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CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 3  |  Page : 62-65

Management of Gorham–Stout disease presenting as intractable pleural effusion in an adolescent: A case report and review of literature


1 Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Pediatric Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Correspondence Address:
Dr. Kirtikumar J Rathod
Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_20_19

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Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.


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