Skip to main content

Advertisement

Log in

Image-Guided Percutaneous Bleomycin and Bevacizumab Sclerotherapy of Orbital Lymphatic Malformations in Children

  • Clinical Investigation
  • Non-Vascular Interventions
  • Published:
CardioVascular and Interventional Radiology Aims and scope Submit manuscript

Abstract

Purpose

To evaluate the effectiveness and safety of image-guided percutaneous sclerotherapy using bleomycin for macrocystic and bevacizumab (Avastin™) for microcystic orbital lymphatic malformations in children.

Materials and Methods

Between October 2015 and July 2018, we prospectively evaluated 10 pediatric patients who presented clinically and radiologically with lymphatic malformations and were treated with percutaneous sclerotherapy. Patients with venous malformations were excluded. Eight females and two males with ages ranging from 3 to 17 years (mean: 8.8, SD: 4.9) were included. Guided with ultrasound and fluoroscopy, macrocysts were treated with bleomycin instillation. For microcystic components in three patients, bevacizumab was injected intralesional. All patients underwent ultrasound and non-contrast MRI to evaluate response to treatment after 6 weeks.

Results

The malformations were macrocystic in seven patients and complex (macro/microcystic) in three. Twenty sclerotherapy sessions were performed, (range: 1–3 sessions, mean: 2, SD: 0.8). Clinically, there was a significant reduction in the proptosis after treatment (P = 0.007) and dystopia (P = 0.018). The local radiological response showed a reduction in the maximum lesions diameters and volumes after treatment (P = 0.005 and 0.005, respectively). Two of the three patients treated with bevacizumab showed a reduction in the lesions volumes by 90.4% and 63.4%, respectively, whereas one patient did not show volume reduction. Transient periorbital edema and ecchymosis occurred following the procedure with no major complications encountered. Follow-up ranged from 9–33 months, mean: 20.3, SD: 7.4.

Conclusion

Bleomycin sclerotherapy is a safe and effective treatment for orbital macrocystic lymphatic malformations. Further use of bevacizumab for microcystic lesions in a larger series is required to outline its efficacy and safety.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Katz SE, Rootman J, Vangveeravong S, Graeb D. Combined venous lymphatic malformations of the orbit (so-called lymphangiomas): association with noncontiguous intracranial vascular anomalies. Ophthalmology. 1998;105(1):176–84.

    Article  CAS  PubMed  Google Scholar 

  2. Greene AK, Burrows PE, Smith L, et al. Periorbital lymphatic malformation: clinical course and management in 42 patients. Plast Reconstr Surg. 2005;115:22–30.

    Article  CAS  PubMed  Google Scholar 

  3. Mulligan PR, Prajapati HJS, Martin LG, Patel TH. Vascular anomalies: classification, imaging characteristics and implications for interventional radiology treatment approaches. Br J Radiol. 2014;87:1035.

    Article  Google Scholar 

  4. Chung EM, Smirniotopoulos JG, Specht CS, Schroeder JW, Cube R. From the archives of the AFIP: pediatric orbit tumors and tumorlike lesions: non-osseous lesions of the extra ocular orbit. Radiographics. 2007;27:1777–99.

    Article  Google Scholar 

  5. Rootman J, Heran MKS, Graeb DA. Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies. Ophthal Plast Reconstr Surg. 2014;30:91–104.

    Article  PubMed  Google Scholar 

  6. Tunc M, Sadri E, Char DH. Orbital lymphangiomas: an analysis of 26 patients. Br J Ophthalmol. 1999;83:76–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Gündüz K, Demirel S, Yagmurlu B, Erden E. Correlation of surgical outcome with neuroimaging findings in periocular lymphangiomas. Ophthalmology (Internet). 2006;113(7):1231–8.

    Google Scholar 

  8. Nassiri N, Rootman J, Rootman DB, Goldberg RA. Orbital lymphaticovenous malformations: current and future treatments. Surv Ophthalmol. 2015;60:383–405.

    Article  PubMed  Google Scholar 

  9. Güneyli S, Ceylan N, Bayraktaroğlu S, Acar T, Savaş R. Imaging findings of vascular lesions in the head and neck. Diagn Interv Radiol. 2014;20(5):432–7.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Harris GJ, Sakol PJ, Bonavolonta G, et al. An analysis of thirty cases of orbital lymphangiomas: pathophysiologic considerations and management recommendations. Ophthalmology. 1990;97:1583–92.

    Article  CAS  PubMed  Google Scholar 

  11. Barnacle AM, Theodorou M, Maling SJ, Abou-Rayyah Y. Sclerotherapy treatment of orbital lymphatic malformations: a large single-center experience. Br J Ophthalmol. 2016;100(2):204–8.

    Article  PubMed  Google Scholar 

  12. Wojno TH. Sotradecol (sodium tetradecyl sulfate) injection of orbital lymphangioma. Ophthal Plast Reconstr Surg. 1999;15:432–7.

    Article  CAS  PubMed  Google Scholar 

  13. Svendsen PA, Wikholm G, Rodriguez M, et al. Direct puncture and sclerotherapy with Sotradecol. Orbital lymphatic malformations. Interv Neuroradiol. 2001;30:193–9.

    Article  Google Scholar 

  14. Poonyathalang A, Preechawat P, Jiarakongmun P, et al. Sclerosing therapy for orbital lymphangioma using sodium tetradecyl sulfate. Jpn J Ophthalmol. 2008;52:298–304.

    Article  CAS  PubMed  Google Scholar 

  15. Hill RH, Shiels WE, Foster JA, et al. Percutaneous drainage and ablation as first-line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Recontr Surg. 2012;28:119–25.

    Article  Google Scholar 

  16. Chiramel GK, Keshava SN, Moses V, Mammen S, David S, Sen S. Percutaneous sclerotherapy of congenital slow-flow vascular malformations of the orbit. Cardiovasc Intervent Radiol. 2015;38:270–9.

    Article  PubMed  Google Scholar 

  17. Patel KC, Kalantzis G, El-Hindy N, Chang BY. Sclerotherapy for orbital lymphangioma—case series and literature review. In Vivo. 2017;31(2):263–6.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Shen CY, Wu MC, Tyan YS, Ou CH, Chen TY, Wong HF. Preliminary experience of percutaneous intralesional bleomycin injection for the treatment of orbital lymphatic-venous malformation refractory to surgery. Clin Radiol. 2012;67:182–4.

    Article  PubMed  Google Scholar 

  19. Gooding C, Meyer D. Intralesional bleomycin: a potential treatment for refractory orbital lymphangiomas. Ophthal Plast Reconstr Surg. 2014;30:65–7.

    Google Scholar 

  20. Paramasivam S, Fay A, Fifi J, Berenstein A. O-015 Image guided bleomycin sclerotherapy for orbital lymphatic malformation. J Neuro Intervent Surg. 2014;6:A8–9.

    Google Scholar 

  21. Harmoush S, Chinnardurai P, El Salek K, et al. Multimodality image-guided sclerotherapy of low-flow orbital vascular malformations: report of single-center experience. J Vasc Interv Radiol. 2016;27(7):987–95.

    Article  PubMed  Google Scholar 

  22. Raichura N, Alam S, Noronha V, Mukherjee B. A prospective study of the role of intralesional bleomycin in orbital lymphangioma. J AAPOS. 2017;21:146–51.

    Article  PubMed  Google Scholar 

  23. Hanif AM, Saunders JA, Hawkins CM, Wojno TH, Kim HJ. Use of percutaneous bleomycin sclerotherapy for orbital lymphatic malformations. Orbit. 2018;14:1–7.

    Article  Google Scholar 

  24. Suzuki Y, Obana A, Gohto Y, et al. Management of orbital lymphangioma using intralesional injection of OK-432. Br J Ophthalmol. 2000;84:614–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Woo YJ, Kim CY, Sgrignoli B, Yoon JS. Orbital lymphangioma: characteristics and treatment outcomes of 12 cases. Korean J Ophthalmol. 2017;31(3):194–201.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Yue H, Qian J, Elner VM, et al. Treatment of orbital vascular malformations with intralesional injection of pingyangmycin. Br J Ophthalmol. 2013;97:739–45.

    Article  PubMed  Google Scholar 

  27. Schwarcz RM, Ben Simon GJ, Cook T, et al. Sclerosing therapy as first-line treatment for low flow vascular lesions of the orbit. Am J Ophthalmol. 2006;141:333–9.

    Article  PubMed  Google Scholar 

  28. Thomas DM, Wieck MM, Grant CN, et al. Doxycycline sclerotherapy is superior in the treatment of pediatric lymphatic malformations. J Vasc Interv Radiol. 2016;27(12):1846–56.

    Article  PubMed  Google Scholar 

  29. Orford J, Barker A, Thonell S, King P, Murphy J. Bleomycin therapy for cystic hygroma. J Pediatr Surg. 1995;30:1282–7.

    Article  CAS  PubMed  Google Scholar 

  30. Baskin D, Tander B, Bankaoglu M. Local bleomycin injection in the treatment of lymphangioma. Eur J Pediatr Surg. 2005;15:383–6.

    Article  CAS  PubMed  Google Scholar 

  31. Muir T, Kirsten M, Fourie P, Dippenaar N, Ionescu GO. Intralesional bleomycin injection (IBI) treatment for haemangiomas and vascular malformations. Pediatr Surg Int. 2004;19:766–73.

    Article  CAS  PubMed  Google Scholar 

  32. Horbach SE, Rigter IM, Smitt JH, et al. Intralesional bleomycin injections for vascular malformations: a systematic review and meta-analysis. Plast Reconstr Surg. 2016;137:244–56.

    Article  CAS  PubMed  Google Scholar 

  33. Ferrara N, Hillan KJ, Gerber HP, Novotny W. Discovery and development of bevacizumab, an anti-VEGF antibody for treating cancer. Nat Rev Drug Discov. 2004;3(5):391–400.

    Article  CAS  Google Scholar 

  34. Pasqualetti G, Danesi R, Del Tacca M, Bocci G. Vascular endothelial growth factor pharmacogenetics: a new perspective for anti-angiogenic therapy. Pharmacogenomics. 2007;8(1):49–66.

    Article  CAS  PubMed  Google Scholar 

  35. Rosenfeld PJ, Brown DM, Heier JS, Boyer DS, Kaiser PK, Chung CY, et al. Ranibizumab for neovascular age-related macular degeneration. N Engl J Med. 2006;355(14):1419–31.

    Article  CAS  PubMed  Google Scholar 

  36. Arevalo JF, Lasave AF, Wu L, Diaz-Llopis M, Gallego-Pinazo R, Alezzandrini AA, et al. Intravitreal bevacizumab plus grid laser photocoagulation or intravitreal bevacizumab or grid laser photocoagulation for diffuse diabetic macular edema: results of the Pan-American Collaborative Retina Study Group at 24 months. Retina. 2013;33(2):403–13.

    Article  CAS  PubMed  Google Scholar 

  37. Wu L, Arevalo JF, Berrocal MH, Maia M, Roca JA, Morales-Cantón V, et al. Comparison of two doses of intravitreal bevacizumab as primary treatment for macular edema secondary to central retinal vein occlusion: results of the pan American collaborative retina study group at 24 months. Retina. 2010;30(7):1002–11.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors wish to acknowledge the help provided by Dr. Alex M Barnacle, Consultant Interventional Radiologist, Great Ormond Street Hospital for Children, NHS, London, UK. We would like to thank her for the expert advice and encouragement throughout this work. Her willingness to give her time so generously in editing the manuscript has been very much appreciated.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Omar Abdelaziz.

Ethics declarations

Conflicts of interest

On behalf of all authors, the corresponding author states that there are no conflicts of interest.

Ethical Standards

All procedures performed were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all legal guardians of the children.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Abdelaziz, O., Hassan, F., Elessawy, K. et al. Image-Guided Percutaneous Bleomycin and Bevacizumab Sclerotherapy of Orbital Lymphatic Malformations in Children. Cardiovasc Intervent Radiol 42, 433–440 (2019). https://doi.org/10.1007/s00270-018-2128-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00270-018-2128-4

Keywords

Navigation