Cavernous Hemangioma 

  • Author: Adam J Cohen, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Dec 13, 2011
 

Background

Cavernous hemangiomas are the most common intraorbital tumors found in adults. These benign, vascular lesions are slow growing and can manifest as a painless, progressively proptotic eye. Most of these tumefactions are exceedingly unilateral. Bilateral cases have been reported but are rare.

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Pathophysiology

Orbital cavernous angiomas can increase intraorbital volume with a resultant mass effect. Although cavernous hemangiomas are histologically benign, they can encroach on intraorbital or adjacent structures and can be considered anatomically or positionally malignant. Visual acuity or field compromise, diplopia, and extraocular muscle or pupillary dysfunction can result from compression of intraorbital contents by the angioma. Lagophthalmos can result in exposure keratopathy, keratitis, and corneal perforation.

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Epidemiology

Frequency

International

Henderson reported an incidence of 4.3% among orbital neoplasms.[1]

Mortality/Morbidity

The morbidity associated with cavernous hemangiomas is the threat of compressive optic neuropathy, extraocular muscle dysfunction, and cosmetic disfigurement.

Mortality can result from intraoperative complications, such as bleeding and the risk of general anesthesia.

Race

No predilection exists for race or ethnicity.

Sex

Harris and Jakobiec found a 7:3 occurrence ratio of women to men, while Henderson reported an almost equal ratio, 8:7 in women and men.[2, 1]

Age

Patients usually manifest symptomatically during the third to fifth decades of life.

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Contributor Information and Disclosures
Author

Adam J Cohen, MD  Eyelid and Facial Aesthetic and Reconstructive Surgery, Diseases and Surgery of the Orbit and Lacrimal System, Cosmetic Laser Surgery

Adam J Cohen, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and American Society of Ophthalmic Plastic and Reconstructive Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Michael Mercandetti, MD, MBA, FACS  Consulting Staff, Department of Surgery, Doctors Hospital of Sarasota

Michael Mercandetti, MD, MBA, FACS is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Ophthalmology, American College of Surgeons, American Society for Laser Medicine and Surgery, American Society of Ophthalmic Plastic and Reconstructive Surgery, Association of Military Surgeons of the US, and Sarasota County Medical Society

Disclosure: Nothing to disclose.

David A Weinberg, MD, FACS  Director, Oculoplastic and Orbital Surgery, Assistant Professor of Neurology and Ophthalmology, Department of Surgery, Division of Ophthalmology, Fletcher Allen Health Care

Disclosure: Nothing to disclose.

Specialty Editor Board

Andrew W Lawton, MD  Medical Director of Neuro-Ophthalmology Service, Section of Ophthalmology, Baptist Eye Center, Baptist Health Medical Center

Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, and Southern Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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 Honoraria Speaking and teaching

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.

References
  1. Henderson GW. Vascular hamartomas, hyperplasias, and neoplasms. In: Henderson GW, ed. Orbital Tumors. New York: Raven Press; 1994:94-100.

  2. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysis of sixty-six cases. In: Jakobiec, ed. Ocular and Adnexal Tumors. Birmingham, Ala: 1978:741-81.

  3. Zauberman H, Feinsod M. Orbital hemangioma growth during pregnancy. Acta Ophthalmol (Copenh). 1970;48(5):929-33. [Medline].

  4. Ko F, Dibernardo CW, Oak J, Miller NR, Subramanian PS. Confirmation of and differentiation among primary vascular lesions using ultrasonography. Ophthal Plast Reconstr Surg. Nov 2011;27(6):431-5. [Medline].

  5. Boari N, Gagliardi F, Castellazzi P, Mortini P. Surgical treatment of orbital cavernomas: clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien). Mar 2011;153(3):491-8. [Medline].

  6. Cho KJ, Paik JS, Yang SW. Surgical outcomes of transconjunctival anterior orbitotomy for intraconal orbital cavernous hemangioma. Korean J Ophthalmol. Oct 2010;24(5):274-8. [Medline]. [Full Text].

  7. Liu X, Xu D, Zhang Y, Liu D, Song G. Gamma Knife surgery in patients harboring orbital cavernous hemangiomas that were diagnosed on the basis of imaging findings. J Neurosurg. Dec 2010;113 Suppl:39-43. [Medline].

  8. Dortzbach RK, Kronish JW. Orbital disease. In: Dortzbach RK, ed. Ophthalmic Plastic Surgery Prevention and Management of Complications. New York: Raven Press; 1994:312-25.

  9. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit. J Neurosurg. Aug 1979;51(2):219-28. [Medline].

  10. Mercandetti M, Cohen AJ. Exophthalmos. eMedicine Journal [serial online]. February 7, 2007;Available at http://emedicine.medscape.com/article/1218575-overview.

  11. Mercandetti M, Cohen AJ. Tumors, orbital. eMedicine Journal [serial online]. February 7, 2007;Available at http://emedicine.medscape.com/article/1218892-overview.

  12. Rodgers IR, Grove AS. Vascular lesions of the orbit. In: Principles and Practice of Ophthalmology. Philadelphia: WB Saunders; 1994:1970-1.

  13. Shields JA, Shields CL. Vascular and hemorrhagic lesions. In: Atlas of Orbital Tumors. Philadelphia: Lippincott, Williams and Wilkins; 1999:50-56.

  14. Yan J, Wu Z. Cavernous hemangioma of the orbit: analysis of 214 cases. Orbit. Mar 2004;23(1):33-40. [Medline].

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Extirpation of an orbital cavernous hemangioma. Note en bloc removal and preservation of capsule. Courtesy of Robert Alan Goldberg, MD.
MRI demonstrates enhancing mass in apex of left orbit. White arrow points to the superior portion of the optic nerve, showing its deviation. Mass was pressing on superotemporal optic nerve and displacing it inferomedially at apex. Patient had 6 months of progressive decreased vision and visual field loss. Courtesy of M. Duffy, MD, PhD.
In A, final preoperative visual field of same patient as in Media file 2, demonstrating significant inferior altitudinal field loss; in B, postoperative visual field at approximately 3 weeks after orbital apex decompression and removal of mass; and in C, postoperative visual field at approximately 6 months.
Intraoperative photo of same patient as in Media file 2. Neurosurgical service performed craniotomy and decompression of the superior orbital fissure and optic canal (yellow arrows) at request of ophthalmology service. Orbital surgery service then opened the periorbita over a bulge (double black arrows) between optic nerve and cranial nerves (single black arrow) and bluntly dissected out mass. Pathology confirmed mass as a cavernous hemangioma. Cranial nerves V and IV were adhered, and careful blunt separation was performed. Postoperatively, a small left hypertropia resolved over 6 weeks. Courtesy of M. Duffy, MD, PhD.
 
 
 
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