Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions

A baby with cough and poor feeding

S. Sonnappa, G. Cohen, A. Ramsay, R. Dinwiddie, A. Jaffé
European Respiratory Journal 2003 22: 182-185; DOI: 10.1183/09031936.03.00105402
S. Sonnappa
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
G. Cohen
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. Ramsay
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
R. Dinwiddie
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. Jaffé
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Case history

A 9‐week-old female baby presented at her local hospital with a 10‐day history of cough and poor feeding. The pregnancy had been uneventful with normal antenatal scans. The baby was born at term by normal vaginal delivery weighing 3.23 kg with good American Paediatric Gross Assessment Record scores and was sent home on the second day after birth. Prior to this presentation, she had exhibited no respiratory symptoms and was maintaining good growth.

On review, the baby was afebrile with no respiratory distress. Chest auscultation revealed reduced breath sounds on the left side. The rest of the physical examination results were unremarkable. Arterial oxygen saturation was 97% in air.

Initial chest computed tomography (CT) results are shown in figure 1⇓. Unfortunately, the initial chest radiograph was “lost”.

Fig. 1.—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 1.—

Initial computed tomography of the chest showing: a) upper and b) lower lung lobes.

One week later, the baby underwent thoracotomy and resection. A chest radiograph taken the day prior to surgery is shown in figure 2⇓.

Fig. 2.—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 2.—

Preoperative chest radiograph.

The histopathology of the lesion is shown in figure 3⇓.

Fig. 3.—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 3.—

Histopathology of lung lesion (haematoxylin and eosin stain). Scale bars a) 200 µm and b) 100 µm.

BEFORE TURNING THE PAGE, INTERPRET THE RADIOGRAPHS, COMPUTED TOMOGRAM OF THE CHEST AND HISTOPATHOLOGICAL RESULTS AND SUGGEST DIAGNOSIS AND FURTHER MANAGEMENT OF THE PATIENT.

Interpretation

Chest computed tomography on presentation

There was marked hyperexpansion of the left upper lobe with multiple cystic lesions and a mediastinal shift to the right (fig. 1a⇑). The left lower lobe was compressed with multiple cysts within (fig. 1b⇑).

Preoperative chest radiograph

The preoperative chest radiograph was taken 9 days after initial presentation (fig. 2⇑). There were cystic changes in the left lower lobe. The changes seen previously in the left upper lobe had completely resolved.

Surgical findings

On surgery, the left upper lobe looked normal, whereas the left lower lobe was congested with signs of infection. The lower lobe was totally adherent to the diaphragm with signs of hyperaemic granulation tissue. Left lower lobe resection was performed.

Histopathology

A 5×4×4‐cm lung lobe was received. On cut section, approximately one-half of the normal lung parenchyma was replaced by variously sized cystic spaces. Microscopic examination showed patchy atelectasis and focal mild interstitial fibrosis together with numerous air-filled cystic spaces (fig. 3⇑). These spaces exhibited a fibrous wall and a lining composed of numerous multinucleate foreign-body-type macrophages. There was no evidence of cystic adenomatoid malformation and the picture was that of persistent pulmonary interstitial emphysema (PIE).

Diagnosis: “Localised persistent pulmonary interstitial emphysema of the left lung”.

Clinical course

The baby progressed well following lobectomy and made an uneventful recovery. She was asymptomatic 6 months after surgery and chest CT results were normal except for a small area of hyperlucency on the left side.

Discussion

PIE in the newborn is a frequent complication of assisted mechanical ventilation. It most commonly occurs in premature infants with respiratory distress syndrome. Occasionally, it occurs spontaneously with minimal or no respiratory symptoms, as in the present patient. PIE may be acute (<7 days duration) or chronic (or persistent) and may be localised to one or more lobes, or distributed diffusely throughout all lobes. The three forms of PIE, i.e. acute PIE, localised persistent PIE (LPPIE) and diffuse persistent PIE (DPPIE), have different clinical, radiographical and pathological features 1–4.

LPPIE usually develops in infants with hyaline membrane disease but can also occur spontaneously with no underlying lung disease, as in the present case. LPPIE is a rare form of interstitial emphysema compared with the diffuse form, with a propensity for localised persistent progressive accumulation of air in the interstitium. This accumulation results in cystic air spaces that are typically associated with mediastinal shift and progressive respiratory distress.

On plain radiography, LPPIE can be differentiated from acute PIE and DPPIE by its expansile multicystic appearance. The cystic air spaces are localised to one or more lobes and are associated with a mediastinal shift and mass effect. Chest CT is helpful in differentiating LPPIE from other lesions, such as congenital cystic adenomatoid malformation and congenital lobar emphysema, as the abnormal air collection is in the interstitium and surrounds the bronchovascular bundles. On CT, the bronchovascular bundle may appear as a soft tissue attenuation nodule or line in the centre of the air-filled cyst (central lines/dots surrounded by lucency) but may be absent or difficult to see 5, as in the present case. Emphysematous distension of the alveoli and air-containing congenital masses do not show this appearance. In equivocal cases, it may be helpful to administer intravenous contrast material that results in enhancement of the vascular component 6.

Pathologically, persistent PIE is characterised by distortion of the pulmonary parenchyma by cysts of various size. These cysts are abnormal air-filled spaces in the interstitium and typically show foreign-body giant cells at their periphery. The postulated pathogenesis is a break in the integrity of the bronchoalveolar system that allows air to escape into the interstitium, where it excites a giant cell and fibroblastic response 7, 8.

Although some cases require surgical resection, in most cases the interstitially leaked air is resorbed, as seen in the present case. Some authors recommend conservative management in the first instance (selective intubation and selective bronchial obstruction and/or decubital positioning) 9–11. Resection of the LPPIE may be beneficial when there is: 1) significant reduction in effective lung volume producing ventilator dependence; 2) atelectasis and recurrent infections; or 3) recurrent pneumothoraces. This is usually indicated in a small fraction of patients (<2%). These patients can be expected to improve significantly after resection and the long-term outcome is generally good 12.

It is important to differentiate localised persistent pulmonary interstitial emphysema from other radiolucent cystic lesions, such as congenital cystic adenomatoid malformation and congenital lobar emphysema, as this can avert unnecessary surgery in some cases. Careful attention to the round or linear soft tissue component seen in the wall or within air-containing spaces, although not present in all cases, is key to making the correct diagnosis on computed tomography of the chest. In retrospect, surgery was probably unnecessary in the present case but was undertaken because of difficulty in making the diagnosis of localised persistent pulmonary interstitial emphysema in the absence of classical computed tomographic changes. However, the diagnosis should be entertained in cystic lesions that resolve spontaneously over a short period of time.

Acknowledgments

The authors would like to thank K. McHugh for helpful comments on the imaging.

    • © ERS Journals Ltd

    References

    1. ↵
      Oh MH, Kim MY, Oh SS, Shin BK, Cho SJ, Kim HK. A case of localized persistent interstitial pulmonary emphysema. J Korean Med Sci 2001;16:225–228.
      OpenUrlPubMedWeb of Science
    2. Cohen MC, Drut RM, Drut R. Solitary unilocular cyst of the lung with features of persistent interstitial pulmonary emphysema: report of four cases. Pediatr Dev Pathol 1999;2:531–536.
      OpenUrlCrossRefPubMedWeb of Science
    3. Crosswell HE, Stewart DL. Radiological case of the month. Arch Pediatr Adolesc Med 2001;155:615–616.
      OpenUrlPubMedWeb of Science
    4. ↵
      Drut R, Drut M. Pathological case of the month. Arch Pediatr Adolesc Med 2000;154:87–88.
      OpenUrlCrossRefPubMedWeb of Science
    5. ↵
      Jabra AA, Fishman EK, Shehata BM, Perlman EJ. Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-patholgic correlation. AJR Am J Roentgenol 1997;169:1381–1384.
      OpenUrlPubMedWeb of Science
    6. ↵
      Donnelly LF, Frush DP. Localized radiolucent chest lesions in neonates: causes and differentiation. AJR Am J Roentgenol 1999;172:1651–1658.
      OpenUrlPubMedWeb of Science
    7. ↵
      Yao JL, Fasano M, Morotti R, Caprio M, Greco MA. Demonstration of communication between alveolus and interstitium in persistent interstitial pulmonary emphysema: case report. Pediatr Dev Pathol 1999;2:484–487.
      OpenUrlCrossRefPubMedWeb of Science
    8. ↵
      Zimmermann H. Progressive interstitial pulmonary lobar emphysema. Eur J Pediatr 1982;138:258–262.
      OpenUrlCrossRefPubMedWeb of Science
    9. ↵
      Cohen RS, Smith DW, Stevenson DK, Moskowitz PS, Graham CB. Lateral decubitus position as therapy for persistent focal pulmonary interstitial emphysema in neonates: a preliminary report. J Pediatr 1984;104:441–443.
      OpenUrlPubMedWeb of Science
    10. Leonidas JC, Hall RT, Rhodes PG. Conservative management of unilateral pulmonary interstitial emphysema under tension. J Pediatr 1975;87:776–778.
      OpenUrlCrossRefPubMedWeb of Science
    11. ↵
      Gurakan B, Tarcan A, Arda IS, Coskun M. Persistent pulmonary interstitial emphysema in an unventilated neonate. Pediatr Pulmonol 2002;34:409–411.
      OpenUrlCrossRefPubMedWeb of Science
    12. ↵
      Schneider JR, St Cyr JA, Thompson TR, Johnson DE, Burke BA, Foker JE. The changing spectrum of cystic pulmonary lesions requiring surgical resection in infants. J Thorac Cardiovasc Surg 1985;89:332–339.
      OpenUrlPubMedWeb of Science
    PreviousNext
    Back to top
    View this article with LENS
    Vol 22 Issue 1 Table of Contents
    • Table of Contents
    • Index by author
    Email

    Thank you for your interest in spreading the word on European Respiratory Society .

    NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

    Enter multiple addresses on separate lines or separate them with commas.
    A baby with cough and poor feeding
    (Your Name) has sent you a message from European Respiratory Society
    (Your Name) thought you would like to see the European Respiratory Society web site.
    CAPTCHA
    This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
    Print
    Citation Tools
    A baby with cough and poor feeding
    S. Sonnappa, G. Cohen, A. Ramsay, R. Dinwiddie, A. Jaffé
    European Respiratory Journal Jul 2003, 22 (1) 182-185; DOI: 10.1183/09031936.03.00105402

    Citation Manager Formats

    • BibTeX
    • Bookends
    • EasyBib
    • EndNote (tagged)
    • EndNote 8 (xml)
    • Medlars
    • Mendeley
    • Papers
    • RefWorks Tagged
    • Ref Manager
    • RIS
    • Zotero

    Share
    A baby with cough and poor feeding
    S. Sonnappa, G. Cohen, A. Ramsay, R. Dinwiddie, A. Jaffé
    European Respiratory Journal Jul 2003, 22 (1) 182-185; DOI: 10.1183/09031936.03.00105402
    del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
    Full Text (PDF)

    Jump To

    • Article
      • Case history
      • Interpretation
      • Clinical course
      • Discussion
      • Acknowledgments
      • References
    • Figures & Data
    • Info & Metrics
    • PDF
    • Tweet Widget
    • Facebook Like
    • Google Plus One

    More in this TOC Section

    • A turbulent cause of bilateral pneumonia
    • An incidental finding in a 34-year-old male under investigation for haemoptysis
    • A 61-year-old female patient with right-sided pleuritic chest pain and fatigue
    Show more Case for Diagnosis

    Related Articles

    Navigate

    • Home
    • Current issue
    • Archive

    About the ERJ

    • Journal information
    • Editorial board
    • Reviewers
    • Press
    • Permissions and reprints
    • Advertising

    The European Respiratory Society

    • Society home
    • myERS
    • Privacy policy
    • Accessibility

    ERS publications

    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS books online
    • ERS Bookshop

    Help

    • Feedback

    For authors

    • Instructions for authors
    • Publication ethics and malpractice
    • Submit a manuscript

    For readers

    • Alerts
    • Subjects
    • Podcasts
    • RSS

    Subscriptions

    • Accessing the ERS publications

    Contact us

    European Respiratory Society
    442 Glossop Road
    Sheffield S10 2PX
    United Kingdom
    Tel: +44 114 2672860
    Email: journals@ersnet.org

    ISSN

    Print ISSN:  0903-1936
    Online ISSN: 1399-3003

    Copyright © 2023 by the European Respiratory Society