Disseminated lymphangiomatosis is a rare vascular tumor characterized by a proliferation of abnormal lymphatic channels that often involves multiple organ systems. One particularly morbid manifestation of this disorder is the presence of bony lytic lesions with associated chylothorax. Because of its unusual nature, this condition is often a diagnostic and therapeutic challenge. In this report, we present the diagnostic features, including a unique radiologic finding, and successful management of a 7-year-old girl with this condition using a combination of aggressive surgery and medical treatment with interferon and pamidronate.
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