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Year : 2017  |  Volume : 24  |  Issue : 1  |  Page : 99-103

Asymptomatic unilateral pulmonary hypoplasia/agenesis in adults: A report of six cases

1 Department of Radiology, New Fujairah Hospital, Fujairah, United Arab Emirates
2 Department of Medicine, New Fujairah Hospital, Fujairah, United Arab Emirates

Date of Web Publication11-Jan-2017

Correspondence Address:
Funsho Komolafe
Department of Radiology, New Fujairah Hospital, P. O. Box 3292, Fujairah
United Arab Emirates
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1115-3474.198151

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Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral pulmonary hypoplasia or agenesis is rare, the vast majority of cases presenting in the newborn, infancy, or early childhood, with respiratory symptoms. Pulmonary hypoplasia or agenesis presenting in adults is extremely rare, and the initial chest radiographs may present significant interpretation challenges. We report six patients encountered incidentally in a 10-year period, during which approximately 400,000 routine adult chest radiographs were reported. In all six cases, the chest X-ray films were equivocal, and multidetector computed tomography (CT) was required to settle the diagnosis. The cases are presented to draw the attention of radiologists and physicians to this rarity and to avert the kind of initial errors that we made in interpreting their chest radiographs. The invaluable role of multidetector CT in resolving this potential confusion is emphasized. A brief review of the relevant literature is undertaken.

Keywords: Adult chest; chest X-ray; computed tomography; congenital pulmonary anomalies

How to cite this article:
Komolafe F, Dahniya MH, Al-Ali Y, Al-Zaabi L, Aziz F. Asymptomatic unilateral pulmonary hypoplasia/agenesis in adults: A report of six cases. West Afr J Radiol 2017;24:99-103

How to cite this URL:
Komolafe F, Dahniya MH, Al-Ali Y, Al-Zaabi L, Aziz F. Asymptomatic unilateral pulmonary hypoplasia/agenesis in adults: A report of six cases. West Afr J Radiol [serial online] 2017 [cited 2022 Aug 12];24:99-103. Available from: https://www.wajradiology.org/text.asp?2017/24/1/99/198151

  Introduction Top

The chest X-ray film remains the most common radiographic examination requested as a part of routine medical fitness assessment, in particular, to exclude communicable diseases such as pulmonary tuberculosis. We report approximately 150 routine adult chest radiographs daily, performed for preemployment, visa, military service, or school admission purposes.

All cases with equivocal chest X-ray findings were subjected to further imaging including computed tomography (CT).

Of the six cases which turned out to have pulmonary hypoplasia or agenesis, their chest X-ray films mimicked confusing features such as lobar collapse, pleural effusion, hydatid disease, and post tuberculous destroyed lung. Multi-detector CT, with coronal and sagittal reformation, was critical to making the correct diagnosis in each case.

  Case Reports Top

Case 1

A 19-year-old male had a chest X-ray done for visa purposes. It showed a large, rounded mass over the right hemidiaphragm [Case 1 [Additional file 1]] and [Case 2 [Additional file 2]]. In view of the patient's country of origin and being from a sheep-rearing community, the possibility of a right basal pulmonary hydatid cyst was considered. He had no significant past or current health issues.

Contrast-enhanced CT of the chest and abdomen [Case 3 [Additional file 3]]a and b confirmed a large, almost pedunculated, herniation of the liver into the thorax, presumably resulting from a hypoplastic right lower lobe.

Case 2

An asymptomatic 28-year-old male had a preemployment chest X-ray which showed a left pleural opacity with a positive meniscus sign, suggesting pleural effusion [Case 1]. This was accompanied by typical layering on the lateral decubitus film [Case 2], as expected of pleural fluid.

Two repeated attempts at percutaneous diagnostic pleural aspiration yielded no fluid. A subsequent ultrasound study also demonstrated no fluid. CT of the chest and upper abdomen [Case 3] demonstrated replacement of most of the left lung by herniated omentum and intestinal loops, and no pleural fluid. A diagnosis of left lung hypoplasia was made.

Case 3

A 13-year-old girl had a routine pre-high school chest X-ray. The film showed opacification of the right base with obliteration of the right hemidiaphragm and slight shift of the heart to the right [Case 1]. The features suggested right lower lobe collapse/consolidation, but she had no symptoms and demonstrated no clinical signs.

CT of the chest showed elevation of the liver into the right hemithorax and cardiac shift to the right [Case 2]. There was no collapse or consolidation. The changes were considered as due to the right lower lobe agenesis or segmental hypoplasia.

Case 4

A 25-year-old male had a preemployment chest X-ray which showed a poorly defined, elevated right hemidiaphragm and cardiac shift to the right [Case 1]. A right lower lobe collapse was considered a possibility although the patient exhibited neither symptoms nor clinical signs.

A CT study [Case 2] and [Case 3] revealed that the liver dome was deformed and elevated into the lower chest. The gallbladder was malpositioned. Since there was no history of previous chest trauma or significant chest disease, the changes were considered due to segmental right pulmonary hypoplasia.

Case 5

A 28-year-old female had a preemployment chest X-ray. The chest film [Case 1] showed a marked mediastinal shift to the left and a largely opaque hemithorax. Although a destroyed left lung from old TB was considered, the patient denied any history of significant chest disease.

Chest CT showed a hypoplastic left bronchus and lung [Case 2]. The right lung showed compensatory hypertrophy, extending across the midline and occupying most of the left hemithorax.

Case 6

A 32-year-old male had a preemployment chest X-ray which showed a largely opaque left hemi thorax, with severe deviation of the mediastinal structures to the left [Case 1]. He denied any significant chest symptoms.

CT of the chest showed the complete absence of the left lung, with compensatory hypertrophy of the right lung, extending to occupy the left hemithorax [Case 2] and [Case 3]. The left bronchus was completely absent.

An anomalous right upper lobe bronchus was noted arising directly from the trachea, high above the level of the carina [Case 3]. The left pulmonary artery was also completely absent [Case 4 [Additional file 4]]. Features were considered as those of complete left pulmonary agenesis. The cases are summarized in [Table 1].{Table 1}

  Discussion Top

The incidence of pulmonary hypoplasia is estimated as approximately 1 in 15,000 live births, with no gender prevalence. Most cases present in neonates, infants, and childhood, but a few cases may remain asymptomatic until adolescence and adulthood and are discovered incidentally. [1],[2],[3],[4]

Pulmonary underdevelopment represents a broad spectrum of abnormalities which are classified as:

  1. Pulmonary agenesis (complete absence of the lung, bronchus, and pulmonary artery)
  2. Pulmonary aplasia (rudimentary blind-ending bronchus, but absent lung and pulmonary artery)
  3. Pulmonary hypoplasia (hypoplastic bronchus with variable lung tissue reduction). [5],[6],[7]

When symptoms occur, they are usually related to the severity of the hypoplasia and may present as recurrent episodes of wheezing or pneumonia, as the underdeveloped lung is more susceptible to bacterial or viral proliferation. The function of the other lung which has undergone compensatory hypertrophy may be seriously impaired by pneumothorax or pulmonary hypertension. [6],[7]

The diagnosis of pulmonary hypoplasia may be difficult on the regular chest radiograph. Two of our cases were wrongly diagnosed as lower lobe collapse, an experience previously reported. [6]

Pulmonary hypoplasia is well known to be associated with diaphragmatic hernia. [7] This was present in two of our cases (Cases 1 and 2). An elevated liver dome with diaphragmatic elevation and ipsilateral cardiac shift was observed in two other cases (Cases 3 and 4).

It is difficult to ascertain if pulmonary hypoplasia is a consequence of the restriction of normal pulmonary development by the presence of a hernia, or if the hernia and mediastinal shift observed are secondary reactions to a reduced volume of the hemithorax engendered by pulmonary hypoplasia.

Contrast-enhanced CT remains the best modality for the evaluation of pulmonary hypoplasia because of its capacity for multiplanar reformation and the excellent demonstration of the vascular system. [8],[9],[10]

Intravenous contrast was not administered in some of our cases because in the context of routine chest examination and the absence of symptoms, once the diagnosis was established on plain CT, contrast administration was considered superfluous and an additional cost.

Apart from CT, other ancillary procedures such as bronchoscopy, bronchography, ventilation- perfusion scintigraphy, and magnetic resonance imaging have also been employed in the evaluation of pulmonary hypoplasia. [9],[10],[11]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Albay S, Cankal F, Tunali S, Ozan H. Unilateral pulmonary hypoplasia. Int J Anat Var 2008;1:23-5.  Back to cited text no. 1
Thomas RJ, Lathif HC, Sen S, Zachariah N, Chacko J. Varied presentations of unilateral lung hypoplasia and agenesis: A report of four cases. Pediatr Surg Int 1998;14:94-5.  Back to cited text no. 2
Pathania M, Lali BS, Rathaur VK. Unilateral pulmonary hypoplasia: A rare clinical presentation. BMJ Case Rep 2013;2013. pii: bcr2012008098.  Back to cited text no. 3
Gulen F, Kar S, Midyat L, Demir E, Ozyurt S, Ozyurek A, et al. A rare clinical presentation of pulmonary hypoplasia. Open J Pediatr 2011;1:75-8.  Back to cited text no. 4
Katsenos S, Antonogiannaki EM, Tsintiris K. Unilateral primary lung hypoplasia diagnosed in adulthood. Respir Care 2014;59:e47-50.  Back to cited text no. 5
Zylak CJ, Eyler WR, Spizarny DL, Stone CH. Developmental lung anomalies in the adult: Radiologic-pathologic correlation. Radiographics 2002;22:S25-43.  Back to cited text no. 6
Comet R, Mirapeix RM, Marín A, Castañer E, Sans J, Domingo C. Pulmonary hypoplasia in adults: Embryology, clinical presentation and diagnostic methods. Our experience and review of the literature. Arch Bronconeumol 1998;34:48-51.  Back to cited text no. 7
Lee EY, Boiselle PM, Cleveland RH. Multidetector CT evaluation of congenital lung anomalies. Radiology 2008;247:632-48.  Back to cited text no. 8
Kurkcuoglu IC, Eroglu A, Karaoglanoglu N, Polat P. Pulmonary hypoplasia in a 52-year-old woman. Ann Thorac Surg 2005;79:689-91.  Back to cited text no. 9
Berrocal T, Madrid C, Novo S, Gutiérrez J, Arjonilla A, Gómez-León N. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: Embryology, radiology, and pathology. Radiographics 2004;24:e17.  Back to cited text no. 10
Georgescu A, Nuta C, Bondari S. 3D imaging in unilateral primary pulmonary hypoplasia in an adult: A case report. Case Rep Radiol 2011;2011:659586.  Back to cited text no. 11


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