patients (16.98%), right upper lobe in 3 cases (5.66%), and right upper and middle lobes in 2 cases (3.77). Postoperative complication encountered in 6 patients; chest infection (2 patients), bronchopleural fistula and empyema (2 patients), wound infection (1 patient), and postoperative atelectasis (1 patient). Each treated accordingly. No intraoperative mortality was recorded, yet postoperative mortality happened in 3 cases, two cases due to respiratory failure and other one due to septicaemia. The significant finding in this review was that, 8 cases out of the 53, during the period of observation and conservative management, they eventually developed complete clinical improvement of their signs and symptoms and complete resolution of their radiologic findings. All other patients (45) required surgical resection, were lobectomy was the commonest type of surgical resection (39 patients), combined lobectomy and wedge resection performed in (6) patients.&#x0D; Conclusions: A group of cases diagnosed with CLE can benefit from a period of observation and monitoring aiming for conservative management

Intra- and interoperator variability of lobar pulmonary volumes and emphysema scores in patients with chronic obstructive pulmonary disease and emphysema: comparison of manual and semi-automated segmentation techniques

We aimed to compare the intra- and interoperator variability of lobar volumetry and emphysema scores obtained by semi-automated and manual segmentation techniques in lung emphysema patients. In two sessions held three months apart, two operators performed lobar volumetry of unenhanced chest computed tomography examinations of 47 consecutive patients with chronic obstructive pulmonary disease and lung emphysema. Both operators used the manual and semi-automated segmentation techniques. The intra- and interoperator variability of the volumes and emphysema scores obtained by semi-automated segmentation was compared with the variability obtained by manual segmentation of the five pulmonary lobes. The intra- and interoperator variability of the lobar volumes decreased when using semi-automated lobe segmentation (coefficients of repeatability for the first operator: right upper lobe, 147 vs. 96.3; right middle lobe, 137.7 vs. 73.4; right lower lobe, 89.2 vs. 42.4; left upper lobe, 262.2 vs. 54.8; and left lower lobe, 260.5 vs. 56.5; coefficients of repeatability for the second operator: right upper lobe, 61.4 vs. 48.1; right middle lobe, 56 vs. 46.4; right lower lobe, 26.9 vs. 16.7; left upper lobe, 61.4 vs. 27; and left lower lobe, 63.6 vs. 27.5; coefficients of reproducibility in the interoperator analysis: right upper lobe, 191.3 vs. 102.9; right middle lobe, 219.8 vs. 126.5; right lower lobe, 122.6 vs. 90.1; left upper lobe, 166.9 vs. 68.7; and left lower lobe, 168.7 vs. 71.6). The coefficients of repeatability and reproducibility of emphysema scores also decreased when using semi-automated segmentation and had ranges that varied depending on the target lobe and selected threshold of emphysema. Semi-automated segmentation reduces the intra- and interoperator variability of lobar volumetry and provides a more objective tool than manual technique for quantifying lung volumes and severity of emphysema.

Effect of Emphysema on CT Scan Measures of Airway Dimensions in Smokers

In CT scans of smokers with COPD, the subsegmental airway wall area percent (WA%) is greater and more strongly correlated with FEV1 % predicted than WA% obtained in the segmental airways. Because emphysema is linked to loss of airway tethering and may limit airway expansion, increases in WA% may be related to emphysema and not solely to remodeling. We aimed to first determine whether the stronger association of subsegmental vs segmental WA% with FEV1 % predicted is mitigated by emphysema and, second, to assess the relationships among emphysema, WA%, and total bronchial area (TBA). We analyzed CT scan segmental and subsegmental WA% (WA% = 100 × wall area/TBA) of six bronchial paths and corresponding lobar emphysema, lung function, and clinical data in 983 smokers with COPD. Compared with segmental WA%, the subsegmental WA% had a greater effect on FEV1% predicted (-0.8% to -1.7% vs -1.9% to -2.6% per 1-unit increase in WA%, respectively; P < .05 for most bronchial paths). After adjusting for emphysema, the association between subsegmental WA% and FEV1 % predicted was weakened in two bronchial paths. Increases in WA% between bronchial segments correlated directly with emphysema in all bronchial paths (P < .05). In multivariate regression models, emphysema was directly related to subsegmental WA% in most bronchial paths and inversely related to subsegmental TBA in all bronchial paths. The greater effect of subsegmental WA% on airflow obstruction is mitigated by emphysema. Part of the emphysema effect might be due to loss of airway tethering, leading to a reduction in TBA and an increase in WA%.

Multidetector computed tomography evaluation in neonatal respiratory distress: Clinical implication

ObjectiveTo assess the value of multiplanar reformation (MPR) and three-dimensional multidetector computed tomography (MDCT) images in evaluating respiratory distress in neonates. Materials and methodsSixteen neonates (⩽30days) who presented with respiratory distress (RD) and admitted in the neonatal intensive care unit of the Assuit university children hospital who were selected from a total of 1295 patients presented with RD in whom chest X-ray was inconclusive, so those 16 neonates underwent 64-rows MDCT in the period between November 2010 and November 2011. The recruited patients were sedated before examination and IV contrast medium administration was needed when there was suspected vascular anomaly. Scans were performed in supine position to cover the root of the neck down to the level of the adrenal glands. The images were sent to the workstation to be reviewed in the multiplanar and 3D images. ResultsThe final radiologic diagnoses included cystic adenomatoid malformation (n=4, 25%), congenital lobar emphysema (n=1, 7%), pneumonia (n=5, 36%), hypoplastic lung (n=3, 22%), diaphragmatic hernia (n=2, 14%) and postoperative hydro-pneumothorax (n=1, 7%). ConclusionThe combination of fast speed, high spatial resolution, and enhanced quality of MPR and 3D images makes MDCT an ideal non-invasive method for evaluating surgical causes of neonatal respiratory distress.

Inadvertent chest tube insertion in congenital cystic adenomatoid malformation and congenital lobar emphysema-highlighting an important problem

Background:Chest tube insertion in congenital cystic lung lesions is an important problem in children with acute respiratory distress having a cystic lucent lesion on chest radiograph.Objective:To evaluate the imaging findings and complications in cases of congenital cystic lung lesions with chest tube insertion and suggest the role of appropriate imaging for management of these patients.Materials and Methods:Chest radiographs and CT scans of children with congenital cystic lung lesions who had inadvertent chest tube insertion preoperatively were retrospectively reviewed for imaging appearances and complications.Results:Fifteen patients comprising 10 cases of congenital cystic adenomatoid malformation (CCAM) and 5 cases of congenital lobar emphysema (CLE) were included. Majority of the cases were infants. CCAM was misdiagnosed as complicated pneumatocele (n = 5) and pneumothorax (n = 5), while CLE was misdiagnosed as tension pneumothorax (n = 5) on the chest radiograph findings. Final diagnosis was made on CT and operative findings with histopathology. Complications noted were pneumothorax, hydropneumothorax, and infection in cases of CCAM, and change in imaging appearance and pneumothorax in cases of CLE.Conclusion:Chest tube insertion in congenital cystic lesions increases the rate of associated complications. Chest CT has a definite role in early diagnosis and deciding appropriate management in these cases.

A case of congenital lobar emphysema in the middle lobe

Congenital lobar emphysema (CLE) is a rare cause of respiratory distress during the neonate period. It is characterized by overinflation of pulmonary lobe, most commonly the left upper lobe or the right middle lobe. We report a case of a 21-day-old baby with the severe respiratory distress. Diagnosis of CLE was made by computed tomography scan and chest X-ray and confirmed by histopathological studies. We would like to draw attention to this rare condition, and discussing their clinical features and management.

Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy

Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy