scholarly journals Flexible Bronchoscopy Findings in Children with Congenital Lobar Emphysema: 8-Year Data from a Single Center Study

Author(s):  
Gokcen Tugcu ◽  
Sanem Eryılmaz ◽  
Şule AKYAN SOYDAŞ ◽  
Ece Ocak ◽  
Murat Gençoğlu ◽  
...  

Introduction:Congenital lobar emphysema (CLE) is a rare developmental lung malformation that involves the hyperaeration of one or more lung lobes due to 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 with surgical or conservative approaches in our clinic to compare our results with those in the literature. Method:We examined the clinical, and radiological data and FB findings of the patients with CLE aged 0–18 years at our center between 2013 and 2020.We also examined the symptoms and findings recorded during the patients’ follow-up. Results:.The median age of 20 patients with CLE at diagnosis was 3.2 years (range, 1 day–17 years).Respiratory distress and mediastinal shift were more prominent in the patients who underwent surgery than the patients who were followed up conservatively and diagnosed at an early age (p = 0.001, p = 0.049, p = 0.001, respectively). Discussion: In line with studies in the literature, the pulmonary symptoms and CLE-related imaging findings in our study regressed during the conservative follow-up.We recommend clinicians consider performing a detailed anamnesis for patients with unresolved respiratory symptoms and unilateral or bilateral increased ventilation,along with appropriate imaging tests and examinations, and should consider CLE in the diagnosis.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fanyi Gan ◽  
Liang Xia ◽  
Yushang Yang ◽  
Qiang Pu ◽  
Lunxu Liu

Abstract Background Congenital lobal emphysema (CLE) is a developmental lung abnormality usually diagnosed in the neonatal period and is rarely observed in adults. Adults with CLE are usually asymptomatic and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress. In imaging studies, the most frequently affected lobe of CLE is the left upper lobe, followed by the right middle lobe. However, multilobar involvement with severe mediastinal shift is extremely rare. Case presentation We report a case of fatal CLE in a 28-year-old puerpera with postpartum respiratory failure. Chest computed tomography (CT) revealed emphysema of the right upper, middle and lower lobes resulting in adjacent atelectasis. Hyperinflation of the right upper lobe crossed the midline, leading to a deviation of the mediastinal structure to the left hemithorax and severe compression of the left lung. Conclusions Early and timely diagnosis of CLE with routine follow-up is necessary for patients. CLE, especially with multilobar involvement or mediastinal shift, could be life-threatening and should be promptly and aggressively treated to prevent severe complications.


2021 ◽  
Vol 24 (3) ◽  
pp. E517-E521
Author(s):  
Montaser Elsawy Abd Elaziz ◽  
Mohamed Gaber Elsayed ◽  
Mohamed Ahmed El-hag Aly

Background: Congenital lobar emphysema (CLE) is a lung malformation characterized by overdistension and air trapping in the affected lobe. It is one of the causes of neonatal and infantile respiratory distress. This study aimed to evaluate our experience regarding perioperative and surgical management in children with CLE. Methods: A retrospective observational study was done for all CLE patients who underwent surgery at Menoufia University Hospital. Perioperative data collected included demographic, clinical, and radiological findings, as well as operative and postoperative data. Results: We included 30 neonates and infants who suffered from CLE between January 2013 and December 2020; the mean age was 111.43 ± 65.19 days, and 21 were males. All cases presented with respiratory distress; 19 had cyanosis, and 15 had recurrent pneumonia and fever. Plain chest x-ray and computed tomography (CT) revealed emphysema in all cases. Lobectomy was done in all cases; the mean age at surgery was 147.58 ± 81.49 days. Postoperative complications occurred in 5 patients, and 2 of them needed mechanical ventilation. The follow-up duration ranged from 3 months to 1 year (except 1 case lost to follow-up after 3 months), and all patients were doing well. Conclusion: CLE is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile respiratory distress. Chest CT is the most useful diagnostic modality. Early management of CLE improves outcome and prevents life-threatening complications. Surgical management is the treatment of choice in our center, without recorded mortality.


1999 ◽  
Vol 43 (2) ◽  
pp. 243-245 ◽  
Author(s):  
Gervais Khin-Lin Wansaicheong ◽  
Chiou Li Ong

2021 ◽  
Vol 57 (2) ◽  
pp. 96-100
Author(s):  
Penny J. Regier ◽  
Aitor Gallastegui ◽  
William F. Craft

ABSTRACT A 5 mo old male intact English bulldog was evaluated at a veterinary referral hospital for acute respiratory distress and chronic difficulty breathing. Thoracic radiographs revealed multifocal pulmonary hyperinflation and hyperlucency suspected in the left caudal and accessory lung lobes. A thoracic computed tomography scan identified severe diffuse enlargement of the caudal subsegment of the left cranial lung lobe and the dorsal process of the accessory lung lobe, with parenchymal hypoattenuation, rounded margins, and thin pulmonary vessels. Based on clinical signs and imaging findings, he was diagnosed with suspect congenital lobar emphysema in multiple lung lobes. A median sternotomy was performed, which revealed a hyperinflated, emphysematous left cranial lung lobe (caudal subsegment) and accessory lung lobe for which two lung lobectomies were performed. The remaining lung lobes were small and atelectatic. Histopathology revealed bronchial cartilage hypoplasia and aplasia and findings consistent with congenital lobar emphysema. The puppy recovered well from surgical treatment of congenital lobar emphysema, requiring multiple lung lobectomies, with subsequent computed tomography–evidenced re-expansion of the remaining lung lobes 3 mo after surgery. The patient is still alive 1 yr after surgery with a normal activity level and no evidence of respiratory compromise.


2013 ◽  
Vol 39 (4) ◽  
pp. 418-426 ◽  
Author(s):  
Daniele Cristina Cataneo ◽  
Olavo Ribeiro Rodrigues ◽  
Erica Nishida Hasimoto ◽  
Aurelino Fernandes Schmidt Jr ◽  
Antonio Jose Maria Cataneo

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.


2003 ◽  
Vol 35 (5) ◽  
pp. 384-391 ◽  
Author(s):  
Uğur Özçelik ◽  
Ayhan Göçmen ◽  
Nural Kiper ◽  
Deniz Doğru ◽  
Embiya Dilber ◽  
...  

2017 ◽  
Vol 07 (01) ◽  
pp. e4-e9
Author(s):  
Hemonta Dutta ◽  
Madhuchanda Bora ◽  
Diganta Saikia

Objective The purpose of this study is to review our experience with neonates and infants with congenital lung lesions emphasizing natural history, management, and outcomes. Methods A total of 29 neonates and infants presented with congenital lung lesions between 2000 and 2015. Two patients died before surgery due to complications, and one patient refused surgery. Overall, 26 of them were subjected to surgical treatment and were included in the study. Demographic data, indications for surgery, operative procedure, complications, hospital stay, and follow-up were assessed. Results A total of 26 children aged 5 to 122 days (mean: 35.5 days, 14 males) presented with various congenital lung malformations: congenital lobar emphysema in 10, congenital cystic adenomatous malformation in 8, bronchogenic cyst in 5, and pulmonary sequestration in 3 patients. Respiratory distress and respiratory tract infection were the most common presenting symptoms noted in 22 patients. In three patients the lesion was detected incidentally on chest X-ray. Lobectomy was the most common operation (19/26). Postoperative complications were noted in 12 patients. One patient died due to postoperative sepsis. Postoperative ventilation was required in 24 patients. Patients in the asymptomatic group recovered without any complications. The follow-up period ranged from 3 months to 15 years (median: 76.3 months). Only 12 patients received epidural anesthesia and had a better recovery than the other patients. Conclusion Congenital lobar emphysema was the most common congenital lung lesion in our series. Respiratory distress and respiratory infection were the most common symptoms. Neonates and infants tolerated lung resection well. Use of epidural anesthesia led to less postoperative complications.


2011 ◽  
Vol 1 (4) ◽  
pp. 101 ◽  
Author(s):  
Sriparna Basu ◽  
Aditya Kumar Gupta ◽  
Ashok Kumar

Congenital lobar emphysema (CLE) is an uncommon congenital malformation of the lung that can present with respiratory distress in early life. We report a full term female baby who presented with intermittent bluish discoloration of the lips and extremities during crying since birth. An initial diagnosis of left sided collapse-consolidation with right-sided compensatory emphysema was made from chest xray. When no improvement was noted after intravenous antibiotic therapy for 7 days, computerized tomographic scan of thorax was done which showed emphysema of the right middle lobe with transherniation and mediastinal shift to the left. Right middle lobectomy was done and postoperative period was uneventful. Histopathological examination of the specimen confirmed the diagnosis of CLE. CLE can mimic more common causes of respiratory distress in neonatal period and needs a high index of suspicion for diagnosis. Early diagnosis and effective surgical treatment is curative.


2020 ◽  
Vol 9 (3) ◽  
pp. 266-271
Author(s):  
Qiaoling Lei ◽  
Wen Zeng ◽  
Rong Ju

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