title: Imaging features of kaposiform lymphangiomatosis.
abstract: BACKGROUND
  Kaposiform lymphangiomatosis is a rare, aggressive lymphatic disorder. The imaging and presenting features of kaposiform lymphangiomatosis can overlap with those of central conducting lymphatic anomaly and generalized lymphatic anomaly.
  
  
  OBJECTIVE
  To analyze the imaging findings of kaposiform lymphangiomatosis disorder and highlight features most suggestive of this diagnosis.
  
  
  METHODS
  We retrospectively identified and characterized 20 children and young adults with histopathological diagnosis of kaposiform lymphangiomatosis and radiologic imaging referred to the vascular anomalies center between 1995 and 2015.
  
  
  RESULTS
  The median age at onset was 6.5 years (range 3 months to 27 years). The most common presenting features were respiratory compromise (dyspnea, cough, chest pain; 55.5%), swelling/mass (25%), bleeding (15%) and fracture (5%). The thoracic cavity was involved in all patients; all patients had mediastinal involvement followed by lung parenchymal disease (90%) and pleural (85%) and pericardial (50%) effusions. The most common extra-thoracic sites of disease were the retroperitoneum (80%), bone (60%), abdominal viscera (55%) and muscles (45%). There was characteristic enhancing and infiltrative soft-tissue thickening in the mediastinum and retroperitoneum extending along the lymphatic distribution.
  
  
  CONCLUSIONS
  Kaposiform lymphangiomatosis has overlapping imaging features with central conducting lymphatic anomaly and generalized lymphatic anomaly. Presence of mediastinal or retroperitoneal enhancing and infiltrative soft-tissue disease along the lymphatic distribution, hemorrhagic effusions and moderate thrombocytopenia (50-100,000/μl) should favor diagnosis of kaposiform lymphangiomatosis.
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA.
authors.forename: Pradeep
authors.initials: P
authors.lastname: Goyal
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA.
authors.forename: Ahmad I
authors.initials: AI
authors.lastname: Alomari
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Harry P
authors.initials: HP
authors.lastname: Kozakewich
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Division of Hematology/Oncology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Cameron C
authors.initials: CC
authors.lastname: Trenor
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Perez-Atayde
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Fishman
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Department of Plastic and Oral Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: Arin K
authors.initials: AK
authors.lastname: Greene
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.|Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA.
authors.forename: Raja
authors.initials: R
authors.lastname: Shaikh
authors.affiliation: Vascular Anomalies Center, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. gulraiz.chaudry@childrens.harvard.edu.|Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA. gulraiz.chaudry@childrens.harvard.edu.
authors.forename: Gulraiz
authors.initials: G
authors.lastname: Chaudry
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging / Q000379:methods*; D005260:Female; D006390:Hemangioendothelioma / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D059885:Kasabach-Merritt Syndrome / Q000000981:diagnostic imaging*; D044148:Lymphatic Abnormalities / Q000000981:diagnostic imaging*; D008297:Male; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000000981:diagnostic imaging*
keywords: Children; Computed tomography; Kaposiform lymphangiomatosis; Lymphatic malformation; Magnetic resonance imaging; Radiography; Ultrasound