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Hemangioma, Capillary

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
Coauthor(s): Orin M Zwick, MD, Oculoplastic Surgeon, Chesapeake Eye Care and Laser Center; Dan D DeAngelis, MD, FRCS(C), Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto; Susan Carter, MD, Clinical Associate Professor of Ophthalmology, Institute of Ophthalmology and Visual Science, New Jersey Medical School
Contributor Information and Disclosures

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.

More on Hemangioma, Capillary

Overview: Hemangioma, Capillary
Differential Diagnoses & Workup: Hemangioma, Capillary
Treatment & Medication: Hemangioma, Capillary
Follow-up: Hemangioma, Capillary
References
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References

  1. Haik BG, Karcioglu ZA, Gordon RA, et al. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. Mar-Apr 1994;38(5):399-426. [Medline].

  2. 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].

  3. 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].

  4. 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].

  5. Goggin M. Astigmatism and periocular hemangioma. Ophthalmology. Oct 2008;115(10):1854-5; author reply 1855. [Medline].

  6. 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].

  7. 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].

  8. Boon LM, MacDonald DM, Mulliken JB. Complications of systemic corticosteroid therapy for problematic hemangioma. Plast Reconstr Surg. Nov 1999;104(6):1616-23. [Medline].

  9. Cruz OA, Zarnegar SR, Myers SE. Treatment of periocular capillary hemangioma with topical clobetasol propionate. Ophthalmology. Dec 1995;102(12):2012-5. [Medline].

  10. Elsas FJ, Lewis AR. Topical treatment of periocular capillary hemangioma. J Pediatr Ophthalmol Strabismus. May-Jun 1994;31(3):153-6. [Medline].

  11. Kushner BJ. Intralesional corticosteroid injection for infantile adnexal hemangioma. Am J Ophthalmol. Apr 1982;93(4):496-506. [Medline].

  12. 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].

  13. 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].

  14. 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].

  15. Weiss AH. Adrenal suppression after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. May 15 1989;107(5):518-22. [Medline].

  16. 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].

  17. 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].

  18. 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].

  19. 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].

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Further Reading

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Keywords

capillary hemangioma, capillary hemangiomas, orbital tumor, benign endothelial cell neoplasm, vascular malformation, vascular anomaly

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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.

 
 
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