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CHEST IMAGE
Year : 2014  |  Volume : 2  |  Issue : 1  |  Page : 47-48

Congenital hypoplasia of lung


1 Department of Tuberculosis and Respiratory Medicine, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, India
2 Department of Community Medicine, Pt. B.D. Sharma, PGIMS, Rohtak, Haryana, India

Date of Web Publication5-Feb-2014

Correspondence Address:
Ruchi Sachdeva
Department of TB and Respiratory Medicine, Pt. B.D. Sharma, PGIMS, Rohtak - 124 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-8775.126515

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How to cite this article:
Sachdeva R, Sachdeva S. Congenital hypoplasia of lung. J Assoc Chest Physicians 2014;2:47-8

How to cite this URL:
Sachdeva R, Sachdeva S. Congenital hypoplasia of lung. J Assoc Chest Physicians [serial online] 2014 [cited 2019 Sep 16];2:47-8. Available from: http://www.jacpjournal.org/text.asp?2014/2/1/47/126515

A 35-year-male smoker admitted in our chest medicine ward with right sided chest pain for 20 days. He had no history of anti-tubercular drug intake or any chronic respiratory diseases. General physical examination was within normal limits. Respiratory system examination revealed smaller right hemi-thorax along with shifting of trachea and heart to right side. Movements were diminished and percussion note was dull over right hemi-thorax. On auscultation, air entry was very poor on right side with diffuse coarse crackles. Sputum for acid-fast bacilli (AFB), pyogenic and fungus samples were negative. Spirometry showed mild restrictive airway disease.

Chest X-ray (PA view) showed homogenous opacity in right lower lobe with signs of volume loss on right side that is rib crowding, tracheal and cardiac shift [Figure 1]. Contrast enhanced computed tomography (CECT) of chest revealed non-visualization of right lung along with mediastinal shift towards right side; right pulmonary artery and vein were also not visualized; right main bronchus was hypoplastic with rudimentary horns and herniation of left lung towards right side [Figure 2]. Fibro-optic bronchoscopy revealed under developed right bronchial tree. Right upper lobe bronchus was seen arising just near the carina and only two small openings were visible; middle and lower lobe bronchus were under developed. Normal development of trachea-bronchial tree of left side was noticed.
Figure 1: Chest X-ray (PA view)

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Figure 2: CECT chest (longitudinal view)

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The incidence of congenital pulmonary hypoplasia may range from 9-11 per 10,000 live births with a very high fatality. [1],[2] This includes both primary and secondary pulmonary hypoplasia. The presentation in adult for the first time is rare and countable cases have been reported. Hoyden classified mal-development into three degrees: (1) Agenesis: Complete absence of lung tissue; (2) Aplasia: Rudimentary bronchus is present but no lung tissue; (3) Hypoplasia: All the normal pulmonary tissues are present but are underdeveloped. The variation in morphological findings depend on cause of hypoplasia and on the time of insult during development. Hypoplastic lungs are smaller with reduction in airway generation to about 50-70% of normal size. The left lung is affected more frequently than the right and the males predominate over females. Poor development of alveolar tissue results in small fibrotic and non-functioning lung. Bronchiectatic changes have also been reported in the hypoplastic lung. There are no clear clinical diagnostic criteria to facilitate identification and management of lung hypoplasia. Treatment of hypoplasia includes medical as well as surgical care, both before and after delivery. Prenatally amino-infusions, antibiotics, and steroids may be given. Oxygen and surfactant administration have been found to improve survival. Adults are treated symptomatically and prophylactic vaccines for respiratory syncitial virus, pneumococcal, and influenza infection are recommended.

 
  References Top

1.Logan JW, Rice HE, Goldberg RN, Cotton CM. Congenital diaphragmatic hernia: A systematic review and summary of best-evidence practice strategies. J Perinatol 2007;27:535-49.  Back to cited text no. 1
    
2.Bush A, Hogg J, Chitty LS. Cystic lung lesions: Prenatal diagnosis and management. Prenat Diagn 2008;28:604-11.  Back to cited text no. 2
    


    Figures

  [Figure 1], [Figure 2]



 

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