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To highlight the clinical importance of inflammatory myofibroblastic tumours of the respiratory tract in children, and to present a case series of three children which illustrates this tumour's variable clinical presentation.
Case history:
The series includes: a nine-year-old girl with a diagnosis of juvenile idiopathic arthritis, who presented with finger clubbing and was found to have an inflammatory myofibroblastic tumour in her right upper lobe; a 15-year-old adolescent with a left main stem bronchial inflammatory myofibroblastic tumour, who presented with breathlessness and chest pain; and a 12-year-old girl with a tracheal inflammatory myofibroblastic tumour who presented with stridor. In each case, the tumour was resected surgically.
Conclusion:
Inflammatory myofibroblastic tumour are a rare but clinically important and pathologically distinct lesion of the respiratory tract in children. The cases in this series highlight some of the varied clinical presentations of inflammatory myofibroblastic tumours, and illustrate some of this tumour's different anatomical locations within the paediatric respiratory tract.
Stridor is usually produced by obstruction in the upper airways. We present a case of stridor referred to the ENT Department in whom an endoscopic examination as far as the lower trachea showed no abnormality. A subsequent bronchoscopy in the Chest Department revealed a tumour in the right main bronchus.
Juvenile respiratory papillomatosis involving the tracheo-bronchial tree imposes a significant management problem and is sometimes life threatening. The mainstay of treatment is repeated vapourization with a CO2 laser. To date, adjunctive medical treatments have been of limited value. A tracheostomized child with extensive laryngo-tracheo-bronchial papillomatosis who has required bronchoscopic lasering at two-weekly intervals for three years was treated with ribavirin, a broad spectrum anti-viral agent. The drug was administered in nebulized form using a small particle aerosol generator (S.P. A.G.) to the lower respiratory tract (6 gm/150 ml over nine hours) on three consecutive nights every two weeks over seven weeks and also administered orally (15 mg/kg/day). Endoscopic assessments were made every two weeks. At 14 days the papillomata were regressing and far less lasering was required. No further lasering was required up to 56 days. One month after stopping the ribavirin, however, a few sessile papillomata in the tracheo-bronchial tree had recurred and were treated with the laser. No adverse reactions were encountered. During the treatment period there was a significant reduction in the frequency of therapeutic endoscopies. This promising response requires further evaluation to define the role of ribavirin in the treatment of juvenile respiratory papillomatosis.
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