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  Table of Contents 
CASE REPORT
Year : 2019  |  Volume : 22  |  Issue : 8  |  Page : 1163-1165

Congenital segmental emphysema in an adult patient


Department of Pneumology, Interdisciplinary Allergy Outpatient Clinic, Lübeck, Germany; Department of Pulmonary Diseases, Celal Bayar University Faculty of Medicine, Manisa, Turkey

Date of Acceptance31-Jan-2019
Date of Web Publication14-Aug-2019

Correspondence Address:
Dr. A Gulsen
Department of Pneumology, Interdisciplinary Allergy Outpatient Clinic, Lübeck; Department of Pulmonary Diseases, Celal Bayar University Faculty of Medicine, Manisa

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_141_18

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   Abstract 


Congenital segmental emphysema (CSE) is a disease characterized by severe hyperinflation of a specific segment of the lung without any intrinsic or extrinsic compression. It is most commonly observed in neonates and young children with acute respiratory distress. It is usually unilateral, and the left upper lobe of the lung is most often affected. Rarely, patients may remain asymptomatic until they are older and, therefore, may be diagnosed later. Cases unexplained until adulthood are sometimes detected incidentally as single-sided hyperinflation on a chest X-ray image. We have presented an extremely rare case where the patient remained asymptomatic until adulthood and did not exhibit any clinical findings.

Keywords: Adult, congenital, emphysema, segmental


How to cite this article:
Gulsen A. Congenital segmental emphysema in an adult patient. Niger J Clin Pract 2019;22:1163-5

How to cite this URL:
Gulsen A. Congenital segmental emphysema in an adult patient. Niger J Clin Pract [serial online] 2019 [cited 2019 Aug 24];22:1163-5. Available from: http://www.njcponline.com/text.asp?2019/22/8/1163/264404




   Introduction Top


Congenital lobar emphysema (CLE) is a disease characterized by severe hyperinflation of a lobe or segment of the lung without any intrinsic or extrinsic compression.[1] Congenital segmental emphysema (CSE), which is one of its subtypes, is very rarely encountered. It is most commonly observed in neonates and young children with acute respiratory distress. It is usually unilateral, and the left upper lobe of the lung is most often affected.[2] There is a male predominance (female:male, 1:3), and it has been reported to be familial.[3]

The pathomechanism of the disease is not yet fully understood. Factors that may play a role in its etiology include viral infections that damage the alveoli, bronchial obstruction by mucous plugs with dark consistency or meconium, absence of or reduction in the cartilage, bronchomalacia, polyalveolar lobes, and external impairment or abnormality of the pulmonary artery.[2]

Respiratory symptoms arise when the condition is accompanied by compression atelectasis and a mediastinal shift. The main symptoms that lead to its diagnosis are shortness of breath, cyanosis, wheezing, intercostal withdrawal, and tachycardia.[4] It is frequently misdiagnosed as a pneumothorax or a diaphragmatic hernia. Posterior-anterior and lateral chest X-ray, computerized tomography, ventilation-perfusion scintigraphy, and bronchoscopy are usually sufficient for diagnosis. Rarely, patients may remain asymptomatic until they are older and, therefore, may be diagnosed later. Cases unexplained until adulthood are sometimes detected incidentally as single-sided hyperinflation on a chest X-ray image obtained for unrelated reasons. We would like to present a case in which CSE was diagnosed in adulthood.


   Case Report Top


A 27-year-old male patient was admitted to our outpatient clinic with a 1-week history of cough, sputum, and fever in 2015. The patient had no history of previous lung disease or chronic respiratory symptoms. He had never smoked cigarettes. On physical examination, his general condition was good; however, crackles in the right lower lung margin and relatively decreased upper left respiratory sounds were found. Body temperature was 38.5°C. Oxygen saturation measured using pulse oximetry was 97%. There was no abnormality detected in other systems. A hemogram showed a white blood cell count of 14,500/μl and a C-reactive protein level of 4.3 mg/dl. Electrocardiography findings were normal. The pulmonary function test did not show any restrictive or obstructive findings, and all findings were normal. A posteroanterior chest X-ray image showed suspicious pneumonic infiltration in the right paracardiac area and unilateral increased radiolucency of the left lung, as well as a mediastinal shift to the right [Figure 1]. Computed tomography of the thorax showed localized hyperlucency and hyperinflation of the superior segment of the left lower lobe, along with infiltration of the right lower lobe of the lung [Figure 2] and [Figure 3]. Antibiotic therapy was started for the treatment of pneumonia, and follow-up was recommended because of the absence of respiratory symptoms and normal pulmonary function parameters. After 1 month, his complaints had completely resolved. There were no respiratory symptoms during the 1-year follow-up of the patient.
Figure 1: Chest X-ray showing suspicious pneumonic infiltration in the right paracardiac area, unilateral increased radiolucency of the left lung, and a mediastinal shift to the right

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Figure 2: Coronal section of chest CT showing localized hyperlucency and hyperinflation of superior segment of the left lower lobe

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Figure 3: Axial section of chest CT image showing hiperinflation and decreased vascularity of superior segment of the left lower lobe, and alveolar infiltration of right lower lobe due to pneumonia

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   Discussion Top


CLE, also known as childhood emphysema, is often associated with sudden respiratory distress, cyanosis, asymmetric breath sounds, and displaced heart sounds in newborns.[5]

The age at presentation is usually 4 weeks to 6 months, and the incidence is 1 in 20,000-30,000.[6] The distribution of affected lobes is as follows: 42% left upper, 30% right middle, and 20% right upper; occasionally, both lungs (20%) may be involved.[7] Pneumatocele, diaphragmatic hernia, foreign body aspiration, mucus plugs, and secondary lobar emphysema should be considered in the differential diagnosis; the disease is often misdiagnosed as pneumothorax.[8]

Three different treatment modalities can be applied: medical, conservative, and surgical treatments. The indication for treatment usually determines the clinical condition of the patient. The most common indicator of surgery is the severity of respiratory distress, which usually occurs at an early age.[5] Definitive treatment is only possible with surgical correction, and this is preferred because of its curative nature. The most appropriate surgical treatment is lobectomy of the affected lobe, which produces very effective results.[3],[9] However, operations under general anesthesia and single lung ventilation are recommended.[5],[10] Intermittent positive pressure ventilation and pressure-controlled volume control mode with low tidal volume (4-6 ml/kg) have achieved successful results.[5],[10] It is stated that the induction of anesthesia in patients is very important because it can increase the amount of air trapping during crying.[10]

Furthermore, in 2010, Paramalingam et al. identified a new subtype called CSE.[11] Their definition was investigated retrospectively in 130 infant patients who were diagnosed with congenital lung parenchymal lesions in the prenatal period, and the postoperative results were evaluated in detail. As only one study has been conducted in this area, we believe that there is a need for new prospective studies. With this newly defined subtype, conservative lung surgery has become especially important for neonates and children. However, it has been argued that segmental resection may be the best alternative to lobectomy if the emphysema is detected at the segmental level.[12] In our case, the left lower lobe was affected, and the effect was at the segmental level. It is recommended that patients are given follow-up care unless the patient is asymptomatic and does not consent to any interventional diagnosis or treatment. In recent years, application of minimally invasive endobronchial occlusion has been reported as an alternative to surgical lobe resection.[13]


   Conclusion Top


Segmental lobar emphysema is a newly defined subtype of CLE, and conservative segmental resection of the lung is recommended for these cases. We have presented an extremely rare case where the patient remained asymptomatic until adulthood and did not exhibit any clinical findings.

Acknowledgments

A.G. study concept and design, data collection, manuscript preparation, and drafting of the manuscript.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Yoshioka H, Aoyama K, Iwamura Y, Muguruma T, Kondo Y, Goto T, et al. Case of congenital lobar emphysema in an 18-month-old boy and review of earlier cases. PediatrInt 2003;45:587-9.  Back to cited text no. 1
    
2.
Orenstein DM. Emphysema and overinflation. In: Behrman RE, Kliegman RM, Jenson HB, editors. Textbook of Pediatrics. 16th ed. Philadelphia: W.B. Saunders Company; 2000. p. 303.  Back to cited text no. 2
    
3.
Al-Salem AH. Congenital lobar emphysema. Saudi Med J 2002;23:335-7.  Back to cited text no. 3
    
4.
Reynolds M. Congenital lesions of the lung. In: Shields TW, LociceroIII J, Reed CE, Feins RH, editors. General Thoracic Surgery, vol 1, 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2009. p. 1017-32.  Back to cited text no. 4
    
5.
Nandihalli MC, Thammaiah SH, Kotekar N, Putran PR. Congenital lobar emphysema: A modified approach to anesthetic management. Indian J Crit Care Med 2015;19:47-9.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Jacob M, Ramesh GS, Narmadha Lakshmi K. Anesthetic management of congenital lobar emphysema in a neonate. Med J Armed Forces India 2015;71(Suppl 1):287-9.  Back to cited text no. 6
    
7.
Mei Zahav M, Konen O, Manson D, Langer JC. Is congenital lobar emphysema a surgical disease? J PediatrSurg 2006;41:1058-61.  Back to cited text no. 7
    
8.
Cooney DR, Menke JA, Allen JE. “Acquired” lobar emphysema: A complication of respiratory distress in premature infants. J PediatrSurg 1977;12:897-904.  Back to cited text no. 8
    
9.
Chandran-Mahaldar D, Kumar S, Balamurugan K, Raghuram AR, Krishnan R, Kannan. Congenital lobar emphysema. Indian J Anaesth 2009;53:482-5.  Back to cited text no. 9
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10.
Nath MP, Gupta S, Kumar A, Chakrabarty A. Congenital lobar emphysema in neonates: Anaesthetic challenges. Indian J Anaesth 2011;55:280-3.  Back to cited text no. 10
[PUBMED]  [Full text]  
11.
Paramalingam S, Parkinson E, Sellars M, Diaz-Cano S, Nicolaides KH, Davenport M. Congenital segmental emphysema: An evolving lesion. Eur J PediatrSurg 2010;20:78-81.  Back to cited text no. 11
    
12.
Krivchenya DU, Rudenko EO, Dubrovin AG. Congenital emphysema in children: Segmental lung resection as an alternative to lobectomy. J PediatrSurg 2013;48:309-14.  Back to cited text no. 12
    
13.
Price M, Happel C, Brinkmann F, Hülskamp G, Price T, Hansen G, et al. A novel non-surgical treatment of symptomatic congenital lobar emphysema. EurRespir J 2016;48(Suppl 60):PA3847.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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