eMedicine Specialties > Ophthalmology > Orbit
Hemangioma, Capillary
Updated: Jun 18, 2009
Introduction
Background
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
Pathophysiology
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.3
Frequency
United States
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.
Mortality/Morbidity
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.
- 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.
Sex
Female patients outnumber male patients with hemangiomas by a ratio of 3:1.
Age
Capillary hemangiomas are present in approximately 1-2% of neonates. All patients who eventually develop hemangiomas have them by age 6 months.
Clinical
History
- The history can be quite variable in this group of patients. Typically, parents may notice a red spot growing in size and thickness in the periorbital area.
- The parents may be acutely aware of a gradual inability of the child to open an affected eye due to progressive involvement of the eyelid.
Physical
- Patients usually present with a unilateral, superonasal eyelid or brow lesion. It typically blanches with pressure, unlike the lesions seen with port-wine stains. The mass lesion may be sufficient to cause a ptosis of the involved eyelid. Alternatively, if the lesion extends posteriorly in the orbit, proptosis and visual loss may be present.
- Examination of the visual system may reveal decreased visual acuity on the ipsilateral side.
- Unfortunately, amblyopia is seen in 43-60% of patients with eyelid hemangiomas.
- Anisometropia also can be found on examination as a result of the mass effect on the cornea. The axis of plus cylinder is usually toward the hemangioma.
Causes
Capillary hemangiomas are believed to be hamartomatous proliferations of vascular endothelial cells.
Differential Diagnoses
Hemangioma, Cavernous
Other Problems to Be Considered
Rhabdomyosarcoma
Lymphangioma
Neuroblastoma
Arterial venous malformations
Workup
Laboratory Studies
- Immunohistochemical staining is positive for factor VIII.
Imaging Studies
- Neuroimaging studies can be of great assistance in establishing the diagnosis.
- Ultrasonography shows a lesion with irregular contour and low-to-medium internal reflectivity.
- CT scan reveals a poorly circumscribed mass with no bony erosion. The lesion usually enhances with intravenous contrast.
- Magnetic resonance imaging (MRI) reveals a lesion that is hypointense to fat on T1-weighted scans and hyperintense to fat on T2-weighted scans.
- A well-circumscribed lobular pattern with prolonged parenchymal staining is seen with angiography. Involuting lesions tend to have less tissue staining.
Histologic Findings
Pathological findings include proliferation of a single layer of endothelial cells and pericytes. Endothelial cells of the basement membrane characteristically have large amounts of endoplasmic reticulum. The small vascular spaces lead to the formation of a high-flow lesion.
Treatment
Medical Care
The indications for treatment can be divided into systemic, ophthalmic, and dermatologic reasons. Systemic reasons for intervention include congestive heart failure, thrombocytopenia, hemolytic anemia, and nasopharyngeal obstruction. Ophthalmic indications for intervention include occlusion of the visual axis, optic nerve compression, severe proptosis, and anisometropia.4,5 Dermatologic indications for intervention include maceration and erosion of the epidermis, infection, and cosmetic disfigurement.
- The first-line treatment of capillary hemangiomas is simple observation. Since most of these lesions regress on their own, there is no need to intervene unless one of the above criteria is met.6
- Corticosteroids, in various formulations, also have been used in the treatment of capillary hemangiomas.7,8,9,10,11,12,13
- Topical steroid formulations, such as clobetasol propionate cream, can be applied topically to the lesion. The response to these treatments, even with the strongest corticosteroid formulations, is slower than other methods because several weeks are required to obtain a response.
- Complications include dermal atrophy, pigmentary changes in the skin, and dermatitis, as well as less common ophthalmic complications, such as elevated intraocular pressure and cataract formation. Systemic absorption also can occur.
- Overall, this modality is not appropriate for vision-threatening lesions.
- Injectable steroid formulations also are used in the treatment of these lesions.
- Endothelial cell sensitization to catecholamines is the mechanism by which intralesional corticosteroids exert their effects. Although there may be a period of temporary enlargement, blanching usually is noted within 2-3 days and involution is seen by 2-4 weeks. Their effectiveness is most pronounced 2 weeks after injection but can be seen up to 2 months later. The overall success rate for this intervention is 75%.
- Risks of injection include lid necrosis, depigmentation, and fat necrosis. The most disconcerting adverse effect concerns reports of central retinal artery occlusion following the intralesional injection of corticosteroids. Although the precise pathogenesis is uncertain, some suggest it may be related to anomalous vessels. A technically slower injection of corticosteroid or a smaller volume of solution may lessen the risk.14,15,16
- Steroid injections can be repeated, but, ideally, they should be separated in time by 2-3 months to allow for maximum benefit to be seen. Adrenal suppression after cutaneous steroid injections has been reported.
- Systemic corticosteroids are used for amblyogenic life-threatening lesions.
- An excellent response rate can be expected in 30% of patients, a questionable response in 40%, and no response in 30% when systemic corticosteroids are used under close observation. The response may be quite dramatic within the first 2 weeks, and the effect may last from 1-4 months. Doses of 2-3 mg/kg may be necessary for up to several months.
- Complications of systemic therapy with corticosteroids include Cushingoid changes, personality changes, gastrointestinal irritation, oral candidiasis, delayed growth, diabetes, hypertension, and rebound growth of hemangiomas on cessation.
- Interferon alfa-2a has emerged as a new modality to combat the life-threatening and vision-threatening hemangiomas of infancy that are resistant to steroid treatment.17 While it has potent effects on these lesions, it is commonly associated with adverse effects of varying severity.
- Interferon alfa-2a exerts its effect by preventing endothelial cell migration in capillary hemangiomas. Authors have shown significant tumor regression after treatment with interferon.
- Unfortunately, adverse effects from treatment may have somewhat dampened the initial excitement with this treatment. Adverse effects include fever, chills, arthralgias, and retinal vasculopathy. Of more significance, the incidence of spastic diplegia has been as high as 20% in some reports, leading some to reevaluate the benefits of its use. The long-term effects of interferon on the developing brain are unknown.
Surgical Care
- Laser surgery has been attempted to ameliorate capillary hemangiomas but is still controversial. The hemostatic effects of the carbon dioxide laser have been used with success to surgically remove these lesions. Other lasers used include the argon laser and the Nd:YAG laser. The pulsed dye laser is only effective for very superficial lesions; its mechanism of action is too slow for sight-threatening hemangiomas. Overall, variable results have been seen with various laser modalities, and the risks of scarring and ulceration often deter its use.
- Incisional surgical techniques also have had variable success. Surgical ligation of the hemangiomas produces equivocal results. Vascular embolization of the lesions should be used for large extraorbital hemangiomas only. Primary excision also has been advocated for infantile hemangiomas. Early surgical intervention can be considered as a primary treatment option in selected, isolated capillary hemangiomas without a significant cutaneous component. Surgery can provide definitive early treatment and prevent astigmatism and occlusion-related amblyopia.18,19
Consultations
- Consultations with a pediatrician may be necessary to monitor for the systemic adverse effects if prednisone is used or if there is suspicion of systemic involvement.
- Pediatric dermatologists may have particular expertise in the various treatment options available.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Prednisone (Deltasone)
Decreases inflammation by stabilizing lysosomal membranes, inhibiting PMN and mast cell degranulation, and decreasing capillary vasoconstriction.
Dosing
Adult
Pediatric
2-3 mg/kg/d for up to several mo may be necessary to cause regression of lesions; close monitoring at these high doses is essential
Interactions
Coadministration with anticholinesterase agents may prolong their effects; corticosteroid-induced potassium loss may potentiate digoxin toxicity and cause severe hypokalemia if used with potassium-depleting diuretics; anticoagulant action may be prolonged with concurrent steroid administration; caution must be taken in patients with diabetes mellitus, osteoporosis, hypertension, or impaired liver function
Contraindications
Documented hypersensitivity; fungal, viral, tubercular skin infections; peptic ulcer disease; hepatic dysfunction; connective tissue infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
With sudden cessation of long-term steroid use, adrenal insufficiency may occur; steroid psychoses, immunosuppression, impaired wound healing, osteoporosis, peptic ulcer disease, and growth suppression are also adverse effects of corticosteroid usage
Clobetasol propionate 0.05% (Temovate)
Decreases inflammation by stabilizing lysosomal membranes, inhibiting PMN and mast cell degranulation. Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.
Dosing
Adult
Pediatric
Apply to affected area qd/bid; not to exceed 50 g/wk
Interactions
None reported
Contraindications
Documented hypersensitivity; cutaneous fungal or tubercular infections, or viral infections (eg, varicella, herpes simplex)
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Significant systemic absorption may occur if used chronically or under occlusive dressings; application to the eyelid skin must be monitored carefully because of the thin skin and higher than normal systemic absorption
Betamethasone (Celestone)
Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.
Dosing
Adult
Pediatric
1 cc of a 6 mg/cc solution combined with 1 cc of a 40 mg/cc solution of triamcinolone
Interactions
Effects decrease with coadministration of barbiturates, phenytoin and rifampin
Contraindications
Documented hypersensitivity; systemic fungal infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Local adverse effects include fat atrophy, skin hyperpigmentation, and central retinal artery occlusion; systemic absorption increases risk of multiple complications, including severe infections, adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, and growth suppression
Triamcinolone (Kenalog)
Treats inflammatory dermatosis that is responsive to steroids. Decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.
Dosing
Adult
Pediatric
1 cc of a 40 mg/cc solution combined with 1 cc of a 6 mg/cc solution of betamethasone (see separate section)
Interactions
Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
Contraindications
Documented hypersensitivity; systemic fungal infections
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Local adverse effects include fat atrophy, skin hyperpigmentation, and central retinal artery occlusion; systemic absorption increases risk of multiple complications, including severe infections, adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, and growth suppression
Follow-up
Further Inpatient Care
- Young patients may require inpatient admission and monitoring if high doses of systemic steroids are administered.
Further Outpatient Care
- Concerns must be reiterated about ensuring that children with potentially amblyogenic capillary hemangiomas obtain frequent assessments of their visual acuity and refraction. Treatment would be indicated if astigmatism is greater than 1.5-2 diopters or if there is occlusion of the visual axis.
Inpatient & Outpatient Medications
- Patients started on systemic steroids may need to be monitored continually on an outpatient basis until either an adequate response is seen or complications preclude its continuation.
Prognosis
- The prognosis for cosmetic and visual recovery is excellent if treatment is instituted at an appropriate time and careful attention is paid to the visual development. Most lesions involute by age 5 years.
Miscellaneous
Medicolegal Pitfalls
- The primary concern with capillary hemangiomas is related to their ability to cause permanent, dense amblyopia with the potential for an irrecoverable loss of vision. Close collaboration with ophthalmologists is essential to ensure that sudden changes in vision are promptly detected and treated.
- Vision loss after intralesional corticosteroid injection is a concern of all specialists managing this problem. Parents should be informed of this risk but also cautioned against withholding treatment on the basis of this rare event.
References
Haik BG, Karcioglu ZA, Gordon RA, et al. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. Mar-Apr 1994;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. Feb 2006;10(1):13-19. [Medline].
Kavanagh EC, Heran MK, Peleg A, et al. Imaging of the natural history of an orbital capillary hemangioma. Orbit. Mar 2006;25(1):69-72. [Medline].
Weiss AH, Kelly JP. Reappraisal of astigmatism induced by periocular capillary hemangioma and treatment with intralesional corticosteroid injection. Ophthalmology. Feb 2008;115(2):390-397.e1. [Medline].
Goggin M. Astigmatism and periocular hemangioma. Ophthalmology. Oct 2008;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. Oct 2008;43(5):605-6. [Medline].
Assaf A, Nasr A, Johnson T. Corticosteroids in the management of adnexal hemangiomas in infancy and childhood. Ann Ophthalmol. Jan 1992;24(1):12-8. [Medline].
Boon LM, MacDonald DM, Mulliken JB. Complications of systemic corticosteroid therapy for problematic hemangioma. Plast Reconstr Surg. Nov 1999;104(6):1616-23. [Medline].
Cruz OA, Zarnegar SR, Myers SE. Treatment of periocular capillary hemangioma with topical clobetasol propionate. Ophthalmology. Dec 1995;102(12):2012-5. [Medline].
Elsas FJ, Lewis AR. Topical treatment of periocular capillary hemangioma. J Pediatr Ophthalmol Strabismus. May-Jun 1994;31(3):153-6. [Medline].
Kushner BJ. Intralesional corticosteroid injection for infantile adnexal hemangioma. Am J Ophthalmol. Apr 1982;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. Jun 2003;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. Nov 7 2008;[Medline].
Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic Surg. Apr 1986;17(4):229-31. [Medline].
Weiss AH. Adrenal suppression after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. May 15 1989;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). Apr 2008;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. Mar 1998;132(3 Pt 1):527-30. [Medline].
Deans RM, Harris GJ, Kivlin JD. Surgical dissection of capillary hemangiomas. An alternative to intralesional corticosteroids. Arch Ophthalmol. Dec 1992;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. Oct 2003;31(5):418-23. [Medline].
Keywords
capillary hemangioma, capillary hemangiomas, orbital tumor, benign endothelial cell neoplasm, vascular malformation, vascular anomaly
Contributor Information and Disclosures
Author
Stuart Seiff, MD, FACS, Emeritus Professor of Ophthalmology, University of California San Francisco; Chief, Department of Ophthalmology, San Francisco General Hospital; Consultant, Oculofacial and Aesthetic Plastic Surgery, California Pacific Medical Center and Mills Peninsula Medical Center
Stuart Seiff, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, and California Medical Association
Disclosure: Nothing to disclose.
Coauthor(s)
Orin M Zwick, MD, Oculoplastic Surgeon, Chesapeake Eye Care and Laser Center
Orin M Zwick, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association
Disclosure: Nothing to disclose.
Dan D DeAngelis, MD, FRCS(C), Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto
Dan D DeAngelis, MD, FRCS(C) is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, California Medical Association, Canadian Medical Association, Canadian Ophthalmological Society, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.
Susan Carter, MD, Clinical Associate Professor of Ophthalmology, Institute of Ophthalmology and Visual Science, New Jersey Medical School
Susan Carter, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Ophthalmic Plastic and Reconstructive Surgery
Disclosure: Nothing to disclose.
Medical Editor
Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.
Pharmacy Editor
Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.
Managing Editor
Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting
CME Editor
Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.
Chief Editor
Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.