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.
- adult intensive care
- malignant and benign haematology
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Contributors As lead/corresponding author, I, AAT, have made substantial contributions to the conception of the work and drafting the work or revising it critically for important intellectual content and produced a final version to be published. SP has made contributions to this work including planning, conception and writing/editing. I agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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