Gorham-Stout disease of the mandible, manubrium and cervical spine presenting as bilateral chylothorax

Gorham-Stout disease (GSD) is an extremely rare musculoskeletal disease of unknown aetiology characterised by non-neoplastic proliferation of vascular and lymphatic channels causing massive osteolysis, typically affecting younger individuals. Chylothorax is a known complication of GSD which is postulated to occur from thoracic spine involvement leading to pleural or thoracic duct invasion. In our case, bilateral chylothorax developed in a 60-year-old woman without any thoracic spine involvement of her disease, challenging the proposed mechanism. Despite bilateral pleural drainage and escalating doses of sirolimus, she ultimately developed respiratory failure and shock and succumbed to her illness. Overall survival of GSD is unknown, but when complicated by chylothorax, prognosis is typically poor. GSD represents a diagnostic and management challenge due to the paucity of knowledge surrounding its aetiology and management. These patients require multidisciplinary coordinated care. It is also important to note its high mortality when associated with chylothorax in particular.

Vanishing Mandible in a 7-year Old Child: Response to Radiation Therapy

Massive osteolysis in a 7-year old child is a rare condition. The etiology of massive osteolysis is unknown and it results in the progressive destruction of bony structures. There is no standard therapy available in the literature. Conservative treatment is often used for its management. Radiotherapy is considered as an accepted form of treatment with greater chance of success when it is used in the early course of disease. There are few case reports in the literature in which radiotherapy has been used for the treatment. This article highlights the literature update on various treatment modalities and a case managed by radiation therapy.