Skeletal Radiology

, Volume 47, Issue 9, pp 1293–1297 | Cite as

Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus

  • Megan K. MillsEmail author
  • Bryn Putbrese
  • Hailey Allen
  • Sarah E. Stilwill
Case Report


Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported. We will review the clinical and imaging manifestations of lymphangiomatosis and provide a differential diagnosis for masses of the lumbosacral plexus. Although lower extremity pain and weakness encountered in the emergency department or outpatient setting is most frequently caused by lumbar spine pathology, occasionally, abnormalities of the lumbosacral plexus may prove to be the cause. While peripheral nerve sheath tumors lead the differential diagnosis of tumor or tumor-like entities involving the lumbosacral plexus, lymphangiomatosis is a rare differential consideration.


Lymphangiomatosis Sacral plexus Lumbosacral plexopathy 



The authors wish to thank Dr. Ulrich Rassner for his role in obtaining imaging parameters. Special thanks to Michael Mozdy and Matthew S. Zabriskie for their assistance in writing, editing, and preparing the manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Bhatti MA, Ferrante JW, Gielchinsky I, Norman JC. Pleuropulmonary and skeletal lymphangiomatosis with chylothorax and chylopericardium. Ann Thorac Surg. 1985;40:398–401.CrossRefPubMedGoogle Scholar
  2. 2.
    Wunderbaldinger P, Paya K, Partik B, Turetschek K, Hörmann M, Horcher E, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. Am J Roentgenol. 2000;174(3):827–32.CrossRefGoogle Scholar
  3. 3.
    Ozturk A, Yousem DM. Magnetic resonance imaging findings in diffuse lymphangiomatosis: neuroradiological manifestations. Acta Radiol. 2007;48(5):560–4.CrossRefPubMedGoogle Scholar
  4. 4.
    Chen H. Lymphangiomas and lymphangiomatosis. In: Atlas of genetic diagnosis and counseling. New York: Springer; 2014.CrossRefGoogle Scholar
  5. 5.
    Maki DD, Nesbit ME, Griffiths HJ. Diffuse lymphangiomatosis of bone. Australas Radiol. 1999;43:535–8.CrossRefPubMedGoogle Scholar
  6. 6.
    Forstner R, Datz C, Dietze O, Rettenbacher L. Sclerotic variant of lymphangiomatosis of bone: imaging findings at diagnosis and long-term follow-up. Skelet Radiol. 1998;27(8):445–8.CrossRefGoogle Scholar
  7. 7.
    Yang DH, Goo HW. Generalized lymphangiomatosis: radiologic findings in three pediatric patients. Korean J Radiol. 2006;7(4):287–91.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© ISS 2018

Authors and Affiliations

  • Megan K. Mills
    • 1
    Email author
  • Bryn Putbrese
    • 1
  • Hailey Allen
    • 1
  • Sarah E. Stilwill
    • 1
  1. 1.Department of RadiologyUniversity of Utah Health Sciences CenterSalt Lake CityUSA

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