9yo

Please see http://www.medhelp.org/perl6/RespiratoryDisorders/messages/670a.html and http://www.medhelp.org/perl6/RespiratoryDisorders/messages/691a.html for past history.

Current:
Bronchial lavage on 9yo revealed inflammation. Advair 250/50 2x per day continued daily, Foradil before exercise. 2 months later, spirometry is worse. Put on Prednisone orally for 5 days at 80mgs per day....spirometry is even worse afterwards. Heliox testing done to see about VCD...negative for VCD. pH probe done overnight shows frequent reflux, almost exclusively when up and moving around, with several episodes as high as the upper airway. Started Prevacid. 1 1/2 months later, spirometry is no better, but atleast stable. Pulmo feels he's been micro-aspirating causing the inflammation.

9yo's sibling, a 6yo girl also with Mitochondrial Disease is seen in May for yearly followup. Last years spirometry and full PFT's were normal. This years spirometry shows a 17% drop in FEV1 and she's now in the low 60's like her brother. Flow loop is also flattened when it was not last year. She too worsens after bronchodilator. She starts Pulmicort 200mcg 2x per day. Spirometry a month later shows a continued decline, while most recent spirometry (July 2003) shows a stabilization but no improvement. Pulmo feels her issues are identical to her brothers.

Current diagnosis for both: Tracheobronchomalacia, Chronic Lung Inflammation, Micro-aspiration of Reflux

1. Any ideas on the course the Tracheobronchomalacia will take in these children?
2. Any other treatments that should be considered?

Thanks,
mom

[National Jewish]

The prognosis for this disorder often depends on the underlying cause.  Stents have been used frequently, and successfully, for correction of the airway collapse.  Please discuss these issues with your children's physicians.  The following article may be of interest to you.
Authors
Masters IB. Chang AB. Patterson L. Wainwright C. Buntain H. Dean BW. Francis PW.

Institution
Department of Respiratory Medicine, Royal Children's Hospital, Herston, Brisbane, Queensland, Australia. brent_masters***@****

Title
Series of laryngomalacia, tracheomalacia, and bronchomalacia disorders and their associations with other conditions in children.

Source
Pediatric Pulmonology. 34(3):189-95, 2002 Sep.

Abstract
Laryngomalacia, bronchomalacia, and tracheomalacia are commonly seen in pediatric respiratory medicine, yet their patterns and associations with other conditions are not well-understood. We prospectively video-recorded bronchoscopic data and clinical information from referred patients over a 10-year period and defined aspects of interrelationships and associations. Two hundred and ninety-nine cases of malacia disorders (34%) were observed in 885 bronchoscopic procedures. Cough, wheeze, stridor, and radiological changes were the most common symptoms and signs. The lesions were most often found in males (2:1) and on the left side (1.6:1). Concomitant malacia lesions ranged from 24% for laryngotracheobronchomalacia to 47% for tracheobronchomalacia. The lesions were found in association with other disorders such as congenital heart disorders (13.7%), tracheo-esophageal fistula (9.6%), and various syndromes (8%).Even though the understanding of these disorders is in its infancy, pediatricians should maintain a level of awareness for malacia lesions and consider the possibility of multiple lesions being present, even when one symptom predominates or occurs alone. Copyright 2002 Wiley-Liss, Inc.