Tracheobronchomegaly Associated with Interstitial Pulmonary Fibrosis

The diagnosis of bronchus cardiacus accessorius dexter (BCAD) has occurred in 25 cases during the bronchoscopic investigations of 30,000 adult patients of the authors. In most of the cases, this bronchial anomaly has been revealed as an accessory phenomenon, nevertheless, in one of the patients, it was the source of a considerable hemorrhage. In another case reported here in detail, it occurred together with multiple developmental anomalies, such as tracheobronchomegaly, mitral valve prolapse, pectus excavatum, hypoplasy of sinus frontalis on the right side, inguinal hernia on the left side and hyperlipidemia type IV. Family analysis did not confirm the presence of any chromosomal disorders or accumulation of similar developmental anomalies. The forms and frequency of associations of the anomalies are surveyed on the basis of literary data. The recognition of BCAD is of diagnostic importance, since it may explain the persistence of some bronchopulmonary symptoms; furthermore, the exploration of the associated abnormal vascular branches may be very useful in case of an eventual thoracic surgical intervention.

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“…Its acquired form is very rare [25,26]. Trachea dilatation with pulmonary fibrosis [27] has been observed in singers [21].…”

Section: Discussionmentioning

confidence: 99%

Bronchus Cardiacus Accessorius Dexter

Barzó

Nagy

1998

Diagnostic and Therapeutic Endoscopy

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Rapidly progressing case of Mounier‐Kuhn Syndrome in early childhood

Hubbard

Masters

Chang

2003

Pediatric Pulmonology

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Reports of Mounier-Kuhn Syndrome in childhood are extremely rare, as it usually presents in the third or fourth decades. In the only other report of a case diagnosed in early childhood, the child was well at the time of publication. We report on a 15-month-old boy who presented with his first respiratory illness at age 3 months. His disease course was more severe than previously reported. Diagnosis was characteristically delayed until acute respiratory failure complicated a respiratory tract infection at 13 months, prompting high-resolution computerized tomography (HRCT) of the chest. He gradually deteriorated, eventually requiring ventilatory support; death occurred at age 15 months. This report illustrates the clinical heterogeneity of the syndrome. We review the theories about etiology and the recognized clinical findings in adults. Incidence may be higher than previously estimated, and tracheobronchomegaly (TBM) should be considered as a cause of respiratory failure and recurrent pneumonia in children where other investigations, including chest X-ray (CXR), are normal. A chest HRCT (cHRCT) scan and bronchoscopy are usually diagnostic.

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Development of the lungs; perinatal and developmental lung disease

Corrin

2011

Pathology of the Lungs

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Tracheobronchomegaly Associated with Interstitial Pulmonary Fibrosis

Cited by 5 publications

References 8 publications

Bronchus Cardiacus Accessorius Dexter

Bronchus Cardiacus Accessorius Dexter

Rapidly progressing case of Mounier‐Kuhn Syndrome in early childhood

Development of the lungs; perinatal and developmental lung disease

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