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Weekly Chest CasesArchive of Old Cases

Case No : 791 Date 2012-12-24

  • Courtesy of Semin Chung, Jae-Seung Suh / Chung Ang University Hospital
  • Age/Sex 0 / M
  • Chief ComplaintKnown lung mass on antenatal US
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

Figure 1

Diagnosis With Brief Discussion

Diagnosis
Congenital Lobar Emphysema
Radiologic Findings
Lund and mediastinal window images of axial and coronal chest CT scans show multiple cystic lesions with or without air-fluid level and overdistended parenchyma in the left lower lobe. There are associated mediastinal shift and adjacent compressive atelectasis of normal parenchyma. (Figs1-6)
Chest radiograph shows a hyperlucent left lung, left rib space widening, and left hemidiaphragm depression causing left to right sided mediastinal shift. (Fig 7)
Brief Review
The term congenital lobar emphysema (CLE) is preferable to the term congenital pulmonary overinflation (CLO) although the term emphysema is technically inaccurate because the alveoli expand and the alveolar walls remain intact. CLE usually affects a single lobe of the lungs and results from progressive lobar overexpansion and subsequent compression of the ipsilateral remaining lung, which can be secondary to an intrinsic cartilaginous abnormality with resultant weak or absent bronchial cartilage or extrinsic compression of an airway. The collapsed airway can act as a one-way valve, resulting in air trapping. Radiography performed during the neonatal period may show a radiodense area in the lung, since the involved section is still filled with fluid. Over time, the fluid will resolve via the lymphatic and capillary systems. After birth, the affected lung will progressively overinflate and become hyperlucent on conventional radiographs as well as CT scans. With continued expansion, there can be associated mediastinal shift, rib space widening, hemidiaphragm depression, and adjacent compressive atelectasis. At imaging, CLE can be confused with a pneumothorax or pulmonary cyst, but the presence of organized pulmonary markings coursing through an overdistended portion of the affected lung should help in making the diagnosis of CLE. At microscopy, CLE have airspace enlargement without maldevelopment whereas congenital cystic adenomatoid malformation (CCAM) have the cysts lined by ciliated or cuboid respiratory epithelium (Fig). In addition, pulmonary interstitial emphysema (PIE) arises from a disruption of the basement membrane of the alveolar wall, allowing air entry into the interstitial space that may be due to aspiration of foreign material or partial occlusion of bronchi or bronchiole in a newborn. Line-and-dot pattern, which consists of multiple soft-tissue density linear and punctuate structures within cystic radiolucencies, is a typical radiologic appearance. For patients who demonstrate mild respiratory distress, treatment can be conservative, with low-volume and low-pressure ventilatory support given as needed. However, with more severe neonatal respiratory distress, emergent surgical resection of the affected lobe may be needed.
References
1. Biyyam DR, Chapman T, Ferguson MR, Deutsch G, Dighe MK. Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation.
2. Kuhn C 3rd, West WW, Craighead JE. Lungs. In: Damjanov I, Linder J, eds. Anderson's pathology. 10th ed. St Louis, Mo: Mosby, 1996; 1471.
3. Stigers KB, Woodring JH, Kanga JF. The clinical and imaging spectrum of findings in patients with congenital lobar emphysema. Pediatr Pulmonol 1992;14(3):160
Keywords
lung, congenital,

No. of Applicants : 103

▶ Correct Answer : 4/103,  3.9%
  • - GHICL , France manuel toledano
  • - , Korea (South) Sanghyuk Ahn
  • - Hopital du Sacre Coeur , Canada Andrea Ojanguren
  • - Teikyo mizonokuchi hospital , Japan Takana Yamakawa
▶ Correct Answer as Differential Diagnosis : 26/103,  25.2%
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - University of British Columbia , Canada Amr Ajlan
  • - SNUH , Korea (South) Euijin Hwang
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Goa Medical College , India Paresh Desai
  • - UWO , Canada S Lee
  • - Dong-A University Hospital , Korea (South) Ji-yeon Han
  • - McGill University Health Center , Canada Alexandre Semionov
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - royal melbourne hospital , Australia kimchi kings
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - HIA Legouest - METZ , France Alban Gervaise
  • - China Medical University , Ditmanson Medical Foundation Chia-Yi Christian Hospital ,Taiwan, R.O.C. , Taiwan Jun Jun Yeh
  • - Suncheon Plus Medicine , Korea (South) Park Sang Hyun
  • - Dae-Joen Bohun Veterans Hospital , Korea (South) KI-TAE HAN
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Hanyang University Hospital , Korea (South) Yo Won Choi
  • - Fukuyama city hospital , Japan Ryotaro Kishi
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - All India Institute of medical sciences , India Justin Moses
  • - Myongji hospital , Korea (South) Ji Ye Lee
  • - chp st martin , France Mariotte benoit
  • - CAC Rennes , France nicolas gautier
  • - NASA SCANS , India RAKESH BHATIA
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