Skip to main content
SpringerLink
Log in

Orbital Vascular Anomalies

  • Chapter
  • First Online:
Clinical Ophthalmic Oncology

  • 477 Accesses

Abstract

Vascular anomalies comprise vascular neoplasms that result from active cell proliferation and vascular malformations which are congenital defects in vascular development. The distinction between tumors and malformations was recognized by the International Society for the Study of Vascular Anomalies (ISSVA) to provide a more clinically useful classification scheme and internationally consistent nomenclature. It focuses on a systematic approach to vascular lesions that correlates predictably with clinical history, disease course, and treatment options. Vascular tumors are further classified as benign, locally aggressive (borderline), or malignant. The most common of these is infantile hemangioma. Vascular malformations, on the other hand, are named after the vessel type(s) that compose them, i.e., venous malformation, lymphatic malformation, arteriovenous malformation, and assorted variations. Fistulae represent related vascular pathologies. Orbital vascular anomalies present unique challenges owing to the compartment they occupy and the critical structures therein. These lesions can be classified independent of tissue type, based on flow radiographic and clinical flow characteristics.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 69.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 89.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 119.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–22.

    Article  CAS  PubMed  Google Scholar 

  2. Haggstrom AN, Drolet BA, Baselga E, et al. Hemangioma Investigator Group. Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics. J Pediatr. 2007;150(3):291–4.

    Article  PubMed  Google Scholar 

  3. North PE, Waner M, Mizeracki A, et al. GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas. Hum Pathol. 2000;31(1):11–22.

    Article  CAS  PubMed  Google Scholar 

  4. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18(6):894–900.

    Article  CAS  PubMed  Google Scholar 

  5. Fay A, Santiago YM, Waner M. Infantile hemangiomas and other vascular neoplasms. In: Fay A, Dolman P. Diseases and disorders of the orbit and ocular adnexa. Elsevier. 2018.

    Google Scholar 

  6. Margileth AM, Museles M. Cutaneous hemangiomas in children. Diagnosis and conservative management. JAMA. 1965;194(5):523–6.

    Article  CAS  PubMed  Google Scholar 

  7. Zallmann M, Leventer RJ, Mackay MT, et al. Screening for Sturge-Weber syndrome: a state-of-the-art review. Pediatr Dermatol. 2018;35(1):30–42.

    Article  PubMed  Google Scholar 

  8. Robb RM. Refractive errors associated with hemangiomas of the eyelids and orbit in infancy. Am J Ophthalmol. 1977;83(1):52–8.

    Article  CAS  PubMed  Google Scholar 

  9. Schwartz SR, Blei F, Ceisler E, et al. Risk factors for amblyopia in children with capillary hemangiomas of the eyelids and orbit. J AAPOS. 2006;10(3):262–8.

    Article  PubMed  Google Scholar 

  10. Haik BG, Karcioglu ZA, Gordon RA, et al. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. 1994;38(5):399–426.

    Article  CAS  PubMed  Google Scholar 

  11. Lowe LH, Marchant TC, Rivard DC, et al. Vascular malformations: classification and terminology the radiologist needs to know. Semin Roentgenol. 2012;47(2):106–17.

    Article  PubMed  Google Scholar 

  12. Waner M, Suen JY. A classification of congenital vascular lesions. . In: Waner M, Suen JY, eds. Hemangiomas and vascular malformations of the head and neck New York: J: Wiley-Liss, 1999.

    Google Scholar 

  13. Pratt AG. Birthmarks in infants. AMA Arch Derm Syphilol. 1953;67(3):302–5.

    Article  CAS  PubMed  Google Scholar 

  14. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649–51.

    Article  CAS  PubMed  Google Scholar 

  15. Fay A, Nguyen J, Waner M. Conceptual approach to the management of infantile hemangiomas. J Pediatr. 2010;157(6):881–8 e1-5.

    Article  PubMed  Google Scholar 

  16. Dieterich-Miller CA, Cohen BA, Liggett J. Behavioral adjustment and self-concept of young children with hemangiomas. Pediatr Dermatol. 1992;9(3):241–5.

    Article  CAS  PubMed  Google Scholar 

  17. Ruttum MS, Abrams GW, Harris GJ, et al. Bilateral retinal embolization associated with intralesional corticosteroid injection for capillary hemangioma of infancy. J Pediatr Ophthalmol Strabismus. 1993;30(1):4–7.

    Article  CAS  PubMed  Google Scholar 

  18. Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic Surg. 1986;17(4):229–31.

    CAS  PubMed  Google Scholar 

  19. Egbert JE, Schwartz GS, Walsh AW. Diagnosis and treatment of an ophthalmic artery occlusion during an intralesional injection of corticosteroid into an eyelid capillary hemangioma. Am J Ophthalmol. 1996;121(6):638–42.

    Article  CAS  PubMed  Google Scholar 

  20. Droste PJ, Ellis FD, Sondhi N, et al. Linear subcutaneous fat atrophy after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. 1988;105(1):65–9.

    Article  CAS  PubMed  Google Scholar 

  21. Sutula FC, Glover AT. Eyelid necrosis following intralesional corticosteroid injection for capillary hemangioma. Ophthalmic Surg. 1987;18(2):103–5.

    CAS  PubMed  Google Scholar 

  22. Sagi L, Zvulunov A, Lapidoth M, et al. Efficacy and safety of propranolol for the treatment of infantile hemangioma: a presentation of ninety-nine cases. Dermatology. 2014;228(2):136–44.

    Article  PubMed  Google Scholar 

  23. Lou Y, Peng WJ, Cao Y, et al. The effectiveness of propranolol in treating infantile haemangiomas: a meta-analysis including 35 studies. Br J Clin Pharmacol. 2014;78(1):44–57.

    Article  CAS  PubMed  Google Scholar 

  24. Bauman NM, McCarter RJ, Guzzetta PC, et al. Propranolol vs prednisolone for symptomatic proliferating infantile hemangiomas: a randomized clinical trial. JAMA Otolaryngol Head Neck Surg. 2014;140(4):323–30.

    Article  PubMed  Google Scholar 

  25. Boon LM, Enjolras O, Mulliken JB. Congenital hemangioma: evidence of accelerated involution. J Pediatr. 1996;128(3):329–35.

    Article  CAS  PubMed  Google Scholar 

  26. Ooi KG, Wenderoth JD, Francis IC, et al. Selective embolization and resection of a large noninvoluting congenital hemangioma of the lower eyelid. Ophthalmic Plast Reconstr Surg. 2009;25(2):111–4.

    Article  PubMed  Google Scholar 

  27. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239–52.

    Article  PubMed  Google Scholar 

  28. Demirci H, Christanson MD. Eyelid angiosarcoma: a case report and review of the literature. Middle East Afr J Ophthalmol. 2013;20(3):259–62.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet Oncol. 2010;11(10):983–91.

    Article  PubMed  Google Scholar 

  30. Morgan MB, Swann M, Somach S, et al. Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol. 2004;50(6):867–74.

    Article  PubMed  Google Scholar 

  31. Harris GJ. Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Orbital society. Am J Ophthalmol. 1999;127(4):453–5.

    Article  CAS  PubMed  Google Scholar 

  32. Wiegand S, Eivazi B, Bloch LM, et al. Lymphatic malformations of the orbit. Clin Exp Otorhinolaryngol. 2013;6(1):30–5.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Greene AK, Burrows PE, Smith L, et al. Periorbital lymphatic malformation: clinical course and management in 42 patients. Plast Reconstr Surg. 2005;115(1):22–30.

    CAS  PubMed  Google Scholar 

  34. Hall N, Ade-Ajayi N, Brewis C, et al. Is intralesional injection of OK-432 effective in the treatment of lymphangioma in children? Surgery. 2003;133(3):238–42.

    Article  PubMed  Google Scholar 

  35. Yue H, Qian J, Elner VM, et al. Treatment of orbital vascular malformations with intralesional injection of pingyangmycin. Br J Ophthalmol. 2013;97(6):739–45.

    Article  PubMed  Google Scholar 

  36. Gandhi NG, Lin LK, O’Hara M. Sildenafil for pediatric orbital lymphangioma. JAMA Ophthalmol. 2013;131(9):1228–30.

    Article  PubMed  Google Scholar 

  37. Swetman GL, Berk DR, Vasanawala SS, et al. Sildenafil for severe lymphatic malformations. N Engl J Med. 2012;366(4):384–6.

    Article  CAS  PubMed  Google Scholar 

  38. Ernemann U, Westendorff C, Troitzsch D, et al. Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations. AJNR Am J Neuroradiol. 2004;25(10):1792–5.

    PubMed  PubMed Central  Google Scholar 

  39. Goldberg RA, Garcia GH, Duckwiler GR. Combined embolization and surgical treatment of arteriovenous malformation of the orbit. Am J Ophthalmol. 1993;116(1):17–25.

    Article  CAS  PubMed  Google Scholar 

  40. Werner JA, Dunne AA, Folz BJ, et al. Current concepts in the classification, diagnosis and treatment of hemangiomas and vascular malformations of the head and neck. Eur Arch Otorhinolaryngol. 2001;258(3):141–9.

    Article  CAS  PubMed  Google Scholar 

  41. Osaki T, Fay A, Waner M. Vascular malformations. In: Fay A, Dolman P, editors. Diseases and disorders of the orbit and ocular adnexa. Edinburgh: Elsevier; 2018.

    Google Scholar 

  42. Multimodal management of arteriovenous malformations of the basal ganglia and thalamus: factors affecting obliteration and outcome. J Neurosurg. 2018:1–10.

    Google Scholar 

  43. Chelliah MP, Do HM, Zinn Z, et al. Management of complex arteriovenous malformations using a novel combination therapeutic algorithm. JAMA Dermatol. 2018;154(11):1316–9.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Calandriello L, Grimaldi G, Petrone G, et al. Cavernous venous malformation (cavernous hemangioma) of the orbit: current concepts and a review of the literature. Surv Ophthalmol. 2017;62(4):393–403.

    Article  PubMed  Google Scholar 

  45. Osaki TH, Jakobiec FA, Mendoza PR, et al. Immunohistochemical investigations of orbital infantile hemangiomas and adult encapsulated cavernous venous lesions (malformation versus hemangioma). Ophthalmic Plast Reconstr Surg. 2013;29(3):183–95.

    Article  PubMed  Google Scholar 

  46. Bruder E, Perez-Atayde AR, Jundt G, et al. Vascular lesions of bone in children, adolescents, and young adults. A clinicopathologic reappraisal and application of the ISSVA classification. Virchows Arch. 2009;454(2):161–79.

    Article  PubMed  Google Scholar 

  47. McNab AA, Wright JE. Cavernous haemangiomas of the orbit. Aust N Z J Ophthalmol. 1989;17(4):337–45.

    Article  CAS  PubMed  Google Scholar 

  48. Xian J, Zhang Z, Wang Z, et al. Evaluation of MR imaging findings differentiating cavernous haemangiomas from schwannomas in the orbit. Eur Radiol. 2010;20(9):2221–8.

    Article  PubMed  PubMed Central  Google Scholar 

  49. Kim JW, Im YS, Nam DH, et al. Preliminary report of multisession gamma knife radiosurgery for benign perioptic lesions: visual outcome in 22 patients. J Korean Neurosurg Soc. 2008;44(2):67–71.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Goh ASC, Kim YD, Woo KI, Lee JI. Benign orbital apex tumors treated with multisession gamma knife radiosurgery. Ophthalmology. 2013;120(3):635–41.

    Article  PubMed  Google Scholar 

  51. Young SM, Kim KH, Kim Y-D, et al. Orbital apex venous cavernous malformation with optic neuropathy – treatment with multisession Gamma knife radiosurgery. Br J Ophthalmol. 2019;. epub ahead of print

    Google Scholar 

  52. Chhabra N, Wu AW, Fay A, et al. Endoscopic resection of orbital hemangiomas. Int Forum Allergy Rhinol. 2014;4(3):251–5.

    Article  PubMed  Google Scholar 

  53. Kiratli H, Bulur B, Bilgic S. Transconjunctival approach for retrobulbar intraconal orbital cavernous hemangiomas. Orbital surgeon’s perspective. Surg Neurol. 2005;64(1):71–4.

    Article  PubMed  Google Scholar 

  54. Brusati R, Goisis M, Biglioli F, et al. Surgical approaches to cavernous haemangiomas of the orbit. Br J Oral Maxillofac Surg. 2007;45(6):457–62.

    Article  CAS  PubMed  Google Scholar 

  55. Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases. Hum Pathol. 1982;13:210218.

    Article  Google Scholar 

  56. Fletcher CDM, Bridge JA, Hogendoorn P, et al. WHO classification of tumours. In: WHO classification of tumours of soft tissue and bone, vol. 5. 4th ed: IARC; Geneva, Switzerland, 2013. http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4005#

  57. Karcioglu ZA, Nasr AM, Haik BG. Orbital hemangiopericytoma: clinical and morphologic features. Am J Ophthalmol. 1997;124(5):661–72.

    Article  CAS  PubMed  Google Scholar 

  58. Bernardini FP, de Conciliis C, Schneider S, et al. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology. 2003;110(7):1442–8.

    Article  PubMed  Google Scholar 

  59. Girnita L, Sahlin S, Orrego A, et al. Malignant solitary fibrous tumour of the orbit. Acta Ophthalmol. 2009;87(4):464–7.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Eye Institute, St. Luke’s Medical Center, Quezon City, Metro Manila, Philippines

    Yvette Marie Santiago

  2. Harvard Medical School, Department of Ophthalmology, Boston, MA, USA

    Aaron Fay

Authors
  1. Yvette Marie Santiago
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aaron Fay
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Division of Orbital and Oculofacial Plastic Surgery, Cole Eye Institute Cleveland Clinic Foundation, Cleveland, OH, USA

    Catherine J. Hwang

  2. Division of Facial Cosmetic and Reconstructive Surgery, University of Utah, Salt Lake City, UT, USA

    Bhupendra C.K. Patel

  3. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland, OH, USA

    Arun D. Singh

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Santiago, Y.M., Fay, A. (2019). Orbital Vascular Anomalies. In: Hwang, C.J., Patel, B.C., Singh, A.D. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-13558-4_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-13558-4_8

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-13557-7

  • Online ISBN: 978-3-030-13558-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation