2016
DOI: 10.1155/2016/4397641
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Right Lung Agenesis with Tracheal Stenosis due to Complete Tracheal Rings and Postpneumonectomy Like Syndrome Treated with Tissue Expander Placement

Abstract: Congenital lung agenesis is an extremely rare condition with an estimated prevalence of 34 in 1,000,000 live births. It is often associated with other congenital malformations of the skeletal, cardiovascular, urogenital, and gastrointestinal systems. We discuss the case of a 5-month-old who presented with increasing stridor over 1 month. Imaging revealed right lung agenesis, complete dextromalposition of heart, and compression of distal trachea. An intrathoracic saline tissue expander was placed which marked i… Show more

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Cited by 9 publications
(14 citation statements)
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“…Two cases involved patients with malignancy, one with long-standing history of Hodgkin’s lymphoma involving the left lung and mediastinum [18] and one with lung cancer [6]. Another case involved a patient with destruction of the right lung due to multiple episodes of pneumonia [19] Several cases of postpneumonectomy-like syndrome due to congenital lung agenesis or hypoplasia have been described [5, 20, 21]. Among seventy-three cases of postpneumonectomy syndrome in adults reported in the literature, the median interval between pneumonectomy and onset of symptoms was 2 years (range 1 month—49 years) [14].…”
Section: Discussionmentioning
confidence: 99%
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“…Two cases involved patients with malignancy, one with long-standing history of Hodgkin’s lymphoma involving the left lung and mediastinum [18] and one with lung cancer [6]. Another case involved a patient with destruction of the right lung due to multiple episodes of pneumonia [19] Several cases of postpneumonectomy-like syndrome due to congenital lung agenesis or hypoplasia have been described [5, 20, 21]. Among seventy-three cases of postpneumonectomy syndrome in adults reported in the literature, the median interval between pneumonectomy and onset of symptoms was 2 years (range 1 month—49 years) [14].…”
Section: Discussionmentioning
confidence: 99%
“…Radiologic findings are usually related to bronchial obstruction and include associated atelectasis, air trapping, obstructing pneumonia and mucoid impaction [4]. Postpneumonectomy-like syndrome is a rare condition caused by either destructive or neoplastic unilateral lung disease or by congenital lung agenesis or hypoplasia, resulting in herniation of the normal lung into the diseased hemithorax with compensatory hyperinflation and air trapping in that lung [5]. Its pathogenic mechanism and treatment are analogous to those of postpneumonectomy syndrome, but without previous lung resection [6].…”
Section: Introductionmentioning
confidence: 99%
“…Most patients with right lung agenesis suffer from airway problems as a result of tracheal or bronchial compression. 2 translocation or the application of tissue expander in the empty pleural cavity), we selected aortopexy and near total thymectomy to relieve tracheal compression, as suggested by Dohlemann et al [1][2][3] As the use of tissue expanders requires some time to fill the pleural cavity, we would not have achieved a rapid response for our patient. We did not consider the use of rapid expansion of a tissue expander for fast weaning from the ventilator to be appropriate because of the risk of haemodynamical instability.…”
Section: Discussionmentioning
confidence: 99%
“…Congenital single lung (CSL) is a rare condition, affecting about 1–3.4 per 100,000 live births [ 1 ]. Although CSL can be unilateral or bilateral, right-sided CSL is more likely to have mediastinal shift causing compression of vascular structures or airway, causing recurrent respiratory infection or respiratory distress during childhood [ 2 ].…”
Section: Introductionmentioning
confidence: 99%