Unique Diagnostic Features and Successful Management of a Patient With Disseminated Lymphangiomatosis and Chylothorax

Abstract

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.

Cutaneous lymphangioma as a diagnostic clue for thoracic lymphangiomatosis

“…Nonspecific CT findings such as interlobular, septal thickening and peribronchovascular cuffing could be suggestive of disseminated pulmonary lymphangiomatosis 5 . Especially, lytic bone lesions, as in our case, which can be a manifestation of bony involvement of lymphangiomatosis, could suggest the thoracic lymphangiomatosis 6 …”

A gene missense mutation in diffuse pulmonary lymphangiomatosis with thrombocytopenia

“…DPL with DIC is considered a coagulation abnormality associated with venous malformations; this so-called localized intravascular coagulation could develop into systemic DIC. [ 7 , 10 ] However, this hypothesis lacks a pathological basis, and the actual etiology needs to be further studied. Two cases of DPL involving the spleen with thrombocytopenia and DIC showed improvement after partial splenic embolization and splenectomy; therefore, such cases may be related to lymphoma involving the spleen.…”

Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis

“…Surgical interventions, such as excision of a localised lesion, pleurodesis, pleurosclerosis, pleuroperitoneal shunting, chest wall resection or thoracic duct ligation in the case of thoracal involvement have been applied with differing results 14 , 21 , 23 – 27. Systemic treatment with corticosteroids, immunomodulating agents and chemotherapy have shown varying results 2 , 9 , 12 , 24 , 26 , 27. Interferon α, a cytokine with antiangiogenic activity, appears to be beneficial and has been used for the treatment of lymphangiomatosis for almost 20 years.…”

“…Interferon α, a cytokine with antiangiogenic activity, appears to be beneficial and has been used for the treatment of lymphangiomatosis for almost 20 years. Clinical improvement has been reported in the majority of cases, although the optimal treatment duration remains unclear and ranges from 3 weeks to several years 7 , 21 , 24 , 26 , 27…”