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Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up

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Abstract

Objective

Generalized cystic lymphangiomatosis is a particularly rare disease with variable involvement of skeletal and extraskeletal sites. The key role of imaging in the diagnosis of this disease is no longer in doubt. The aim of our study was to demonstrate the contribution of whole-body magnetic resonance imaging (WB-MRI) at the diagnostic stage and during the follow-up in the pediatric population.

Subjects and methods

Three children were included from 2008. The inclusion criteria were radiological images (conventional radiographs, computed tomography, and MRI) compatible with histological confirmation. Each child included received WB-MRI at the diagnosis stage and during follow-up.

Results

WB-MRI detected multiple hypointense T1 and hyperintense T2-STIR cystic bony lesions. One unnoticed mass in the retroperitoneum on computed tomography was easily detected by MRI. Mediastinal involvement was observed in one asymptomatic case. Histology was contributory in all cases. Preventive intramedullary nailings was done in one case. Follow-up WB-MRI detected new asymptomatic lesions in all cases. One child presented a third episode of lymphangitis of the right thigh during follow-up.

Conclusions

Due to its high sensitivity to diagnose, ability to eliminate differential diagnoses and make exhaustive lesions assessment, and its non-radiating character for long-term follow-up, WB-MRI is highly recommended for generalized cystic lymphangiomatosis in the pediatric population.

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References

  1. Aviv RI, McHugh K, Hunt J. Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. Clin Radiol. 2001;56:184–90.

    Article  PubMed  CAS  Google Scholar 

  2. Maki DD, Nesbit ME, Griffiths HJ. Diffuse lymphangiomatosis of bone. Australas Radiol. 1999;43:535–8.

    Article  PubMed  CAS  Google Scholar 

  3. Wunderbaldinger P, Paya K, Partik B, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. AJR Am J Roentgenol. 2000;174:827–32.

    Article  PubMed  CAS  Google Scholar 

  4. Lohrmann C, Foeldi E, Langer M. Assessment of the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging. Eur J Radiol. 2011;80:576–81.

    Article  PubMed  Google Scholar 

  5. Yang DH, Goo HW. Generalized lymphangiomatosis: radiologic findings in three pediatric patients. Korean J Radiol. 2006;7:287–91.

    Article  PubMed Central  PubMed  Google Scholar 

  6. Sokmensuer C, Sungur A, Tokgozoglu M, Ruacan S. Lymphangiomatosis of bone. A case report. Int Orthop. 1995;19:63–4.

    PubMed  CAS  Google Scholar 

  7. Renjen P, Kovanlikaya A, Narula N, Brill PW. Importance of MRI in the diagnosis of vertebral involvement in generalized cystic lymphangiomatosis. Skelet Radiol. 2014;43:1633–8.

    Article  Google Scholar 

  8. Cohen MD, Rougraff B, Faught P. Cystic angiomatosis of bone: MR findings. Pediatr Radiol. 1994;24:256–7.

    Article  PubMed  CAS  Google Scholar 

  9. Warin M, Bonnaire B, Deramond H. Generalized cystic lymphangiomatosis of bone with splenic involvement: minor variant of a systemic disease. J Radiol. 2010;91:907–10.

    Article  PubMed  CAS  Google Scholar 

  10. Olmos JM, Fernandez-Echevarria A, Sampedro MF, Landeras R, Gonzalez-Macias J. Disseminated bone lymphangiomatosis. Eur J Radiol. 2007;64:103–6.

    Article  Google Scholar 

  11. Klemola R, Karttunen A, Laine M, Liisanantti A, Halonen J, Ilkko E. Nontraumatic dens fracture in a patient with lymphangiomatosis: radiographic, CT and MR findings. Emerg Radiol. 2001;8:119–22.

    Article  Google Scholar 

  12. Lala S, Mulliken JB, Alomari AI, Fishman SJ, Kozakewich HP, Chaudry G. Gorham-Stout disease and generalized lymphatic anomaly—clinical, radiologic, and histologic differentiation. Skelet Radiol. 2013;42:917–24.

    Article  Google Scholar 

  13. Kwag E, Shim SS, Kim Y, Chang JH, Kim KC. CT features of generalized lymphangiomatosis in adult patients. Clin Imaging. 2013;37:723–7.

    Article  PubMed  Google Scholar 

  14. Marom EM, Moran CA, Munden RF. Generalized lymphangiomatosis. AJR Am J Roentgenol. 2004;182:1068.

    Article  PubMed  Google Scholar 

  15. Khung S, Budzik JF, Amzallag-Bellenger E, et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013;4:569–79.

    Article  PubMed Central  PubMed  Google Scholar 

  16. Bousson V, Rey-Jouvin C, Laredo JD, et al. Fibrous dysplasia and McCune-Albright syndrome: imaging for positive and differential diagnoses, prognosis, and follow-up guidelines. Eur J Radiol. 2014;83:1828–42.

    Article  PubMed  Google Scholar 

  17. Jee WH, Choi KH, Choe BY, Park JM, Shinn KS. Fibrous dysplasia: MR imaging characteristics with radiopathologic correlation. AJR Am J Roentgenol. 1996;167:1523–7.

    Article  PubMed  CAS  Google Scholar 

  18. Trenor 3rd CC, Chaudry G. Complex lymphatic anomalies. Semin Pediatr Surg. 2014;23:186–90.

    Article  PubMed  Google Scholar 

  19. Kotecha R, Mascarenhas L, Jackson HA, Venkatramani R. Radiological features of Gorham’s disease. Clin Radiol. 2012;67:782–8.

    Article  PubMed  CAS  Google Scholar 

  20. Rigopoulou A, Saifuddin A. Intraosseous hemangioma of the appendicular skeleton: imaging features of 15 cases, and a review of the literature. Skelet Radiol. 2012;41:1525–36.

    Article  Google Scholar 

  21. Schmidt GP, Reiser MF, Baur-Melnyk A. Whole-body imaging of the musculoskeletal system: the value of MR imaging. Skelet Radiol. 2007;36:1109–19.

    Article  Google Scholar 

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Authors and Affiliations

  1. Service de Radiologie, CHU Amiens-Picardie, 80054, Amiens Cedex 1, France

    Cécile Herruela-Suffee, Mathilde Warin, Mélody Castier-Amouyel, Florine Dallery, Bruno Bonnaire & Jean-Marc Constans

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  1. Cécile Herruela-Suffee
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  2. Mathilde Warin
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  3. Mélody Castier-Amouyel
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  4. Florine Dallery
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  5. Bruno Bonnaire
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  6. Jean-Marc Constans
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Correspondence to Cécile Herruela-Suffee.

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Herruela-Suffee, C., Warin, M., Castier-Amouyel, M. et al. Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up. Skeletal Radiol 45, 177–185 (2016). https://doi.org/10.1007/s00256-015-2280-8

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  • DOI: https://doi.org/10.1007/s00256-015-2280-8

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