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Year : 2016  |  Volume : 2  |  Issue : 1  |  Page : 22-24

A Rare Case of Right Lung Agenesis with Dextrocardia: An Unusual Cause of Breathlessness

1 Department of T. B. & Chest, IGIMS, Patna, Bihar, India
2 Department of Radiodiagnosis, IGIMS, Patna, Bihar, India
3 Department of Cardiology, IGIMS, Patna, Bihar, India
4 Department of General Medicine, IGIMS, Patna, Bihar, India

Date of Web Publication12-Feb-2016

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Agenesis of lung, a rare congenital anomaly, defined as complete absence of bronchus, parenchyma, and vessels which may present in adult life with features of recurrent chest infections and chest X-Ray may present as opaque hemithorax with ipsilateral shifting of mediastinum. Here, a case of an adolescent presenting with frequent attacks of cough, expectoration and dyspnea since childhood, proved to be a case of right pulmonary agenesis along with dextrocardia is to be discussed.

Keywords: Recurrent chest infections, Opaque hemithorax, Agenesis, congenital anomaly

How to cite this article:
Shankar M, Kumari M, kumar N, Kumar G. A Rare Case of Right Lung Agenesis with Dextrocardia: An Unusual Cause of Breathlessness. J Indira Gandhi Inst Med Sci 2016;2:22-4

How to cite this URL:
Shankar M, Kumari M, kumar N, Kumar G. A Rare Case of Right Lung Agenesis with Dextrocardia: An Unusual Cause of Breathlessness. J Indira Gandhi Inst Med Sci [serial online] 2016 [cited 2022 Oct 2];2:22-4. Available from: http://www.jigims.co.in/text.asp?2016/2/1/22/303363

  Introduction: Top

An extremely rare congenital anomaly i;e Agenesis of the lung which represents failure of development of the primitive lung bud. The term ‘agenesis’ is taken to mean almost complete absence of growth in the lung[1]. The rarity of this condition is evident by the infrequent reporting of such cases in literature with prevalence of 34 per million live births.[2] Unilateral agenesis of the lung is less rare and may present with varying degrees of severity.

The condition was first discovered at the autopsy of an adult female in 1673, by De Pozze[3]. From India, the first case was reported by Muhamed4in 1923, of a left sided pulmonary agenesis.

Here we report a case of an adolescent with right pulmonary agenesis with dextrocardia

  Case Report: Top

A 15 year old male came in the OPD with the complain of recurrent cough, fever and shortness of breath since childhood. There was no history of orthopnea, palpitation, wheezing, chest pain, hemoptysis, anorexia and weight loss. He had no past history of pulmonary tuberculosis. He was a non-smoker. His perinatal history was insignificant. On examination, he was a thin built male, moderately nourished and tachypnic. Pallor, icterus, clubbing, engorged neck veins and lymphadenopathy were absent. On inspection of chest, accessory muscles of respiration were working, drooping of shoulder seen in right side. On palpation, chest movement was diminished in right side with rib crowding, trachea deviated to right and apex beat placed at right 4th intercostal space with normal heart sound. Vocal fremitus diminished throughout the right side. On percussion, right side had impaired note 5th ICS downward along mid axillary line and scapular line. On auscultation, reduced vocal resonance on right side, Breath sounds were absent over most of the right lung field. Other systems were within normal limits. Routine examination of blood, and sputum for AFB two consecutive samples were non-contributory. Chest radiograph [Figure 1] showed homogenous opacity in the right lower zone, obliterating the right costophrenic angle with gross shifting of trachea and mediastinal structures to the affected side. His FVC was 1380cc (73% of predicted), FEV1 1240cc (78% of predicted), and FEV1/FVC 106% of predicted. PEFR was 2280cc (56% of predicted). ECG showed right axis deviation. Echo Doppler study revealed situs solitus, dextrocardia. Contrast enhanced computed tomogram [Figure 2] and [Figure 3] showed absence of right lung, right main bronchus and right pulmonary artery and herniation of left lung to the right. CT pulmonary angiography could not be done. Fibreoptic bronchoscopy could not be done as it was refused by the patient. Skiagram of skeletal system and ultrasonography abdomen were carried out in order to detect any other congenital defect, but none was found. He was diagnosed as right sided agenesis of lung with right pulmonary artery atresia and dextrocardia.
Figure 1: Chest X-Ray shows opaque right hemi thorax associated with ipsilateral shift of heart and mediastinum.

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Figure 2: CECT Thorax (axial view):non-visualization of right lung and shifting of heart towards right. Main pulmonary artery is continued as left pulmonary artery. Right pulmonary artery is absent with herniation of left lung (compensatory hypertrophied) towards right.

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Figure 3: CECT Thorax (coronal view): shows the presence of trachea and is continued further as left main bronchus with absent right bronchus. Main pulmonary trunk is also continued as left pulmonary artery with absent right pulmonary artery. Herniated portion of left lung to the right (marked by arrow).

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  Discussion: Top

Many cases of pulmonary agenesis, aplasia and hypoplasia have been reported at different ages prenatally in newborns, infants, children and adults.[5] Lung agenesis or aplasia is very rare. Complete absence of bronchus, parenchyma and vessels is defined as Pulmonary agenesis., No pleural cavity can be found on the affected side in case of complete unilateral agenesis. Whole of the thorax is occupied by heart and opposite lung. Mediastinal shift with heart rotation and kinking and compression of trachea by the displaced aortic arch and truncus arteriosus may be present.[6] The right lung is affected less frequently than the left, and patients with aplasia of left lung have shown better prognosis. Bilateral pulmonary aplasia or agenesis does not support life. Dextrocardia with situs solitus and normally related great arteries without intracardiac malformation may be present, secondary to agenesis or hypoplasia of right lung.[6] The most of cases exhibit other congenital abnormalities like patent ductus arteriosus, pulmonary artery atresia, cardiac malformation, and horse-shoe kidney.

Schneider7classified agenesis into three groups, which has been subsequently modified by Boyden[8]. Depending upon the stage of development of the primitive lung bud, pulmonary agenesis is classified into three categories:

Type l(Agenesis), Type 2 (Aplasia), Type 3 (Hypoplasia)

Our patients would classify as Type 1 pulmonary agenesis.

Perhaps due to the failure of the bronchial analogue to divide equally between the two lung buds one side develops normally while the other fail completely (agenesis / aplasia) or partially (dysplasia or hypoplasia). Respiratory difficulty with tracheal deviation is a clue to this condition , with clinically symmetric chest and Chest X-ray suggestive of massive atelectasis with mediastinal shift. Pulmonary agenesis presents with variable symptoms. This anomaly usually presents during infancy because of recurrent chest infections, cardiopulmonary insufficiency or due to associated congenital anomalies. Nearly 50% cases of pulmonary agenesis have associated congenital defects[9]. Our patient had no associated congenital defect. However, patients with one lung can survive well into adulthood without much complaint. Our patients had recurrent respiratory infections since childhood. Diagnosis was suspected from routine radiological examination of chest, where possibilities of atelectasis or agenesis of the right lung were considered. The diagnosis was confirmed by CT scan thorax. The sole lung is larger than normal and this enlargement is true hypertrophy.

Left sided agenesis is more common and have a longer life expectancy as compared to right sided agenesis. Right sided agenesis can lead to excessive mediastinal shift and malrotation of carina which impaires proper drainage of the functioning lung and increases chances of respiratory infections. However, some reports have shown patients with right lung agenesis living upto old age because of absence of critical anomalies in other organs.[10] Patients usually present with recurrent respiratory infections, cough, tachypnea, strider, wheezing, and cyanosis from an early age. CT Chest is considered to be the most definitive investigation to diagnose agenesis as chest radiograph is not diagnostic. The characteristic CT findings are opaque hemithorax with mediastinal shift toward the affected side and symmetrical bony cage. Pulmonary CT angiography or MRI Angiography shows the absence of ipsilateral pulmonary vessel. In our case these could not be done as the patient could not afford them. Surgery is rarely required for agenesis or aplasia. Functional integrity of the remaining lung as well as the presence of associated anomalies decides the prognosis. Asymptomatic cases requires no treatment. Treatment is necessary for lower respiratory tract infections. The severity of associated congenital anomalies and involvement of the normal lung in any disease process decides the prognosis. Survival for the first five years without major infection can lead to an almost normal life span.

  Conclusion: Top

Congenital pulmonary agenesis along with dextrocardia is an extremely rare anomaly which may present acutely with severe respiratory distress and may need urgent intervention. Invasive diagnostic procedures and prophylactic surgery are not required in asymptomatic patients however close follow-up is needed.

  References Top

Spencer H. Pathology of the lung. Oxford: Pergamon Press; 1985.  Back to cited text no. 1
Mardini MK, Nyhan WL. Agenesis of the lung: Report of four patients with unusual anomalies. Chest. 1985; 87:522-7.  Back to cited text no. 2
De Pozze, S. cited by Brescia M.A., Amermon E.E., Sharma K.K. (1960). Agenesis of the Left Lung. Arch. Paediatric, 77,485-490  Back to cited text no. 3
Muhamed, K.S.N. (1923). Absence of left lung. Ind. Med. Gaz. 58. 262-64.  Back to cited text no. 4
Holstein A, Weber M. An extraordinary finding- accidental diagnosis of complete pulmonary aplasia in a 90 yr old lady. Age and Aging 2009;38:487  Back to cited text no. 5
Dohlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: Airway obstruction and its release by aortopexy. J Pediatr Surg 1990; 25:290-3.  Back to cited text no. 6
Schneider P, Schwatbe E. Part 2. Die Morphologie der Missbildungen Des Menschen Under Thiere, vol. 3. Jena: Gustav Fischar; 1912. 817e822.  Back to cited text no. 7
Boyden E. Developmental anomalies of lung. Am J Surg 1955;89:79e88  Back to cited text no. 8
Sbokos C.B. and McMillan I.R.K. (1977). Agenesis of the lung. Brit J Dis Chest, 3,183-197  Back to cited text no. 9
Ueda T, Nozoe M, Nakamoto Y, Irie Y, Mizushige K. Right pulmonary agenesis in an elderly woman complicated by transient ischemic attack. Intern Med. 2011; 50:463-6.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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