title: Thoracic lymphangiomatosis with massive chylothorax after a tumor biopsy and with disseminated intravenous coagulation--lymphoscintigraphy, an alternative minimally invasive imaging technique: report of a case.
abstract: Thoracic lymphangiomatosis in childhood is a rare disorder that frequently has poor response to medical therapy as well as a poor prognosis. This disease is often misdiagnosed, and a definitive diagnosis is usually delayed because of the rarity and slow course of the disease. No previous reports have so far described the usefulness of lymphoscintigraphy in the diagnosis of lymphangiomatosis, although some authors have reported the efficacy of lymphoscintigraphy for evaluating chylothorax. A 6-year-old boy presented with a diffuse mediastinal mass and received an open mediastinal biopsy for a definitive diagnosis, which led to the occurrence of massive chylothorax postoperatively. A diagnosis of lymphangiomatosis was finally made based on the lymphoscintigraphic findings demonstrating an obstruction of the thoracic duct. This report describes a rare case of thoracic lymphangiomatosis diagnosed at autopsy, and suggests that the minimally invasive technique of lymphoscintigraphy should be employed to obtain a definitive diagnosis at an early stage.
authors: Department of Pediatric Surgery, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka, 830-0011, Japan.
authors: Suguru
authors: S
authors: Fukahori
authors: Tomomitsu
authors: T
authors: Tsuru
authors: Kimio
authors: K
authors: Asagiri
authors: Hirotaka
authors: H
authors: Nakamizo
authors: Takahiro
authors: T
authors: Asakawa
authors: Hiroaki
authors: H
authors: Tanaka
authors: Yoshiaki
authors: Y
authors: Tanaka
authors: Jun
authors: J
authors: Akiba
authors: Hirohisa
authors: H
authors: Yano
authors: Minoru
authors: M
authors: Yagi
mesh_terms: D018952:Antigens, CD34 / Q000032:analysis; D001706:Biopsy; D002648:Child; D002916:Chylothorax / Q000175:diagnosis* / Q000473:pathology; D004211:Disseminated Intravascular Coagulation / Q000473:pathology*; D006801:Humans; D008171:Lung Diseases / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008200:Lymphangiectasis / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D061305:Lymphoscintigraphy / Q000295:instrumentation*; D008297:Male; D013909:Thorax / Q000473:pathology*
chemical_list: D018952:Antigens, CD34