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Pediatric Radiology

, Volume 46, Issue 9, pp 1282–1290 | Cite as

Imaging features of kaposiform lymphangiomatosis

  • Pradeep Goyal
  • Ahmad I. Alomari
  • Harry P. Kozakewich
  • Cameron C. TrenorIII
  • Antonio R. Perez-Atayde
  • Steven J. Fishman
  • Arin K. Greene
  • Raja Shaikh
  • Gulraiz Chaudry
Original Article

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.

Materials and 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.

Conclusion

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.

Keywords

Children Computed tomography Kaposiform lymphangiomatosis Lymphatic malformation Magnetic resonance imaging Radiography Ultrasound 

Notes

Acknowledgments

We are very grateful to David Zurakowski, PhD, MA, MS, Statistics, Director of Biostatistics at Boston Children’s Hospital, for his help in biostatistics.

Compliance with ethical standards

Conflicts of interest

None

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Pradeep Goyal
    • 1
    • 2
  • Ahmad I. Alomari
    • 1
    • 2
  • Harry P. Kozakewich
    • 1
    • 3
  • Cameron C. TrenorIII
    • 1
    • 4
  • Antonio R. Perez-Atayde
    • 1
    • 3
  • Steven J. Fishman
    • 1
    • 5
  • Arin K. Greene
    • 1
    • 6
  • Raja Shaikh
    • 1
    • 2
  • Gulraiz Chaudry
    • 1
    • 2
  1. 1.Vascular Anomalies CenterBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Division of Vascular and Interventional RadiologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  3. 3.Department of PathologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  4. 4.Division of Hematology/OncologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  5. 5.Department of SurgeryBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  6. 6.Department of Plastic and Oral SurgeryBoston Children’s Hospital and Harvard Medical SchoolBostonUSA

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