alexa Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy | Open Access Journals
ISSN: 2161-105X
Journal of Pulmonary & Respiratory Medicine
Like us on:
Make the best use of Scientific Research and information from our 700+ peer reviewed, Open Access Journals that operates with the help of 50,000+ Editorial Board Members and esteemed reviewers and 1000+ Scientific associations in Medical, Clinical, Pharmaceutical, Engineering, Technology and Management Fields.
Meet Inspiring Speakers and Experts at our 3000+ Global Conferenceseries Events with over 600+ Conferences, 1200+ Symposiums and 1200+ Workshops on
Medical, Pharma, Engineering, Science, Technology and Business

Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy

Yaron Perry1*, Monica Espiritu2 and Emilee Schroeder1

1Minimally Invasive Thoracic Surgery, Memorial Health Medical Center, Savannah, GA, USA

2Tufts University School of Medicine, Boston, MA, USA

*Corresponding Author:
Yaron Perry, MD, FACS
Director- Minimally Invasive Thoracic Surgery
Memorial Health Medical Center
Savannah, GA, USA
E-mail: [email protected]

Received date: March 11, 2013; Accepted date: April 16, 2013; Published date: April 19, 2013

Citation: Perry Y, Espiritu M, Schroeder E (2013) Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy. J Pulmon Resp Med S14:003. doi:10.4172/2161-105X.S14-003

Copyright: © 2013 Perry Y, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Visit for more related articles at Journal of Pulmonary & Respiratory Medicine

Keywords

Congenital lobar emphysema; Video assisted thoracoscopy

Case Report

A 25-year old Puerto Rican female presented with increased shortness of breath, cough and chest pain. Her past medical history included diagnosis of asthma In Puerto-Rico at the age of five, treated with advair. During her third pregnancy, at the age of 23, the patient experienced shortness of breath and reported “fainting”. A chest CT at that time diagnosed her with lobar emphysema.

In the United States, she presented to an outside hospital with exacerbation of shortness of breath, where she was thought to have a pneumothorax, and a right chest tube was placed. She was then transferred to the Medical Center ICU. At presentation, She was afebrile, exhibiting tachycardia (117 bpm), with 28 respirations per minute, oxygen saturation of 91%, and decreased breath sounds on the right chest field.

Pulmonary function test suggested of Restrictive Lung Disease (RLD) (Table 1). The patient’s initial X-rays, as seen in Figure 1, indicate hyperlucency on the right side, flattening of the right diaphragm, and a slight shift of mediastinum to the left. The right lung is collapsed inferiorly. There are a severe number of bullae within the right hemithorax, with some consolidation or collapse in the region of the right perihilar region.

pulmonary-respiratory-Pre-operative-chest-X-ray

Figure 1: Pre-operative chest X-ray.

CT taken before surgery documented large bullae, mediastinal shift, and compressed right lung. (Figure 2 and 3).

pulmonary-respiratory-Large-bullae-mediastinal

Figure 2: CT demonstrating: Large bullae, mediastinal shift, compressed right Lung.

pulmonary-respiratory-normal-lung-tissue

Figure 3: CT; lower cuts show some relatively normal lung tissue. Note the leftward mediastinal shift and the compression of the left lung.

Treatment and Surgical Findings

Since the patient’s shortness of breath was not successfully managed with Oxygen support and chest tube drainage, she was scheduled for a bronchoscopy and a Video Assisted Thoracoscopic Surgery (VATS) - right upper lobectomy.

On Bronchoscopy

The Right Upper Lobe (RUL) takeoff was a slit-like lumen, slightly distorted - confirms the diagnosis of CLE.

VATS right upper lobectomy was performed with general endotracheal anesthesia with a double lumen tube and one lung ventilation. We used 3 ports; 10mm port in the seventh intercostal space in anterior axillary line, 10mm trocar in posterior axillary line and a working port in the fifth intercostal space in mid axillary line.

Using a 10 mm 30 degree Olympus scope

The right upper lobe composed entirely of large bullae. The bullae did not deflate and were densely adherent to the chest wall. There were also aberrant blood vessels from the chest wall to the bullae, with no apparent superior pulmonary vein, pulmonary artery, or bronchi going to the bullous portion. However, the segmental bronchi and major pulmonary vessels were found attached to the area of the hilum of the atritic lobe. An endoscopic EndoGIA (Covidian) used to divide the vessels and the rudimentary bronchus. The lobe removed with an endobag. A 28 french chest tube was left in the chest.

Pathology

Microscopic examination of the resected lobe showed dense fibrosis, smooth cell proliferation with entrapped cuboidal respiratory epithelial-lined spaces. The large bullae found in the periphery of the emphysematous lobe were fibrous-walled. These findings confirmed chronic changes consistent with congenital lobar emphysema and appear to be secondary to bronchial atresia. No evidence of bronchial muscle proliferation to support the diagnosis of asthma.

The patient’s postoperative course was uneventful; the drain was removed on postoperative day 3 and she was discharged home 4 days after her surgery. In the beginning of July 2009, the patient revisited the hospital for a follow-up exam. Her thoracoscopy incisions had completely healed and she was feeling much better clinically.

Post-Op Chest X-Ray shows expansion of the right middle and lower lobe, the right hemidiaphragm is no longer flattened, and the mediastinum has shifted back to the midline (Figure 4).

pulmonary-respiratory-Post-Op-Chest-X-Ray

Figure 4: Post-Op Chest X-Ray shows expansion of the right middle and lower lobe. The right hemidiaphragm is no longer flattened. The mediastinum has shifted back to the midline.

Pulmonary Function tests (PFTs) were repeated, and were remarkably better (Table 1).

Pre-op 90 days post-op
FVC 2.47 liters 68% the predicted value 3.6 liters 99% the predicted value
FEV1 2.02 liters 67% the predicted value 3 liters, 94% predicted
TLC 4.3 liters 4.9 liters
FRC 1.79 liters 2.55 liters

Table 1: Pulmonary function test before and after surgery.

Summary and Discussion

Congenital lobar emphysema involves over-inflation and distention of one or more pulmonary lobes. It usually presents as respiratory distress in the first few days of life, and a majority of reported cases of congenital lobar emphysema have documented its presentation in neonates [1-10]. Approximately 50% of cases identified in the first month of life, and only 5% of cases are detected after the age of six months [11]. We discussed the successful diagnosis and treatment of a rare case of congenital lobar emphysema in a 25- year old woman.

Bronchial atresia is suggested etiology for lobar emphysema, it is a congenital condition in which the bronchus lacks communication within the central airway. This disorder is characterized by a bronchocele, a localized dilation of the bronchus, due to a mucus-filled bronchus that has prematurely,blindly terminated. It involves hyperinflation of the peripheral lung. Seo et al. [12] outlines two cases of congenital lobar emphysema that have been caused by lobar bronchial atresia. In cases in which CLE is directly linked to bronchial atresia, the pulmonary parenchyma distal to the lesion may be aerated by collateral ventilation [12]. This is not as effective in expiration as it is in inspiration, resulting in a significant amount of air trapping. The pathogenesis has been described as “localized interruption of the blood supply to an already formed bronchus, resulting in bronchial infarction and luminal obstruction, or developmental abnormalities of bronchial cartilage after completion of bronchial branching [13]. In patients in whom congenital lobar emphysema is caused by bronchial atresia, the affected emphysematous lobe is extremely large, with concomitant compression of the normal lung tissue, displacement of the mediastinum, and herniation of the emphysematous lobe across the antrerior mediastinum into the opposite side of the chest [12]. These findings are consistent with the most typical presentations of CLE.

In the case we presented The CT scan showed narrowed RUL bronchus but it was not atretic, the bronchoscopy documented formed RUL bronchus although narrow and smaller compared to the patients other bronchi, and no evidence of mucocele. The pathologic analysis documented abnormal bronchus wall and structure suggesting a variant of bronchial atresia. We presume that this is the cause of the lobar emphysema in our patient; the late presentation can be explained by an incomplete atresia of the upper lobar bronchus.

The adult patient treated in our case had underlying compromise of her respiratory lung function, yet, since she was breathing spontaneously, did not require positive pressure ventilation.

However, the mediastinal shift associated with CLE makes intubation difficult [14]. Intubation should thus be supplemented with fiberoptic bronchoscopy in case of tracheal deviation, and should be attempted in an anesthetized but spontaneously breathing patient [15].

A thoracotomy is often recommended for patients with CLE who are more challenging thoracoscopically because of inadequate lung collapse and poor visualization, but with the new advancement and the accumulative experience in VATS lobectomy, our patient was treated thoracoscopically [16,17]. In the literature there are few reports on treatment of CLE with thoracoscopic approach while most of it in childhood [18-23]. Thus, in adult we found one case report by Muramatsu [24] while no long-term follow up or pulmonary function improvements were mentioned.

Our case report helps to make the medical community aware of this rare pathology in adults and supports VATS lobectomy as an ideal approach to treating highly symptomatic CLE adult patients.

References

Select your language of interest to view the total content in your interested language
Post your comment

Share This Article

Recommended Conferences

Article Usage

  • Total views: 11762
  • [From(publication date):
    specialissue-2013 - Nov 20, 2017]
  • Breakdown by view type
  • HTML page views : 7969
  • PDF downloads : 3793
 

Post your comment

captcha   Reload  Can't read the image? click here to refresh

Peer Reviewed Journals
 
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
 
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri & Aquaculture Journals

Dr. Krish

[email protected]

1-702-714-7001Extn: 9040

Biochemistry Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Business & Management Journals

Ronald

[email protected]

1-702-714-7001Extn: 9042

Chemistry Journals

Gabriel Shaw

[email protected]

1-702-714-7001Extn: 9040

Clinical Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Engineering Journals

James Franklin

[email protected]

1-702-714-7001Extn: 9042

Food & Nutrition Journals

Katie Wilson

[email protected]

1-702-714-7001Extn: 9042

General Science

Andrea Jason

[email protected]

1-702-714-7001Extn: 9043

Genetics & Molecular Biology Journals

Anna Melissa

[email protected]

1-702-714-7001Extn: 9006

Immunology & Microbiology Journals

David Gorantl

[email protected]

1-702-714-7001Extn: 9014

Materials Science Journals

Rachle Green

[email protected]

1-702-714-7001Extn: 9039

Nursing & Health Care Journals

Stephanie Skinner

[email protected]

1-702-714-7001Extn: 9039

Medical Journals

Nimmi Anna

[email protected]

1-702-714-7001Extn: 9038

Neuroscience & Psychology Journals

Nathan T

[email protected]

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

Ann Jose

[email protected]

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

[email protected]

1-702-714-7001Extn: 9042

 
© 2008- 2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version
adwords