The clinical and imaging spectrum of findings in patients with congenital lobar emphysema

Stigers, Kimberly B.; Woodring, John H.; Kanga, Jamshed F.

doi:10.1002/ppul.1950140305

Cited by 87 publications

(35 citation statements)

References 21 publications

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“…Our findings raise the possibility that developmental abnormalities might also contribute to CF lung disease. However, our data indicate that the changes in CF airways are not as severe as those in other congenital lung diseases, such as congenital lobar emphysema, in which approximately 50% of cases are caused by a ball-valve-type defect, airway lumen obstruction, or external airway compression causing airspace enlargement (38,39). The remaining cases have no identifiable cause, suggesting additional etiologies.…”

Section: Discussionmentioning

confidence: 63%

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

Adam¹,

Michalski²,

Bauer³

et al. 2013

Am J Respir Crit Care Med

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Section: Discussionmentioning

confidence: 63%

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

Adam¹,

Michalski²,

Bauer³

et al. 2013

Am J Respir Crit Care Med

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“…Later in life, radiography demonstrates hyperlucent expanded upper or middle lobe, which can cause mediastinal shift and compression of unaffected lobes (38). CT demonstrates hyperexpansion with decreased CT attenuation of the involved lobe, helping to differentiate CLO from CPAM type I or other cystic lung lesions such as pneumatoceles, or persistent pulmonary interstitial emphysema (Fig.…”

Section: Congenital Lobar Overinflationmentioning

confidence: 99%

Broncopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings

Kocaoğlu¹,

Frush²,

Uğurel³

et al. 2008

Diagn Interv Radiol

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Bronchopulmonary foregut malformations are a heterogeneous but interrelated group of abnormalities that may contain more than one histologic feature. Familiarity with the presentation and imaging features of bronchopulmonary foregut malformations presenting as a congenital mass or mass-like lesion is important. Moreover, imaging plays a central role in the evaluation of these lesions since, when symptomatic, clinical features are usually nonspecific. With imaging, the presence of and features of the lesion can be determined, facilitating appropriate management to prevent the potential complications including infection, respiratory compromise and, very rarely, malignancy.

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“…Congenital deficiency of bronchial cartilage is the most common cause of CLE. Less common causes include intraluminal bronchial obstruction, bronchostenosis, bronchial torsion, bronchial mucosa thickening, obstructing mucosal flaps, cartilagineous septae, bronchial atresia, and extrinsic bronchial compression [5].…”

Section: Discussionmentioning

confidence: 99%

An infant with respiratory distress and failure to thrive

Morini

Cozzi

Conforti³

et al. 2002

Eur Respir J

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A 4-month-old male was referred to the Dept of Paediatric Surgery at the authors9 institution, for the investigation of respiratory distress, failure to thrive and complete situs viscerum inversus since birth. A chest radiograph obtained a few hours after birth showed situs viscerum inversus, left apical atelectasis, hyperinflation and hyperlucency of the remaining left lung, and a mild shift of the mediastinum toward the right. The right lung was normal. The infant was born at term, after an uneventful pregnancy, by elective caesarean section. The parents were consanguineous and the baby had a positive maternal family history for situs viscerum inversus and chronic respiratory diseases.On admission, the infant9s general condition was poor, his body weight was 5.490 kg (5th percentile) and he was 62.5 cm in height (10th percentile). Clinical examination disclosed tachypnoea (70 breaths?min -1 ), chest retractions and barrel chest. The arterial oxygen saturation in room air was 90% when the child was awake, reaching 97% with 2 L?min -1 of oxygen. The cardiac frequency was 150 beats?min -1 . On examination of the chest, auscultation revealed diffuse bilateral expiratory wheezing and right posterobasal crackles. The remainder of the examination was compatible with situs viscerum inversus and negative. Cardiac evaluation (electrocardiogram and echocardiogram) was compatible with dextrocardia. Arterial blood gas analysis at room air showed an arterial oxygen tension of 6.8 kPa (51 mmHg) and an arterial carbon dioxide tension of 5.7 kPa (43 mmHg). Red blood cells were found at a level of 5.05610 12 ?L -1 , haemoglobin at 14 g?dL -1 , white blood cells at 12.66 10 9 L -1 (neutrophils 37%, eosinophils 3%, basophils 2%, lymphocytes 51%, and monocytes 7%), and the erythrocyte sedimentation rate at the first hour was 13. C-reactive protein, immune profile, blood chemistry, and sweat test were normal. Microbiological studies for common bacteria, Mycoplasma pneumoniae, Chlamidia trachomatis, respiratory viruses, cytomegalovirus, and Epstein Barr virus, were negative. A chest radiograph was repeated ( fig. 1). Flexible bronchoscopy showed a slightly right-shifted trachea and a normal inverted bronchial tree, without signs of inflammation or mucus plugs. The child also underwent perfusion ( fig. 2) and ventilation lung scintigraphy, with measurement of the ventilation/perfusion ratio (V9/Q9 ratio), and virtual computed tomography (CT) scan ( fig. 3). The V9/Q9 ratio calculated from the lung scan showed a moderate mismatch involving the right upper lung (V9/Q9 ratio: 2.2-2.3), with a normal right lower lobe (V9/Q9 ratio: 0.86-1.08) and left lung (V9/Q9 ratio: 0.85-1.04).

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The clinical and imaging spectrum of findings in patients with congenital lobar emphysema

Cited by 87 publications

References 21 publications

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

Broncopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings

An infant with respiratory distress and failure to thrive

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