Diagnosis Of Congenital Lobar Emphysema Research Articles

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.

Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery.

Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by partial obstruction and occurs at a rate of 1/20,000-30,000 live births. Here, we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature. We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18years at our center between 2013 and 2020. In addition, we examined the symptoms and findings recorded during the patients' follow-up in this retrospective descriptive study. The median age of 20 patients with CLE at diagnosis was 3.2years (range 1day-17years). Respiratory distress and mediastinal shift were more prominent in patients who underwent surgery, and they were diagnosed at an earlier age compared with patients who were followed up conservatively (p = 0.001, 0.049, 0.001, respectively). Neither the pulmonary lobe involvement nor the bronchoscopy findings were found to be indicative of surgery. We observed that respiratory distress and mediastinal shift were more prominent in patients with a diagnosis of CLE who underwent surgery compared with patients who were conservatively followed up. Moreover, we observed that those who underwent surgery were diagnosed with CLE at an earlier age. In line with the literature, the pulmonary symptoms and CLE-related imaging findings in our study were reduced during conservative follow-up.

Surgical cause of respiratory distress in a newborn

Common causes of respiratory distress in a term infant include transient tachypnoea of newborn, sepsis/congenital pneumonia and congenital heart diseases. Surgical causes of distress in a newborn are rare and should be considered when other causes have been ruled out. We report a case of an early term female neonate who developed respiratory distress immediately after birth which was gradually progressive and required respiratory support. On evaluation with chest radiography and CT, a diagnosis of congenital lobar emphysema (CLE) was made. Immediate surgical resection was planned and done on day 8 of life. Post operatively she had no respiratory distress and discharged within a week. Baby is now healthy with normal growth and development. Half of the cases of CLE are diagnosed within the first 6 months of life but very few cases present with respiratory distress since birth. Timely diagnosis and early surgical excision in neonates <2 months improve the outcome, as seen in our case.

Unilateral Lung Agenesis in an infant with prenatal diagnosis of Congenital Lobar Emphysema

Abstract Infants in respiratory distress presenting with congenital lobar emphysema (CLE) are surgical emergencies. However, several other congenital lung lesions (CLL) have similar prenatal imaging, highlighting the importance of early postnatal evaluation. We present a case of a newborn transferred for respiratory distress. Prenatal imaging suspected a CLE in the left upper lobe with concern for decompensation in the early postnatal period. Upon arrival, the patient demonstrated reasonable cardio-respiratory stability after initial apnea and cyanosis; due to prenatal concerns, we proceeded to CT imaging, which demonstrated congenital absence of the right pulmonary artery, agenesis of the right lung, and a larger left lung herniating into the right side. Surgical intervention was therefore not required. This case emphasizes the importance of postnatal imaging in the CLL workup. Providing “pathological” diagnoses to CLL can affect postnatal counseling and influence surgical decision-making. Here, early postnatal imaging clarified the diagnosis and prevented both unnecessary surgery and significant morbidity. The patient's clinical status should determine the urgency of CLL investigation. Stable patients may be followed as outpatients but should be assessed within 1–2 weeks after birth. Symptomatic patients should be transferred to tertiary care centers with pediatric surgical expertise for further investigation and possible intervention.

AB182. 141. Congenital lobar emphysema: an important diagnosis in the infant with respiratory distress

: Congenital lobar emphysema (CLE) is a rare congenital malformation of the lung characterised by hyperinflation of one or more lobes which usually presents in the neonatal period or in early infancy. Males are three times more likely to be affected than females and the left upper lobe is the area most commonly involved. The diagnosis and management are often difficult and if not detected may be mismanaged leading to significant respiratory distress and haemodynamic instability. We present the case of a six-week old male who presented to the emergency department with respiratory distress and was admitted with a diagnosis of respiratory syncytial virus positive bronchopneumonia. However after an acute deterioration on the ward he required admission to the intensive care unit where he was ventilated for management of type two respiratory failure. Further deterioration while on convential ventilation lead to commencement of high frequency oscillatory ventilation and inotropic support for persistent respiratory acidosis and hemodynamic instability. A CT Thorax was later preformed showing mediastinal shift with hyperinflation of the right middle lobe confirming the diagnosis an underlying diagnosis of congenital lobar emphysema which was likely exacerbated by ventilation. An emergency thoracotomy and right middle lobectomy was preformed with extracorporeal life support on standby. The surgery was successful and the patient had an uneventful post operative recovery. Here we emphasise the importance of early consideration of underlying congenital lung disease in cases where the clinical picture does not correlate with the working diagnosis especially in the infant population. We also look at anaesthetic challenges associated with congenital lobar emphysema and how best they can be managed.

Congenital Lobar Emphysema–A case Series for 2 Years

Background: Congenital lobar emphysema (CLE), a rare variety of congenital malformation of lung characterized by over distension of a lobe of a lung due to partial obstruction of the bronchus, can be a diagnostic and therapeutic challenge for the treating pediatrician. Aim: To study the way of presentation, diagnosis, management and prognosis of CLE and comparing our results with other studies done in other centers. Method: A case series of ten patients with CLE. All of our patients were managed in the department of pediatric surgery center at AL-KHANSAA hospital in Mosul city between January 2016 to January 2018. Results: We have found that the right upper lobe is more affected than the left upper lobe and more than one lobe can be affected. The surgical treatment lobectomy of the affected lobe. The mortality rate was high in patients having congenital heart diseases and those with late presentation and complication. Ten patients, five male and five females, their ages ranged from 7 days to 6 months. Conclusion: CLE is a rare congenital anomaly, most of our patients are infant less than 6 months old and most of them presented with respiratory distress, recurrent chest infection and some of them presented with pneumothorax.

An 18-year-old man with recurrent pneumothorax since he was 10-year-old

An 18-year-old male patient was referred to the department of pediatric pulmonology with a history of recurrent pneumothorax. Initial pneumothorax occurred at the age of 10. Following diagnosis of congenital lobar emphysema, he had five episodes of pneumothorax and subsequently underwent right-lower lobe anterobasal segmentectomy. Based on thoracic computed tomography (CT) and clinical manifestation, Birt-Hogg-Dube (BHD) syndrome was suspected and confirmed following genetic testing. BHD syndrome is a rare tumor predisposition syndrome first described in 1977. The syndrome is characterized by skin fibrofolliculomas, lung cysts, recurrent spontaneous pneumothorax, and renal cell cancer. The underlying cause is a germline mutation in the folliculin (FLCN) gene located on chromosome 17p11.2. Clinical manifestation usually appears after the age of 20 years. In this case, we report a case of BHD with episodes of recurrent pneumothorax, the first of which occurred at the age of 10 years. Pulmonologists should be aware of this syndrome in patients with a personal and family history of pneumothoraces and CT findings of multiple pulmonary cysts as additional evaluation and testing may be warranted. Pediatr Pulmonol. 2016;51:E41-E43. © 2016 Wiley Periodicals, Inc.

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

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 and Intercostal Drainage Tube Insertion: The Common Fate of an Uncommon Disease

Congenital Lobar Emphysema (CLE) is one of the rare cystic malformations of the lung. This malformation is generally confused with pneumothorax of the lung and most often, the placement of an intercostal drainage tube is entertained in an emergency situation, but the non- improvement of the distress and non-expansion of the lung are eye openers for a treating paediatrician. We are describing a case of a 26 days old baby who presented with respiratory distress in the Emergency Department. On the basis of the chest X-ray (CXR), we suspected it to be a case of pneumothorax and an intercostal drainage tube was inserted. But as he did not improve, CT of the chest was done and the baby was diagnosed to have congenital lobar emphysema. In spite of the advanced diagnostic techniques, the diagnosis of CLE may present a diagnostic challenge and a high index of suspicion is needed if the diagnosis has to be made promptly.

Intralobar pulmonary sequestration masquerading as congenital lobar emphysema

Intrapulmonary sequestrations are quite uncommon in pediatric age group. The preoperative diagnosis of pulmonary sequestration is not possible in most of the cases. A 2-year-old boy presented with recurrent episodes of chest infections and respiratory distress. A preoperative diagnosis of congenital lobar emphysema was made on the basis of chest radiograph and computed tomography scan. At operation, an intralobar pulmonary sequestration was found. The sequestration cyst was excised with uneventful recovery.

Congenital lobar emphysema in an adult

Congenital lobar emphysema (CLE) is a clinico-radiological diagnosis, seen usually by four-six weeks of age (50% of patients) and rarely (<5% of patients) after the age of six months. Here, we report a young male with gradual onset of mild exertional breathlessness and physical examination revealing the features of right sided pneumothorax. X-ray of chest, with subsequent CT of chest, leads to the diagnosis of CLE. The pulmonary function tests, bronchoscopic examination and α1-antitrypsin level are normal. Patient is managed conservatively.

Prenatal Diagnosis of Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is a rare developmental anomaly of the lower respiratory tract, which is characterized by hyperinflation of 1 or more of the pulmonary lobes. The routine use of prenatal sonography has resulted in early identification and serial evaluation of congenital lung lesions. 1 Prenatal diagnosis of CLE is rarely reported in the literature. 2 Here we report a case of a fetus with an echogenic lung presenting in the second trimester suspected of CLE. A discussion of the causes and natural course of CLE is presented.

Congenital lobar emphysema and increased nuchal translucency

Congenital lobar emphysema and increased nuchal translucency

Unilobar congenital pulmonary lymphangiectasis mimicking congenital lobar emphysema: an underestimated presentation?

Background Congenital lobar emphysema (CLE) is characterized by unilobar alveolar distension secondary to bronchomalacia or absent cartilage. In contrast, congenital pulmonary lymphangiectasis (CPL) is defined as distended lymphatics in the bronchovascular bundle, in the interlobular septa, and in the subpleural space. Little information is available regarding the radiologic presentation of CLE as it correlates with histological diagnosis. Methods In a retrospective chart review from 1995 to 2002, 8 patients (5 boys and 3 girls) with clinical and radiologic diagnosis of CLE were reviewed. Results The mean age at diagnosis was 26 months (range, 11 days to 10 years). All but one had classic respiratory symptoms of CLE. Six of 7 chest computed tomography (CT), scans were suggestive of CLE. Of 8 patients, 3 were treated without pulmonary resection with resolution of symptoms. Five patients underwent lobectomies, and histology results showed CPL in 3. CT failed to identify CPL in all cases. Conclusions Diagnosis of CLE is not as straightforward as the literature suggests. Even retrospectively, radiologic distinction between CLE and CPL could not be achieved by an experienced pediatric radiologist. CPL, thus, mimics CLE clinically and radiologically and, therefore, should be considered in the differential radiologic diagnosis of CLE.

Congenital lobar emphysema – the disappearing chest mass: antenatal ultrasound appearance

Prenatal diagnosis of congenital lobar emphysema is rarely reported in the literature. This case presents a fetus with an echogenic lung diagnosed at 18 weeks' gestation, that resolved completely during the pregnancy. This case is unique in that sonographic increased echogenicity was first noted at 18 weeks' gestation and had disappeared by 29 weeks' gestation. The diagnosis was made in the postnatal period as a result of follow up of the prenatal findings. This case reinforces the importance of continuing postnatal investigations of prenatal sonographic abnormalities even when they appear to have disappeared. A discussion of the causes of congenital lobar emphysema is presented.

Congenital lobar emphysema: Diagnostic and therapeutic considerations

Background/Purpose: Congenital lobar emphysema (CLE) is characterized by overinflation of a pulmonary lobe and may present as a diagnostic and therapeutic dilemma. The authors have reviewed their experience to clarfy the controversies about the diagnosis and management of CLE in children. Methods: Children who had CLE and were treated at our department between 1991 and 1998, inclusive, were reviewed. Results: Fourteen children consisting of 8 boys and 6 girls (age range, 25 days to 2.5 years) had CLE. Eight children (57%) were symptomatic at birth. Major presenting symptoms were dyspnea (n = 8, 57%), cyanosis (n = 6, 43%) and recurrent respiratory tract infection (n = 4, 28.5%). Chest x-rays and computerized tomography scans showed hyperaeration of the affected lobes in 14 and 12 patients, respectively. Pulmonary perfusion scan was performed in 8 patients and showed loss of perfusion in the affected lobe in 7 cases. The affected sites were left upper lobe (n = 8, 57%), right middle lobe (n = 4, 28.5%) and right upper lobe (n = 2, 14.5%). Congenital cardiac anomalies were encountered in 2 patients (14.5%). Ten children underwent lobectomy, and postoperative course was uneventful. Nonoperative management was performed in 4 cases (28.5%) presenting at older age with milder symptoms. In the latter, although symptoms subsided, radiological abnormalities persisted during the follow-up period of 3 months to 4 years. No mortality was encountered in the series. Conclusions: The diagnosis of CLE is established by combined radiological and scintigraphic imaging methods. Surgical excision of the affected lobe is the appropriate treatment in all infants under 2 months of age and in infants older than 2 months presenting with severe respiratory symptoms. Infants older than 2 months presenting with mild to moderate respiratory symptoms associated with normal bronchoscopic findings can be treated conservatively. In conservative management, the children should be under close follow-up, and the family should be alarmed about the disease. Further studies are required to determine the long-term outcome of conservative management.

Solubilized xenon 133 lung scintigraphy

Lung scanning using solubilized xenon 133 can provide important information concerning both pulmonary perfusion and ventilation. This technique proved valuable in establishing the diagnosis of congenital lobar emphysema in a 7-month-old baby.

The role of pulmonary scanning in the diagnosis of congenital lobar emphysema: F. Maxton Mauney, Jr., and David C. Sabiston, Jr. Amer. Surg. 36:20–27 (January), 1970

The role of pulmonary scanning in the diagnosis of congenital lobar emphysema: F. Maxton Mauney, Jr., and David C. Sabiston, Jr. Amer. Surg. 36:20–27 (January), 1970