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Year : 2015  |  Volume : 6  |  Issue : 2  |  Page : 172-174

Agenesis of left lung in an adult female: A rare case report

Department of Chest Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India

Date of Web Publication13-Jul-2015

Correspondence Address:
Dr. Rupam Kumar Ta
120, Vivekananada Road, Chotonilpur, Sripally, Burdwan - 713 103, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-9727.160708

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Pulmonary agenesis is a rare congenital anomaly presenting usually with recurrent chest infection in early childhood with an annual incidence of 1 out of 100,000 births. Nearly half of the cases were usually associated with congenital co-anomaly of other organ(s). Diagnosis in adulthood is very rare. We reported here an extremely rare case of left lung agenesis in a 19 year girl presenting with dry cough and breathlessness for one month with an abnormal chest x-ray. Diagnosis was made with the aid of contrast enhanced Computed Tomotram [CECT] thorax and fibre-optic bronchoscope. Prognosis depends on affected side, existing lung tissue and other anomalies (if present). However, we managed the case conservatively. We want to add this rare case into the pool of lung agenesis cases report.

Keywords: Agenesis, bronchus, left lung

How to cite this article:
Ta RK, Banerjee SN, Chatterjee K. Agenesis of left lung in an adult female: A rare case report. Muller J Med Sci Res 2015;6:172-4

How to cite this URL:
Ta RK, Banerjee SN, Chatterjee K. Agenesis of left lung in an adult female: A rare case report. Muller J Med Sci Res [serial online] 2015 [cited 2023 Mar 21];6:172-4. Available from: https://www.mjmsr.net/text.asp?2015/6/2/172/160708

  Introduction Top

Pulmonary agenesis is a rare congenital anomaly that can present either isolated or associated with anomalies of other systems. Patients usually present with recurrent chest infection since early childhood. Diagnosis in adulthood is very rare. However, patients with a mild form of this disease or without any comorbid anomalies can reach adulthood. The oldest patient cited by Oyamada et al. [1] was 72 years old. Its annual incidence occurs approximately in 1 out of 100,000 births. [2] We report here a case of pulmonary agenesis diagnosed late into an adulthood and want to add this into the pool of pulmonary agenesis case reports.

  Case Report Top

A 19 year child presented to our out-patient department with dry cough and exertional dyspnoea for last 1 month. Her symptoms were not relieved after initial treatment by local physician. She denied any history of wheeze, palpitation, chest pain, haemoptysis, fever and weight loss or prior hospitalisation. She had uneventful birth history and was immunized properly. Her general survey was unremarkable except for pallor and tachypnoea. Respiratory system examination revealed flattened left hemithorax with its diminished movement, tracheal shift to left and non-localized apex beat. Dull percussion was noted in left hemithorax except left infrascapular area and along left parasternal line where resonant note was elicited alike right side. Breath sound was absent in left hemithorax except left parasternal area and a small left infrascapular area where harsh vesicular breath sound was present like right side. Late inspiratory rales were present in right lung in a scattered manner. Traube's space situated from left 5 th intercoastal space. Other systems were clinically normal.

Routine blood examination with biochemistry was normal except for microcytic hyprochromic red blood cells (RBCs) with haemoglobin level of 9gm/dl. Her sputum smear was negative for any acid fast bacilli [AFB].Culture of sputum grew no Mycobacteria tuberculosis complex. Her chest x-ray showed an opaque homogenous opacity in left lower hemithorax with same sided mediastinal shift and rib crowding [Figure 1]. Left lateral chest x-ray showed increased translucency in retrosternal area additionaly. Contrast enhanced computed tomgram [CECT] scan of thorax revealed absence of left lung with its supplying vasculature and bronchi along and herniation of right lung into left hemithorax and mediastinal shift to left side [Figure 3]. Pulmonary function test [PFT] was of restrictive pattern. Fibre-optic bronchoscopy [FOB] was done and it showed rudimentary left main bronchi narrowed to a slit like opening within 2 cm of the carina but right side was normal [Figure 2]. Ultrasonography of abdomen and echocardiography with doppler study were of normal study. So, a diagnosis of left pulmonary agenesis [Type 2] was made. Patient was treated with parenteral antibiotics for 2 weeks and bronchodilators and oral iron therapy. She improved gradually and now kept under follow up.
Figure 1: Chest xray showing left lower dense opacity with rib crowding and ipsilateral mediastinal shift with signs of right lung hyperinfl ated

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Figure 2: Bronchoscopic view from carina showing blinded rudimentary left main bronchus with normal right main bronchus

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Figure 3: CECT thorax showing absent left main bronchus and left lung and left pulmonary artery and left mediastinal shift and herniation of right lung into left hemithorax

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  Discussion Top

Pulmonary agenesis means undeveloped pulmonary vessels, bronchi and parenchyma. It can be unilateral or bilateral. Depending on the stage of development of the primitive lung bud, pulmonary agenesis is classified into three categories which was later modified by Boyden. [3]

Type 1 (Agenesis) - Complete absence of lung bronchus and no vascular supply to the affected side. Type 2 (Aplasia) - Rudimentary bronchus with complete absence of pulmonary parenchyma. Type 3 (Hypoplasia) - Presence of variable amounts of bronchial tree, pulmonary parenchyma and supporting vasculature. Our patient had Type 2 disease. Its etiology is multifactorial. Genetic, teratogenic and mechanical factors could have played a role. Nearly half of the cases have been associated congenital malformation of the cardio-vascular, skeletal, gastrointestinal or genitor-urinary system. [4] But co-anomalies were absent in our patient. Incidence of lung agenesis occurs nearly in 1 out of 100,000 births. [2] Its incidence are nearly same in males and females. [5] Left sided agenesis is more common and patients live longer than that of right sided agenesis. [1] The onset of symptoms in pulmonary agenesis is variable and begins usually since childhood with history of recurrent chest infections, cardiopulmonary insufficiency or due to associated congenital anomalies. Sometime diagnosis also was suspected from chest x-ray done for other ailments. Although many patients with unilateral lung aplasia (agenesis) are believed to die usually in the neonatal period, there are some patients who survive upto the adulthood, sometime without any symptoms. [6] The oldest patient cited by Oyamada et al. [1] was 72 years old. The average life expectancy of right lung agenesis was reported as 6 years and that of 16 years with left lung agenesis. [7] Our patient had been asymptomatic since birth till this current age of 19 year.

The diagnostic difficulty arises from many reasons. There are no characteristic symptoms or clinical signs of pulmonary agenesis. The clinical picture depends upon the degree of pulmonary abnormality and other congenital coanomalies. Pulmonary aplasia (agenesis) could predispose to chest infections. [6] Chronic respiratory failure and eventually respiratory infection might develop in adults. [8] The chest wall is likely to develop normally and symmetrical. Other authors (Smart, 1946; Hochberg and Naclerio, 1955) [9],[10] have confirmed the symmetry of the two sides in cases of unilateral agenesis. But our patient had flattened left hemithorax. The differential diagnosis of this condition in adults includes lung collapse, thickened pleura, destroyed lung and pneumonectomy, diaphragmatic hernia and lung tumour.

The restrictive pattern in PFT detected in our case was thought to be secondary to the aplasic lung disorder. In chest radiography, mediastinum was deviated to the diseased side and signs of compensatory hyperinflation of right lung. CECT scan thorax revealed rudimentary bronchus and absent parenchymal tissue in the left lung area. Right lung compensated by herniating to the left side. Right pulmonary artery was quite long. Left pulmonary artery did not exist and heart and the main vascular structures moved into left hemithorax. The compensated hyperinflation of right lung explained the harsh vesicular breath sounds on auscultation. In our case, the diagnosis was made with noninvasive methods such as x-ray chest, CECT thorax [11] and FOB. Today's advanced radiological technique has now obviated the need for older invasive process of pulmonary artery angiography. The patient improved conservatively with the use of parenteral antibiotics for 2 weeks along with bronchodilator and haematinic. She is now under follow up. Overall prognosis in these cases depends upon the affected side, functional integrity of the remaining lung tissue as well as the presence of associated anomalies.

  Conclusion Top

We concluded that agenesis of lung might manifest in adult life with chest infection and chest x-ray mimicking lung collapse. High index of suspicion needs to diagnose the case.

  Acknowledgement Top

General support from our departmental staffs for technical and material support and help.

  References Top

Oymada A, Gasul B.M, Holinger P.M. Agenesis of the Lung. Am J Dis Child 1953;85:182-7.  Back to cited text no. 1
Conway K, Gibson RL, Perkins J, Cunningham ML. Pulmonary agenesis: Expansion of the VCFS phenotype. Am J Med Genet 2002;113:89-92.  Back to cited text no. 2
Boyden E. Developmental anomalies. AM J Surg 1955;89:79-88.  Back to cited text no. 3
Razaque MA, Singh S, Singh T. Pulmonary agenesis. Indian J Chest Dis Allied Sci 1980;22:174-8.  Back to cited text no. 4
Nowotny T, Ahrens BC, Bittigau K, Buttenberg S, Hammer H, Kalache KD, et al. Right-sided pulmonary aplasia: Longitudinal lung function studies in two cases and comparison to results from term healthy neonates. Pediatr Pulmonol 1998;26:138-44.   Back to cited text no. 5
Richard S. Pulmonary Abnormalities of Developmental Origin. In: Richard S, editor. Synopsis of Diseases of the Chest. 2 nd ed. Philadelphia: W. B. Saunders Company; 1994. p. 25686.  Back to cited text no. 6
Black PR, Welch KJ. Pulmonary agenesis (aplasia), oesophageal atresia, and tracheoesophageal fistula: A different treatment strategy. J Pediatr Surg 1986;21:936-8.  Back to cited text no. 7
Viora E, Sciarrone A, Bastonero S, Errante G, Campogrande M. Prenatal diagnosis of isolated unilateral pulmonary agenesis in the second trimester. Ultrasound Obstet Gynecol 2002;19:206-7.  Back to cited text no. 8
Smart J. Complete congenital agenesis of a lung. Q J Med 1946;15:125-39.  Back to cited text no. 9
Hochberg LA, Naclerio EA. Congenital pulmonary agenesis. Dis Chest 1955;28:275-81.  Back to cited text no. 10
Schwartz MZ, Ramachandran P. Congenital malformations of the lung and mediastinum - a quarter century of experience from a single institution. J Pediatr Surg 1997;32:44-7.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 Late presentation of unilateral lung agenesis in adulthood
Arshed Hussain Parry,Mujahed Abdulsattar Ibrahim Raheem,Hussam Hassan Ismail,Osama Sharaf
Egyptian Journal of Radiology and Nuclear Medicine. 2021; 52(1)
[Pubmed] | [DOI]


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