Annals of Thoracic Medicine Official publication of the Saudi Thoracic Society, affiliated to King Saud University
 
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Year : 2006  |  Volume : 1  |  Issue : 1  |  Page : 26-27
A young woman with an opacity on the left hemithorax


Department of Medicine, Security Forces Hospital, Riyadh, Saudi Arabia

Correspondence Address:
Mohammed Alanezi
Department of Medicine, Security Forces Hospital, Riyadh
Saudi Arabia
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DOI: 10.4103/1817-1737.25867

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How to cite this article:
Alanezi M, Al-Fifi M. A young woman with an opacity on the left hemithorax. Ann Thorac Med 2006;1:26-7

How to cite this URL:
Alanezi M, Al-Fifi M. A young woman with an opacity on the left hemithorax. Ann Thorac Med [serial online] 2006 [cited 2014 Nov 25];1:26-7. Available from: http://www.thoracicmedicine.org/text.asp?2006/1/1/25/25867


A 30-year-old, previously healthy, Saudi woman presented to the emergency department in our hospital, with a one-year history of gradually progressive shortness of breath. She denied history of cough, hemoptysis, chest pain, or wheezing.

On examination, she was looking well with normal vital signs. There was no cyanosis, clubbing, lymphadenopathy, or edema. There was decreased chest expansion of the left hemithorax. The trachea and the heart were shifted to the left. The left hemithorax was dull to percussion. Breath sounds were absent on the left side. The right hemithorax was normal. Cardiovascular, abdomen, musculoskeletal and neurology systems were normal.

Chest radiograph, selected CT scan and bronchoscopic images, are seen in the following [Figure - 1],[Figure - 2],[Figure - 3].

1. What does the chest radiograph and CT show?

2. What are the findings on the bronchoscopic image suggest?

3. What would be the likely differential diagnosis?

What is your diagnosis?

Answers

[Figure - 1]: Chest radiograph showing diffuse opacity in the left hemithorax with ipsilateral shift of the heart.

[Figure - 2] a and b: Contrast enhanced computed tomogram of the chest showing a small left main bronchus, the trachea and the mediastinum is completely shifted towards the left side with the absence of the left lung

[Figure - 3]: Fiberoptic bronchoscopy showed a short left main bronchus ending suddenly in a rudimentary bud.

Diagnosis

Unilateral pulmonary agenesis.


   Discussion Top


Pulmonary agenesis is an very rare congenital anomaly of the respiratory system, representing failure of development of the primitive lung bud.

This condition was first reported by De Pozze,[1] who discovered it accidentally at the autopsy of an adult female in 1673. Munch Meyer,[2] first diagnosed unilateral agenesis of the lung, clinically in 1885.

It can be seen alone, or may be associated with other cardiac, abdominal, or urological anomalies.[3] An abdominal ultrasound and an echocardiography of the heart were performed for our patient both of which were normal.

Pulmonary agenesis is classified into three types:

a. Agenesis-Complete absence of lung and bronchus and no vascular supply to the affected side.

b. Aplasia-Rudimentary bronchus with complete absence of pulmonary parenchyma.

c. Hypoplasia-Presence of variable amounts of bronchial tree, pulmonary parenchyma and supporting vasculature.

However, Spencer divided pulmonary agenesis into: (1) bilateral complete agenesis, (2) unilateral agenesis with (a) Complete absence of bronchi, (b) rudimentary bronchus present but no pulmonary tissue, or (c) Poorly developed main bronchus with poorly organized parenchyma and (3) lobar agenesis.[3]

Males predominate over females, left sided agenesis is more common and the subjects have a longer life expectancy than those with right-sided agenesis. This is may be due to excessive mediastinal shift and malrotation of carina in right-sided agenesis, that hinders proper drainage of the functioning lung and increases chances of respiratory infections. Parental consanguinity through an autosomal recessive mode of inheritance, may be a predisposing factor.[4] The incidence of this condition, though not exactly known, ranges between 0.0034 and 0.0097 percent.[5]

Most patients suffer from recurrent chest infections during childhood and about half of the affected individuals, die within the first five years of life. However, as in this case, many patients remain asymptomatic until adulthood and may survive up to old age. In contrast, bilateral pulmonary agenesis is clearly incompatible with life.

They are often misdiagnosed for the more common diseases affecting the lung and a high index of suspicion is needed to make an early diagnosis. Many patients with congenital lung diseases present during adult life, when a clinical diagnosis becomes more difficult. Consequently, those patients are misdiagnosed as collapsed, thickened pleura, destroyed lung and pneumonectomy. Other differential diagnoses include adenomatoid cystic malformations, diaphragmatic hernia and sequestrations.

In conclusion, this condition must be suspected in differential diagnosis, in an adult patient presenting with features of lung collapse.

 
   References Top

1.De Pozze S, Brescia MA, Amermon EE, Sharma KK. Agenesis of the Left Lung. Arch Paediatr 1960;77:485-90.  Back to cited text no. 1    
2.Munch, Meyer, Ferguson FF, Meuhauser EB. Congenital absence of lung and other anomalies of tracheo bronchial tree. Am J Reontgen 1944;52:459-71.  Back to cited text no. 2    
3.Gould SJ, Hasleton PS. Congenital abnormalities. In : Hasleton PS, ed Spencer's Pathology of the Lung; 5th ed. McGraw-Hill: New York; 1996. p. 57-114.  Back to cited text no. 3    
4.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. 4  [PUBMED]  
5.Oymada A, Gasul BM, Holinger PM. Agenesis of the fung. Am J Dis Child 1953;85:182-7.  Back to cited text no. 5    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]

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