Orbital Vascular Anomalies

  • Yvette Marie Santiago
  • Aaron Fay


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.


Orbital vascular anomalies Orbital vascular tumors Hemangioma Orbital vascular malformations Lymphatic malformations Venous malformations AV fistula AV malformation Hemangiopericytoma 


  1. 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.PubMedGoogle Scholar
  2. 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.PubMedGoogle Scholar
  3. 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.PubMedGoogle Scholar
  4. 4.
    Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18(6):894–900.PubMedGoogle Scholar
  5. 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. 6.
    Margileth AM, Museles M. Cutaneous hemangiomas in children. Diagnosis and conservative management. JAMA. 1965;194(5):523–6.PubMedGoogle Scholar
  7. 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.PubMedGoogle Scholar
  8. 8.
    Robb RM. Refractive errors associated with hemangiomas of the eyelids and orbit in infancy. Am J Ophthalmol. 1977;83(1):52–8.PubMedGoogle Scholar
  9. 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.PubMedGoogle Scholar
  10. 10.
    Haik BG, Karcioglu ZA, Gordon RA, et al. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. 1994;38(5):399–426.PubMedGoogle Scholar
  11. 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.PubMedGoogle Scholar
  12. 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. 13.
    Pratt AG. Birthmarks in infants. AMA Arch Derm Syphilol. 1953;67(3):302–5.PubMedGoogle Scholar
  14. 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.PubMedGoogle Scholar
  15. 15.
    Fay A, Nguyen J, Waner M. Conceptual approach to the management of infantile hemangiomas. J Pediatr. 2010;157(6):881–8 e1-5.PubMedGoogle Scholar
  16. 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.PubMedGoogle Scholar
  17. 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.PubMedGoogle Scholar
  18. 18.
    Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic Surg. 1986;17(4):229–31.PubMedGoogle Scholar
  19. 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.PubMedGoogle Scholar
  20. 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.PubMedGoogle Scholar
  21. 21.
    Sutula FC, Glover AT. Eyelid necrosis following intralesional corticosteroid injection for capillary hemangioma. Ophthalmic Surg. 1987;18(2):103–5.PubMedGoogle Scholar
  22. 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.PubMedGoogle Scholar
  23. 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.PubMedGoogle Scholar
  24. 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.PubMedGoogle Scholar
  25. 25.
    Boon LM, Enjolras O, Mulliken JB. Congenital hemangioma: evidence of accelerated involution. J Pediatr. 1996;128(3):329–35.PubMedPubMedCentralGoogle Scholar
  26. 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.PubMedGoogle Scholar
  27. 27.
    Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239–52.PubMedGoogle Scholar
  28. 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.PubMedPubMedCentralGoogle Scholar
  29. 29.
    Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet Oncol. 2010;11(10):983–91.Google Scholar
  30. 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.PubMedGoogle Scholar
  31. 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.PubMedGoogle Scholar
  32. 32.
    Wiegand S, Eivazi B, Bloch LM, et al. Lymphatic malformations of the orbit. Clin Exp Otorhinolaryngol. 2013;6(1):30–5.PubMedPubMedCentralGoogle Scholar
  33. 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.PubMedGoogle Scholar
  34. 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.PubMedGoogle Scholar
  35. 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.PubMedGoogle Scholar
  36. 36.
    Gandhi NG, Lin LK, O’Hara M. Sildenafil for pediatric orbital lymphangioma. JAMA Ophthalmol. 2013;131(9):1228–30.PubMedGoogle Scholar
  37. 37.
    Swetman GL, Berk DR, Vasanawala SS, et al. Sildenafil for severe lymphatic malformations. N Engl J Med. 2012;366(4):384–6.PubMedGoogle Scholar
  38. 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.PubMedGoogle Scholar
  39. 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.PubMedGoogle Scholar
  40. 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.PubMedGoogle Scholar
  41. 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. 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. 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.PubMedPubMedCentralGoogle Scholar
  44. 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.PubMedGoogle Scholar
  45. 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.PubMedGoogle Scholar
  46. 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.PubMedGoogle Scholar
  47. 47.
    McNab AA, Wright JE. Cavernous haemangiomas of the orbit. Aust N Z J Ophthalmol. 1989;17(4):337–45.PubMedGoogle Scholar
  48. 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.PubMedPubMedCentralGoogle Scholar
  49. 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.PubMedPubMedCentralGoogle Scholar
  50. 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.PubMedGoogle Scholar
  51. 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 printGoogle Scholar
  52. 52.
    Chhabra N, Wu AW, Fay A, et al. Endoscopic resection of orbital hemangiomas. Int Forum Allergy Rhinol. 2014;4(3):251–5.PubMedGoogle Scholar
  53. 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.PubMedGoogle Scholar
  54. 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.PubMedGoogle Scholar
  55. 55.
    Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases. Hum Pathol. 1982;13:210218.Google Scholar
  56. 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.
  57. 57.
    Karcioglu ZA, Nasr AM, Haik BG. Orbital hemangiopericytoma: clinical and morphologic features. Am J Ophthalmol. 1997;124(5):661–72.PubMedGoogle Scholar
  58. 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.PubMedGoogle Scholar
  59. 59.
    Girnita L, Sahlin S, Orrego A, et al. Malignant solitary fibrous tumour of the orbit. Acta Ophthalmol. 2009;87(4):464–7.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Yvette Marie Santiago
    • 1
  • Aaron Fay
    • 2
  1. 1.Eye InstituteSt. Luke’s Medical CenterQuezon CityPhilippines
  2. 2.Harvard Medical School, Department of OphthalmologyBostonUSA

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