The Annals of Thoracic Surgery, Vol 39, 185-187, Copyright © 1985 by The Society of Thoracic Surgeons
Surgical palliation of respiratory insufficiency secondary to massive exuberant polyostotic fibrous dysplasia of the ribs
RM King, WS Payne, S Olafsson and KK Unni
A middle-aged man with long-standing polyostotic fibrous dysplasia had
severe progressive restrictive lung disease with hypoxemia and pulmonary
hypertension with heart failure because of exuberant intrathoracic,
extraosseous proliferation of dysplastic tissue. Subtotal resection of this
benign tissue mass ameliorated the respiratory insufficiency and led to
sustained improvement in exercise tolerance, increase in pulmonary reserve,
and decrease in signs of heart failure and pulmonary hypertension.