title: Sudden death of a 14-year-old girl with lymphangiomatosis.
abstract: A 14-year-old girl presented with progressively worsening intermittent orthopnea. Imaging studies showed cardiomegaly, extensive pericardial effusion, and a mediastinal tumor. By pericardial drainage, approximately 8,000-mL fluid was collected over 10 days. Left thoracoscopic pericardial fenestration and mediastinal tumor biopsy were performed, revealing lymphangiomatosis. Chylous pleural effusion developed post-surgery. Although control was attempted, her condition worsened. Eight months later, she died of sudden cardiopulmonary arrest. Autopsy revealed systemic spread of the primary lesion from the hyperplastic lymph ducts to the parietal pleura and mediastinum confirming systemic lymphangiomatosis.
authors.affiliation: Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan. k167999@is.icc.u-tokai.ac.jp.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Nakagawa
authors.affiliation: Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Koizumi
authors.affiliation: Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Kana
authors.initials: K
authors.lastname: Oiwa
authors.affiliation: Division of Pathology, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Chie
authors.initials: C
authors.lastname: Inomoto
authors.affiliation: Division of Pathology, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Go
authors.initials: G
authors.lastname: Ogura
authors.affiliation: Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Ryota
authors.initials: R
authors.lastname: Masuda
authors.affiliation: Division of Radiology, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Yamashita
authors.affiliation: Division of Pathology, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Naoya
authors.initials: N
authors.lastname: Nakamura
authors.affiliation: Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Iwazaki
mesh_terms: D000293:Adolescent; D001706:Biopsy; D003645:Death, Sudden / Q000209:etiology*; D004724:Endoscopy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008202:Lymphangioma / Q000150:complications / Q000473:pathology*; D008479:Mediastinal Neoplasms / Q000150:complications / Q000473:pathology*; D008482:Mediastinum / Q000473:pathology; D010490:Pericardial Effusion / Q000150:complications* / Q000473:pathology; D010994:Pleura / Q000473:pathology
keywords: Chylothorax; Lymphangiography; Pericardial effusion; Systemic lymphangiomatosis