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Year : 2013  |  Volume : 16  |  Issue : 2  |  Page : 151-152
Agenesis of the lung


Department of Anaesthesia and Critical Care Medicine, Fortis Escorts Heart Institute, New Delhi, India

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Date of Web Publication29-Mar-2013
 

How to cite this article:
Dutta D, Karlekar A, Saxena R. Agenesis of the lung. Ann Card Anaesth 2013;16:151-2

How to cite this URL:
Dutta D, Karlekar A, Saxena R. Agenesis of the lung. Ann Card Anaesth [serial online] 2013 [cited 2019 Dec 13];16:151-2. Available from: http://www.annals.in/text.asp?2013/16/2/151/109775


A 32-year-old female patient presented for surgical decompression of Carpal tunnel syndrome. The patient gave history of being a diagnosed case of congenital absence of left lung. The chest X-ray showed homogenous opacity in the left hemithorax with tracheal deviation to the left and herniation of the hyper-inflated right lung across the mediastinum [Figure 1]. Her oxygen saturation on room air was 96%. The patient denied history of breathing difficulty. On clinical examination, the breath sounds were absent on the left side of chest. She underwent Carpal tunnel decompression under brachial plexus block. Post-operative course was uneventful.
Figure 1: Chest X-ray showing total opacity of the left hemithorax; tracheal shift to the left (dotted arrow). The right lung is hyperinflated and there is a marked herniation of the right lung across the mediastinum (solid arrows)

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Agenesis of the lung represents failure of development of the primitive lung bud. This condition was first described by Pozze, et al., [1] they discovered it accidently at the autopsy of an adult female in 1673. Schneider and Schawatbe [2] classified agenesis into three groups, which was subsequently modified by Boyden [3] depending upon the stage of development of the primitive lung bud. The pulmonary agenesis is classified into three categories: (1) Agenesis - complete absence of lung and bronchus and no vascular supply to the affected side. (2) Aplasia - rudimentary bronchus with complete absence of pulmonary parenchyma. (3) Hypoplasia - presence of variable amounts of bronchial tree, pulmonary parenchyma, and supporting vasculature.

The agenesis of the lung is an uncommon, but not a rare congenital anomaly. The key question is - how should an anesthesiologist proceed to plan anesthesia in presence of agenesis of lung? During preanesthetic evaluation, diagnosis should be suspected/confirmed on the chest X-ray. In case of doubt, the diagnosis can be confirmed by bronchography or pulmonary angiography. Imaging technique like CT scan, and three-dimensional reconstruction helical chest CT [4] are equally helpful in the diagnosis and classification of the agenesis. Differential diagnosis of lung agenesis includes collapse, destroyed lung, post-pneumonectomy presentation, and thickened pleura. Clinical findings include asymmetry and flattening of the affected side, scar of pneumonectomy and lagging on inspiration; however, the shape and movements of the chest may be normal. Agenesis by itself does not give rise to symptoms unless complicated by broncho-pulmonary disease. Many patients, like the present one, remain asymptomatic and the diagnosis may not be made until adulthood. [5],[6] Asymptomatic patients can be diagnosed by accidental finding on a routine chest roentgenogram or by absence of breath sounds during routine clinical examination. Symptomatic patients may present with cough, wheezing, respiratory distress, and occasionally cyanosis. Agenesis of lung occurs more often on the left side than the right. [7] Right-sided pulmonary agenesis have a graver prognosis than that of the left, the mortality is observed more often and earlier in the patients with right-sided agenesis of lung. [8] A poorer prognosis for the right-sided defect could be due to a higher incidence of associated cardiac abnormalities [9] or to the greater mediastinal shift produced by right-sided agenesis, leading to more significant distortion of the bronchial and vascular structures. Majority of reported cases of pulmonary agenesis have associated congenital defects, involving cardiovascular, skeletal, gastrointestinal, and genitourinary systems. Anesthesiologist should look for these associated anomalies which if present could be a major cause of morbidity and mortality. [10] Presences of associated anomalies as well as functional integrity of the remaining lung decide the prognosis in these cases.

In conclusion, agenesis of the lung is an uncommon congenital anomaly. Asymptomatic patients are generally well compensated and do not pose any special challenges during conduct of anesthesia. However, the finding of absent lung should warrant careful assessment of functional status of the patient, the class of agenesis because hypoplastic lung is prone for atelectasis and pulmonary infection and associated congenital anomalies. Awareness about such a condition helps anesthesiologists to equip themselves better for safe conduct of an anesthetic.

 
   References Top

1.Pozze D, Brescia MA, Amermon EE, Sharma KK. Agenesis of the Left Lung. Arch Paediatric 1960;77:485-90.  Back to cited text no. 1
    
2.Schneider P, Schwalbe E. Die morphologie der missbildungen des menschen under thiere. Jena: Fischer 1912;3:812-22.  Back to cited text no. 2
    
3.Boyden EA. Developmental anomalies of the lungs. Am J Surg 1955;89:79-89.  Back to cited text no. 3
    
4.Wu CT, Chen MR, Shih SL, Huang FY, Hou SH. Case report: Agenesis of the right lung diagnosed by three-dimensional reconstruction of helical chest CT. Br J Radiol 1996; 69:1052-4.  Back to cited text no. 4
    
5.Shenoy SS, Culver GJ, Pirson HS. Agenesis of lung in an adult. AJR Am J Roentgenol 1979; 133:755-7.  Back to cited text no. 5
    
6.Kisku KH, Panigrahi MK, Sudhakar R, Nagarajan A, Ravikumar R, Daniel JR. Agenesis of lung-a report of two cases. Lung India 2008; 25:28-30.  Back to cited text no. 6
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7.Valle AR. Agenesis of the lung. Am J Surg 1955;89:90-100.  Back to cited text no. 7
    
8.Schaffer AJ, Rider RV. A note on the prognosis of pulmonary agenesis and hypoplasia according to the side affected. J Thorac Surg 1957;33:379-82.  Back to cited text no. 8
    
9.Thurlbeck WM. Postnatal growth and development of the lung. Am Rev Respir Dis 1975; 111:803-44.  Back to cited text no. 9
    
10.Sbokos CG, McMillan IK. Agenesis of the lung. Br J Dis Chest 1977;71:183-97.  Back to cited text no. 10
    

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Correspondence Address:
Devesh Dutta
Department of Anaesthesia and Critical Care Medicine, Fortis Escorts Heart Institute, New Delhi - 110 025
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.109775

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This article has been cited by
1 Unilateral Agenesis of the Lung: A Rare Entity
Muralidharan Ramaraj
American Journal of Case Reports. 2015; 16: 69
[Pubmed] | [DOI]



 

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