title: Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease.
abstract: BACKGROUND
  Complex lymphatic anomalies are intractable lymphatic disorders, including generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), and kaposiform lymphangiomatosis (KLA). The etiology of these diseases remains unknown and diagnosis is confused by their similar clinical findings. This study aimed to clarify the differences in clinical features and prognosis among GLA, KLA, and GSD, in Japanese patients.
  
  
  METHODS
  Clinical features, radiological and pathological findings, treatment, and prognosis of patients were obtained from a questionnaire sent to 39 Japanese hospitals. We divided the patients into three groups according to radiological findings of bone lesions and pathology. Differences in clinical findings and prognosis were analyzed.
  
  
  RESULTS
  Eighty-five patients were registered: 35 GLA, 9 KLA, and 41 GSD. Disease onset was more common in the first two decades of life (69 cases). In GSD, osteolytic lesions were progressive and consecutive. In GLA and KLA, 18 patients had osteolytic lesions that were multifocal and nonprogressive osteolysis. Thoracic symptoms, splenic involvement, and ascites were more frequent in GLA and KLA than in GSD. Hemorrhagic pericardial and pleural effusions were more frequent in KLA than GLA. GSD had a significantly favorable outcome compared with combined GLA and KLA (P = 0.0005). KLA had a significantly poorer outcome than GLA (P = 0.0268).
  
  
  CONCLUSIONS
  This survey revealed the clinical features and prognosis of patients with GLA, KLA, and GSD. Early diagnosis and treatment of KLA are crucial because KLA has high mortality. Further prospective studies to risk-stratify complex lymphatic anomalies and optimize management for KLA are urgently needed.
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan.
authors.forename: Michio
authors.initials: M
authors.lastname: Ozeki
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Fujino
authors.affiliation: Department of Pathology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuoka
authors.affiliation: Department of Radiology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Nosaka
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Fukao
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D044148:Lymphatic Abnormalities / Q000000981:diagnostic imaging / Q000401:mortality / Q000628:therapy; D008297:Male; D010015:Osteolysis, Essential / Q000000981:diagnostic imaging / Q000401:mortality / Q000628:therapy; D011859:Radiography; D011795:Surveys and Questionnaires; D015996:Survival Rate
keywords: Gorham-Stout disease; complex lymphatic anomaly; generalized lymphatic anomaly; kaposiform lymphangiomatosis; lymphatic malformation; osteolysis