Introduction. Fibrous dysplasia is a noninherited benign skeletal disorder
associated with abnormal bone development. Single bone involvement, the
monostotic form, accounts for 70 ? 80% of cases, while the polyostotic form,
with multiple bone involvement, accounts for 20 ? 30% of cases. Cystic
degeneration and occasional aneurysmal bone cyst formation may be found in
fibrous dysplasia lesions, particularly in the costal lesions. Case Report.
A 51-year-old man presented with acute shortness of breath after sustaining
simple chest wall injury. Chest computed tomography showed multiple massive
osteolytic rib lesions, as well as a massive left-sided pleural effusion
with compression atelectasis of the lung parenchyma. Osteolytic lesions of
the anterior 2nd and 7th thoracic vertebral body were found, along with a
well defined osteolytic lesion in the body of the sternum. Video-assisted
thoracoscopy of the left pleural space was performed and frozen sections,
collected using endoscopic biopsy forceps of the cystic wall and solid parts
of the tumors, were sent for ex tempore histopathological analysis. Results
showed fibrous dysplasia with suspected malignancy. Talc pleurodesis was
performed based on the obtained results. At present, the patient is
asymptomatic with his daily routine uninterrupted by his medical condition.
Conclusion. Treatment of pleural effusion caused by a cyst rupture of
unresectable degenerated polyostotic fibrous dysplasia of the ribs
represents a surgical challenge. Surgical drainage of the cysts followed by
chemical pleurodesis seems to be a reasonable solution in cases where
pulmonary functions are impacted by combined effects of pleural effusion and
cystic compression.
A narrative literature review on the diagnostic and therapeutic intervention approaches of polyostotic fibrous dysplasia in bones: A bangladeshi case report
