Year : 2013  |  Volume : 16  |  Issue : 2  |  Page : 151--152

Agenesis of the lung


Devesh Dutta, Anil Karlekar, Ravindra Saxena 
 Department of Anaesthesia and Critical Care Medicine, Fortis Escorts Heart Institute, New Delhi, India

Correspondence Address:
Devesh Dutta
Department of Anaesthesia and Critical Care Medicine, Fortis Escorts Heart Institute, New Delhi - 110 025
India




How to cite this article:
Dutta D, Karlekar A, Saxena R. Agenesis of the lung.Ann Card Anaesth 2013;16:151-152


How to cite this URL:
Dutta D, Karlekar A, Saxena R. Agenesis of the lung. Ann Card Anaesth [serial online] 2013 [cited 2020 Aug 9 ];16:151-152
Available from: http://www.annals.in/text.asp?2013/16/2/151/109775


Full Text

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}

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

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