Surgical Palliation of Respiratory Insufficiency Secondary to Massive Exuberant Polyostotic Fibrous Dysplasia of the Ribs

Abstract

Listen

Translate article

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.