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Pediatric Radiology

, Volume 45, Issue 4, pp 562–569 | Cite as

Pediatric lymphangiectasia: an imaging spectrum

  • Ladonna J. Malone
  • Laura Z. Fenton
  • Jason P. Weinman
  • Miran R. Anagnost
  • Lorna P. Browne
Original Article

Abstract

Background

Lymphangiectasia is a rarely encountered lymphatic dysplasia characterized by lymphatic dilation without proliferation. Although it can occur anywhere, the most common locations are the central conducting lymphatics and the pulmonary and intestinal lymphatic networks. Recent advances in lymphatic interventions have resulted in an increased reliance on imaging to characterize patterns of disease.

Objective

To describe the patient populations, underlying conditions, and imaging features of lymphangiectasia encountered at a tertiary pediatric institution over a 10-year period and correlate these with pathology and patient outcomes.

Materials and methods

We retrospectively reviewed the pathology database from 2002 to 2012 to identify patients with pathologically or surgically proven lymphangiectasia who had undergone cross-sectional imaging. Medical records were reviewed for patient demographics, underlying conditions, treatment and outcome.

Results

Thirteen children were identified, ranging in age from 1 month to 16 years. Five had pulmonary lymphangiectasia, four intestinal and four diffuse involvement. Pulmonary imaging findings include diffuse or segmental interlobular septal thickening, pleural effusions and dilated mediastinal lymphatics. Intestinal imaging findings include focal or diffuse bowel wall thickening with central lymphatic dilation. Diffuse involvement included dilation of the central lymphatics and involvement of more than one organ system. Children with infantile presentation and diffuse pulmonary, intestinal or diffuse lymphatic abnormalities had a high mortality rate. Children with later presentations and segmental involvement demonstrated clinical improvement with occasional regression of disease. Three children with dilated central lymphatics on imaging underwent successful lymphatic duct ligation procedures with improved clinical course.

Conclusion

Lymphangiectasia is a complex disorder with a spectrum of presentations, imaging appearances, treatments and outcomes. Cross-sectional imaging techniques distinguish segmental involvement of a single system (pulmonary or intestinal) from diffuse disease and may show dilated central conducting lymphatics, which may benefit from interventions such as ligation or occlusion.

Keywords

Lymphangiectasia Magnetic resonance imaging Magnetic resonance lymphangiogram Child 

Notes

Conflicts of interest

None

References

  1. 1.
    Laurence KM (1959) Congenital pulmonary lymphangiectasis. J Clin Pathol 12:62–69CrossRefPubMedCentralPubMedGoogle Scholar
  2. 2.
    Faul J, Berry G, Colby T et al (2000) Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 161:1037–1046CrossRefPubMedGoogle Scholar
  3. 3.
    Blei F (2008) Congenital lymphatic malformation. Ann N Y Acad Sci 1131:185–194CrossRefPubMedGoogle Scholar
  4. 4.
    Barker PM, Esther CR, Fordham LA et al (2004) Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J 24:413–419CrossRefPubMedGoogle Scholar
  5. 5.
    Esther CR Jr, Barker PM (2004) Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol 38:308–313CrossRefPubMedGoogle Scholar
  6. 6.
    Arrivé L, Azizi L, Lewin M et al (2007) MR lymphography of abdominal and retroperitoneal lymphatic vessels. AJR Am J Roentgenol 189:1051–1058CrossRefPubMedGoogle Scholar
  7. 7.
    Derhy S, El Mouhadi S, Ruiz A et al (2013) Non-contrast 3D MR lymphography of retroperitoneal lymphatic aneurysmal dilatation: a continuous spectrum of change from normal variants to cystic lymphangioma. Insights Imaging 4:753–758CrossRefPubMedCentralPubMedGoogle Scholar
  8. 8.
    Liu NF, Lu Q, Wang CG (2008) Magnetic resonance imaging as a new method to diagnose protein losing enteropathy. Lymphology 41:111–115PubMedGoogle Scholar
  9. 9.
    Bellini C, Boccardo F, Campisi C et al (2006) Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis 1:43CrossRefPubMedCentralPubMedGoogle Scholar
  10. 10.
    Chung CJ, Fordham LA, Barker P et al (1999) Children with congenital pulmonary lymphangiectasia: after infancy. Am J Roentgenol 173:1583–1588CrossRefGoogle Scholar
  11. 11.
    Mettauer N, Agrawal S, Pierce C et al (2009) Outcome of children with pulmonary lymphangiectasis. Pediatr Pulmonol 44:351–357CrossRefPubMedGoogle Scholar
  12. 12.
    Nobre LF, Müller NL, de Souza Júnior AS et al (2004) Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging 19:56–59CrossRefPubMedGoogle Scholar
  13. 13.
    Strober W, Wochner RD, Carbone PP et al (1967) Intestinal lymphangiectasia: a protein-losing enteropathy with hypogammaglobulinemia, lymphocytopenia and impaired homograft rejection. J Clin Invest 46:1643–1656CrossRefPubMedCentralPubMedGoogle Scholar
  14. 14.
    Holzknecht N, Helmberger T, Beuers U et al (2002) Cross-sectional imaging findings in congenital intestinal lymphangiectasia. J Comput Assist Tomogr 26:526–528CrossRefPubMedGoogle Scholar
  15. 15.
    Sharma R, Wendt JA, Rasmussen JC et al (2008) New horizons for imaging lymphatic function. Ann N Y Acad Sci 1131:13–36CrossRefPubMedCentralPubMedGoogle Scholar
  16. 16.
    Laor T, Hoffer FA, Burrows PE et al (1998) MR lymphangiography in infants, children, and young adults. AJR Am J Roentgenol 171:1111–1117CrossRefPubMedGoogle Scholar
  17. 17.
    Zeng Y, Wang F, Williams ED et al (2005) Lymphatics in the alimentary tract of children in health and disease: study on mucosal biopsies using the monoclonal antibody D2-40. Pediatr Dev Pathol 8:541–549CrossRefPubMedGoogle Scholar
  18. 18.
    Juszczyk K, Waugh R, Sandroussi C (2013) Lymphangiography as therapeutic management of chylothorax. J Med Imaging Radiat Oncol 57:460–461CrossRefPubMedGoogle Scholar
  19. 19.
    Campisi C, Bellini C, Eretta C et al (2006) Diagnosis and management of primary chylous ascites. J Vasc Surg 43:1244–1248CrossRefPubMedGoogle Scholar
  20. 20.
    Patel KV, Goel RM, Wong T (2013) Diffuse large B-cell lymphoma recurrence complicating primary intestinal lymphangiectasia. Clin Gastroenterol Hepatol 11:e86–87CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Ladonna J. Malone
    • 1
  • Laura Z. Fenton
    • 1
  • Jason P. Weinman
    • 1
  • Miran R. Anagnost
    • 2
  • Lorna P. Browne
    • 1
  1. 1.Department of RadiologyChildren’s Hospital ColoradoAuroraUSA
  2. 2.Department of PathologyChildren’s Hospital ColoradoAuroraUSA

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