- Author: Stuart Seiff, MD, FACS; Chief Editor: Hampton Roy, Sr, MD more...
Capillary hemangiomas are one of the most common benign orbital tumors of infancy. They are benign endothelial cell neoplasms that are typically absent at birth and characteristically have rapid growth in infancy with spontaneous involution later in life. This is in contrast to another known group of childhood vascular anomalies, vascular malformations. Vascular malformations, such as lymphangiomas and arteriovenous malformations, are present at birth and are characterized by very slow growth with persistence into adult life.[1, 2]
Capillary hemangiomas are believed to be hamartomatous proliferations of vascular endothelial cells. They are now thought to be of placental origin due to a unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Periorbital capillary hemangiomas follow a similar course to hemangiomas on other parts of the body. They generally exhibit 2 phases of growth, a proliferative phase and an involutional phase. The proliferative phase of rapid growth typically occurs from 8-18 months. Pathologically, it is characterized by an increased number of endothelial and mast cells, the latter being a stimulus for vessel growth. Endothelial cell proliferation returns to normal following the proliferation phase.
The involutional phase is characterized by slow regression of the hemangiomas. One half of all lesions will involute by age 5 years, and 75% will involute by age 7 years. During this phase, mast cell numbers decrease to normal and there is a decrease in endothelial and mast cell activity. These vascular spaces become lined with endothelial cells without muscular support.
As many as 50% of systemic capillary hemangiomas can occur in the head and neck region. Of all the patients who eventually develop capillary hemangiomas, 30% of them have evidence of their presence at birth, while 100% have manifest them by age 6 months.
Systemic involvement with hemangiomas can be a significant source of morbidity and mortality.
The Kasabach-Merritt syndrome is associated with mortality rates from 30-50%. These patients characteristically present with a consumption coagulopathy and thrombocytopenia secondary to platelet sequestration in large visceral hemangiomas. Disseminated intravascular coagulation may occur. These patients also experience high-output congestive heart failure. Nasopharyngeal hemangiomas can be associated with respiratory obstruction. Surgical intervention often is required in these cases.
PHACES syndrome is associated with large segmental hemangiomas of the head and neck. The acronym refers to P osterior fossa abnormalities (Dandy-Walker malformation), H emangiomas, A rterial lesions, C ardiac abnormalities (coarctation), E ye abnormalities, and Sternal clefting. As a spectrum of severity exists in these patients, they must be closely monitored for progressive cardiovascular and neurovascular deterioration.
The ophthalmic morbidity of hemangiomas is largely related to their space-occupying effects. The most devastating ophthalmic complication of hemangiomas relates to their ability to cause deprivation amblyopia in the affected eye if the lesion is large enough to directly occlude the visual axis. If the lesion is large enough to cause corneal distortion and astigmatism, anisometropic amblyopia will result.
Female patients outnumber male patients with hemangiomas by a ratio of 3:1.
Capillary hemangiomas are present in approximately 1-2% of neonates. All patients who eventually develop hemangiomas have them by age 6 months.
Haik BG, Karcioglu ZA, Gordon RA, et al. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. 1994 Mar-Apr. 38(5):399-426. [Medline].
Rosca TI, Pop MI, Curca M, et al. Vascular tumors in the orbit--capillary and cavernous hemangiomas. Ann Diagn Pathol. 2006 Feb. 10(1):13-19. [Medline].
Kavanagh EC, Heran MK, Peleg A, et al. Imaging of the natural history of an orbital capillary hemangioma. Orbit. 2006 Mar. 25(1):69-72. [Medline].
Fernández-Vega Cueto L, Tresserra F, de la Paz MF. De novo growth of a capillary hemangioma of the conjunctiva. Arch Soc Esp Oftalmol. 2012 Dec 20. [Medline].
Weiss AH, Kelly JP. Reappraisal of astigmatism induced by periocular capillary hemangioma and treatment with intralesional corticosteroid injection. Ophthalmology. 2008 Feb. 115(2):390-397.e1. [Medline].
Goggin M. Astigmatism and periocular hemangioma. Ophthalmology. 2008 Oct. 115(10):1854-5; author reply 1855. [Medline].
Wittenberg L, Ma P. Treatment of a von Hippel-Lindau retinal capillary hemangioma with photodynamic therapy. Can J Ophthalmol. 2008 Oct. 43(5):605-6. [Medline].
Assaf A, Nasr A, Johnson T. Corticosteroids in the management of adnexal hemangiomas in infancy and childhood. Ann Ophthalmol. 1992 Jan. 24(1):12-8. [Medline].
Boon LM, MacDonald DM, Mulliken JB. Complications of systemic corticosteroid therapy for problematic hemangioma. Plast Reconstr Surg. 1999 Nov. 104(6):1616-23. [Medline].
Cruz OA, Zarnegar SR, Myers SE. Treatment of periocular capillary hemangioma with topical clobetasol propionate. Ophthalmology. 1995 Dec. 102(12):2012-5. [Medline].
Elsas FJ, Lewis AR. Topical treatment of periocular capillary hemangioma. J Pediatr Ophthalmol Strabismus. 1994 May-Jun. 31(3):153-6. [Medline].
Kushner BJ. Intralesional corticosteroid injection for infantile adnexal hemangioma. Am J Ophthalmol. 1982 Apr. 93(4):496-506. [Medline].
O'Keefe M, Lanigan B, Byrne SA. Capillary haemangioma of the eyelids and orbit: a clinical review of the safety and efficacy of intralesional steroid. Acta Ophthalmol Scand. 2003 Jun. 81(3):294-8. [Medline].
Friling R, Axer-Siegel R, Ben-Amitai D, et al. Intralesional and sub-Tenon's infusion of corticosteroids for treatment of refractory periorbital and orbital capillary haemangioma. Eye. 2008 Nov 7. [Medline].
Thoumazet F, Léauté-Labrèze C, Colin J, Mortemousque B. Efficacy of systemic propranolol for severe infantile haemangioma of the orbit and eyelid: a case study of eight patients. Br J Ophthalmol. 2012 Mar. 96(3):370-4. [Medline].
Aletaha M, Salour H, Bagheri A, Raffati N, Amouhashemi N. Successful treatment of orbital hemangioma with propranolol in a 5-year-old girl. Orbit. 2012 Feb. 31(1):18-20. [Medline].
Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic Surg. 1986 Apr. 17(4):229-31. [Medline].
Weiss AH. Adrenal suppression after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. 1989 May 15. 107(5):518-22. [Medline].
Deboer MD, Boston BA. Failure-to-thrive in an infant following injection of capillary hemangioma with triamcinolone acetonide. Clin Pediatr (Phila). 2008 Apr. 47(3):296-9. [Medline].
Barlow CF, Priebe CJ, Mulliken JB, et al. Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy. J Pediatr. 1998 Mar. 132(3 Pt 1):527-30. [Medline].
Al Dhaybi R, Superstein R, Milet A, Powell J, Dubois J, McCuaig C, et al. Treatment of periocular infantile hemangiomas with propranolol: case series of 18 children. Ophthalmology. 2011 Jun. 118(6):1184-8. [Medline].
Missoi TG, Lueder GT, Gilbertson K, Bayliss SJ. Oral propranolol for treatment of periocular infantile hemangiomas. Arch Ophthalmol. 2011 Jul. 129(7):899-903. [Medline].
Fridman G, Grieser E, Hill R, Khuddus N, Bersani T, Slonim C. Propranolol for the treatment of orbital infantile hemangiomas. Ophthal Plast Reconstr Surg. 2011 May-Jun. 27(3):190-4. [Medline].
Ni N, Guo S, Langer P. Current concepts in the management of periocular infantile (capillary) hemangioma. Curr Opin Ophthalmol. 2011 Sep. 22 (5):419-25. [Medline].
Ni N, Langer P, Wagner R, Guo S. Topical timolol for periocular hemangioma: report of further study. Arch Ophthalmol. 2011 Mar. 129 (3):377-9. [Medline].
Guo S, Ni N. Topical treatment for capillary hemangioma of the eyelid using beta-blocker solution. Arch Ophthalmol. 2010 Feb. 128 (2):255-6. [Medline].
Xue K, Hildebrand GD. Deep periocular infantile capillary hemangiomas responding to topical application of timolol maleate, 0.5%, drops. JAMA Ophthalmol. 2013 Sep. 131 (9):1246-8. [Medline].
Deans RM, Harris GJ, Kivlin JD. Surgical dissection of capillary hemangiomas. An alternative to intralesional corticosteroids. Arch Ophthalmol. 1992 Dec. 110(12):1743-7. [Medline].
Slaughter K, Sullivan T, Boulton J, et al. Early surgical intervention as definitive treatment for ocular adnexal capillary haemangioma. Clin Experiment Ophthalmol. 2003 Oct. 31(5):418-23. [Medline].