Lobar Emphysema Research Articles

Congenital cystic lesions of the lungs: The perils of misdiagnosis - A single-center experience.

Background:A majority of cystic lesions in the western world are detected antenatally, whereas, the diagnosis in our setup occurs once the child becomes symptomatic. Surgical management is primarily dictated by the presence of symptoms, recurrent infection, and rarely by the potential risk of malignant transformation.Materials and Methods:A retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from January 2000 through June 2011 for antenatal diagnosis, presentation, diagnostic modalities, treatment, and complications.Results:Forty cystic lung lesions were identified. Only 8% were antenatally detected. Out of 40, the final diagnosis was congenital cystic adenomatoid malformation in 19, congenital lobar emphysema in 11, and bronchogenic cysts and pulmonary sequestration in five each. Of these, 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center. Postoperative morbidity in the form of bronchopleural fistula, pneumothorax, and non-expansion of the residual lung was noted in 10% of the patients.Conclusion:Antenatal diagnosis of these lesions is still uncommon in third world countries. Prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion, due to incorrect diagnosis.

Surgical Treatment for Congenital Lung Parenchyma and Non Lung Parenchyma Disorder: Center Experience

Objective: Non parenchyma lung disorders are rare entity with life threatening outcome. Early surgerical intervention is the clue for life saving and avoid life threatening complications. Methods: From Aug 2008 to dec 2014, 101 cases operated in Zagazig University Hospital for congenital non parenchyma disorder data are collected regarding preoperative, intra operative and post operative results. Results: The mean age of our patients was 27 month (1 week - 120 month). F\M 61\40. Mean symptom is respiratory distress and frequent chest infection. Tracho-esophygeal fistula 24 cases, Congenital diaphragmatic hernia 17, Diaphragmatic event ration 6, congenital lobar emphysema 37, cystic adenomatoid malformation (n=9), pulmonary sequestration (n=7) and arteriovenous malformation (n=1). Conclusion: Early surgerical intervention for parenchyma and non parachyanma l disorder is the primary curative to avoid life threatening complications.

Congenital lobar emphysema: Intubation and ventilation strategies.

Sir, The case report entitled “congenital lobar emphysema: A modified approach to anesthetic management” by Nandihalli et al.[1] made for interesting reading. Tracheal intubation at the time of anesthetic induction and the subsequent ventilation strategies are the cornerstone of successful anesthetic management of such cases and may influence the outcome. We would like to make certain observations on the described management of this case. In the abstract, they have written that they adopted gentle manual ventilation maintaining the airway pressure before thoracotomy as described by Cote and Payne et al. This fact has been repeated in the discussion section also. In the case report, it has been mentioned that deliberate endobronchial intubation was performed and intermittent positive pressure ventilation (IPPV) was carried out using low tidal volumes (4-6 ml) on pressure regulated volume control mode. Later on they went on to conclude that “if IPPV is necessary, gentle manual ventilation or pressure controlled ventilation with a pressure limit of 20-25 cm of H2O can be carried out until thoracotomy”. To us it is not clear actually which ventilation mode was applied. We would also like to know the names of the breathing circuit and the anesthesia workstation that was used to manage this case because that will give a clue to the method employed. Deliberate endobronchial intubation using a tracheal tube is described in literature. However, some important measures from the safety point of view should be kept in mind. In children, it is suggested to use a tracheal tube 0.5-2.0 mm smaller in diameter than recommended for the particular patient.[2] Their patient was somewhat older at presentation as he was symptom free till the age of 9 months and a tube of 4.5 mm internal diameter was used. Another issue that should be kept in mind with right endobronchial intubation is the possible blockage of the right upper lobe bronchus by the tube. Different ventilation strategies for management of such cases are well known. There is nothing new in their description of endobronchial intubation, gentle manual ventilation or pressure regulated volume controlled ventilation that has been already described in literature.[3] So, it is not clear what modification was employed in their management as suggested by the title of the case report.

Anesthetic Management in Children with Congenital Lobar Emphysema

Background: The cause of congenital lobar emphysema (CLE) is unknown and characterized by hyperinflation of one or more lobes of the lung. The purpose of this retrospective study was to present the anesthetic management of children with congenital lobar emphysema (CLE) receiving treatment in our center.Methods: Ten children underwent CLE-related surgical treatment in our center between March 1995 and August 2014. All cases were diagnosed on the basis of postero-anterior chest radiography and computerized tomography. Age, sex, preoperative clinical findings, location of lesions, surgical and anesthetic procedures, results of anesthesia and duration of hospitalization were evaluated.Results: Six patients were male (60%) and four female (40%). Their ages ranged from 40 days to 6 years. Dyspnea was present in all cases and severe in four of them. Four patients had emphysema in the left upper lobe, three in the right middle lobe, one in the right upper lobe and one in the left lower lobe. All patients were extubated in the operating room and none experienced post-operative respiratory distress. Post-operative analgesia was provided via the previously placed intrapleural catheter. All the patients were extubated in the operation room. There was no post-operative mortality or morbidity.Conclusions: Anesthetic management of patients with CLE is challenging. In these patients the surgical team and anesthetists should collaborate closely and the time between anesthesia induction and thoracotomy should be as short as possible.

Congenital lobar emphysema of right middle lobe-transition from an opaque lung to hyperlucent lung in a neonate

Congenital Lobar Emphysema (CLE) is a rare congenital malformation of the lung characterized by over distension of a lobe of a lung due to partial obstruction of the bronchus. CLE often presents a diagnostic and therapeutic dilemma. We report a case of a 3 week old neonate who presented with sudden onset of respiratory distress related to CLE affecting the right middle lobe. Chest X-ray showed overexpansion of the right middle lobe, collapse of the ipsilateral lower lobe and shift of mediastinum. Lobectomy was performed successfully under general anaesthesia. Chest X-ray done at 6 hours of age showed an opaque right middle lobe which progressively became hyperlucent at 3 weeks of age requiring surgical intervention.

Congenital cystic lesions of lung in the paediatric population: A 5-year single institutional study with review of literature.

Background:The aim was to evaluate the clinical presentation, investigation modalities, operative management, pathology, outcome (morbidity and mortality) and short term follow-up of congenital cystic lesions of the lung.Materials and Methods:This is a retrospective study. Study period was 5 years (December 2008-November 2013) in the Department of paediatric surgery, Nil Ratan Sircar Medical College. Study population: Total number = 10 patients. Age range: 2 days-7 years. (Neonate-4). Male and female ratio = 1:1.Result:Among 10 cases of lung cyst four having congenital lobar emphysema, four having congenital pulmonary airway malformation, one sequestration and one teratoma. All patients have undergone surgical excision in terms of lobectomy or excision of the lesion. Post-operative histopathology confirmed the diagnosis. Recovery was uneventful.Conclusion:Although our experience is limited, operative management of lung cysts seems to be safe with rewarding results. However we are yet to encounter many of the other varieties of the cysts found in the lung, which may be associated with other congenital anomalies and have an impact on prognosis.

Acquired lobar emphysema in a case of interstitial chronic lung disease. Surgical implications and review of the literature

Acquired lobar interstitial emphysema (ALIE), initially described in infants with bronchopulmonary dysplasia is now known to occur in other conditions such as a secondary post-traumatic or inflammatory injury as in patients requiring ventilatory sup- port with chronic lung disease. The ALIE leads to progressive deterioration of lung function with massive distension and com- pression of lung parenchyma with consequent mediastinal shift in the course of chronic interstitial lung disease. This situation requires adequate clinical and radiological interpretation in order to make the proper patient selection i.e., between a candidate for lung biopsy for the benefit of a definitive diagnosis or one who in the course of a superimposed ALIE requires a diagnostic and therapeutic lobectomy. Two cases of this entity and descriptive decision-making diagnostic and surgical about pre-and intraop- erative findings are presented.

Diagnosis and management of fetal congenital lobar emphysema

Objective Fetal congenital lobar emphysema (CLE) is generally confused with congenital cystic adenomatoid malformation type Ⅲ(CCAM Ⅲ) by prenatal ultrasound as both are hyperechoic cystic mass with the same blood supply, which is difficult to differentiate through the prenatal ultrasound.As they have different clinical outcomes, and in order to improve the understanding of CLE, the diagnosis and treatment of neonatal outcome of fetal CLE were discussed. Methods A retrospective analysis was conducted of the experience in prenatal diagnosis and treatment of postnatal CLE at Department of Fetal Medicine, Guangdong Women and Children's Hospital from Mar.2011 to May 2013. Results On the basis of prenatal ultrasound for fetal chest cystic malformations, there were 52 cases of CCAM Ⅲ in which 6 cases(11%, 6/52 cases) were confirmed as CLE by CT scan after birth and 5 cases had early operation.Emphysema was detected in all pathological specimens.Of 3 cases presenting symptoms of tachypnea after birth, 2 had acute lobectomy and 1 gave up therapy.The other 3 cases underwent lobectomy on 7 day, 19 day and 3 months respectively after birth because of tachypnea and respiratory distress.The most common affected lobe was the upper lobe (83%, 5/6 cases). The lobectomy included 2 left upper lobe, 1 right upper lobe, 1 right upper and middle lobe, and 1 right lower lobe.The mean age of lobectomy was 20 days(from 3 to 90 days). All the cases were cured through lobectomy. Conclusions Prenatal CLE can not be differentiated from CCAM Ⅲ on ultrasound, but it exhibited early pulmonary distress at birth or after birth leading to neonatal emergency and treatment.It can be confirmed by computed tomography scan.Previously CCAM Ⅲ prognosis was thought to be poor and it remains unconfirmed whether CLE had been misdiagnosed as CCAM Ⅲ need to know.For the CLE with neonatal acute respiratory symptoms early surgical treatment is effective. Key words: Fetal; Congenital lobar emphysema; Congenital cystic adenomatoid malformation type Ⅲ; Neonatal surgery

Congenital Lobar Emphysema Diagnosed in Adult Age: A Case Report

Congenital Lobar Emphysema (CLE) is a rare congenital pulmonary malformation and it is characterized by hyperinflation of one or more lobes of the lungs. The disease is usually encountered in infants aged less than 6 months and it is very rare in adulthood. Herein, we presented a patient with CLE, who received asthma treatment for dyspnoea complaint in adult age but was switched to anxiolytic medications as no benefit was achieved from asthma treatment. The diagnosis of the patient was made clinically, radiologically and scintigraphycally. Although it is a rarely encountered condition, CLE should be considered among the prediagnosis, in patients with localized unilateral hyperinflation on chest x-ray, presenting with dyspnoea in adult age.

English

Congenital high airway obstruction syndrome (CHAOS) results in a predictable constellation of findings: large echogenic lungs flattened or inverted diaphragms, dilated airways distal to the obstruction, and fetal ascites and/or hydrops. 1 The finding of CHAOS on prenatal ultrasound examination is diagnostic of complete or near-complete obstruction of the fetal upper airway, most likely caused by laryngeal atresia. A greater understanding of the natural history of CHAOS may permit improved prenatal and perinatal management. INTRODUCTION: Congenital chest malformations are rare, and may involve the lung parenchyma, bronchi, arterial supply, and venous drainage. They include congenital cystic adenomatoid malformation (CCAM), congenital diaphragmatic hernia (CDH), bronchopulmonary sequestration (BPS), congenital hydrothorax, and congenital lobar emphysema. Rarer entities include congenital high airway obstruction syndrome (CHAOS), congenital bronchogenic cyst, bronchial atresia, pulmonary Arterio Venous malformation (PAVM), congenital pulmonary lymphangiectasia, pulmo- nary hypoplasia-aplasia, mediastinal teratoma, and mediastinal lymphangioma.2 Sometimes there may be compromise in normal pulmonary development, causing hypoplastic lung formation. Pulmonary abnormalities are not mutually exclusive, since abnormalities frequently occur together as hybrid conditions. MATERIALS AND METHODS: Here we present a case of a fetus post-delivery found to have large hyperechoic lungs, flattened diaphragm and hydrops in the prenatal scan, which form a triad in the detection of CHAOS. A brief review of literature is discussed. CASE PRESENTATION: In our case we had a twenty six years old pregnant lady with twenty two weeks history of amenorrohea who had come for a routine antenatal scan. She was the mother of a two year old child which was born normally and was doing well. On antenatal ultrasound of the present pregnancy, the fetus was found to be of approximately twenty weeks gestation. The fetus was found to have large hyperechoic lungs, flattened diaphragm and hydrops which form a triad in the detection of CHAOS. (Fig: 1) The patient and her husband were counseled regarding the risks involved on progression of the fetus till birth. The parents agreed to terminate the pregnancy voluntarily. A dead male fetus was delivered and sent for autopsy subsequently. An interesting finding noted during dissection of neck and thoracic organs was an abrupt ending of the trachea approximately 11 mm caudal to the lower end of larynx. (Fig: 2 & Fig: 3). The subsequent histopathology showing swollen cells confirmed the diagnosis (Fig: 4)

Miller–Dieker Syndrome Associated with Congenital Lobar Emphysema

Miller–Dieker syndrome (MDS) is a rare genetic syndrome associated with lissencephaly, developmental delay, and high mortality. We describe a patient who was diagnosed postnatally with both MDS and congenital lobar emphysema. We believe that this is the first reported case of the two conditions presenting in the same patient.

Emergency Neonatal Chest Imaging: "Challenges and Peculiarities"

: The neonatal chest and lung abnormalities can be divided into congenital and acquired or iatrogenic disorders, mostly causing respiratory distress. Congenital lesions include various types of abnormalities such as congenital diaphragmatic hernia, congenital cystic adenoid malformation (CCAM), bronchopulmonary sequestration, lobar emphysema and others. Such conditions are frequently associated with other additional organ malformations. The primary acquired neonatal pulmonary diseases are hyaline membrane disease of premature neonate, meconium aspiration syndrome, transient tachypnea of newborn and neonatal pneumonia. Both congenital and acquired pulmonary disorders in the neonatal period mostly need interventional therapy with the use of mechanical ventilation, oxygen therapy or high frequency ventilation. All these procedures may be complicated by pulmonary air leakage with various intra- and extrapulmonary changes, initially, mostly as interstitial emphysema followed by pneumothorax, pneumomediastinum, pneumopericardium and pneumoretroperitoneum. Such events are always complicated with recurrent atelectasis, acidosis and hypoxemia resulting mostly in chronic lung disease (bronchopulmonary dysplasia). Together with the pulmonary infection, sepsis and other extrapulmonary problems such as intraventricular hemorrhage and necrotizing enterocolitis they result in a high mortality and morbidity rate in this group of infants. The introduction of surfactant therapy in respiratory distress syndrome contributed largely to the reduction of complications in neonatal pulmonary diseases. The imaging of congenital intrathoracic malformations is now frequently recognized by antenatal sonography or MRI if available. The conventional chest X-ray after birth is the first step in the diagnosis of all congenital and acquired neonatal lung disorders for the detection of intrathoracic complications. The prevention of iatrogenic incidents is especially a challenge to the radiologist in this field. The aim of this workshop will be a systematic interpretation of the typical and atypical neonatal chest films including congenital and acquired diseases. Special attention should be paid to the various pulmonary and extrapulmonary complications demonstrated by different pathological signs. Additionally, the radiological signs of patent ductus Botalli on the chest film and the different types and development of chronic lung disease (bronchopulmonary dysplasia) will be shown.

Segmental resection for the treatment of congenital pulmonary malformations

PurposeThe purpose of this study was to compare clinical outcomes of segmental resection to lobectomy as increasing antenatal diagnosis of congenital pulmonary malformations has led to a shift in surgical management. MethodsA retrospective institutional review for patients undergoing surgical excision of congenital pulmonary malformations was performed. ResultsSixty-two patients with congenital pulmonary malformations were reviewed between 2001 and 2012. Forty-five were included for analysis. Malformations were subdivided into two groups, including congenital lobar emphysema (CLE) (n=11, 24%) and intrapulmonary (IP) lesions (n=34, 76%). Nineteen (56%) IP patients underwent segmental resection, and 15 (79%) were performed thoracoscopically without conversion to thoracotomy. None of these patients had recurrent disease. Lobectomy was performed in 11 (100%) CLE and 15 (44%) IP patients, and the majority were by thoracotomy. Median hospital stay was longer for the lobectomy group at 7days when compared to the segmentectomy group at 2days (p<0.001). There was not a difference in complication rate (21% vs. 19%, p=1.000) or in median number of chest tube days (2 vs. 3days, p=0.079) for segmentectomy versus lobectomy patients. ConclusionsSegmental resections of congenital pulmonary malformations can be performed safely while conserving healthy lung tissue.

Congenital lobar emphysema causing discrepancy between size and symptoms of ventricular septal defect

Three small infants presented with severely symptomatic ventricular septal defect, thought to require cardiac surgery, although the defect was not very large. Surgery for the associated congenital lobar emphysema led to recovery, and cardiac surgery was not necessary.

Case of congenital lobar lung emphysema

Abstract The authors describe a case of congenital lobar emphysema in child at the age of 45 days and discuss the clinical manifestations as well as the features of histological diagnostic of the disease. This case is of particular

Congenital lobar emphysema: anaesthetic considerations

Congenital lobar emphysema (CLE) is a rare clinical entity that usually presents as acute respiratory distress. It is potentially reversible, though possibly life-threatening, cause of respiratory distress in the neonate. It poses dilemma in diagnosis and management. We are presenting a 40 days old baby who presented with a sudden onset of respiratory distress related to CLE affecting the left upper lobe. Lobectomy was performed under general anaesthesia with spontaneous and controlled lung ventilation. Strategies to prevent hyperinflation and anaesthetic consideration of various techniques adopted for lung separation in infants have been reviewed.

Congenital Lobar Emphysemma: A Rare Case Report

We describe case of congenital lobar emphysema presented as a case of pneumoniawith pneumothorax in a newborn. Later on with HRCT we found to have left upperlobe emphysema for which the left upper lobectomy was done. After surgery the patient improved dramcatically, successfully extubated and discharged.

Three Cases of ALCAPA with Associated Anomalies

There are several potential issues that affect the treatment and diagnostic pattern of anomalous left coronary artery arising from the pulmonary artery. We report three cases of infants who presented with anomalous left coronary artery arising from the pulmonary artery with severe left ventricular dysfunction and severe mitral regurgitation along with associated anomalies. One patient had congenital lobar emphysema of the right midde lobe. Another patient had left main stem bronchus compression, collapse of basal segments of left lower lobe and panlobular emphysema in medial basal segment of right lower lobe. The third patient had cleft lip and palate. All patients underwent successful repair. The hemodynamic stability was compromised when the infant with congenital lobar emphysema had spontaneous pneumothorax after extubation and she needed an intercostal drainage. The infant with lung collapse had to be reintubated on the second day since she became hypoxic due to recollapse of the lung once the airway positive pressure was removed. She needed chest physiotherapy, vigorous endotracheal suctioning and inhaled bronchodilator therapy. The patient who had cleft palate succumbed to aspiration pneumonitis in the postoperative period. Follow-up of other two patients after three months showed very good improvement in left ventricular systolic function.

Congenital lobar emphysema presenting at late childhood: A rare case report.

Congenital lobar emphysema presenting at late childhood: A rare case report.

Case series: unusual presentations of congenital lung malformations

Congenital Lung Malformations (CLM) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. Congenital lung malformations encompass several conditions such as Congenital Cystic Adenomatoid Malformation (CCAM), Congenital Lobar Emphysema (CLE), Diaphragmatic hernia, sequestration of lung. A high index of suspicion is necessary to diagnose CLM in children irrespective of the age. Here we present a series of three cases of congenital lung malformations with unusual presentations.