Skip to main content

Advertisement

SpringerLink
Log in

Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Background

Multifocal lymphangiomatosis is a rare systemic disorder affecting children. Due to its rarity and wide spectrum of clinical, histological and imaging features, establishing the diagnosis of multifocal lymphangiomatosis can be challenging.

Objectives

The purpose of this study was to describe a new imaging sign in this disorder: paraspinal soft tissue and signal abnormality at MRI.

Materials and methods

We retrospectively reviewed the imaging, clinical and histopathological findings in a cohort of eight children with thoracic involvement from this condition.

Results

Evidence of paraspinal chest disease was identified at MRI and CT in all eight of these children. The changes comprise heterogeneous intermediate-to-high signal parallel to the thoracic vertebrae on T2-weighted sequences at MRI, with abnormal paraspinal soft tissue at CT and plain radiography.

Conclusion

Multifocal lymphangiomatosis is a rare disorder with a broad range of clinicopathological and imaging features. MRI allows complete evaluation of disease extent without the use of ionising radiation and has allowed us to describe a previously unreported imaging sign in this disorder, namely, heterogeneous hyperintense signal in abnormal paraspinal tissue on T2-weighted images.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Levey DS, MacCormack LM, Sartoris DJ et al (1996) Cystic angiomatosis: case report and review of the literature. Skeletal Radiol 25:287–293

    Article  PubMed  CAS  Google Scholar 

  2. Enzinger FM (1994) Tumors of lymphatic vessels. In: Enzinger FM WS (ed) Soft tissue tumours, 3rd edn. Mosby, St. Louis, pp 679–700

  3. Wong CS, Chu TYC (2008) Clinical and radiological features of generalised lymphangiomatosis. Hong Kong Med J 14:402–404

    PubMed  CAS  Google Scholar 

  4. Rostom AY (2000) Treatment of thoracic lymphangiomatosis. Arch Dis Child 83:138–139

    Article  PubMed  CAS  Google Scholar 

  5. Yang DH, Goo HW (2006) Generalized lymphangiomatosis: radiologic findings in three pediatric patients. Korean J Radiol 7:287–291

    Article  PubMed  Google Scholar 

  6. Marom EM, Moran CA, Munden RF (2004) Generalized lymphangiomatosis. AJR 182:1068

    PubMed  Google Scholar 

  7. Wunderbaldinger P, Paya K, Partik B et al (2000) CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. AJR 174:827–832

    PubMed  CAS  Google Scholar 

  8. Kupeli S, Arac A, Yalcin B et al (2008) Lymphangiomatosis in a child: eight years' follow-up without treatment. Pediatr Hematol Oncol 25:614–619

    Article  PubMed  Google Scholar 

  9. Wadsworth DT, Newman B, Abramson SJ et al (1997) Splenic lymphangiomatosis in children. Radiology 202:173–176

    PubMed  CAS  Google Scholar 

  10. Aviv RI, McHugh K, Hunt J (2001) Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. Clin Radiol 56:184–190

    Article  PubMed  CAS  Google Scholar 

  11. Konez O, Vyas PK, Goyal M (2000) Disseminated lymphangiomatosis presenting with massive chylothorax. Pediatr Radiol 30:35–37

    Article  PubMed  CAS  Google Scholar 

  12. Agarwal PP, Matzinger FR, Seely JM (2008) Case 132: lymphangiomatosis. Radiology 247:288–290

    Article  PubMed  Google Scholar 

  13. Laffan EE, O'Connor R, Ryan SP et al (2004) Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging. Pediatr Radiol 34:472–480

    Article  PubMed  Google Scholar 

  14. Humphries PD, Wynne CS, Sebire NJ et al (2006) Atypical abdominal paediatric lymphangiomatosis: diagnosis aided by diffusion-weighted MRI. Pediatr Radiol 36:857–859

    Article  PubMed  Google Scholar 

  15. Kwee TC, Takahara T, Vermoolen MA et al (2010) Whole-body diffusion-weighted imaging for staging malignant lymphoma in children. Pediatr Radiol 40:1592–1602

    Article  PubMed  Google Scholar 

  16. Davies SG (2009) Aids to radiological differential diagnosis, 5th edn. Saunders Elsevier, USA

  17. McHugh K (2007) Renal and adrenal tumours in children. Cancer Imaging 7:41–51

    Article  PubMed  Google Scholar 

  18. McHugh K, Barnacle A, Shah N (2009) First description of three patients with multifocal lymphangiomatosis and protein losing enteropathy following palliation of complex congenital heart disease with total cavo-pulmonary connection. Pediatr Cardiol 30:571–572

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK

    Vikas Shah, Sachit Shah, Alex Barnacle & Kieran McHugh

  2. Department of Histopathology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK

    Neil J. Sebire

  3. Department of Oncology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK

    Penelope Brock

  4. Department of Dermatology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK

    John I. Harper

Authors
  1. Vikas Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sachit Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Alex Barnacle
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Neil J. Sebire
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Penelope Brock
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. John I. Harper
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kieran McHugh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Vikas Shah.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shah, V., Shah, S., Barnacle, A. et al. Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. Pediatr Radiol 41, 985–992 (2011). https://doi.org/10.1007/s00247-011-2124-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-011-2124-1

Keywords

Search

Navigation