Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
Search Ahead of print Current Issue Archives Instructions Subscribe e-Alerts Login 
Home Email this article link Print this article Bookmark this page Decrease font size Default font size Increase font size


 
Table of Contents   
POSTGRADUATE CLINICAL SECTION
Year : 2011  |  Volume : 6  |  Issue : 4  |  Page : 231-234
A 38-year-old man with lung cysts


1 Department of Pulmonary Medicine, Era's Lucknow Medical College, Lucknow, India
2 Department of Pulmonary Medicine,B. Y. L. Nair Hospital and T.N. Medical College, Mumbai, India

Date of Submission01-Apr-2011
Date of Acceptance05-May-2011
Date of Web Publication12-Sep-2011

Correspondence Address:
Jyotsna M Joshi
Head of Department of Pulmonary Medicine, B. Y. L. Nair Hospital and T.N. Medical College, Dr. A. L. Nair Road, Mumbai - 400 008
India
Login to access the Email id


DOI: 10.4103/1817-1737.84779

PMID: 21977070

Rights and Permissions



How to cite this article:
Gothi D, Joshi JM. A 38-year-old man with lung cysts. Ann Thorac Med 2011;6:231-4

How to cite this URL:
Gothi D, Joshi JM. A 38-year-old man with lung cysts. Ann Thorac Med [serial online] 2011 [cited 2019 Nov 14];6:231-4. Available from: http://www.thoracicmedicine.org/text.asp?2011/6/4/231/84779


A 38-year-old man gave history of cough with expectoration and dyspnea since childhood. He had 2-3 exacerbations a year. He denied history of wheezing or hemoptysis. He had no history of exanthematous fever or pneumonia in childhood. There was no relevant occupational history or systemic complaint. He was an ex-smoker, having quit smoking 10 years ago; he had a smoking index of 7.5 pack years. He was married, with three children. His maternal aunt was diagnosed to have bronchial asthma. The physical examination was normal.

The chest radiograph and high-resolution computed tomography (HRCT) of thorax are shown in [Figure 1] and [Figure 2]a and b, respectively. Hemogram and biochemical investigations were normal. Spirometry showed forced vital capacity (FVC): 2.23 L (55% of predicted), forced expiratory volume in one second (FEV 1 ): 1.94 L (56% of predicted), and FEV 1 /FVC: 87%; there was improvement in FEV 1 of 200 mL following inhalation of 200 μg of salbutamol.
Figure 1: Chest radiograph (posteroanterior view)

Click here to view
Figure 2: (a)High-resolution computed tomography of thorax (transverse section)
Figure 2b: High-resolution computed tomography of thorax (sagittal section)


Click here to view



   Questions Top


  1. What do the chest radiograph and HRCT show?
  2. What are the possible differential diagnoses on HRCT thorax? What is the most likely diagnosis if the history and HRCT thorax are correlated?
  3. Which one of the two is the correct terminology: 'congenital pulmonary airway malformation' or 'congenital cystic adenoid malformation'? What is the new classification of this condition?



   Answers Top


  1. The chest radiograph shows multiple ill-defined air lucent opacities in the left upper lobe. On HRCT of the thorax, however, the air lucent opacities appear to be due to multiple confluent cysts of 2-5 cm size each.
  2. The various causes of cystic lesions on imaging are given in [Table 1]. [1],[2],[3] Amongst the congenital malformations, congenital lobar emphysema (CLE) is unlikely because it usually presents before 6 months of age. Also, in CLE, HRCT thorax will show hyperinflated lungs. Bronchogenic cysts are usually single and are seen in the middle third of the lung. The possibility of intralobar sequestration can be considered as the lesions are cystic; however, sequestration is usually seen in the medial aspect of a posterior lung base and hence is unlikely in this case. Congenital pulmonary airway malformation (CPAM) appears to be the most plausible amongst the congenital etiologies. Acquired causes like infection and cysts with interstitial lung disease are unlikely because the cysts are thin walled and the remaining lung parenchyma is normal. The cysts are too large and are located in the upper lobe and hence bronchiectasis is also improbable. A cystic neoplasm is the other possible differential diagnosis. Thus, amongst the congenital malformations and acquired causes of cysts, CPAM and cystic neoplasm are the most likely diagnoses.The patient was subjected to left upper lobectomy. The resected specimen confirmed the presence of multiple large intercommunicating cysts filled with gelatinous mucoid material [Figure 3]. The histopathology showed the cysts to be lined by pseudostratified ciliated columnar epithelium [Figure 4], with underlying fibromuscular bands; the appearance was suggestive of type 1 CPAM.
  3. Table 1: Causes of cystic lung


    Click here to view
    Figure 3: Specimen of resected lung showing multiple intercommunicating cysts

    Click here to view
    Figure 4: High-resolution photomicrograph of resected lung

    Click here to view
  4. CPAM was earlier known as congenital cystic adenomatoid malformation. [4] Recently, it has been renamed as CPAM because not all the lesions are cystic or adenomatoid. [5] On the basis of the apparent site of origin, five types [Figure 5] have been described in the new classification[6] [Table 2]: Type 0 - alveolar dysplasia or dysgenesis involving the proximal tracheobronchial tree; type 1 - proximal acinus (bronchial/bronchiolar); type 2 -midacinus (bronchiolar); type 3 - bronchiolar/alveolar duct; and type 4 - alveolar saccular/distal acinar.
Table 2: Types of congenital pulmonary airway malformation


Click here to view
Figure 5: Types of congenital pulmonary airway malformation

Click here to view



   Discussion Top


CPAM is a rare hamartomatous developmental anomaly that results from unsystematic spread of tubular bronchioles and enlarged alveolar tissue. [5] It was first described by Chin and Tang in 1949. It is seen in about 1 in 25000-35000 live births.[4] CPAM type 0 is incompatible with life and hence always presents before or immediately after birth. The other types are diagnosed during the neonatal period, when respiratory distress can be caused by inflation of the cysts with spontaneous respiration, or during childhood or adulthood, when the condition causes repeated respiratory tract infection, pneumothoraces, or hemoptysis. [7] CPAM type 1 may sometimes remain asymptomatic.[7] There is no sex predilection or chromosomal anomaly observed with CPAM. Radiologically, type 1 and type 4 have large cysts that may be up to 10 cm in size and type 2 and type 3 have small cysts that are <2 cm in size.[8],[9] Histopathology is essential to confirm the diagnosis and ascertain the type of CPAM.

CPAM may be associated with congenital anomalies such as congenital heart disease (e.g., left heart hypoplasia); pulmonary malformations (e.g., sequestration, pulmonary hypoplasia); skeletal anomalies (e.g., pectus excavatum, genu varum, scoliosis); and renal anomalies (e.g., renal agenesis). [10] Some of these anomalies are common with specific types of CPAM [Table 2]. If CPAM is diagnosed during the early neonatal period, conservative management is recommended, as postnatal regression of the lesion may occur. Surgical intervention remains the gold standard for CPAM diagnosed at later ages because remissions are infrequent and because of the risk of repeated infections and malignant transformation. [11]

 
   References Top

1.Chadha R, Singh D, Kathuria P, Sharma S, Choudhury SR, Jain M. Congenital cystic adenomatoid malformation associated with ipsilateral eventration of the diaphragm. Indian J Pediatr 2006;73:832-4.  Back to cited text no. 1
[PUBMED]    
2.Nuchtern JG, Harberg FJ. Congenital lung cysts. Semin Pediatr Surg 1994;3:233-43.  Back to cited text no. 2
[PUBMED]    
3.Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: Focal and diffuse. Mayo Clin Proc 2003;78:744-52.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg 2008;17:2-8.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Kilinc N, Önen A, Yayla M. Congenital pulmonary airway malformation: Case report. Perinatal Journal 2007;15:47-19.  Back to cited text no. 5
    
6.Stocker JT. Congenital and developmental lung disease. In: Dail and Hammer's Pulmonary Pathology. Non-neoplastic lung disease. Tomashefski JF, Editor. Vol. 1. 3rd ed. NY: Springer Science + Business Media LLC; 2008. p. 154-61.  Back to cited text no. 6
    
7.Roberts DH, Lai PS, Cohen DW, DeCamp MM, Fazio S. A 40-year-old woman with an asymptomatic cystic lesion in her right lung. Chest 2009;136;622-7.  Back to cited text no. 7
    
8.Couluris M, Schnapf BM, Gilbert-Barness E. Intralobar pulmonary sequestration associated with a congenital pulmonary airway malformation type II. Fetal Pediatr Pathol 2007;26:207-12.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Stocker JT, Husain AN. Cystic lesions of the lung in children: Classification and controversies. Eur Respir Mon 2007;39:1-20.  Back to cited text no. 9
    
10.Bassam A, Rabbeah A, Nassar S, Mobaireek K, Rawaf A, Banier H, et al. Congenital cystic disease of the lung in infants and children (experience with 57 cases). Eur J Pediatr Surg 1999;9:364-8.  Back to cited text no. 10
    
11.Liao S, Lai1 SH, Hsueh C, Wong KS. Comparing late-onset and neonatally-diagnosed congenital cystic adenomatoid malformation of the lung. Chang Gung Med J 2010;33:36-42.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2]

This article has been cited by
1 result 1 Document Angeborene Störungen der Lungen und Atemwege - Übersicht der Krankheitsfälle der Kinderklinik Erlangen aus den Jahren 2000 bis 2011 | [Congenital disorders of the lungs and airways - Survey of cases at the paediatric hospital erlangen in the period 2000 to 2011]
Meyer, S., Zimmermann, T.
Pneumologie. 2013;
[Pubmed]



 

Top
Print this article  Email this article
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,556 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


   Questions
   Answers
   Discussion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed4420    
    Printed94    
    Emailed0    
    PDF Downloaded502    
    Comments [Add]    
    Cited by others 1    

Recommend this journal