CTICasesGorham-Stout Disease (Vanishing Bone Disease)
▶ Watch on YouTube — age-restricted, cannot embed here
Imaging findings
Initial chest X-ray and CT showed a left pleural effusion and multiple lytic bony abnormalities in the ribs (first, second, third, and fourth) and other bones (transverse processes, hips, legs, thighs). These lytic changes were filled with material of fluid-to-fat attenuation. Following chest tube placement for the chylous effusion, air was observed to track into these lytic bone lesions, indicating communication between the lymphatic system, pleural space, and bone. After tube removal, the air resolved. Later imaging showed a large right paratracheal lesion and other lymphatic malformations throughout the body. An esophagram surprisingly revealed branching structures, presumed to be lymphatic channels, filling with oral contrast after ingestion.
Key takeaways
Gorham-Stout disease, also known as vanishing bone disease, is a rare congenital lymphatic malformation characterized by progressive osteolysis and replacement of bone by lymphatic or vascular tissue. The lymphatic channels in these patients can communicate with various surrounding structures, including the pleural space and bones. This communication can be dramatically demonstrated by the passage of air from a chest tube into lytic bone lesions, or the filling of lymphatic channels with oral contrast during an esophagram. Patients often present with chylous effusions and widespread lymphatic malformations.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Acute Fibrinous Organizing PneumoniaILD
- Actinomyces graevenitzii PneumoniaInfection
- Arrhythmogenic Right Ventricular Dysplasia (ARVD)Congenital
- Diffuse Adenocarcinoma in SclerodermaNeoplastic
- Recurrent Malignant Solitary Fibrous Tumor of the PleuraNeoplastic
- Systemic-to-Pulmonary Venous Shunt with CirrhosisVascular
- Subtle Pneumothorax on Supine Chest X-rayTrauma
See all cases from November 19, 2014 →
Related Congenital cases
- Anomalous Origin of Right Subclavian Artery from Pulmonary Artery
- Neurofibromatosis Type 1 with Cystic Lung Disease
- Melorheostosis with Tracheobronchial Involvement
- Anomalous Left Main Coronary Artery from Right Coronary Artery with Septal Course
- Partial Anomalous Pulmonary Venous Connection (PAPVC) with Sinus Venosus ASD
- Persistent Left Superior Vena Cava and Tracheal Bronchus