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Capillary Hemangioma

  • Author: Stuart Seiff, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Oct 06, 2015
 

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]

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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]

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Epidemiology

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.

PHACES syndrome is associated with large segmental hemangiomas of the head and neck. The acronym refers to osterior fossa abnormalities (Dandy-Walker malformation), emangiomas, rterial lesions, ardiac abnormalities (coarctation), 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.

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.

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Contributor Information and Disclosures
Author

Stuart Seiff, MD, FACS Emeritus Professor of Ophthalmology, University of California, San Francisco, School of Medicine; 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, California Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Dan D DeAngelis, MD, FRCSC Assistant Professor of Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Ophthalmologist, Department of Ophthalmology and Vision Sciences, Hospital for Sick Children

Dan D DeAngelis, MD, FRCSC 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, 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, American Society of Ophthalmic Plastic and Reconstructive Surgery

Disclosure: Nothing to disclose.

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, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Brian A Phillpotts, MD, MD 

Brian A Phillpotts, MD, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, National Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

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