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J Thorac Cardiovasc Surg 2004;128:645-647
© 2004 The American Association for Thoracic Surgery

Brief communication

Successful bilateral lung volume reduction in a child with emphysema from bronchiolitis obliterans

Maurizio Mancuso, MDa,*, Donatella Pacchioni, MDc, Enrico Ruffini, MDa, Antonio Cavallo, MDa, Pierluigi Filosso, MDa, Aurelio Viale, MDb, Paolo Solidoro, MDd, Elisabetta Bignamini, MDe

a Division of Thoracic Surgery, San Giovanni Battista University Hospital, Torino, Italy
b Respiratory Intensive Care Unit, San Giovanni Battista University Hospital, Torino, Italy
c Department of Pathology, San Giovanni Battista University Hospital, Torino, Italy
d Pulmonary Division, San Giovanni Battista University Hospital, Torino, Italy
e Pulmonary Division, Children's Hospital Regina Margherita, Torino, Italy

Received for publication December 5, 2003; revisions received March 10, 2004; accepted for publication March 30, 2004.

* Address for reprints: Maurizio Mancuso, MD, Division of Thoracic Surgery, San Giovanni Battista University Hospital, Via Genova 3, 10126 Torino, Italy
mmancuso@molinette.piemonte.it

The first 20% of the full text of this article appears below.


Dr Mancuso


Lung hyperinflation occurs not only because of emphysema but also because of small airways diseases with distal air trapping. In infancy bronchiolitis is quite common among airways diseases. We describe the case of a 9-year-old child with end-stage emphysema caused by bronchiolitis and associated chest wall deformity, who was excluded from lung transplantation as a result of severe osteoporosis and who underwent successful bilateral lung volume reduction (LVR) through a median sternotomy.

Clinical summary

At the age of 18 months, because of dyspnea and pulmonary infiltrates, the infant underwent an open middle lobe biopsy with evidence of aspecific bronchiolitis obliterans. Furthermore, the patient had a chest wall abnormality consistent with pectus carenatum and kyphosis (Figure 1). Pulmonary function tests (PFTs) throughout childhood and chest radiographs were consistent with chronic obstructive pulmonary disease. {alpha}1-Antytripsin levels and sweat test results were normal.


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  		Figure 1. Chest wall deformity with pectus carenatum and kyphosis.

 
At the age of 7 years, he worsened, with severe exertional dyspnea necessitating frequent hospitalizations. Prednisone was augmented to 1 mg/kg. The patient was referred to a lung transplantation program abroad because of his age. Meanwhile, his osteoporosis became symptomatic, with spontaneous fracture of the right clavicle, and the child was again referred to us. Recalcification therapy was then started, along with tapering of prednisone to 0.3 mg/kg. The bone disease improved, but a further worsening of respiratory function occurred.

He quit going to school and was very disabled at home in New York Heart Association class III. Forced expiratory volume in 1 second (FEV1) decreased to 26%, total lung capacity (TLC) was 132%, RV was 310%, and RV/TLC was 226% of predicted value. The diffusing capacity of the lung for CO . . . [Full Text of this Article]






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