Primary bronchomalacia in infants and children,☆☆

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Abstract

Objective: To determine the natural history of primary bronchomalacia in infants and children.

Study design: Retrospective chart review and follow-up telephone questionnaire of 17 patients with bronchoscopically confirmed primary bronchomalacia.

Results: All patients had initial symptoms within the first 6 months of life, and all patients were thought by their primary care physicians to have reactive airways disease. In no patient was the diagnosis of bronchomalacia considered before referral to our center. All patients had a harsh, monophonic wheeze loudest over the central airway and intermittently present between illnesses. All 17 patients had bronchomalacia of the left main-stem bronchus; two had mild tracheomalacia associated with the bronchomalacia; and one had bilateral bronchomalacia. One patient had associated laryngomalacia. Twenty-five percent of our patients had reactive airway disease in addition to bronchomalacia. With growth, all patients have shown a gradual improvement and a cessation of daily symptoms. All three patients older than the age of 5 years report limitation of vigorous exercise.

Conclusions: Bronchomalacia should be considered in the differential diagnosis of the persistently wheezing infant and should be evaluated appropriately. More severe forms of bronchomalacia appear to predispose patients to exercise limitation later in life, which suggests that the lesion does not completely resolve with growth. (J Pediatr 1997;130:59-66)

Section snippets

CASES

Summaries of the cases of 17 patients with primary bronchomalacia and tracheobronchomalacia are included in the Table. All patients but one were seen in the pulmonology clinic of the Children's Hospital of Pittsburgh and resdie in western Pennsylvania.

Table. Case summaries: Patients 1 to 17

Patient No.SexAge at diagnosisHistoryExaminationBronchoscopic findingDuration of follow-upFollow-up resultOther
1M5.5 mo“Congested breathing” since birth; intermittent wheezing with coldsRight-sided wheeze

OVERVIEW

Bronchomalacia refers to chondromalacia of a main-stem bronchus. It can be found in association with tracheomalacia (tracheobronchomalacia). The affected bronchus lacks rigidity because of insufficient cartilage or extrinsic compression. During quiet respiration, small changes in the diameter of the affected bronchus can be inapparent on physical examination. Forced expiration will cause collapse of the nonrigid portion of the bronchus and can result in a wheeze that is often audible without a

History

Historical findings in patients with bronchomalacia are fairly consistent. All patients have symptoms within the first 6 months of life, with “noisy breathing” and a harsh wheeze on forceful expiration. Many parents do not note the wheeze until the infant's first cold. All infants have coughing and wheezing with colds and continue to have symptoms between colds. The breathing is generally quiet in sleep. The diagnosis of gastroesophageal reflux is often entertained before the diagnosis of

TREATMENT

A twofold therapeutic approach to these patients, mechanical and pharmacologic, is useful. To improve clearance of the affected side in the younger infants, parents can turn the affected side of the infant upward during sleep—allowing for the lung with poor clearance to drain into the normal side, which then can clear secretions normally. All parents should learn chest physiotherapy and perform it when their children have colds. Gravitational drainage techniques are useful when the patient has

NATURAL HISTORY

The presence or absence of small-airways hyperreactivity partly determines the course of this disorder. In general, patients without RAD in general have a smoother course and gradually outgrow the symptoms of large-airway obstruction. Between birth and maturity, the trachea and bronchi double in length and triple in diameter. It is the tripling of the airway diameter that is responsible for the resolution of daily symptoms. All three patients with primary bronchomalacia older than 5 years of

CONCLUSION

Primary bronchomalacia and tracheobronchomalacia are much more common than was previously thought. Bronchomalacia and tracheobronchomalacia should be considered in the infant with persistent wheezing and should be evaluated. Failure of standard antiasthma therapy is a clue that the wheezing appreciated on examination may reflect an anatomic obstruction of the central airway. An absence of hyperinflation in the face of wheezing on examination also suggests central airway obstruction. The natural

Acknowledgements

I am indebted to David M. Orenstein, MD, for his critical reading of the manuscript and for his encouragement and advice. I also gratefully acknowledge Mr. Steve Walczak and Ms. Rebecca Mutich for technical assistance with the bronchoscopies.

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Reprint requests: Jonathan D. Finder, MD, Children's Hospital of Pittsburgh, 3705 Fifth Ave., Pittsburgh, PA 15213.

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